key: cord-015306-us58wwmp
authors: nan
title: Abstracts for the IPNA Congress, 30 August - 3 September 2013, Shanghai, China
date: 2013-06-21
journal: Pediatr Nephrol
DOI: 10.1007/s00467-013-2518-4
sha: 
doc_id: 15306
cord_uid: us58wwmp

nan

first 5 days after the diagnosis. DWI-MRI was performed without application of contrast medium and without general anaesthesia. Results: DWI-MRI examination confirmed the inflammatory infiltration in kidney parenchyma in all our patients (100 %). On the other hand, static renal scintigraphy confirmed inflammation only in 15 children (60%). Six months later, none of the two follow-up examinations showed any signs of inflammation or scarring in 17 children examined so far. Conclusion: In conclusion, nuclear magnetic resonance (DWI-MRI) imaging seems more beneficial and accurate in the diagnostics of acute pyelonephritis when compared with static renal scintigraphy. Moreover, DWI-MRI provides more accurate information on the extent of kidney damage.

Objective: To evaluate the most proper radiologic investigation algorithm in detecting high grade vesicoureteral reflux (VUR) and renal cortical scarring after first febrile urinary tract infections in infants aged less than one year . Methods: A total of 408 infants aged less than one year with a first febrile urinary tract infection who completed the diagnostic follow up of renal bladder ultrasound (RBUS), voiding cystourethrography (VCUG) and late 6 months technetium 99 dimercaptosuccinic acid renal scan (DMSA) were enrolled in the study.The most proper radiologic investigation algorithm that could highly detected both high grade vesicoureteral reflux and renal scar in infants after the first febrile urinary tract infection considering high benefit, low cost, low radiation exposure were assessed. Results: Abnormal renal bladder ultrasound (RBUS) was identified in 101 (24.7 %) infants. Vesicoureteral reflux (VCUG) was identified in 92 (22.5%) with high grade reflux (grade 4 and 5) in 18 (4.4%) infants.Abnormal renal parenchyma including renal scar was identified in 32 (7.8%) infants.The top down approach with late 6 months DMSA scan showed high benefit in detecting all abnormal renal parenchyma including renal scars, with high sensitivity ( 72%) in detecting high grade vesicoureteral reflux (VUR) whereas reducing the unnecessary investigation for low grade VUR (94.6%) and radiation exposure per patient (0.89 mSv). Conclusion: Currently, there is no ideal diagnostic radiologic investigation after a first febrile urinary tract infection in infants and children.The study suggest performing the top down approach with late 6 months DMSA scan that could detect all renal scars and highly detection of high grade VUR.

Abstract# P-SAT010 Association between postvoid urine bladder volume and urinary tract infection in infants and children: A retrospective cohort study Orpheus Monakil, Ivy Avilla Department of Pediatrics, Da La Salle University Medical, Philippines

Objective: To determine the association between post void urine bladder volume residual and urinary tract infection(UTI) in children. Methods: Medical records of pediatric patients aged 0 to 18 years old with diagnosis of urinary tract infection were reviewed. Demographic data, urinalysis and urine culture results and kidney and urinary bladder(KUB) ultrasound findings were tabulated and analyzed using the 95% confidence interval. Relative risks were computed with a level of significance of p value < 0.05. Results: A total of 803 patients were included in the study. Five hundred forty-four(67.8%) belongs to age group < 5 years old. Majority were females with a 0.97:1 male to female ratio. Escherichia coli is the most common organism isolated. Patients with post void urine bladder residual are at risk for having growth in the urine culture. In sub group analysis for age and sex, statistically significant results were noted among patients belonging to the 3-5 years with the RR of 1.337, 95% CI (1.082, 1.651) p-value of 0.003 and males with RR 1.186 (95% CI 1.024, 1.374) p value 0.0168. Conclusion: There is an association between the occurrence of urinary tract infection and the presence of post void bladder volume residual. Males and those patients aged 3 to 5 years of age are more at risk for urinary infection. Objective: To evaluate if a relationship exists between renal ultrasound and voiding cystourethrography (VCUG) findings among children with UTI and determine whether the renal ultrasound findings/results can serve as a guide if a procedure such as VCUG is needed to be done in the patient. Methods: Medical records of infants and children diagnosed as having UTI who underwent renal ultrasound and VCUG were reviewed. Demographic data, urine culture and results of imaging studies were tabulated and analyzed. Kappa statistics was used to determine the agreement between renal ultrasound and VCUG results. McNemars test was utilized to determine the statistical significance of the agreement. Level of significance was place at p value of < 0.05. Results: A total of 146 patients were included in the study. Thirtyeight(26%) had vesicoureteral reflux. There were more females with a male to female ratio of 1:1.3 while cases with reflux had a 1:1.5 male to female ratio. Most common chief complaint was fever. E. coli is the most commonly isolated etiologic organism from the urine. Twenty(52.7%) patients had primary VUR while 18(47.3%) were secondary. Fifty two of 146 children had abnormal sonogram; of duplex collecting system (DCS) diagnosed during postnatal USC on Objective: The aim was to evaluate the relationship of laboratory investigations, therapeutic delay time (TDT), and therapeutic response time (TRT) with acute renal damage and to verify these parameters in the presence of non refluxing and refluxing urinary tract infection (UTI). Methods: A prospective study was conducted in 67 children. All patients received voiding cystourethrography (VCUG) and dimercaptosuccinic acid (DMSA) renal scintigraphy. Statistical analyses were applied to assess all parameters with DMSA renal scintigraphy. Results: Abnormal DMSA renal scintigraphy was detected in 20/67 (29.9%) patients. There were no significant differences in peak temperature, TDT and treatment duration. However, white blood cells (WBC) count, percentage of serum polymorphonuclear cells (%PMN) and TRT had significant differences at p-values 0.042, <0.001 and 0.001, respectively. The area under ROC curve for WBC count, %PMN and TRT was 0.653 (95%CI 0.509-0.798) at p-values 0.055, 0.799 (95%CI 0.681-0.888) at p-values <0.001 and 0.760 (95%CI 0.637-0.858) at p-value 0.001, respectively. Overall, the optimal cutoff value for %PMN was 56.0 with sensitivity 84.2% (60. 4-96.4 ) and specificity 60. 9% (45.4-74.9 ). The optimal cut-off value for TRT was 22 hours with sensitivity 80.0% (56.3-94.1) and specificity 63. 6% (47.8-77.6) . In 50 patients with no vesicoureteral reflux (VUR), there was significant difference in TRT at p-values 0.002. The area under ROC curve for TRT was 0.824 (95%CI 0.693-0.955) at p-value 0.004. The optimal cut-off value for TRT was 25 hours with sensitivity 88.9 % (95%CI 51.7-98.2) and specificity 68.4% . In VUR patients, there were no significant differences in TDT, %PMN and TRT between normal and abnormal DMSA renal scintigraphy at p-value 0.750, 0.191 and 0.313, respectively. Conclusion: %PMN ≥56% and TRT ≥22 hours predict renal damage in the first episode of UTI. However, in patients with no VUR, TRT ≥25 hours predict renal damage. DMSA renal scintigraphy in the first episode of UTI should be considered in those patients. susceptibility data. Knowledge of local antimicrobial susceptibility all children with the first episode of febrile urinary tract infection traditionally. Nowadays it has been revealed that the presence of renal scar is more important than the presence of VUR regarding renal and patient outcome. Our aim was to assess the relationship between the severity of VUR and the severity of DMSA scan changes which is performed in the first week of the first febrile UTI. Methods: Children with the first febrile UTI who were admitted in Ali asghar children hospital were evaluated prospectively. All DMSA scans have been observed and graded by our nuclear medicine specialist without any knowledge of presence or absence of VUR. Renal damages in DMSA scan were classified to 8 grades as follows: Grade 0: normal kidney, grade 1: decreased uptake in one pole with intact border, grade 2: decreased uptake in two poles with intact borders, grade 3: diffuse decreased uptake with intact border, grade 4: decreased uptake in one pole with scar, grade 5: decreased uptake in two poles with scar, grade 6: multiple scars, grade 7: diffuse decreased uptake with one pole scar, grade 8: diffuse decreased uptake with multiple scars. Then DMSA findings in patients with no VUR, with low grade VUR and high grade or dilating VUR were compared with each other. Results and Conclusion: One hundred and six patients with the first febrile UTI were included in this study, thus 212 kidneys were evaluated for the presence or absence of VUR and DMSA grading. The mean age of our patients was 4±2.9 years old. 12.3% of our patients were male. 44.8% of kidneys did not have any VUR, low grade VUR (grades 1,2,3) was seen in 34.4% of kidneys and high grade VUR 9grades 4, 5) was found in 20.3% of kidneys. 76.2% of patients with low grade VUR and/or without VUR had DMSA scoring.

Abstract# P-SAT040 Fibronection in reflux nephropathy, is it a marker of grade of reflux?

Nahid rahimzadeh Tehran university of medical science, associated professor, Tehran, Iran

Objective: Vesicoureteral reflux (VUR) is one of the most common urinary tract abnormalities in patients with urinary tract infection. Nowadays noninvasive diagnostic methods are suggested to recognize VUR and its severity. Methods: We measured urinary and serum fibronectin in 51 children with VUR. Results: The mean serum fibronectin was 318.3±112.1 in children with low grade VUR versus 356.1±189.9 in children with high grade VUR (Pv>0.05). The mean urinary fibronectin was also 31.5±12.9 in low grade VUR and 25.9±14.2 in high grade VUR (Pv>0.05). Thus we didn't find any association between the severity of VUR and the amount of fibronectin in serum and urine of patients. We also didn't find any relationship between DMSA changes at the acute phase of UTI and serum and urine fibronectin. Conclusion: In contrast to some previous studies, we showed the serum and urinary fibronectin cannot preclude the severity and grade of VUR and hence it is not suitable surrogate marker for imaging techniques for VUR diagnosis.

Abstract# P-SAT041 The use of serum procalcitonin level in the prediction of high grade vesicoureteral reflux in urinary tract infection Nahid rahimzadeh Tehran university of medical science, associated professor, Tehran, Iran

Objective: Procalcitonin is a reliable and specific marker of bacterial infection such as urinary tract infection. Some authors suggest measurement of serum procalcitonin as a predictor of vesicoureteral reflux. We investigated this association in children who admitted because of acute pyelonephritis. Methods: Forty eight children with the first febrile UTI were included. Twelve patients had low grade VUR, nine patients had high grade VUR ((≥ 3) and twenty seven patients didn't have any VUR in their imaging assessment. Results: There was a significant association between high grade VUR and higher levels of procalcitonin (Pv=0.04). The sensitivity of procalcitonin level ≥ 0.31 ng/ml was 90% and specificity was 32% for diagnosis of high grade VUR. Conclusion: We concluded that serum procalcitonin concentration is a sensitive and promising predictor of high grade vesicoureteral reflux.

hydronephrosis, parenchymal scarring and to study the rate of resolution of VUR on follow up. Methods: This was a retrospective study conducted by reviewing case records of all infants and children with primary VUR who had minimum follow up of 3 years, at our nephro-urology clinic over the last 10years.The imaging evaluation (Renal Ultrasound, Voiding cystourethrogram, DMSA scan) was done based on the Indian Society of Pediatric Nephrology guidelines. Severity of VUR was classified as mild (grade I, II), moderate (grade II, III) and severe (grade V) Results: Of the 218 children screened for primary VUR, 107 with complete data were included for analysis. The mean age was 25.68 ±22.45 months with male to female ratio of 1.4:1. The age at presentation was significantly lower for moderate to severe VUR as compared to mild VUR (p=0.033). Thirty children (28%) had abnormal antenatal scans. Majorities (80%) of children were diagnosed to have VUR during evaluation for urinary tract infection.Among107 patients, six had solitary kidneys and thus the total number of systems evaluated was 208. Of these, 164 systems had VUR (24% had mild, 66% had moderate and 10% had severe VUR). Hydronephrosis was seen in 56% of patients with VUR. Of these, 45% were unilateral and 55% were bilateral.There was no significant difference in the presence of hydronephrosis and grade of VUR. There was no significant difference in presence of scars between mild and mod-severe VUR. At the end of 3 years of follow up, complete resolution of reflux was seen in 53%, 43% and 55% of children with mild, moderate and severe VUR respectively. Conclusion: Severity of VUR did not necessarily correlate with hydronephrosis and parenchymal scarring. Over a 3 year follow up, complete resolution of reflux was seen in nearly 50% of children irrespective of the grade of reflux.

Abstract# P-SAT044 Growth in children with dilating VUR -a follow up of the Swedish Reflux Trial Per Brandstrom, Sverker Hansson Pediatricuronephrologic center, University of Gothenburg, Gothenburg, Sweden

Objective: The Swedish Reflux Trial included 203 children, 1-2 years of age, with VUR grade 3-4, diagnosed after UTI in 194 and prenatal urinary tract dilatation in 9. DMSA was abnormal at start in 124 children (61%). The children were randomized to antibiotic prophylaxis, endoscopic injection with Deflux™ or surveillance. There have been reports on growth retardation in children with VUR and catch up after VUR resolution. The mechanism behind these findings is unclear. The children of the Swedish Reflux Trial constitute a high risk group with dilating VUR, recurrent febrile UTI and high prevalence of renal parenchymal defects. We have searched the growth pattern of these children for differences related to gender, treatment group, UTI recurrence, VUR grade at follow up or renal defects on DMSA. Methods: Height and weight Z-scores, compared to standardized Swedish growth charts, were registered during the 2 year follow up in 199 of the children in the trial and at outpatient visits thereafter in 129 patients to the age of 5.3 years (median, range 2.0-11.8). Change in height Z-score between first and last visit was used to measure growth over time.

Results: The first and last recorded height and weight Z-scores were all within normal range. There was a larger gain of height in children with renal defects compared to those without (Z-score difference 0.42 vs. 0.13, p=0.009). There were no differences in growth related to gender, treatment group, VUR-grade at follow up or recurrent UTI. Conclusion: The children of the Swedish Reflux Trial constitute a high risk group with dilating VUR, recurrent febrile UTI and high prevalence of renal parenchymal defects. They have normal weight, height and height gain at follow up for up to 10 years. There was no sign of growth inhibition in these children related to dilating VUR. The larger height gain seen in those with renal defects seem to be due to their slightly shorter stature at study start, although within normal range, which could be related to more severe urinary tract and renal problems during their first 1-2 years of life.

Engin Kose, Caner Alparslan, Serdar Saritas, Cengizhan Elmas, Fatma Mutlubas Ozsan, Onder Yavascan, Nejat Aksu Tepecik Training and Research Hospital, Pediatric Nephrology, Izmir, Turkey Objective: The management of vesicoureteral reflux (VUR) is varied and remains controversial. Conservative therapy is based on the understanding that VUR can resolve spontaneously, mostly in young patients with low-grade reflux. In this study, we wanted to evaluate the spontaneousresolution rate of low-grade VUR (grades I and II) in children.

Methods: Children with low-grade (I-II) VUR treated in our hospital from May 2010 to May 2012 were prospectively studied. Patients with low-grade (I-II) VUR and those who showed normal DMSA findings were included into the study. Initially, a DMSA scintigraphy was performed in all patients and those who experienced acute febrile urinary tract infection (UTI) during the follow-up period. Treatment success was defined as complete VUR resolution. No patients were prescribed antibiotic prophylaxis. All parents were informed by being given an explanation of the clinical significance of personal hygiene methods used after urinating or defecating. Our Institutional Review Board approved to collect the data, retrospectively. Statistical analysis was made by using IBM SPSS 20.0 software. Results: The study sample comprised 21 infants (10 boys and 11 girls) all of whom showed low-grade reflux (grades I-II). Bilateral reflux was seen in 7 (33.3 %) of cases. Median age at diagnosis was 8 months (range: 1-37 months). Median follow-up time was 14 months (range: 9-28 months). The spontaneousresolution rate of reflux was 89.3 % (25 out of 28 renal units). The frequency of febrile UTI was 0.74±0.7 episode/year (median: 0.8 episode/year). During the follow-up no patients with febrile UTI experienced scar on DMSA scintigraphy. Conclusion: Infants with low-grade reflux show a low risk of febrile UTI and a high spontaneous resolution rate without antibiotic prophylaxis. Therefore, these children should be managed primarily by conservative therapy.

Ji-Nan Sheu 1,2 , Hai-Lun Sun 1, 2 , Shan-Ming Chen 1, 2 , Yu-Hua Chao 1, 2 , Min-Sho Ku 1, 2 , Pen-Fen Liao 1 , Ko-Huang Lue 1, 2 1 Pediatrics, Chung Shan Medical Unversity Hospital, Taichung, Taiwan 2 School of Medicine, Chung Shan Medical University Taichung, Taiwan

Objective: To assess the usefulness of procalcitonin (PCT) as a marker for predicting dilating (grades III-V) vesicoureteral reflux (VUR) in young children with a first febrile urinary tract infection (UTI). Methods: Children aged≤ 2 years old with a first febrile UTI were prospectively evaluated. Serum samples were tested for PCT measurements upon admission to a tertiary hospital. All children underwent renal ultrasonography (US), 99m Tc-dimercaptosuccinic acid renal scan, and voiding cystourethrography. The diagnostic characteristics of PCT test for acute pyelonephritis (APN) and dilating VUR were calculated. Results: Of 272 children analyzed (168 boys and 104 girls; median age, 5 months), 169 (62.1%) had APN. There was VUR in 97 (35.7%), including 70 (25.7%) with dilating VUR. The median PCT value was significantly higher in children with VUR than in those without (P< 0.001). Using a PCT cutoff value of ≥1.0ng/mL, the sensitivity and negative predictive value for predicting dilating VUR were 94.3% and 95.4%, respectively, for PCT, and 97.1% and 97.8%, respectively, for the combined PCT and US studies, whereas the positive and negative likelihood ratios were 2.03 and 0.107, respectively, for PCT, and 1.72 and 0.067, respectively, for the combined studies. By multivariate analysis, high PCT values and abnormalities on US were independent predictors of dilating VUR. Conclusion: PCT is useful for diagnosing APN and predicting dilating VUR in young children with a first febrile UTI. A voiding cystourethrography is indicated only in children with high PCT values (≥1.0 ng/mL) and/or abnormalities found on a US. Objective: To evaluate the accuracy of acute 99m Tc-dimercaptosuccinic acid (DMSA) scan in predicting dilating vesicoureteral reflux (VUR) among young children with febrile urinary tract infection (UTI). Methods: The medical records of children (age≤2 years), presenting with febrile UTI between January 2000 and December 2011, were retrospectively reviewed. The sensitivity, specificity, positive and negative predictive value, positive and negative likelihood ratio of acute DMSA scan in predicting dilating VUR in young children with febrile UTI were calculated. Results: A total of 523 children were included, of which 397(75.9%) had abnormal DMSA results and 178(34.0%) were identified as VUR on micturatingcystourethrography (MCU). Among all the patients, the number of dilating VUR was 151(28.9%). The rate of abnormal results on DMSA of dilating VUR group was significantly higher than the rates of non-VUR and low-grade VUR groups (P<0.01). In age<6 months group and age≥6 months group, the sensitivities of DMSA in predicting dilating VUR were 96.15%, 100.0% respectively, while the negative predictive value were 97.26%, 100.0% and negative likelihood ratio were 0.0911,0.0000, respectively. Conclusion: For children of age≤2 years with febrile UTI, acute DMSA scan possesses certain values in excluding dilating VUR. The possibility to detect dilating VUR by MCU is rather low when the result of DMSA is negative.

CAKUT: Voiding disorders Abstract# P-SAT048 Mono-symptomatic and Non-mono symptomatic Nocturnal Enuresis: A Clinical Evaluation Mitra Naseri 1 , Mehran Hiradfar 2 1 Dr Sheikh children hospital/pediatric nephrology department, Mashhad University of Medical Sciences, Mashhad, Iran 2 Dr Sheikh children hospital/pediatric surgery department, Mashhad University of Medical Sciences, Mashhad, Iran Objective: Nocturnal enuresis is divided into mono-symptomatic nocturnal enuresis(MNE) and non mono-symptomatic nocturnal enuresis(NMNE).This study was conducted to review clinical and ultrasonography findings in enuretic children, and compare organic and functional pathologies of lower urinary tract (LUT) in children with mono-MNE with those who have NMNE. Methods: 111 neurologically normal children with chief complaint of enuresis enrolled in the study including 60 boys and 51 girls, aged 5-17 years old, 43 (38.8%) with MNE and 68 (61.2%) with NMNE. Urine analysis, urine culture and kidney bladder ultra sonography was done for all .Some patients underwent voiding cystoureterography (VCUG), urodynamic study (UDS), or both. Results: Patients were divided in to 3 groups: MNE, NMNE daytime incontinence and NMNE +daytime incontinence. Constipation, encopresis and urge incontinence were significantly more frequent in patients with NMNE +daytime incontinence (P= 0.011, 0.003, 0.001 respectively) . Bladder wall thickness was the most common US findings..One patient with MNE and 9 with NMNE+ daytime incontinence had vesico -ureteral reflux(VUR) )(P=0.016).Posterior urethral valve was reported in one patient with NMNE. Evidences of bladder dysfunction were noted in about half of the patients who underwent UDS, with higher prevalence in cases with NMNE + daytime urinary incontinence(P=0.297). Bowel symptoms and VUR were significantly more prevalent in cases with NMNE +daytime incontinence. Conclusion: We recommend doing VCUG in enuretic children who have daytime incontinence.In addition our study revealed that symptoms suggestive of over active bladder are not good indicators for bladder dysfunction.

Abstract# P-SAT049 Correspondence between urinary calcium, Ca 2+ concentration and osmolality in enuretic children Agata Korzeniecka-Kozerska, Tadeusz Porowski Department of Paediatrics and Nephrology, Medical University of Bialystok, Bialystok, Poland

Objective: Among many factors predisposing to enuresis hypercalciuria may play an important role. Hypercalciuria is be observed in patients both in patients with nocturnal polyuria and without. Hence, we decided to assess urine concentration of calcium (mmol/L) and Ca 2+ (mmol/L) in patients with monosymptomatic enuresis and answer the question if patients with enuresis present calcium balance disturbances Methods: The study was conducted on 204 children (83 enuretic aged median 9.66 (4.16-16.98 ) yrs diagnosed with monosymptomatic enuresis after 6 months of unsuccessful non farmacological treatment and 121 healthy children aged median 9.99 (4.15-16.86 ) yrs. We collected 24-h urine samples from all children enrolled to the study. Calcium concentration, Ca 2+ , pH, osmolality of urine and additionally daily sodium excretion were estimated and compared between two groups. Statistical analysis were performed using Statistica ver. 10.0 (StatSoft,Tulsa, Ok). The Mann-Whitney U test was used for comparisons between two independent parameters. Correlations were made with Spearman test. A p value of <0.05 was considered to be statistically significant.

Results: There were no differences in age, gender and parameters of physical development between both studied groups and between girls and boys among groups. Urinary calcium concentration in enuretic children did not differ compared to reference group ( p=0.993). We found statistically significant differences in urinary Ca 2+ concentration (p= 0.001) and osmolality (p=0.02) between both studied group. Ca 2+ in urine correlated negatively with age and parameters of physical Physical development only in enuretic patients. Additionally, positive correlation was found between Ca 2+ and calcium concentration (R = 0.7923; p<0.005) and between Ca 2+ and osmolality (R=0.2795; p<0.05) in urine of enuretic children. Positive correlation was also observed between Ca 2+ and daily natrium excretion (R=0.318; p<0.05) in enuretic children. Conclusion: Disturbed calcium balance may play an important role in pathogenesis of monosymtomatic enuresis. It's a need to assess calcium and natrium excretion in enuretic children.

Abstract# P-SAT050 Usefulness of the application of questionnaires to detect attention deficit hyperactivity disorder (ADHD) and other psychiatric disorders in children with functional voiding disorders Sandra Gautreaux Pediatric Nephrology, Complejo Asistencial Universitario de Leon, Spain

Objective: Although the relationship between functional voiding disorders (FVD) and the presence of a psychological problem remains controversial, the greater frequency of ADHD among children with this condition is well known. The purpose of this study was to determine the diagnostic performance of the application of questionnaires to detect ADHD and other psychiatric disorders in children with functional voiding disorders in general pediatrics consultations Methods: The study was conducted on 32 children between 6 and 13 years of age (20 males) diagnosed with FVD (patient group) and 32 children of the same age (21 males) who had no urinary symptoms (control group). The parents of these children responded to the questions in two standardized questionnaires: the Strengths and Difficulties Questionnaire (SDQ) to screen for mental health disorders and the Questionnaire for the detection of ADHD of the DSM-IV psychiatric disorders of American Academy of Psychiatry manual. The variables obtained from the questionnaire responses were compared between the two groups of children using the Student's t-test for unpaired samples when variables were quantitative and the chi-square test if the variables were qualitative. It was considered significant when p<0.05 Results: No significant differences were found between the two groups in the SDQ questionnaire in any of its sections (emotional symptoms, conduct problems, hyperactivity, peer problems and prosocial behavior) or global assessment test, in which only 2 patients of each group had an abnormal result. The questionnaire for the detection of ADHD, presented an altered overall result of 17.18% and 14.06% patients in the control group (p=ns). There were also no differences between the two groups on the results of this test concerning the inattention or hyperactivity-impulsivity sections. Conclusion: The results of the application of questionnaires to detect ADHD and other psychiatric disorders in children with FVD are similar to those of the general population. The routine application of this type of questionnaires to all the patients in pediatrics consultations does not seem necessary.

Abstract# P-SAT051 Characterization of voiding dysfunction in chidren with attention deficit-hyperactivity disorder Jun Yonug Kim, Kun Hee Lee, Jung Won Lee Department of Pediatrics, Hallym University Kangnam Sacred Heart Hospital, Seoul, Korea Objective: Attention deficit hyperacitivity disorder (ADHD) has been associated with impairment of frontal inhibitory function and catecholaminergic system. ADHD is diagnosed in 3~5 % of children. Children with ADHD seem to suffer from various forms of urinary problems such as nocturnal enuresis, dysfunctional voiding and diurnal incontinence. However, no data exist to confirm in Korean ADHD children. We investigate the clinical findings of voiding dysfunction in ADHD children. Methods: Between October 2009 and March 2011, a total of 63 children (33 children with ADHD and the other 30 children with upper respiratory infection as control group) were enrolled in Gangnam Sacred Heart hospital, Hallymuniversity. ADHD children were diagnosed under diagnostic and statistical manual of mental disorders (DSM)-IV criteria. A comprehensive survey of voiding and defecating were administered. Results: The patient group included 28 boys and 5 girls, and the control group 15 boys and 15 girls. Mean age were 9.09+/−2.8 year in ADHD group and 8.58+/−3.1 in control group. Children with ADHD had statistically significant higher incidence of enuresis (P=0.017), urgency (P=0.017), urge incontinence (P=0.033) and constipation (P=0.007). There was no significant differences in straining, intermitteny, holding maneuvers (P>0.05). Conclusion: Children with ADHD in Korea have significantly higher rates of enuresis, urgency, urge incontinence and constipation than those without ADHD.

The psychological correlates of primary nocturnal enuresis Weiran zhou, Xiaomei liu, Ying shen Nephrology, Beijing Children's Hospital, Beijing, China

Objective: Previous studies based on clinical samples have reported that enuresis in children is associated with behavioural problems and reduced self-esteem, anxiety, but the relationship remains controversial. This study investigated psychological correlates of enuresis in a group of children suffering primary nocturnal enuresis(PNE). Methods: This survey involved 98 parents and their children with PNE aged 7-14 years. Clinical datas of enuresis were collected through parents' reports and individual administrations to all children. Parents completed the Child Behaviour Checklist. Children completed Piers Harris Children'sSelf concept Scale, the Screen for Child Anxiety-Related Emotional Disorders (SCARED)and Depression Self Rating Scale for Children (DSRSC) . Results: Of the 98 children, 60 are boys and 38 are girls. 65 are monosymptomatic primary nocturnal enuresis and, 33 are nonmonosymptomatic. 72 children accord with the severest form of bedwetting(>=7 times/week). 64.3%(n=63)children have behaviour problems and, girls get higher scores in withdraw and internalizing problems than boys. Boys are more aggressive. 42.9%(n=42) get positive results through SCARED. 9.2%(n=9) get positive results in DSRSC. Children with the severest form of bedwetting are likely to have more complex form of psychological problems. They have greater social problems, get higher scores in behaviour problem (P=0.026) and lower scores in Piers Harris Children's Self concept Scale (P=0.034). But children with or without the severest form of bedwetting have no significant difference in SCARED . MNE have no significant difference with NMNE in scores of all the three children's self-evaluated scales. Conclusion: Most children with PNE had different psychological problems. The severer the symptoms are, the more complex the psychological correlates are. These preliminary findings support the view of enuresis closely related with psychological problems. Psychological problems are affected by many factors except for enuresis, so further researches need to be conducted to determine whether there is a causal relationship between psychopathology and enuresis.

The current situation of treatment of primary nocturnal enuresis in children Xiaomei liu, Lu chen, Weiran zhou Nephrology, Beijing Children's Hospital, Beijing, China

Objective: We would like to discuss the treatment of primary nocturnal enuresis in children. Methods: Children, diagnosed with primary nocturnal enuresis, paid outpatient visits to Beijing Children's Hospital from January 2011 to May 2012, are enrolled in the study. The information of previous clinical experience, compliance and expected treatment goals are collected and analyzed. Results: 623 children, with the mean age of 8.69±2.33 are enrolled. 449 children have visited doctors before, 295 have unsatisfied clinical experience, such as late intervention, less standardized treatment and over-treatment. 64.36% have poor compliance with doctor's suggestion of behaviour therapy and alarm therapy. 262 children have been suggested to take medicines such as Chinese traditional medicine DDAVP, but 17.8% of them refused the suggestion anxious of the side effects or stop the usage on their own. The primary expected treatment goal of parents is to improve the symptoms, accounting for 86.04%. Excluding the underlying diseases, Alleviating parents' burden, treating the symptoms of hyperactivity or inattention and avoiding adverse effects on intellectual and fertility are also included. For children, they'd like to improve the symptoms so they won't be published by parents or laughed by their fellows. Most parents (79.94%) preferred medicines as the first choice. 12.40% parents and children are inconsistent with the behavior therapy (the rate of Newly diagnosed and non newly diagnosed children were 8.04%, 14.03% respectly. χ 2 test P>0.05). 38 (6.10%) don't accept alarm therapy (the rate of Newly diagnosed and non newly diagnosed children were 2.30%, 8.02% respectly. χ 2 test P>0.05). Conclusion: Children with primary enuresis need early intervention and standardized treatment. The effect of treatment largely depends on cooperation and joint participation of children and parents. This study found that the compliance of behaviour training and alarm therapy is lower than medicine. The goals of children and parents, living conditions and other factors should be fully considered in the treatment of enuresis to improve short-term remission rates and long-term cure rate of the disease.

Beware of the sleeping bladder in monosymptomatic nocturnal enuresis (MNE) Britt Borg, Konstantinos Kamperis, Birgitte Mahler, Soren Rittig Pediatrics, Aarhus University Hospital, Skejbv, Denmark

Objective: Bladder reservoir function in children with nocturnal enuresis is assessed by maximal voided volumes (MVV) registered on frequency-volume charts. Although a degree of association is evident, MVV does not always reflect the nocturnal bladder reservoir function in MNE. We aimed to evaluate the nocturnal bladder reservoir function during the night in children with apparently normal MVV. Methods: Data from 557 children aged 5-15 treated for MNE in a tertiary referral centre was analyzed. Data from 135 children was excluded due to reduced MVV according to ICCS standardization and 156 due to lack of home recordings. The remaining were divided into two groups, based on whether MVV was above (n=91) or below (n=175) the average nocturnal urine production during wet nights (NUPw) . First morning voids were not included in MVV values. Results: 34 % of the children with MNE and a normal bladder capacity had an average nocturnal urine production during wet nights below their MVV, one third of these had NUPw below 65 % of MVV expected for age. These children did not differ significantly in terms of demographic characteristics, frequency of wet nights or treatment time needed to achieve dryness. Urine output during dry nights was not statistical significant between the groups (p=0.077, mean diff.=28.47 ml). The group with MVV>NUPwet shared significantly higher MVV to MVV expected for age ratios (mean diff=0.16, p<0.05). Urine output during wet nights was lower in the group of children with MVV>NUPw (diff: 96.45 ml, p<0.05) . Conclusion: Children with MNE and apparently normal bladder reservoir function during daytime may experience wet nights with urine volumes well below their MVV and MVV expected for age. The fact indicates bladder reservoir function abnormalities during sleep that is not assessed by day recordings. Physicians treating children with MNE should consider anticholinergic treatment.

One year experience of a multidisciplinary investigation on Nocturnal Enuresis in a Brazilian Tertiary Care Facility Objective: To characterize a cohort of children, 6-16 yrs old, with nocturnal enuresis, defined by 2010 ICCS criteria on the basis of a multidisciplinary evaluation including renal, neurological, psychological and physical therapy approaches. Chronic clinical conditions and genetic disorders constituted exclusion criteria. Methods: After IRB approval, families were invited to participate in the project through press releases, 130 children completed quality of life evaluation through clinically validated questionnaires, followed by a one -day multidisciplinary clinical evaluation. Sleep, urinary and intestinal diaries were evaluated. Urinary sonography, nocturnal polysomnography, urinary and blood analysis were scheduled. Results: 96/130 participants (pts)were male (73,8%), mean age 8 ±1 yrs. Twenty two children were excluded due to noncompliance /chronic clinical conditions. 118/130 pts were evaluated. 93/130 parents of children/adolescents, answered the CBCL questionnaire, 25/93 (27%) of which resulted in scores compatible with clinical psychological conditions. Monosymptomatic enuresis (MoE) was diagnosed in 89/118 children (75.4%). In the MoE group, 67/89 pts were diagnosed with intestinal constipation; 7/89 pts with obstructive sleep apnea, 2/89 pts with hipercalciuria and 2/89 were characterized with ADHD. Area and velocity of center or pressure displacement (VM) were used for postural control evaluation and resulted in 3,67 ±2,95 cm 2 and 20,35±13,04 cm.s-1, respectively; 22/89 pts were also assessed for postural alignment exhibiting pelvic anteversion (10,27 ±3,71 grades) and head protusion (0,40±1,86 grades). These results corroborate with a smaller range of motion found for hip flexion (94,86±17,14 grades) and hip extension (2±3,89 grades) assessed using a goniometer. Conclusion: Multidisciplinary evaluation of enuretic children may be the key to optimize therapy on the basis of the underlying etiology of the process Abstract# P-SAT056 Weight depending dosing of desmopressin (dDAVP) in nocturnal enuresis Pauline De Bruyne 1 , Ann De Guchtenaere 1 , Charlotte Van Herzeele 1 , Ann Raes 1 , Jo Dehoorne 1 Piet Hoebeke 2 , Erik Van Laecke 2 , Johan VandeWalle 1 1 Paediatric Nephrology, Ghent University Hospital, Ghent, Belgium 2 Department of Urology, Ghent University Hospital, Ghent, Belgium Objective: In children as well as in adults, a uniform starting dose of desmopressin is prescribed. This uniformity is based on the inability to detect a weight-dependent dose-concentration correlation as this correlation is probably blurred by the wide intra-and interindividual differences in plasma concentration for a fixed desmopressin dose.

Recently, a smaller variation in plasma concentration was shown for the oral lyophilisate formulation of desmopressin (compared to tablet formulation). Therefore, this study assessed a possible correlation between weight-corrected dose and plasma concentration for both formulations. Methods: 23 children with monosymptomatic nocturnal enuresis were recruited in a tertiary centre. Two tests were performed on two separate days (at two weeks interval) in identical, standardized conditions: on day 1 desmopressin tablet 200μg and on day 2 desmopressin oral lyophilisate 120μg was administered. Plasma concentrations were measured at one, two and six hours post dosing. The nonparametric Spearman's rank correlation coefficient was used for assessing the correlation between weight corrected dose and plasma concentration. Results: Mean (SD) age and body weight of the patients were respectively 12.7 (2.9) years and 50.1 (15. 2) kg. A positive correlation between plasma concentration of dDAVP was found for the oral lyophilisate formulation at 2 hours (P-value 0,021) and 6 hours (Pvalue 0,005) post dosing. This is not the case for the tablet formulation. Results are shown graphically in figure 1 and 2. Figure 1 and 2: correlation of dose corrected by weight to plasma concentrations at 2 and 6 hours post dosing Conclusion: To the best of our knowledge, this is the first pharmacokinetic study showing a significant dose (normalized for size) -concentration correlation for desmopressin. Nevertheless, this correlation was only significant for the oral lyophilisate group. This result is clinically important as it is a strong indication for more predictable plasma concentrations for the oral lyophilisate formulation, and thus preventing elevated concentrations of desmopressin. Objective: Increased nocturnal urine production and/or bladder hyperactivity in primary nocturnal enuretic (NE) patients could possibly be associated with autonomic nervous system dysfunction. Reports of studies on autonomic nervous system dysfunction in NE are limited. To investigate autonomic nervous system function in enuretic children by performing ambulatory blood pressure monitorisation (ABPM) for 24 hours. Methods: Children with primary NE were enrolled in this study and they get 24 hour ABPM. Their results were compared with healthy children. Urinalysis,urine electrolyte levels, urinary culture and urinary system ultrasound were carried out in all the children. They also requested to have a diary about daily fluid intake and volume of daily urine. Results: The enuretic group consisted of 28 children (M/F:19/9) and the control group of 27 healthy children (M/F:14/13). The mean ages were 7.9±2.2 years and 8.77±2.65 years, respectively. The mean 24-hour BP and daytime DBP did not differ between the groups however, the mean systolic BP was significantly higher in enuretic children (p<0.05). The mean nighttime systolic BP, DBP and MAP were significantly higher as well in the patient group. Daytime BP load did not found to be elevated in both groups.Elevated night time systolic BP load was observed in 4 patients and was not observed in control group (14.2 % vs 0%) (p<0.01). Nighttime diastolic BP load was elevated in 7 of 28 patients, compared with 1 of 27 control subjects (p<0.01). The nocturnal BP dip was significantly reduced in the patient group compared with control group for diastolic blood pressure ( 67.8 % vs 14.7 %, respectively; p=0.037). Patients with elevated night time BP load was found to have higher frequency of urinary incontinence per week as well as per night while compared with enuretic children with normal night time BP load (r=0.72, p<0.01; r=0.69, p<0.01 respectively). Conclusion: Nocturnal BP loads were significantly higher in children with enuresis. These subtle abnormalities of circadian blood pressure regulation, loss of nocturnal dip and observation of decreased nocturnal pulse rate may reflect autonomic nervous system dysfunction and pathogenesis of EN.

Objective: Bladder dysfunction and especially OAB plays a major role in nocturnal enuresis, not only in the non monosymptomatic (NMNE) but as well in the monosymptomatic patients (MNE) . If the enuresis is related to a mismatch in nocturnal diuresis and maximal functional bladder capacity, then the bladder volume should be a major parameter, but is sofar not taken as a parameter for subtyping into NMNE in the ICCS standardization. Maximal voided volume in a diary is the golden standard, although relation with outcome is poorly studied. Methods: Aim of the study was to evaluate the optimal parameter for estimation of bladder volume as maximal voided volume in a diary, during forced diuresis, bladder volume during 3 uroflow + uroflow), correlating with cystomanometry (if indicated). Studypopulation 398 patients age 5 to 18 years, >6/7days wet, only 48 cystomanometries. Results: If we compare the data from the bladder volume against the reference frame from Rittig (Aarhus), then 60,4% of patients had a MVV in diary < 2,5% percentile demonstrating that a majority of patients had a small for age voided volume in their diary. Correlation between MVVdiary, MVV forced diuresis, bladder capacity (uroflow+ residu) and cystomanometry show that there is a strong correlation between the 4, But especially for the last 3 parameters (r 2 0.48-0.62, p <0.01),but results with MVV diary are worse (r 2 0.34-0.48, p 0.04-0.018) . There is no sex or gender difference in this observation. Correlation with response to therapy at 1 year shows a superior correlation with MVV forced diuresis and during uroflow than MVV diary (p0.04). Since cystomanometry is only performed in refractory cases, this voided value had no correlation with clinical outcome. Conclusion: Bladder volume can be estimated in several ways, each with their advantages and pitfalls. Our data demonstrate that the alternative non invasive methods during force diuresis and in center during 3 uroflow correlate best to each other and to the cystomanometricvalues. But as well to the one year outcome (p 0.04).

Abstract# P-SAT060 Evaluating nocturnal diuresis (polyuria)

Claire DeBusschere, Delphine Guenter, Sophie Wouters, Johan VandeWalle, Ann Raes, Joke Dehoorne Pediatric, Nephrology, Gent, Belgium

Objective: Nocturnal enuresis is more than bedwetting, but a symptom of a disorder involving multiple pathogenetic factors in circadian rhythm of diuresis/solute excretion and bladder dysfunction. Where the sum of diaper weight and morning voided volume, is the standard to evaluate nocturnal diuresis (polyuria), it does not give indices about pathophysiology. The Aarhus concept : in center studies during standardised intake, offers the advantage of standardized conditions, and reliability of the values, but is restricted to specific research centres in limited patients for budgetary reasons). The Ghent concept : A home based 24 hours concentration prophyle with 4 time daytime and 4 nighttime collections offers an alternative. This one day test was not validated against 14 days diuresis nighttime registration, and was criticized since waking up the patient overnight might increase diuresis and solute excretion, as is demonstrated in sleep deprivation Aim of the study: to validate nocturnal diuresis and solution excretion to evaluate nocturnal polyuria against 14 days nighttime diary and 2 days daytime diary. Methods: To the study the incidence of nocturnal polyuria, and to evaluate the value of the morning osmolality in the study of nocturnal polyuria. Results and Conclusion: 401 children (262M), mean age 8 y (5-18y) , 24 h diuresis 1023+/−445 ml, daytime 663+/−367 ml nighttime 365+/−203ml, No sex difference. Correlation between volumes in diary and 24 h concentration prophyle during 24 h (p 0.036), night (r 2 0.336 p 0.013) and day (p 0.012). Only 13% of patients have a nocturnal polyuria (&gt;130%EBV= absolute nocturnal polyuria), but up to 28% have a nocturnal diuresis higher than 100% EBV and &gt; than their MVV (relative polyuria). There is no correlation between early morning osmolality and nocturnal diuresis-rate (nocturnal polyuria).

Objectives, Methods and Results: We present a 16 months old albanian female with fever, urinary retention and constipation. This problems has occurred one week before hospitalization. No history of trauma. Life history has no remarkable data. No history of UTI or constipation before. Physical examination: weight and stature on 50 centile. No respiratory problems. The big overfilled bladder was palpated. On the left sacral region, was a tumorous mass very painful and worm. Perianal reflex, bulbocavernosus were weak. Other reflexes were normal. There were a hard stools in the rectum. Other systems examination was unremarkable. High ES rate and pathological urine sed with infection indicators. Other Laboratory examination was unremarkable. Ultrasound examination resulted with dilatation of the left kidney pyelon (9mm), distended and overfilled bladder lumen (>400ml) with thin bladder wall. Rectum distended and full with fecal masses.Lateroposterior there is a sonolucent cystic formation with (volume 7ml). That cystic formation was supected for ureterocellae first. The urinary catheter and rectal clismas were done. That formation was abscess and it was drained. And two weeks after was better. Voiding cystourethrogram and Computer Tomography was done and resulted that are no anatomical defects in the Urinary tract. Because of the collapsed bowels there's not good visualization of the region around the sigma and rectum. Irigografy resulted: dolihosigma and suspected for Hirschprung. Two months later the abscess relapsed and needed drainage again. Conclusion: Since the abscess has repeated the next step will be biopsy. Hirschprung, Tailgut cysts and other presacral masses should be included in the differential for patients with recurrent abscess in the perisacral space with clinical manifestation of urinary retention and constipation.

Abstract# P-SAT062 Treatment failure to enuresis alarms: Challenges and factors influencing adherence to treatment Indra Ganesan 1 , Jessica XJ Hooi 2 , Jasmine JY Goh 2 , YH Ng 1 , SM Chao 1 1 Nephrology Service, KK Women's and Children's Hospital, Singapore, Singapore 2 Department of Pediatrics, KK Women's and Children's Hospital, Singapore, Singapore

Objective: The study aims to identify factors influencing treatment failure to enuresis alarm and the factors influencing adherence to treatment. Methods: All patients aged 6 to 16 years referred to the voiding clinic in KK Women's and Children's Hospital, Singapore with Primary nocturnal enuresis who opted for enuresis alarm treatment, over 6 year period from 2007-2013, were prospectively studied. Data, via direct interview and parental questionnaire was collected on demographics, age at presentation, presence of family history, frequency of nocturnal enuresis per week and per night, on whether child awakens after wetting, or associated constipation in the child and parental perception of why primary enuresis happens.

Results: Seventy-nine of 111 (71%) children with primary nocturnal enuresis with complete data were included. Mean age at presentation was 9.6±2.1 years and 45 (57%) were male. Twenty-five children had a family history of nocturnal enuresis in a 1 st degree relative. Eighteen children had constipation. Fifty-one (64 %) children achieved the targeted 21 consecutive dry nights. In the remaining 28 children, the main reasons for treatment failure were non adherence to usage of enuresis alarms mainly due to an inability to wake up to the alarm in deep sleep (71%) and underlying neurobehavioural conditions (21%) (ADHD, autism, depression and a chromosomal disorder). The only significant clinical factor predicting treatment failure was the presence of an underlying neurobehavioural condition (OR 2.8, and developmental delay . Factors such as male gender, family history in first degree relatives, frequency of enuresis, arousal when wet, and the presence of constipation, were not significant. Conclusion: In our population, 64% of children achieved nocturnal urinary continence with enuresis alarms. Treatment failure of enuresis alarms is higher in the presence of an underlying neurobehavioural condition and developmental delay. Non adherence to treatment is mainly due to inability to arouse from deep sleep.

Long-term follow-up of children with nocturnal enuresis -do enuretics become nocturics?

An-Sofie Goessaert 1 , Bente Schoenaers 2 , Olivier Opdenakker 2 , Karel Everaert 1 , Johan VandeWalle 3 1 Urology, Ghent University Hospital, Ghent, Belgium 2 Ghent University, Ghent, Belgium 3 Pediatric Nephrology, Ghent University Hospital, Ghent, Belgium

Objective: Although an overlap between both nocturnal enuresis (NE) and nocturia is known, research on the occurrence of both conditions in one patient is lacking. This study aims to investigate the prevalence of nocturia and other urinary symptoms in patients who have suffered from NE. Methods: A questionnaire was sent to 1265 patients treated more than 3 years ago in the University Hospital of Ghent for NE evaluating the history and current status of enuresis and validated questionnaires on urinary incontinence (ICIQ-UI) and overactive bladder symptoms (ICIQ-OAB). Medical files of all subjects were analysed on the history of enuresis. All subjects were asked to sign an informed consent. Results: Of the 516 (41%) subjects who completed the questionnaire, 183 reported nocturia (36%), with a sex ratio (M/F) of 101/81 in the nocturic group versus 230/103 in the non-nocturic group, mean age is 18 versus 17 years old, respectively. Comparing the nocturic and nonnocturic group, retrospective analysis of the history of enuresis shows an older age at which the subjects were cured (10 vs 9.5, respectively) and a higher percentage of non-monosymptomatic NE (74% vs 63%, respectively) in the nocturic group. No differences in past treatment for NE were found between both groups. Prospective analysis shows a significantly higher prevalence (p<0.001) of voiding frequency during daytime, urge and urinary incontinence in nocturics compared to nonnocturics. With an increase in number of nocturic episodes per night, the percentage of female subjects increases (p<0.001) and the percentage of subjects with non-monosymptomatic NE increases as well (p<0.011). Conclusion: Over one out of 3 former enuretic patients develops nocturia, often accompanied by other urinary symptoms and with significant bother. Cure of nocturnal enuresis does not necessarily equals cure of the urological pathology and some of the nocturic patients, who remain to suffer from bothersome symptoms, might benefit from continuous treatment for the underlying urological condition, such as overactive bladder syndrome or nocturnal polyuria.

Objectives, Methods and Results: A 3 year old girl was admitted to our hospital due to unusual pattern of voiding. When she wanted to urinate, she was writhing in pain; she cried, became pale and sweated. Since the newborn period parents have observed sudden episodes when the girl started to cry and curled up her legs. She stopped when she began to urinate. The girl's father occasionally had pains in the jaw between yawning. The girl's voiding was like parents described: when she was trying to begin urinating, she was squatting on the toilet seat, crying and became pale and sweated. When voiding occurred the girl seemed to relax. The voiding volume was small and the urinary stream was weak. All investigations results were normal. According to the clinical course we assumed that the girl had an unusual expression of Paroxysmal extreme pain disorder (PEPD). The diagnosis was confirmed by the detection of the heterozygous pathogenic mutation in the SCN9A gene, a missense mutation in exon 5. The same mutation was also confirmed in her father. The girl was started on carbamazepine and the pain attacks almost disappeared. PEPD is a rare autosomal dominant neuropathy linked to mutation in the SCN9A gene which encodes voltage-gated sodium channels. Abnormal pain sensitivity occurs due to changes in the properties of the channels. PEPD's onset is in the neonatal period with the most characteristic clinical features being attacks of excruciating pain in the rectum, genitalia, face and limbs. In literature we did not find any citation of patients with attacks triggered by the voiding stimulus. Carbamazepine has been effective in relieving symptoms but the response is often incomplete (1) (2) (3) , as in our patient. Conclusion: The case described is interesting because of the rare clinical expression of PEPD with pain triggered by voiding. The same mutation was recently found in patients with SFN (4) . The study of functional importance of this mutation showed that the result of the mutation is increased frequency of firing pain-signaling neurons (5) .

Rita Pavione Rodrigues Pereira 1 , Vera HerminaKalika Koch 2 , Simone Nascimento Fagundes 2 , Aline Rossi 1 , Juliane de Oliveira Marques Vieira 1 , Clarice Tanaka 1 1 Physical Therapy, University of São Paulo, São Paulo, Brazil 2 Communication Disorders and Occupational Therapy, University of São Paulo, São Paulo, Brazil

Objective: To assess the postural control in children and teenagers with enuresis compared to a control group Methods: 22 enuretic (EG) patients (11 boys and 11 girls) with mean± SD of 10.52±2.77 years, ranging from 7 to 16 years old, and 19.39±3.50 of BMI were paired with assyntomatic kids (CG) of equal age, gender and BMI. Three trials of 20 seconds were collected while standing on a forceplate at 60 Hz of frequency with standard position of feet in four different sensorial conditions: (1) open eyes and stable surface; (2) closed eyes and stable surface;

(3) open eyes and unstable surface, and; (4) closed eyes and unstable surface. The analized variables were area (A) and velocity (Vm) of center of pressure displacement (CoP). Student t-tests were used to compare variables between groups for each sensorial condition. The level of significance was 95% for all the analysis.

Results: When compared to CG, the EG revealed larger area of CoP displacement in the condition (1) (mean± SD of 2,46±2,11 cm 2 for CG and 4,16±2,76 cm 2 for EG; p=0,027), and in condition (4) (mean± SD of 4,29±2,45 cm 2 for CG and 7,33±6,46 cm 2 for EG; p=0,05). No difference in Vm were identified. Conclusion: When compared to assymptomatic control group, the enuretics children and teenagers presented larger area of CoP displacement under normal sensorial input (condition 1) and when vestibular input alone was offered (condition 4).

The hip and spine mobility is diminished in enuretic children and teenagers Rita Pavione Rodrigues Pereira 1 , Vera Herminakalika Koch 2 , Monica Maria Ribeiro Goncalves 2 , Thais de Souza Milhoratti 1 , Daniela Castro Pacheco 1 , Clarice Tanaka 1 1 Physical Therapy, University of São Paulo, São Paulo, Brazil 2 Communication Disorders and Occupational Therapy, University of São Paulo, São Paulo, Brazil

Objective: To assess the hip and spine mobility in children and enuretic teenagers compared to a control group. Methods: 22 enuretic (EG) patients (11 boys and 11 girls) with mean ± SD of 10.52±2.77 years, ranging from 7 to 16 years old, and 19.39±3.50 of BMI were paired with assyntomatic kids (CG) of equal age, gender and BMI. The range of motion of hip flexion and extension was measured using a goniometer. Spine flexibility was measured using Schober e Stibor test and bank of wells. Student t-tests were used to compare variables between groups. The level of significance was 95% for all the analysis. Results: When compared to CG, the EG revealed diminished range of motion of hip extension bilaterally (2±3.89 for EG and 10±7.99 for CG at right side; p<0.01; GE 2.05±3.88 for EG and 10.4±7.22 for CG at left side; p<0.01). No difference was found for hip flexion range of motion, however compared to CG, EG showed lower values bilaterally (94.9±17.1 for EG and 100.32±8.52 for CG at right side; 98.3±11.7 for EG and 101±11.1 for CG at left side). The spine flexibility did not presente any difference for Schober (14.88±3.18 for EG and 16.18±1.16 for CG; p =0.07), and Stibor (50.21±6.2 for EG and 51.93±7.37 for CG; p=0.40) and bank of wells (25.58±7.67 for and 23.66±7.42 for CG; p=0.63). Conclusion: When compared to assymptomatic control group, the enuretics children and teenagers presented diminished range of motion of hip extension suggesting transversal misalignment of the pelvis.

More concern about enuresis children: an epidemiological study of primary nocturnal enuresis in elementary schools in Shanghai Yibing Zheng 1 , Yinv Gong 1 , Hong Xu 1 , Keli Wang 1, 2 , Zhonghui Ni 1,2 , Dandan He 1,3 1 Nephrology and Rheumatism, Shanghai, China 2 Xuhui Health Bureau, Shanghai, China 3 Minhang Center for Disease Control and Prevention, Shanghai, China

Objective: (1) To assess the prevalence of primary nocturnal enuresis (PNE) and its risk factors in children of elementary school age in Shanghai.

(2) To evaluate the impact of enuresis on these children and their parents, and to identify the methods and effectiveness of managing enuresis. Methods: A randomly selected cross-sectional study was conducted in four elementary schools in two districts inShanghai. The parents of these children were asked to complete questionnaires anonymously which included items about the presence and frequency of enuresis, its risk factors and its perceived impact and management. The distress caused to the family by enuresis and the outcome of any management was evaluated using a 5-point visual analogue scale (VAS). PNS was defined as an involuntary voiding of urine during sleep, with a frequency of more than two times a week for three consecutive months, in the absence of congenital or acquired defects of the central nervous system. Results: A total of 7600 questionnaires were distributed. The overall response rate to the questionnaire was 97.1%, girls 45.2% and boys 51.8%. The prevalence of PNE declined with age from 6.3% at 7 years old to 0.5% at 11 years old. Of all enuresis children, 10.1% had daytime urinary symptoms and 11.7% had a positive family history. 58.8% of parents were concerned and 17.6% were very concerned about enuresis. 41.2% of children were worried about and 5.9% were very worried about enuresis. Only 35.3% of PNE children sought for treatment and the common strategies (23.5%) were adjusting lifestyle such as restriction of water intake at night, 11.9% used alarm clock and 5.8% were take medication. 48.6% of parents felt that the current treatment was effective. Conclusion: We conducted a relatively scientific epidemiological survey on the prevalence of PNE in two districts in Shanghai. We find enuresis still has great impact on children and their parents, but only a small part of them will seek for treatment. And only half of the parents feel that the current treatment is effective. Therefore, it would be desirable to create a series of standardized management and follow-up processes for enuresis children.

Abstract# P-SAT068 Renal manifestations of Tuberous Sclerosis: a descriptive analysis Sophy Korula 1 , Alka Ekbote 2 , Naresh Kumar 1 , Sumita Danda 2 , Indira Agarwal 1 , Swasti Chaturvedi 1 1 Paediatrics, Christian Medical College, Vellore, India 2 Clinical Genetics, Christian Medical College, Vellore, India

Objective: To describe the renal manifestations in children 0-18 years of age diagnosed with Tuberous Sclerosis Complex (TSC) at a Tertiary Hospital in South India. Methods: Data of children with TSC who presented to Christian Medical College Vellore Hospital from January 2008 onwards, were analysed by a retrospective chart review. The cases were identified from outpatient records and underwent ultrasonography, urine analysis and serum creatinine to recognize renal involvement. Results: Twenty-five children with TSC were identified. Two children did not imaging studies available and were excluded from the analysis. The age of included children ranged from 5 days to 15 years with a median of 8 years. Seventy four percent (17/23) were males. Ten of the 23 children had evidence of renal involvement (43.5%). Of the ten children with renal involvement, six had angiomyolipoma (60%), five had renal cysts (50%) and one had suspected renal cell carcinoma. In two children both angiomyolipoma (AML) and cysts were noted. None were symptomatic. One child was found to have proteinuria and another had reduced creatinine clearance of 52.5 ml/min/m 2 with normal BP and urinalysis. The rest of children had no evidence of proteinuria and had normal creatinine clearance. Conclusion: We conclude that all children with TSC should be screened for renal involvement and regular follow up should be arranged. 

Abstract# P-SAT069 Sonographic growth charts for kidney length in normal Korean children: a prospective observational study Objective and Methods: Kidney length was measured by sonography in a prospective observational study of 343 normal children from 0 to 12 years of age. Results and Conclusion: There was a good correlation between kidney length and somatic values including age, weight, and height. The rapid growth of height during the first 2 years of life was intimately associated with a similar increase in kidney length. The values of weight and height showed good correlation with kidney length in children from 2 to 12 years of age. Height should be considered the important factor to correlate with kidney length. For the children aged 2 years or older, the regression equation was obtained: kidney length (cm) = 2.677 + 0.046 x height (cm). For the younger than 2 years, the equation was: kidney length (cm) = 1.120 + 0.066 x height (cm).

Objective: The outcome of children with unilateral nonfunctioning MCDK.

Methods: 24 children (13 pts with right MCDK), afunction confirmed by renal scan, 12 boys, mean age of 6 years (range 1.5 month -21.5 years), mean observation period of 6.7 years with a range of 1.5 month -21.5 years. Prenatally diagnosis was made in 83 %. Abnormalities of the contralateral kidney were found in 4 /24 pts (13 %) -dystopia (n=2), hydronephrosis due to ureterovesical obstruction (n=1), VACTER syndrome (n=1). In 2 pts MCDK had been removed (in period 1991-1997) . Collected data include eGFR (calculated by Cystatin C), urine protein/creatinine ratio, microalbumin/creatinine ratio, A1microglobulin (A1M)/creatinine ratio, urine Beta-2microglobulin (B2M), urine NGAL, urine NAG, blood pressure (BP), renal length. Results: Hypetrophy of the contralateral kidney (length >2 SDS) was detected in 19/24 pts (79%) to 2 years of age. Complete involution (in 33 %) or decrease of size (in 30%) of MCDK occured in the first 5 years. Hyperfiltration (defined as eGFR of 149 ml/min/1.73m2) was seen in 4 pts (17 %). None of 24 pts demonstrated decreased eGFR, proteinuria (mean value of 24.8 mg/mmolcreatinine), microalbuminuria (mean value of 2.0 mg/mmolcreatinine).Urine A1M (mean value of 5.8 mg/mmolcreatinine), urine B2M (mean value of 0.2 mg/l), urine NAG (mean value of 7.3 Ukat/mmolcreatinine), urine NGAL (mean value of 10.4 ng/l) were in a normal range. All pts were normotensive -BP defined as <95th percentile for age and gender. The combination of hepatoblastoma with renal failure due to congenital dysplastic kidneys in children is extremely rare. We report two children in renal failure since birth due to congenital cystic dysplastic kidneys. Hepatoblastoma was subsequently diagnosed in both of them. Methods and Results: Case1 A male infant was born at 35 weeks gestation with an antenatal diagnosis of bilateral dysplastic kidneys and bladder outflow obstruction secondary to a prolapsing ureterocele, anhydramnios, and pulmonary hypoplasia. He underwent surgical repair of other congeniatal abnormality after birth. Peritoneal dialysis was initiated at 3 months. Treatment with Erythropoietin (EPO) was commenced at 6 weeks of age. Case 2 A male infant with congenital cystic renal dysplasia, bilateral vesicoureteric reflux pulmonary hypoplasia and oligohydramnios was born at 36 weeks gestation. Peritoneal dialysis was commenced at day 20 due to anuria from birth. EPO was commenced at 6 months of age. Both of them required high dose EPO to maintain adequate haemoglobin. Hepatoblastoma was diagnosed in both of them at 2-3 years, following an incidental finding in abdominal imaging done for different reasons.

Conclusion: Hepatoblastoma has been reported in association with cystic renal dysplasia in 5 children in the literature to date. None of these children had evidence of impaired renal function prior to the diagnosis of hepatoblastoma. In our report both the child were treated with high dose erythropoietin prior to diagnosis of hepatoblastoma. Erythropoietin (Epo) is the primary regulator of erythropoiesis through specific interaction with its receptor (Epo R). EpoR expression has been demonstrated in common paediatrictumour cells and such expression is reported to promote tumour cell survival through release of angiogenic growth factors. Angiogenesis is the primary requirement for tumour growth. Epo production has been observed in patients with hepatomas and in the HepG-2 cell line in hepatoblastoma. We report two cases of hepatoblastoma detected incidentally in children with established renal failure and propose a putative role of EPO in the development and / or progression of hepatoblastoma in this population. Objective: Hypercalciuria is the most common metabolic cause of renal stone. Long term immobilization is associated with hypercalciuria and bone loss. The effect of short term immobilization on hypercalciuria was the main objective of this study.

Methods: In a prospective study all orthopedic patients less than 40 years with pelvic fracture who were assigned for immobilization with traction were enrolled in this study. Serum (calcium, phosphorous, alkaline phosphatase, sodium, potassium, uric acid, BUN, creatinine)and fasting urine calcium, creatinine, sodium, potassium and uric acid were checked within 48hours of hospitalization and also in 1st,2nd and 3rd weeks of immobilization and then after 2to 3months of mobilization. Student's t-tests used for statistical analysis. Results: Fifty five patients 45 male and 10 female with the mean age of 19.4 ± 12.7 years were studied. Urine calcium /creatinine (U Ca/Cr)* ratio before immobilization U Ca/Cr0 was 0.13±0.06. One, two and 3 weeks following immobilization U Ca/Cr ratio was 0.17±0.11, 0.22±0.12 and 0.29±0.17respectively.Multivariate tests revealed a significant rise in U Ca/Cr ratio during hospital stay when this value checked before immobilization and throughout immobilization as soon as end of 1st week and following in 2nd and 3rd

week ( Objective: Importance of renal biopsy(RB) in diagnosis and treatment of kidney disease is the same for children as for adults. However there are some differences between institutions in practice of this invasive method. The aim of this study is to identify the current status and complications associated with the renal biopsy of childhood kidney disease.

Methods: A retrospective study was conducted based on medical records from all patients who had undergone native renal biopsies with 16-gauge needles from April 1997 to November 2012 in our center. We analyzed the number of experiments, sex, age, indication, the types of harvest (percutaneous or open) and anesthesia (general or local), histopathological findings, and complications. Results: A total of 158 renal biopsies were performed in 141 patients (82 boys, 59 girls). The mean age at first procedure was 9.4 years. About 70 % of the indications for a biopsy showed moderate proteinuria and hematuria in the annual urinary screening program in Japan, and approximately 20 percent of those were refractory childhood nephrotic syndrome. Open renal biopsies were practiced in only 3 cases, and the rest of children underwent percutaneous ultrasound-guided kidney biopsies. The most procedures of patients aged 10 or younger were executed under general anesthesia due to the non-cooperation of them. About 30 % of the biopsy-proven kidney diseases showed immunoglobulin A nephropathy, and approximately 20 percent of those were Henoch-Schonlein purpura nephritis. For the post biopsy complications, 5 out of all performances administered hemostatic agents for moderate hematoma, but none of them required blood transfusion or surgical intervention. Conclusion: We suggested that renal biopsy could be supportively performed under local anesthesia in the patients more than the upper grades of elementary school. In addition, 17 patients aged 3 or younger, including 12 months, could undergo the percutaneous ultrasound-guided renal biopsies without procedural and general anesthesia complications. Therefore, our results revealed that this procedure was considered safe even in infants.

Abstract# P-SAT084 Fifteen years review of indications and results of renal biopsy in children from a single center in Egypt There were 20 insufficient biopsies. In pathologically diagnosed 1226 specimens primary glomerulonephritis was the most common finding (n=826, 67.4%) followed by secondary glomerulonephritis (n=238, 19.4%) and end stage renal disease (n=50, 4.1%). The most common causes of primary glomerulonephritis were minimal change disease (n=267, 21.8%), diffuse proliferative glomerulonephritis (n=188, 15.3%), focal proliferative glomerulonephritis (n=164, 13.3%) and focal segmental glomerulosclerosis (n=129, 10.5%). Lupus nephritis (n=209, 17%) was the most common cause of secondary glomerulonephritis followed by hemolytic uremic syndrome (n=16, 1.3%) and amyloidosis (n=10, 0.8%). Only one mortality has been reported as a complication of renal biopsy (uncontrolled bleeding).

Conclusion: This study introduces the first biopsy based epidemiological information of pattern of renal diseases in Egyptian children from a single tertiary pediatric center in which minimal change disease was the most common histopathological finding and steroid resistant nephrotic syndrome was the most frequent indication for biopsy. Results: A total of 112 children were enrolled in the study. Most of them (71%) had isolated microhematuria, and were proved mild lesion of glomeruli (60%) by renal biopsy. About 30% of them, however, might have progressive glomerulonephritis, such as IgA nephropathy, focal segmental glomerulosclerosis, Alport syndrome and so on. These asymptomatic children were found urine abnormalities due to either school urine screening study, health examination, or during diagnosis of non-renal diseases. In center A, IgA nephropathy was the most case diagnosed, while in center B and C, minor glomerular abnormalities was the most case diagnosed. The difference among three centers was significant and this variation might be due to different indications of renal biopsy.

Conclusion: Our findings confirm that urinalysis may help early detecting progressive glomerulonephritis in asymptomatic children. Children with isolated microhematuria has relatively low risk of severe pathologic lesion of glomeruli, thus isolated microhematuria per se might not be suggested as indication of early renal biopsy. Long-term follow-up with appropriate further examination is of great importance for these asymptomatic children.

Abstract# P-SAT089 Twenty-three-year review of disease patterns from renal biopsies: an experience from a pediatric renal center There were 59 episode of peritonitis with an incidence of 1 episode per 16.8 patient/months. The commonest organisms isolated were gram negative rods 12(20.3%), followed by staph aureus 11(18.6%). Sixteen (27.1%) were culture negative and 3(5.1%) were fungal peritonitis. Catheter removal related to peritonitis was performed in 20 patients in whom only one patient returned back to PD. Exit site infection (21 episodes) and tunnel infection were reported in 20 and 10 patients respectively. Catheter malfunction was the most common non infectious complication seen in 27 (61.3%) children. Other noninfectious complications include haemorrahgic effluent 5 (11.3%) children while Catheter leak, umbilical hernia and inflow pain where seen in 2 (4.5%) children each. At the end of the study 17 (18.7%) children remained PD active, 26 (28.6%) transferred to HD, 13(14.3%) transplanted, 9(9.9%) suspended from PD, 5(5.5%) lost follow up and 21(23.1%) children died. Conclusion: In the setting of our country with limited resources and investment, CAPD is efficient and successful with complications comparable to most parts of the world. Attempts to produce PD fluids locally and to train and educate health care workers will greatly improve the use of PD in developing countries.

profile and response to therapy in patients with idiopathic membranous nephropathy from March 2010 to December 2012. Renal biopsies were studied for light microscopy, immunofluorescence and electron microscopy. Antibodies to the M-type phospholipase A2 receptor (PLA2R), bovine serum albumin (BSA), and cationic BSA were measured by Western blotting with patient sera (when available) and then detecting for both totalIgG as well as specifically for the IgG4 subclass.

Results: 80 renal biopsies were performed during the study period, out of which 11 patients had membranous nephropathy. Five were secondary to SLE and 6 patients (7.5 %) had idiopathic membranous nephropathy. Mean age at presentation was 13.16+/-1.94 years with 50 % having hematuria and 66.6% hypertension at presentation. Estimated GFR was 160.57+/-49.33. Renal biopsy showed findings consistent with membranous nephropathy on light microscopy with with<5% of the interstitium showing tubular atrophy and interstitial fibrosis. Immunofluorescence studies showed granular IgG and C3 along the peripheral capillary walls. Electron microscopy showed subepithelial deposits, with formation of basement membrane spikes between the deposits. Few intramembranous deposits were also present. Subendothelial and mesangial deposits were not present. There were no antibodies to BSA or cationic BSA detected in any of the five sera available. One of the five patients had circulating antibodies to PLA2R. All patients received prednisolone, angiotensin converting enzyme inhibitors and oral calcium supplements. Two patients continued to have nephrotic range proteinuria inspite of prednisolone and were started on cyclosporine. None of the patients developed any complications. Conclusion: Immunosuppressive therapy is beneficial in children with idiopathic membranous nephropathy. Anti-PLA2R antibodies can be seen as early as 13 years of age.

Abstract# P-SAT108 Hypercalcemic crisis and nephrogenic diabetes insipidus due to vitamin D intoxication Methods: Twenty-three children (46 kidneys) with first UTI with P-fimbriatedE.coli were enrolled in the study. Children were aged between few months and 10 years. All children were treated with adequate antibiotic therapy. DMSA scintigraphy was performed in a few days after infection and control scintigraphy was performed 5 months to 36 months later. DMSA findings were categorised as normal, generally diminished uptake of activity, focally diminished uptake of activity and clearly pathologic with renal scars. Results: The first DMSA scintigraphy immediately after infection was normal in 12 kidneys (26.1%), generally diminished activity was found in 21 kidneys (45.7%) and focally diminished activity in 6 kidneys (13% ). Renal scars were present in 7 kidneys (15.2%) . On the control scintigraphy 20 kidneys (43.5%) had normal findings. Generally diminished activity were found in 6 kidneys (13%) and focal diminished uptake in 8 kidneys (17.4%). Significantly higher number of scars, in 12 kidneys (26.1%), despite of antibiotic treatment were found on the control scintigraphy (p< 0.01). Conclusion: DMSA scintigraphy demonstrated that the infection with P-fimbriatedE.coli can result in permanent renal damage, which clearly points to UTI with P-fimbriatedE.coli as a risk factor in renal damage. Our results confirm obligatory scintigraphic follow-up of children with positive P-fimbriatedE.coli infection. Objective: Efficacy of mannitol with furosemide was compared with that of albumin with furosemide in the treatment of diuretic resistant oedema in childhood nephrotic syndrome. Methods: Forty patients fulfilling the criteria for "resistant oedema" in nephrotic syndrome cases were enrolled in this descriptive cross sectional study. Resistant oedema was diagnosed based on failure to achieve therapeutic response to diuretics or a weight loss of <1% body weight daily. All nephrotic syndrome patients with severe oedema, age 1 year to 15 years of both sexes were hospitalized and were managed with fluid restriction, no added salt and bed rest. Beside these 2 mg/kg/day oral furosemide or combination of furosemide and spironolactone, were given for 3 days to achieve desired diuresis. Those patients who did not get response were divided into two groups (Group-A, Group-B) in consecutive fashion. The Group-A study population, was with intravenous mannitol 0.5-1 gm/kg/day in single daily dose over 1-2 hrs followed by intravenous furosemide 1 mg/kg/day for 5 days. The Group-B study population was with intravenous albumin, 0.5 -1 gm/ kg /day in single daily dose over 1-2 hrs followed by intravenous furosemide 1 mg/kg/day in every alternate day, total 3 doses. Conclusion: This boy presented with Rhabdomyolysis, myohemoglobinuria and ARF. (Similar Pr Similar Presentation can be rarely seen in human being, but often seen in dogs). Blood exchange transfusion in time must be necessary for preventing "water fall effect" established, for it can eliminate inflammatory factor and toxic products such as BUN, Ccr, and creatinasein blood effectively. Blood tranfusion in combination with Azithromycin and Clindamycin must be a good choice replace the "Babesia-infected" RBC and eliminate pathogens in blood.

Beta-2-microglobulinuria in Human immunodeficiency virus infected children

Objective: To determine whether HIV infection in children is associated with increased urinary excretion of β2-microglobulin, which is a marker of tubular defect.

Methods: A prospective observational study was done of HIV infected children attending outpatient immunology clinics to investigate urinary β2-microglobulin excretion by measuring urine β2-microglobulin to creatinine ratio. In addition serum sodium, potassium, urea, bicarbonate and creatinine levels were documented and urine dipstick test was done to quantify proteinuria.

Results: Thirteen (13) children aged 2 months to 49 months (mean 20+/-4 months) were enrolled. All children had normal estimated glomerular filtration rate. Eleven children were on different HAART regimes (see table 1), one was receiving nevirapine prophylaxis and one was not on any treatment. The duration of HAART use was 1-16 months (mean of 8+/-2 months). Seven (7) children were on cotrimoxazole for Pneumocystis Jiroveci prophylaxis and 4 on anti-tuberculosis drugs, namely rifampicin, ethambutol, isoniazid and pyrazinamide. None received any nephrotoxic drugs. The HIV viral load was >1000 copies/ml in 8 children and <1000 copies/ml in 5 children. Of 13 HIV-infected children, 4(31%) had no abnormal urine protein excretion and nine (69%) had elevated urinary levels of β2-microglobulin.Of note only 2 (22%) of the 9 children with β2-microglobulinuria had associated proteinuria. Ten children ( 76%) had metabolic acidosis with the mean serum bicarbonate (CO 2 ) level of 16.9 mmol/l ( range 10-20 mmol/l). Conclusion: Proteinuria is believed to be the earliest finding for the diagnosis of HIV associated renal diseases. In our study β2microglobulinuria and metabolic acidosis were the prominent findings suggesting that HIV induces tubular dysfunction in children without clinical evidence of renal disease. Objective: Acute kidney injury (AKI) is an important cause of mortality in sub Saharan Africa because access to renal replacement therapy is limited. There are few reports on haemodialysis in childhood AKI in the sub region. We therefore performed a preliminary review of data on children who received intermittent haemodialysis for AKI in our centre. Method: A retrospective review of case records and haemodialysis registers of children in AKI who received haemodialysis in our centre from January 2006 to December 2012. Results: 62 children, including a child aged 3years, received haemodialysis for AKI over the period but full details were available for 23 children and were further reviewed for this study. There were 13 males (56.5%). The children were aged 5-13 (8.4+/-2.4) years. The primary aetiology of AKI was related to intravascular haemolysis (IVH) with massive haemoglobinuria (n=12), septicaemia (n=4), acute glomerulonephritis (n=2), malignancies (n=2), malaria (n=1), HIV (n=1) and haemolyticuraemic syndrome (n=1). The aetiology of IVH was secondary to glucose-6-phosphate dehydrogenase (G6PD) deficiency in 4 patients, autoimmune haemolyticanaemia in 1 and was unknown in the others. One of the patients with IVH secondary to G6PD deficiency also had malaria. The number of sessions of dialysis ranged from 1 to 5 sessions per patient with a modal value of 2 Objectives, Methods and Results: A twelve-day old, formula-fed, full term neonate with unremarkable perinatal history was admitted for poor oral feeding and significant (12%) weight loss. Physical examination was otherwise unremarkable. Investigation showed normal anion gap metabolic acidosis, hyperkalemia (6.5mmol/L), hyperurecemia (14.5mmol/L) but normal creatinine (28umol/L). Metabolic screen and other investigation was unremarkable. On further questioning parents has been feeding baby with goat milkbased formula since day 5. Baby was switched back to normal cow milk-based formula with prompt resolution of metabolic disturbance and without recurrence of acidosis on follow-up.

Conclusions: Goat milk is becoming increasing popular to parents because of the myths associated (organic, easier digested, hypoallergenic, better nutrient content etc). However there is good evidence suggesting against these misbelief and there are even reported morbidities associated with ingestion of goat milkbased formula. With increasing appealing but not necessarily accurate information available on the Internet, what the public need is education. Objective: There have been great inroads made in controlling the scourge of HIV in South Africa. The national PMTCT program has reduced the MTCT rate to 3.5% and children infected prior to these interventions are now on effective treatment programs. These children are now surviving longer and so we are seeing increased numbers of HIV related renal pathology presenting to our clinics. Before the widespread availability of ARV's in South Africa these children would have been denied access to our program but now we are able to offer them CRRTand a transplant. The biggest challenge facing our program is one of the social disintergration of much of our indigent population. The vast majority of our patients come from social backgrounds that are incompatible with adhering to the rigours of a chronic renal program. This is compounded when the care givers are themselves ill or when the child has a second serious illness such as HIV to deal with. Methods: We would like to present our experience with admitting 8 HIV positive children with CKD5 to our chronic program and will highlight the challenges that face these families and their health care providers. We will describe the social issues affecting each family and how they impact on the child's care.

Results: All 8 families had serious social issues on top of having to deal with their HIV disease. All 8 families had extreme difficulties in adhering to our program. 2/7 died as a result of non adherence and of the remaining 5 children only 1 is really coping well with the program. 3/7 children have no living parents and 1 is foster care. The rest are either with a single parent or with a family relative. Conclusion: The huge difficulties facing these patients is forcing us to rethink the criteria that should be applied to admitting these patients to our program. Restricting the program to children with these problem seems cruel and we feel that all options should be considered before denying these children access to life saving care.

Abstract# Most of the patients presented during autumn and winter. The incidence of APSGN has decreased in the past 11 years in Shenyang, but the proportion of the patients with acute kidney injury (AKI) or nephroticrange proteinuria was increased. The proportion of children with macroscopic hematuria remained almost the same during these years. Macroscopic hematuria resolved in 2-3 weeks and non-nephroticproteinuria resolved in 1-3 weeks. The treatment of APSGN patients with nephrotic-proteinuria was the same with the children with primary nephrotic syndrome and prednisone was withdrawn in 3-4 months. Some AKI patients have been followed up for 5 years and no patients with chronic renal failure. All patients with microscopic hematuria at onset and 30% patients with microscopic hematuria after 2 years. Conclusion: The incidence of APSGN was decreased during recent years. The proportion of APSGN patients with AKI or nephrotic-range proteinuria was increased. The short-term prognosis of APSGN was good and minority of the patients persisted with microscopic hematuria.

Abstract# P-SAT122 Analysis of clinical manifestations and prognosis in 43 children of acute poststreptococcal glomerulonephritis Objective: Crescentic glomerulonephritis (GN) is characterized by severe infiltration of massive inflammatory cells into glomeruli and crescent formation. Although renin angiotensin system (RAS) is the key player in renal injury, the impact of direct renin inhibitor on glomerular crescent formation is not elucidated yet.

Methods: To examine whether direct renin inhibitor ameliorate renal injury in crescentic GN, we investigated renal injury induced by antiglomerular basement membrane (GBM) antibodies in Wistar Kyoto rats treated with direct renin inhibitor, aliskiren. In addition, using cultured glomerular mesangial cell (MCs) and parietal epithelial cell (PECs), we examined whether recombinant renin could induce monocyte chemoattractant protein-1 (MCP-1) expression and cell proliferation, respectively. Results: An anti-GBM nephritis model developed progressive proteinuria and glomerular crescent formation, accompanied by increased expression of MCP-1 and (pro)renin receptor. Interestingly, (pro)renin receptor expressed strongly in the crescent formation area in diseased glomeruli. Proteinuria was significantly reduced by the treatment of aliskiren. Then, aliskiren markedly ameliorated renal injury (% glomerular crescent: 26.0 +/− 1.7 %) compared to vehicle treatment (59.6 +/− 3.6 %). Excretion of urinary protein in a group of rats treated with aliskiren were significantly reduced compared to vehicle-treated rats. Aliskiren treatment markedly decreased MCP-1 and (pro)renin receptor mRNA levels in the diseased kidney. Next, primary cultured MCs stimulated by recombinant renin showed significant increases of MCP-1 mRNA expression. Furthermore, primary cultured PECs showed an increase in recombinant renininduced cell proliferation.

Conclusion: These data suggest that therapeutic strategy of direct renin inhibitor may prove beneficial for crescentic GN by the suppression of the RAS activation and the decrease of inflammation and cell proliferation in glomerular crescent via (pro)renin receptor.

An update on management of acute glomerulonephritis in children Objective: To analyze the clinical effects, safety, and significance of leflunomide combined with hormone in the treatment of children with refractory nephrotic syndrome. Methods: Collected 60 cases patients in our hospital in children with refractory nephroticsyndrome,and randomly divided into 30 cases of the control group and treatment group. Control group was treated with mycophenolatemofetil (MMF)andhormone,the treatment group was treated with leflunomide (LET) and hormone.Measured and compared the changes before and after treatment of 24h urinary protein excretion,TC,ALT,Cr,BUN Clinical efficacy and complications.

Results: The two groups after 6 months of treatment, 24 h urinary protein, plasma albumin, total serum cholesterol, serum creatinine and blood urea nitrogen and other indicators were significantly better than before treatment and has a statistically significant (P <0.05); The overall response rate in the control group and treatment group were 83.33% and 86.67%. Conclusion: The application of leflunomide combined with hormone treatment of children with refractory nephrotic syndrome can make the disease to be effectively alleviated, The clinical results were satisfactory, safe, and fewer complications, It is worthy of promotion. Objective: Post-streptococcal acute glomerulonephritis (PSAGN) is a familiar disease in children. Misdiagnosis might occur when it is presented with atypical symptoms. Here we report a 12-year-old girl presented with acute heart failure which was finally diagnosed comorbidity of PSAGN and Graves' disease. Methods: retrospectively review the history of the patient.

The girl was admitted to our hospital with cough, shortness of breath and chest tightness, without any other obvious symptoms and history of illness. The examination of cardiac color ultrasound showed mitral insufficiency with severe mitral regurgitation and heart enlargement. Other examinations also found she had hypertention, mild anomalies of urinalysis, hypocomplementemia, hypoalbuminemia, hyperthyroidism and elevated antistreptolysin O. First we diagnosed her illness as rheumatic disease. After two weeks treatment with rest, fluid and sodium restriction, controlling blood pression, diuresis and methimazole, the girl recovered very well. She had no any symptom with normal urinalysis and blood pressure, the result of reexamination of Cardiac color ultrasound was normal, her hypocomplementemia and hyperthyroidism were improved significantly. Finally she was diagnosed as co-morbidity of PSAGN and Graves' disease. Conclusion: PSAGN with atypical symptoms at presentation should be paid more attention, especially when it is also has co-morbidity.

Abstract# P-SAT128 Report of a child with idiopathic cryoglobulinemia Idiopathic cryoglobulinemia is rare to see in child. Here we report a child with typical manifestations of cryoglobulinemia. The subjected child was male, 6 years and 4 months, He had repeated rashes, itching, desquamation on his fingertips for more than two years, accompanied with recurrent miliary reddish rashes on the trunk and limbs for more than one year, transient arthralgia for 2 weeks, and edema and oliguria for 10 days before he was admitted to our hospital. He was treated as atopic dermatitis all the time. But the rashes were recurrent. Erythema could be seen after desquamation. 2 weeks before admission, he had arthralgia on the right knee, 2 days later on left knee without swelling, both were retrieved spontaneously. X-ray of the knees revealed no abnormal. Ten days before admission, edema and decreased urine output was notice without gross hematuria. Physical examination in local hospital revealed blood pressure 140/108 mmHg, Needlelike erythematous maculopapules in his hands and feet. Urine test revealed protein 5g/L, RBC 164/ul, no casts. Serum BUN was 32.41mmol/L, Cr 116umol/L, TCO2 11mmol/L, C3 0.47g/L. He was sent to another hospital 2 days later. There, prednisone 25mg per day was given. As a result, the urine output increased gradually, but serum BUN and Cr remained high, so he was referred to our hospital. Renal biopsy indicated membrane proliferation glomerular nephritis with abundant microthrombus. The diagnosis of cryoglobulinemia was suggested. After that serum cryoglobulin and Serum protein electrophoresis were tested 18 days after prednisone was given, both were normal. Etiological examinations, included hepatitis virus A, B, C, D, E, G were all negative; antibodies of HIV, syphilis, Toxo, CMV, RVB, RSV, HSV, EB, ADV, and Rickett's organism were all negative, MP-IgM 1:40. Antinuclear antibodies of SLE, ANCA and antiphospholipid antibodies were all negative. So idiopathic cyoglobulinemia was more favored. The treatment of prednisone 25mg per day was continued after admission. He restored gradually. Prednisone was taped down gradually. Now prednisone has been stopped for more than one month, he seems well during that period of time. Methods: Children with HSP over 3 years of age were enrolled in the study. They were evaluated for demographic, anthropometric, clinical and laboratory data including urinalysis, complete blood count, serum albumin, creatinine, IgA levels. In addition, anti-tissue transglutaminase IgA (ELISA), anti-endomysium IgA (IFAT), antigliadin (GAF3X, deaminated) IgA (IFAT) and anti-gliadin (GAF3X, deaminated) IgG (ELISA) antibody levels were determined. Seropositive patients were evaluated by endoscopic small bowel biopsy. The rate of CD seropositivity in HSP patients was compared to the rate in healthy Turksih children by the test for the statistical significance of two percentages. Results: Celiac serology was evaluated in 42 children (25 male, mean age 11.2 +/-3.6 years) with HSP. There was no patient with growth failure or having symptoms associated with CD like abdominal pain, abdominal distention or diarrhea. In addition, none of the patients had IgA deficiency, anemia or hypoalbuminemia. Celiac serology was positive in 5 (12%) children. Endoscopic evaluation was performed in 3 patients and one of them was diagnosed as CD. Prevalence of CD in children with HSP was significantly higher compared to healthy Turkish children (p<0.001).

Conclusions: Celiac seropositivity was 12% in children with HSP and this rate is significantly higher than the rate in healthy children. Although the number of children with HSP is small in this preliminary study, this result suggests that celiac screening may be considered in children with HSP. (14) groups. The glomerular and tubulointerstitial lesions were scored by Katafuchi criteria and Oxford classification of IgAN,respectively. Results:1.The baseline data of different clincal or pathological groups were no significant differences (P more than 0.05),but 24h protienuria were significant increased in grade 3 and 4 groups than grade 2 group(P less than 0.05).2.According Katafuchi critiria,the severity scores of glomerullar and tubulointerstitial lesions were positively correlated with either different clincal or pathological groups(P less than 0.01).3.According to Oxford classification, only the severity scores of mesangial hypercellularity and segmental glomerulosclerosis/adhension were positively correlated with different clinical groups(P less than 0.01),but the severity of mesangial hypercellularity, endocapilarity hypercellularity, segmental glomerulosclerosis/adhension,tubular atrophy/interstitial fibrosis and cellular/fibrocellular crecents were all positively correlated with different pathological groups (P less than 0.01). [1990] [1991] [1992] [1993] [1994] [1995] [1996] [1997] [1998] [1999] [2000] [2001] [2002] [2003] [2004] [2005] [2006] [2007] [2008] [2009] were evaluated retrospectively. Patients were analyzed comparing those hospitalized during the first and the second ten-year period.The long-term prognosis was evaluated in 60/64 patients (94%) with HSP nephritis followed-up for 1 to 11 years (mean 5.7±3.6 years) based on urine analyses, serum creatinine and blood pressure measurements. Results: Of 156 patients (mean age 6.9±3.5 years, range 1-17 years, M: F ratio 1.0) with palpable purpura (100%), other symptoms were more common during the first ten-year period including arthralgias (62% vs. 52%, p=0.282), gastrointestinal symptoms (63% vs. 44 %, p=0,018) and renal involvement (55% vs. 21%, p=0.001). Among 64 patients (41%) with HSP nephritis, 42 had isolated hematuria and/or non-nephrotic range proteinuria (66%), 15 nephritic syndrome (23%) and 6 nephritic/nephrotic syndrome (10%). Hematuria and/or nonnephritic proteinuria was less common (p=0.0005) in the second tenyear period while the incidence of nephritic/nephrotic syndrome was insignificant (p=0.807). Recurrences of purpura had 11 patients within 2 years. 6 patients had renal biopsy including one with 30% of crescents and one another with IgA nephropathy diagnosed seven years after initial presentation of HSP. Long-term prognosis was favourable in most patients with abnormal urine analyses in 12 (20%), in one with hypertension but normal serum creatinine and in all with nephritic/nephrotic syndrome at disease onset. Conclusion: clinical evaluation of patients with HSP nephritis treated at our institution showed a good long-term prognosis. Urine and blood pressure abnormalities in followed-up patients were associated with nephritis at disease onset. -up (f-up) . eGFR was estimated using Schwartz formula; decline in renal function was defined as the slope of eGFR over the f-up. Results: 72% were males, with mean age at renal biopsy of 12.7 ± 3.7 y and a median f-up of 4.7 y (IQ range 2.4-7.8 y); more than 80% presented with eGFR > 90 ml/min/1.73m 2 . End-stage renal disesase (ESRD) was reached by 4% of children, 50% loss of initial eGFR by 5%; 7% reached the combined end point (ESRD or 50% loss of initial eGFR). At renal biopsy 57% presented with mesangial proliferation (M1), 24% with endocapillary proliferation (E1), 35% with segmental glomerulosclerosis (S1) and 9% with tubular atrophy/interstitial fibrosis (TA/IF; T1/2). Patients with segmental sclerosis and TA/IF showed a significantly worse eGFR slope over the f-up (S0 vs S1 p=0.04; T0 vs T1/2 p=0.003). At univariate linear regression, clinical data at renal biopsy (eGFR, proteinuria and MAP) were not associated with renal function decline, while data at 6-12 and 12-24 months and TA-proteinuria and MAP significantly predicted eGFR slope.A multivariate linear regression model (including proteinuria and MAP at 12-24 months together with the difference of eGFR at renal biopsy and at 12-24 months as independent variables) performed well in predicting eGFR slope (R 2 =0.39). This model was used to derive a formula able to estimate eGFR slope with good performance (mean bias between estimated and really observed eGFR slope of 0.05 ± 6.6 ml/min/1.73m 2 ). Conclusion: The Oxford classification of IgAN was well applicable to this pediatric population. A formula was developed that estimates renal function decline over the f-up based on proteinuria, MAP and eGFR loss after 1-2 years of observation, which will need a validation on other cohorts.

Sixteen-year experience with pediatric IgA nephropathy and validation of the Oxford classification as a risk predictor (M1), segmental sclerosis (S1), endocapillary proliferation (E1), and tubulointerstitial fibrosis (T1 or T2) was 32 (35.2%), 15 (16.5%), 9 (9.9%), and 6 (6.6%), respectively. There was no significant decrease in GFR within the entire cohort. However, five patients showed decreased renal function, and of them, two reached stage III or IV CKD. The five patients' changes in the K/DOQI CKD stage were 2 to 4, 1 to 3, 1 to 2, 1 to 2, and 1 to 2. Their pathological classifications were Haas 4/M1 S1 E0 T0, Haas 4/M1 S0 E1 T1 with global sclerosis, Haas 1/M0 S1 E0 T0, Haas 1/M0 S0 E0 T0, and Haas 1/M0 S0 E0 T0 with global sclerosis. In the Oxford classification, global sclerosis was the only factor correlated with decreased renal function, whereas the Haas classification showed no significant correlation with renal function. Conclusions: Global sclerosis was correlated with decreased renal function. However, in our cohort, there was no significant decrease in renal function during the follow-up period (P = 0.216). Thus, we could not clearly correlate the Haas and Oxford classifications with renal outcome. The value of the Oxford classification as a predictor of renal outcome remains unclear in Korean children with IgA nephropathy.

Meng-jie JIANG, Xiao-yun JIANG, Ying MO, Li-zhi CHEN, Li-ping RONG Pediatric, The First Affiliated Hospital of Sun Yat-sen University, Guangzhou, China

Objective: To analyze the clinicopathological characteristics of primary Immunoglobulin A Nephropathy (IgAN) manifested as macroscopic hematuria in children.

The clinicopathological characteristics of 48 cases with primary IgAN manifested as macroscopic hematuria were analyzed retrospectively. According to the lasting time of macroscopic hematuria, 15 cases were assigned to group A (no more than seven days) and 33 cases were assigned to group B (more than seven days).

Results: The manifestations comprised: acute glomerulonephritis (40% in group A, 33.3% in group B), nephrotic syndrome (20% in group A, 15.1% in group B), hematuria and proteinuria (6.7% in group A, 12.1% in group B) and isolated hematuria (33.3% in group A, 39.4% in group B). 20.0% cases in group A and 21.2% cases in group B were accompanied with abnormal renal function. There was no significant difference between group A and B (P>0.05). The urinary lysozyme or β2-MG increased (6.7% in group A, 30.3% in group B). In group A, subclass II was the most common histopathological type (53.3%), followed by III (33.3%) and I (13.3%) while the predominant histopathological types in group B were subclass III (45.5%), IV (27.3%), II (21.2%) and I (6.1%). None in group A and 15.2% cases in group B showed crescent. Besides, only one glomerulosclerosis was in group A while 8 (24.2%) cases of glomerulosclerosis were in group B. The difference between group A and group B was statistically significant (P<0.05).No differences were found in balloon stenosis between group A (6.7%) and group B (12.1%), as well as the renal tubular and interstitial damage (33.3% in group A, 39.4% in group B). 46.7% cases in group A and 60.1% cases in group B were found with simple IgA deposition in mesangial area. There was no significant difference between group A and group B (P>0.05).

The children with primary IgAN manifested as macroscopic hematuria lasting for more than seven days are easily appearing renal tubular damage, crescent formation and glomerulosclerosis. Subclass III and IV are the most common histopathological types.

A case report of IgA nephropathy accompanying Crohn's disease Akihiko Shirasu 1 , Akira Ashida 2 , Hideki Matsumura 2 , Hyogo Nakakura 2 , Tomoki Aomatsu 2 , Atsushi Yoden 2 , Motoshi Hattori 3 , Hiroshi Tamai 2 , 1 Pediatrics, Hirakata City Hospital, Hirakata, Japan 2 Pediatrics, Osaka Medical College, Takatsuki, Japan 3 Pediatric Nephrology, Tokyo Wemen's Medical University, Tokyo, Japan

There have been several reports of IgA nephropathy accompanying Crohn's disease in which the clinical course of the two diseases was linked. We recently experienced a case of IgA nephropathy with deterioration of renal function complicated by Crohn's disease. A 15- year-old boy diagnosed as having Crohn's disease underwent total colectomy at the age of 7 years. The patient was referred to pediatric gastroenterology unit at the age of 13, and thereafter maintained a state of remission form Crohn's disease while receiving 5-aminosalicylic acid (5-ASA). Two months prior to referral to our pediatric nephrology unit, when the patient was 15 years old, a school health examination had revealed microscopic hematuria and mild proteinuria. At that time, laboratory tests suggested deterioration of renal function. Renal biopsy was performed and histological examination by light microscopy demonstrated focal segmental mesangial proliferation and moderate tubule atrophy with a diffuse interstitial inflammatory infiltrate consisting predominantly of lymphocytes. Immunofluorescence assays revealed 2+ diffuse granular staining for IgA and 1+ staining for C3 in the mesangium. Therefore, the patient was diagnosed as having IgA nephropathy and chronic interstitial nephritis. The 5-ASA therapy was stopped considering the possibility that it had induced interstitial nephritis. The renal function of the patient has since remained quiescent for 7 months. Patients with Crohn's disease who present with abnormal urinalysis findings commonly have urological complications, such as urolithiasis and urinary tract infections. However, the possibility of renal parenchymal disease including IgA nephropathy should also be considered, even if there is no apparent aggregative linkage of urinalysis findings with gastrointestinal symptoms of Crohn's disease.

The duration of heavy proteinuria determined long-term outcome of Henoch-Schönlein purpura nephritis in children Results: Among the 9 patients, 7 (77.78%) cases presented with hematuria and nephrotic syndrome (NS); 1 (11.11%) with hematuria and nephrotic range proteinuria (>50mg/kg/24h); 1 (11.11%) with hematuria and moderate proteinuria (25~50mg/kg/24h). The typical pathological features, such as the diffuse glomerular mesangial and endocapillary proliferation, mesangial interposition, and double contour formation, were shown in all specimens. Moreover, the podocyte hypertrophy, shedding, and cytoplasmic absorption dropletswere also observed in most specimens under light microscopy. The percentage of small cellular crescents varied from 4.25-25%. 9 patients were followed up for 2 to 4 years, and all had recovered. Conclusion: The predominant clinical manifestation of ISKDC grade VI HSPN in children was NS accompanying with hematuria. The well outcome might be associated with the prompt use of steroids and/or immunosuppressive drugs at the onset of ISKDC grade VI HSPN with very mild glomerulosclerosis and tubulointerstitial lesion. In our future study, all patients should be followed for five or ten years, and more specimens should be observed under electron microscope to investigate whether the podocyte lesions of ISKDC grade VIHSPN should be taken into account in the clinical pathological report and a future new histologic classification of HSPN. Objective: The aim of the study was to assess efficacy of immunosuppressive treatment in children with IgA nephropathy (IgAN) and Henoch-Schoenlein nephritis (HSN) based on clinical manifestation and histopathological Oxford (O-C) and WHO (WHO-C) classifications.

The study group consisted of 18 children with IgAN-9 and HSN -9, (mean age 10.44 ±3.94 years) , treated with azathioprine (AZA) 2 mg/kg/day -12 months with Prednison (Pred) 2mg/kg/day (nephrotic syndrome-NS), 1mg/kg/day (nephritic syndrome -NPS) 8 weeks, gradually diminished within 15 months. It recognize clinical remission at normal renal function and absence of proteinuria at the end of treatment. In all patients we estimated: proteinuria, GFRs (calculated using Schwartz's formula) at the onset of study and after 15 months. They had performed renal biopsy on average 0.69 ± 0.95 years after the onset of illness and at the end of treatment. We were analyzed the histopathologic evaluation of renal biopsy specimens using the WHO-C (grade I-V) and Oxford classification : (Mmesangial hypercellularity, E-endocapillary hypercellularity, Ssegmental sclerosis, T-tubular atrophy/interstitial fibrosis; absent =0, present=1).

Results: In patients with IgAN we observed: NS-1, NPS-8; with HSN : NS-7, NPS-2. After the treatment AZA/Pred we noted clinical remission in all children with IgAN and 89% with HSN. GFR was normal before and after treatment. At the onset of illness IgAN patients had: grade III-6, grade IV-3 according to WHO-C; HSN had: grade II -2, grade III-6, grade IV -1; at the end of therapy we observed positive effect in WHO-C only in 2 (22%) IgAN patients and in 5 (56%) HSN , without progression properly 6 (67%) and 4 (44%). In O-C of IgAN patients we observed nobody with total regression of changes, only 3 children had M0 or E0 or S0 after treatment. In patients with HSN we noted total regression of changes in 4 (44%), in other 3-M0 or E0 or T0.

Conclusion: AZA/Pred therapy used in children with IgAN and HSN can make clinical remission in both groups, but histopathological regression-more frequent in HSN. Oxford and WHO classifications are very useful for estimation of efficiency of treatment.

10-year follow-up of pediatric Hennoch Schönlein purpura with renal involvement

Elena Tudorache 1 , Christine Azema 1 , Stéphane Decramer 2 , Georges Deschênes 3 , Tim Ulinski 1 1 Pediatric Nephrology, Armand Trousseau Hospital, Paris, France 2 Pediatric Nephrology, CHU Toulouse, Toulouse, France 3 Pediatric Nephrology, Robert Debré Hospital, Paris, France

Objective: The aim of this study was to determine the long-term outcome in children with HSP nephritis who underwent a renal biopsy and to identify possible correlation between disease parameters and treatment.

We included retrospectively all patients with renal biopsy proven IgA nephropathy related to HSP of three pediatric nephrology centres over a 10-year period. Results: 142 patients were included. Nephrotic range proteinuria was present in 28% with grade II, 60% with grade III and 90% with grade IV lesions. Renal function was impaired in 14%. Significant proteinuria >500 mg/L was found in 9/48 patients 3 years post renal biopsy, in 8/25 patients at 5 years and in 3/14 patients at 10 years. There was no correlation between risk for proteinuria at 3, 5, or 10 years with initial histological lesions. There was a tendency to higher residual proteinuria in patients with nephrotic proteinuria at disease onset and also for patients who were not treated with steroid pulses at disease onset with 310 vs. 175 mg/L (p=0.06) at three months, 100 vs. 50 mg/L (p=0.93) at one year, 720 vs. 150 mg/L (p=0.1) at five years, and 520 vs. 355 mg/L (p=0.35) at ten years. There was a tendency to less proteinuria in the long term for those with early steroid pulses (<15 days vs. >16 days after proteinuria onset (p=0.16).In patients with ACEi/ARB treatment within 15 days after renal disease onset, compared to those who were treated later (>one month), proteinuria was significantly lower at 6 (p=0.02) and 12 months (p=0.03). Among 18 control kidney biopsies, 50% show fibrosis of different degree unrelated to initial histology. Conclusion: There is a risk to underestimate long term disease severity in patients with low ISKDC classes. There is need for prospective long term studies to explore the benefit of early ACEi/ARB and/or steroid pulses.

Abstract# P-SAT147 Good outcome of biopsy-proven Henoch-Schonlein purpura nephritis in children in Shanghai single center Objective: Severe HSP nephritis considered a risk factor for chronic kidneys disease development especially in patients with nephrotic syndrome (NS). Methods: We present our experience with tacrolimus (Tac) as a steroid sparing agent for a treatment of severe HSP nephritis in 17 years boy. Our patient has been observing at the Republic Center of Pediatric Nephrology Minsk for 5 years. He presented with a typical purpuric rash with recurrences after infections. One year later during acute pneumonia macrohematuria, proteinuria (1-1.5 g/24h) occured and ACE inhibitors (iACE) were prescribed. Despite this, boy developed nephrotic range proteinuria (3.8-7.7 g/24h), biochemical protein 52g/l, albumin 27g/l, cholesterin 6,5mmol/l. Renal biopsie showed mesangial proliferation with IgA deposits. Prednisolon (Pred) 80mg/daily with heparin failed to decrease proteinuria and activity. Cyclosporin A 200 mg/daily plus Pred on alternate day were started and followed during 8 months with positive dynamic (proteinuria 1,7g/24h, decreased clinical and laboratory activity) after which pathogenic treatment was stopped. Arterial hypertension (AG) was treated by iACE. 6 months later proteinuria (3.5g/24h) and activity increased again.

With the presence of NS resistance to steroids combined with hematuria and AG, medrol (64mg/daily) plus Tac 1 mg twice daily were prescribed. Duration of Tac therapy was 5 months. Tac was succesfully withdrawn with no rebound disease activity observed (during 9 months). At present the patient receiving iACE only.

Our results indicate that Tac may be a promissing steroid sparing agent for treatment of severe HSP nephritis.

Anti-proteinuric effect of cyclosporine A treatment for IgA deposit diseases Objective: To evaluate the therapeutic effect of cyclosporine A (CsA) on nephrotic-range proteinuria in children with Immunoglobulin A (IgA) deposit diseases in mesangium such as IgA nephropathy (IgAN) and Henoch-Schonlein purpura nephritis (HSPN). Methods: Fifty four children (36 children with IgAN, 18 children with HSPN) who were diagnosed with IgA deposit diseases in mesangium at renal biopsy were analyzed retrospectively. All the patients developed nephrotic-range proteinuria (> 40 mg/m2/day) for more than 3 months. The starting dose of CsA was 5 mg/kg per day that was given in two divided doses, and the drug level was maintained between 100 and 200 ng/mL. The degree of proteinuria was measured before and after CsA treatment. Steroids were tapered off and stopped gradually after initiation of CsA.

Results: Mean duration of CsA treatment was 10.7 +/-5.6 months (range 1.4 -32.7 months). Mean follow-up duration was 3.7 +/-3.1 yr (range 0.7 -15.2 yr) from the beginning of CsA treatment. The mean ratios of protein to creatinine decreased from 3.7 +/-1.5 to 0.6 +/-0.4 after CsA treatment for 12 months. Thirty-two (59.2%) patients achieved complete remission. Renal function was preserved in all patients 12 months after CsA treatment. There were no severe complications in patients with CsA treatment. Conclusion: Our findings indicate that CsA is an effective agent to treat nephrotic-range proteinuria of IgA deposition glomerular diseases such as IgAN and HSPN and the duration of CsA treatment should be at least more than 3 months for efficacy. Objective: To observe the therapeutic effects of hemoperfusion combined with corticosteroid in treating children with Henoch-Schonlein purpura.

Methods: A total of 180 patients (101 inthe hemoperfusion group (HP) and79 inthe control group) were included and followed up for 12 months. Both groups were treated with corticosteroids and other supportive therapy. Patients in the HP group received 2 hours of hemoperfusion each day for 3 consecutive days. Clinical features at the acute phase, relapses in a year and renal involvement at 1, 3, 6, 12 months were compared. The blood levels of IgA and such cytokines as, TNF-alpha-IL-1, IL-6, LTB4 were measured by ELISA technique before and 1, 2, 3 days after hemoperfusion.

Results: Hemoperfusion significantly reduced the severity and duration of abdominal and joint pains. For patients with renal involvement at acute phase the HP group had quicker relief than the control (log rank P=0.000). For patients without renal involvement at acute phase the HP group had less occurrences of renal involvement compared with the control during follow-ups(log rank P=0.001). The rash was also alleviated after hemoperfusion though the difference was not significant. Patients' levels of IgA were elevated compared with the healthy control before treatment ( day0) Patients'levels of cytokines also significantly increased compared with the healthy control on day0, they began to decrease 1 day after hemoperfusion and there were significant differences among those groups. Less relapses were seen in the HP group compared with the control (P=0.011).

Conclusion: Hemoperfusion combined with corticosteroid was more effective than corticosteroid alone in improving extrarenal symptoms, alleviating and preventing renal involvement. The effects may be achieved by IgA and cytokine reduction after hemoperfusion.

The treatment of Henoch-Schonlein purpura nephritis in children combined with mycophenolate mofetil and corticosteroid 

nephritic syndrome type, 13 cases(39.4%). About histological degrees, 32 cases were in grade 3 (mesangial proliferation, all accompanied by <50% crescent formation), 1 case was in grade 5 (moderate to severe mesangial proliferation, accompanied by 88% crescent formation). Treated with new therapy, 30 cases were complete remission (91%), 2 cases were improved, and one died. The complete remission rate of this therapy was significantly higher than that of the past treatment. Objective: Steroid pulse therapy (SPT) has been reported to be effective for improving urinary abnormalities and preventing renal deterioration in patients with IgA nephropathy. However, some patients are refractory to SPT or have relapse after SPT, resulting in renal impairment. The efficacy of tonsillectomy combined with SPT has been discussed. The aim of our study is to evaluate the efficacy of tonsillectomy combined with SPT in childhood IgA nephropathy.

Methods: 30 children, aged 3 to 14 years, who had been followed up for more than 3 years after the first biopsy between 2001 and 2009, were enrolled in this study. All patients received SPT (methylprednisolone, 20~30 mg/kg intravenously 3 times per week for consecutive 3 weeks). 8 patients received tonsillectomy combined with SPT (group A), and 22 patients received SPT alone (group B). Tonsillectomy was performed one month before or after SPT. The therapy was followed by oral prednisolone and tapered off for 12 to 24 months, with warfarin, dipyridamole and RAS inhibitor. All of 30 patients underwent repeat biopsy. Clinical features and pathological findings were retrospectively analyzed.

Results: The disappearance rate of proteinuria was 87.5% for group A vs 100% for group B at 1 year, and that of hematuria was 100% vs 72.7% at 2 years. For group A, all the cases which of urinary abnormalities had improved by the treatment could maintain remission, but for group B, 27.3% of the cases had recurrence of proteinuria and 18.2% had recurrence or did not achieve disappearance of hematuria at the last observation. Histlogically, the percentage of glomeruli that showed crescents was significantly reduced in both groups. But the cases which still showed crescent formation at the second biopsy were 0% for group A vs 22.7% for group B.

Conclusion: The differences of recurrence rate for urinary abnormalities after the treatment and disappearance rate of crescents at second biopsy may suggest that tonsillectomy can improve the long-term prognosis.

Objective: To observe the efficacy and security of tripterygium wilfordii polyglycosidium (TWP) in treating children with severe Henoch-schonlein Purpura nephritis (HSPN). Methods: 25 children failed to intravenous pulse methylprednisolone for 6 days for their severe HSPN entered our study and were given oral TWP for 3-6 month. We evaluated the efficacy and security of TWP through examinations of urinalysis, blood routine, function of liver and renal, myocardial enzyme series, level of sex hormone and electrocardiogram (ECG) before treatment, 1-2 month after giving TWF and 4 weeks after stopping medication respectively. Results: Proteinuria disappeared in all patients. The remission time was 4 weeks in 22 children, 12 weeks in 2, 15 weeks in 1 respectively. During treatment, elevated glutamic-pyruvic transaminase and Glutamic-Oxaloacetic occurred in 2 patients and returned to normal after stopping medication. Abnormal findings in examination of ECG were observed in 2 patients, one with left ventricular high voltage, another with knot parallel rhythm of the heart. After stopping medication, one patient still had T wave change in ECG. We didn't find any abnormalities of blood routine, renal function and level of sex hormone. Conclusion: The TWP has good efficacy and security on the treating children with severe HSPN.

The efficacy and side effects of glucocorticoids in treating children with Henoch-Schonlein purpura

Kai-yun Liu, Ling Lu Pediatrics, irst Affiliated Hospital of Anhui Medical University, Anhui, China

Objective: To observe the efficacy and safety of glucocorticoid in the treatment of children with Henoch-Schonlein purpura.

Methods: 50 patients with HSP were divided into mild and severe group according to clinical manifestations. 30 children in mild group were given hydrocortisone sodium succinate (HCSS) therapy. 20 children in severe group were given methylprednisolone ( MP ) therapy. A follow-up record system was set up for every patient. We observed the recovery time of clinical symptoms and side effects of glucocorticoid.

Results: Symptoms of skin rashes, joint and gastrointestinal in all children were disappeared within 3 months of treatment. The urinalysis in 13 of 14 patients with renal involvement returned to normal in 4 weeks. The other patient failed to MP therapy was given oral Tripterygium and got normal urinalysis in 8 weeks after disease onset. The Body Mass Index (BMI) values in all children after glucocorticoid treatment were significantly higher than before treatment. After stopping medication, there was no significant difference in BMI value compared with before treatment and between two groups. During the treatment, 1 patient in each group had ECG abnormal, and their ECG returned to normal after stopping drug. We didn't find any abnormalities of blood routine, renal function and level of sex hormone. Objective: To investigate the role of Th17/Treg imbalance in the pathogenesis of childhood Henoch-Schonlein purpura(HSP). And further explore the immunomodulatory affects of compound/lycyrrhizin(GL) on the Th17/Treg deviation in HSP. Methods: 31 HSP patients were chose as GL-treated group, another 5 patients were used as conventional therapy group. Besides (0.5-2)ml/kg/L was enjected in L-treated group, other treatments were same, any steroid or immunosuppressants treatment was prohibited. 15 age and sex-matched healthy children were used as healthy controls. Blood samples of patients were obtained at the acute stage and after 5 days treatment. Using intracellular staining, the frequency of peripheral CD3+CD8-IL17+(Th17) and CD4+CD25+FOXP3+ Treg cells was detected by flow cytometry All patients received methyl-prednisone pulses iv (1 g/1.73 m2 for 3 consecutives days) and subsequently oral Prednisone with gradual withdrawal in six months, and MMF at dose of 10-20 mg/kg/12 h for 24 months. Results: After six months of therapy all patients but one had persistent microscopic hematuria (ME), only 3/11 patients had recurrent MA. Patients with grade II HSPN showed significant reduction or normal proteinuria. Only 3/11 patients with grade III HSPN had proteinuria still >1gr/24 h, 4/11 patients had proteinuria <1g/24 h, in 4/11 proteinuria was normal. Mean period of follow-up was 3 years. After one year, only 1 patient had recurrent MA, 10 patients showed persistent ME. Two patients with persistent proteinuria >1 g/24 h showed a significant reduction (< 1g/24 h in 2 pts, normal in 1 pt). Proteinuria did not reappear in all patients.The effect persisted after 2 years and after therapy withdrawal. No side effects was recorded. Conclusion: our experience shows the efficacy and safety of MMF in HSPN and has to be confirmed by larger controlled studies.

The Analysis of Blood Perfusion Therapeutic Effect on 45 Cases of Allergic Purpura CHENG Guo-qiang Xi 'an children's hospital urology department, Xi'an, Shanxi, China

Objective: To observe the auxiliary therapeutic effect on severe allergic purpura.

Methods: 45 children with severe allergic purpura were randomly divided into the control group (19 cases) and the treatment group (26 cases). The children of control group were treated with conventional therapy (anti-inflammatory, anti-allergic, anticoagulation and symptomatic treatment). The children of treatment group were treated with conventional therapy and blood perfusion. Two groups of patients were both treated for 14 days, and were followed up for 9 months. The amelioration time and the regression time of clinical symptoms such as abdominal pain, bloody stool, rash, joint symptoms, visible hematuria and proteinuria, was compared in two groups before and after the treatment.

The amelioration time of the clinical symptoms of children in the treatment group, such as acute abdominal pain, bloody stool, rash, joint symptoms, abdominal pain and bloody stool, visible hematuria, proteinuria, and other clinical symptoms, was shorter than the time in the control group (P < 0.05), and the late symptoms such as joint symptoms, abdominal pain and bloody stool iterations was also less than those of the control group (P < 0.05). Methods: IgA nephropathy model was established in male Sprague-Dawley rats. At week 10, rats in the model group were randomly assigned to either remain in the model group (n=5), or to receive treatment with rednisone (n=6), 1,25(OH) 2 D 3 group (n=6), or prednisone plus 1,25(OH)2D3 (n=6). At week 12, serum interleukin-17 level was detected by ELISA, and the level of Treg cells in the blood was assayed using a flow cytometry method.

Results: (1) The proteinurine and the number of blood cells in urine from rats in the model group was significantly higher than in rats in the control, prednisone, 1,25(OH)2D3, and prednisone plus 1,25(OH)2D3 treatment groups (P < 0.01).

(2) Serum interleukin-17 in the model group was significantly increased compared with control and treatment groups (P < 0.01), and the levels were decreased in turn in the prednisone treatment group, 1,25(OH)2D3 treatment group, and prednisone plus 1,25(OH)2D3 treatment group. In addition, the 1,25(OH)2D3 and prednisone plus 1,25(OH)2D3 treatment groups showed lower levels than the prednisone treatment group (P < 0.05).

(3) The level of Treg cells in the model group was significantly decreased compared with the control and treatment groups (P < 0.01), with the levels showing a slight increase in the prednisone treatment group, a larger increase in the 1,25(OH)2D3 treatment group, and the greatest increase in the prednisone plus 1,25(OH)2D3 treatment group when compared with the model group. Furthermore, the 1,25(OH)2D3 group and prednisone plus 1,25(OH)2D3 treatment groups showed higher levels than the prednisone treatment group (P < 0.01). Conclusion: A Th17/Treg disorder exists in rats with IgA nephropathy, with the levels of interleukin-17 increased and the levels of Treg cells decreased. Vitamin D3 can regulate the Th17/Treg balance and reduce the level of protein and blood in the urine in rats with IgA nephropathy. Objective: To explore the therapeutic effect of prednisone (Pred) combined with Mycophenolate Mofetil (MMF) and Cyclosiporin A(CsA) in children with severe purpura nephritis (HSPN). Methods: we collected 6 patients with severe HSPN (from ISKDC IIIa to I) whose proteinuria symptom of Nephrotic Syndrome did not significantly relieve after 4 weeks which treated with oral Pred and MMF (20-30 mg/(kg.d)); and (or) the gross hematuria did not disappear after 2 courses which treated with large doses of methylprednisolone. All patients were treated with oral CsA 2-4mg/(kg.d) to induce remission therapy for 3-6 months, then gradually reduced CsA to 1-3 mg/(kg.d). The follow-up was 8-30 months.

Results: Combined therapy with immunosuppressant treated a month later, gross hematuria were disappeared in all 5 patients, 1 patient (17%) was partial remission (urinary protein and urine erythrocyte continued to decrease 25%-49% than before),5 patients (83%) were significantly remission (urinary protein and urine erythrocyte continued to decrease over 50% than before), 24-hour urinary protein was 30.50±19.35mg/kg, urine erythrocyte was 15.23±11.39×10 4 /ml.3 months later, 5 patients (83%) were significantly remission, 1 patient was complete remission (urinary protein was completely negative, urinary sediment red blood cells <8000/ml), 24-hour urinary protein was 18.67±10.10mg/kg, urine erythrocyte was 10.71±8.55×10 4 /ml. 6 months later, 5 patients (83%) were significantly remission, 1 patient was complete remission, 24-hour urinary protein was 7.66±6.31mg/kg, urine erythrocyte was 5.18±3.81×10 4 /ml. 4 patients were complete remission at last and the median duration of complete remission was 8.5±4.93 months. 2 patients had not been reached complete remission, which follow-up was 8-9 months. 3 patients were relapse during withdraw or reduction, other side effects included hairy (4 patients Objective: Increasing evidence suggest that the Cosmc(1-beta-3galactosyltransferase-specific molecular chaperone ) might play a vital role in the pathogenesis of IgA nephropathy (IgAN). However, the mechanism is not clear. Methods: We investigated whether Cosmc gene( C1GALT1C1 )methylation is an important mechanism in IgAN in children. Methods 33(M:F=25:8) primaryIgAN children and 39(M:F=28:11) healthy controls were enrolled randomly. The methylation status of the C1GALT1C1 was detected using bisulfite-specific polymerase chain reaction (BSP)-based sequencing analysis. Results: The male patients had obviousely high methylation percentage than the male controls(p=0.047). Three significant hypermethylation sites were identified in the male patients: CpG site 20 (p = 0.034), site 21 (p = 0.039), andsite 26 (p = 0.047). In the female groups,the patients and the normal controls all had high level methylation without significance (p=0.947). Conclusion: Methylation of C1GALT1C1 might have the vital significance in the susceptibility to the male IgAN patients.

Objective: The aim of this research is to investigate the value of serum and urinary TGF-β1and MMP-9 level, the noninvasive and easy repeatable biomarker, for assessing the severity and disease progression in IgAN children.

Methods: 54 children with biopsy-proven IgAN and 55 normal children(control group) were enrolled between July 2009 and January 2013.(Fingure 1)The IgAN group was divided into 3 clinical groups according to their clinical features: isolated hematuria group (IH group, 20 patients), hematuria and proteinuria group (HP group, 16), and nephritic syndrome group (NS group, 18). Patients were divided into two groups according to their Lee's pathologic classification: grade I+II (Lee's I+II, 39 patients), grade III+ IV (Lee's III+ IV ,15) groups. IgAN group was classified according to the renal function into two groups(Renal failure Group vs Normal group). IgAN group was also divided into four groups according to their immunophenotype: IgA(7patients),IgA+IgG(9),IgA+IgM(10),IgA+IgG+IgM(28) group.

Results: The level of TGF-β1 and MMP-9 inserum and urinary of the IgAN group was significantly higher than that in the control group (P<0.05). Both the level of TGF-β1 and MMP-9 inserum and urinary of the three clinical groups in the IgAN children were not statistically different. The serum TGF-β1 and MMP-9 levels were significantly higher in Lee' s III+ IV group compared to that in Lee's I+II group(P<0.05). The difference was not statistically significant between urinary TGF-β1 and MMP-9 levels in the two Lee's group.The serum TGF-β1 and MMP-9 levels of renal failure group were significantly higher than that of normal renal function group in IgAN children(P<0.05),and the urinary TGF-β1 and MMP-9 levels of the two group were not statistically significant different. The serum and urinary TGF-β1 and MMP-9 levels were not statistically different in all immune classification.

Conclusion: The serum TGF-β1 and MMP-9 plays an important role in the severity and progression of children with IgAN. TGF-β1 and MMP-9 may be two more noninvasive and easy repeatable biomarkers to be used in evaluating the progression of IgAN in children.

Abstract# P-SAT163 Glomerular ACE2 expression is enhanced in pediatric IgA nephropathy Yusuke Seki, Maki Urushihara, Takahiro Tayama, Takashi Nagai, Ariunbold Jamba, Shuji Kondo, Shoji Kagami Department of Pediatrics, The University of Tokushima Graduate School, Tokushima, Japan

Objective: Angiotensin converting enzyme (ACE)2 is a homolog of ACE and is thought to be a potent counter-regulator against ACE activity. While renin-angiotensin system (RAS) plays a critical role in the progression of IgA nephropathy (IgAN), the role of ACE2 has not been investigated in pediatric patients with IgAN. This study was performed to examine the relationship between ACE2 expression and the development of pediatric IgAN. Methods: We performed immunohistochemical analysis of ACE2 and ACE in kidney tissues from 39 patients with pediatric IgAN and 14 patients with minor glomerular abnormalities (MGA) using specific antibodies and examined the correlation with clinical parameters and pathological findings. In addition, we elucidated the effects of various cytokines on ACE2 expression in cultured human mesangial cells (MCs) by quantitative real time PCR and western blot analyses.

Results: ACE2 expression levels in glomeruli (r = 0.5732, P < 0.0001) and tubules (r = 0.4917, P = 0.0002) were positively correlated with the mesangial hypercellularity score, while ACE expression levels in glomeruli (r = 0.1331, P = 0.3420) and tubules (r = 0.0743, P < 0.5959) are not. Multiple regression analysis showed that the mesangial hypercellularity score correlated with ACE2 expression level in glomeruli and urinary protein-creatinine ratio (r = 0.6295, P < 0.0001). In IgAN patients not treated with a RAS blocker (ACE inhibitors or angiotensin II type 1 receptor blockers), ACE2 expression levels in glomeruli were significantly increased compared to patients with MGA (P < 0.0001). IgA patients treated with a RAS blocker did not show this increase in ACE2 exrepssion. Furthermore, ACE2 mRNA and protein expression was enhanced by interleukin-1 beta, a pro-inlammatory cytokine, and suppressed by transforming growth factor-beta 1 , a pro-fibotic factor in the progression of IgAN. Conclusion: These data indicate that ACE2 expression in glomeruli is associated with mesangial hypercellularity in the active phase of pediatric IgAN.

Abstract# P-SAT164 Increased serum interleukin-17 and peripheral Th17 cells in children with Henoch-Schonlein purpura nephrology (HSPN) xiaoshan shao Nephrology, guiyang children's hospital, Guizhou, China

Objective: Interleukin (IL)-17 and Th17 cells have been involved in many autoimmune diseases. The aim of this study is to investigate the involvement ofIL-17 and Th17 cells in the pathogenesis of childhood Henoch-Schonlein purpura (HSPN).

Methods: Serum and supernatant levels ofcytokines and chemokines were analyzed by enzyme-linked immunosorbent assay (ELISA). Using intracellular staining, the frequency ofperipheral Th17 and Th1 cells was studied by flow cytometry.

Results: Children with HSPN had significantly higher serum levels of IL-17, IL-6and transforming growth factor-beta than healthy controls. The IL-17 levels in culture supernatants of peripheral blood mononuclear cells with anti-CD3 and CD28 antibody stimulation were much higher in patients with HSPN (212.2 +/− 71.4 vs. 34.7 +/− 12.6 pg/ml, p = 0.021). Thepatients also had more Th17 cells (1.47 +/− 0.23% vs. 0.61 +/− 0.10%, p = 0.012) but not Th1 cells in peripheral blood. Moreover, IL-17 could promote human endothelial cells to produce chemoattractants IL-8 and monocyte chemotactic protein-1.

The increased frequency of peripheral Th17 cells and serum IL-17 levels are shown in childhood HSPN that may in part contribute to vascular inflammation, suggesting cellular immunity is likely to be involved in the process of HSPN.

Expression and significance of TGF-beta, Smad7, FN, Ub and Smurf2 in the renal tissues of children with IgA nephropathy CHEN Li-zhi, JIANG Xiao-yun, LING Yi-hong, MO Ying, SUN Liang-zhong Pediatrics, the First Affiliated Hospital of Sun Yat-sen University, Guangzhou, China

Objective: In this study, we investigated the expression of TGF-beta 1, Smad7, SnoN, FN, Ub and Smurf2 inthe renal tissues in children with IgAN, in order to explore the potential cross-talk between ubiquitinproteasome pathway and TGF-beta 1 signaling and even their role in the progressive renal fibrogenesis of IgAN. Methods: Sixty children with IgAN were divided into three groups according to their clinical features: isolated haematuria group (IHgroup, 20 patients), hematuria and proteinuria group (HP group, 21), and nephrotic syndrome group (NS group, 19) . Patients were also divided into three groups according to pathologic grades: grade 2 (26 patients), grade 3 (20) and grade 4 (14) groups. Six normal renal specimens (four was non-tumor kidney tissues from patients who had renal tumor and underwent nehprectomy, another two was healthmismatched donor renal tissues before renal transplantation) were used as the control group. The expression of Ub, Smurf2, TGF-1, Smad7, SnoN, and FN in renal tissues were determined by immunohistochemistry (two-step PowerVisionTM) and semi-quantitatively analyzed by the software of Image-Pro Plus 6.0. The degrees of glomerular and tubulointerstitial lesions were scored according to the Katafuchi semi-quantitative criteria.

Results: The expression of TGF-beta 1, FN, Ub and Smurf2 inIgAN kidneys was significantly higher than that in the control group(Pless than 0.01), while the increased expression of Smad7 was only found in glomeruli but not in renal tubular interstitium(P less than 0.01). The highest expression of TGF-beta 1, FN, and Ub were found in the NS and grade 4 groups(P less than 0.01), Smurf2 in the IH and grade 2 groups(P less than 0.01), while the lowest expression of Smad7 was found in the grade 4 group(P less than 0.01), closely associated with different clinical and pathological groups. (P less than 0.01) Conclusion: These results demonstrate that UPP and TGF-beta 1/Smads signaling pathway were both activated in children with IgAN.Both UPP and TGF-beta 1/Smads signaling pathway may play an important role in the progress of glomerular sclerosis, renal tubular injury and renal interstitial fibrosis in children with IgAN.

Abstract# P-SAT166 Objective: The Proteasome (PS) plays a key role in the activation of transcriptional factors, cytokines and presentation of HLA-I restricted peptides. In immune mature cells, particularly dendritic cells, the PS, under the action of interferon gamma and alpha, becomes an immunoproteasome (iPS), by substituting 3 catalytic units beta1, beta2, beta5 with other low molecular weight proteins (LMP2 and LMP7) and an endopeptidase-like complex (MECL-1). This modification confers an optimal catalytic property for professional presentation of specific peptides to MHC Class I. Our group previously demonstrated that adult patients with IgA-Nephropathy (IgAN) have an increased expression of iPS catalytic subunit, which correlated with the severity of renal disease.Aim of this study was to investigate the switch from PS to iPS in children with primary IgAN and with Henoch-Schoenlein purpura (HSP), a vasculitis sharing with IgAN several immune system abnormalities. Methods: Peripheral Lymphomonocytes (PBMC) from 18 children with IgAN, 57 with HSP and 33 healthy control subjects (HC), isolated by centrifugation gradient, were tested with real time PRC (Taqman) to assess quantitatively the mRNA levels of PS alpha subunit (constitutive), of the active subunit of PS (beta1, beta2, beta5) and of iPS (LMP2, LMP7 and MECL-1). Results: Objective: Recent studies suggest that dysregulated innate immunity plays an important role in the pathogenesis of IgAnephropathy (IgAN). Interleukin-20 subfamily and its receptor, interleukin-22 receptor alpha-1 (IL-22R1), were recently identified as immunomodulators in human diseases, acting as mediators of mucosal host defense. However, the potential role of IL-22R1 in the pathogenesis of IgAN has not been explored.

Methods: In the current study, one hundred ninety four patients with IgAN and 287 normal controls were genotyped for coding polymorphisms of the IL-22R1 gene and the association between the polymorphisms and IgAN was investigated. Local expression of IL-22R1 was examined in patients with IgAN and healthy controls using immunohistochemistry.

Results: Our case-control analysis showed that genotypes of rs3795299 were associated with childhood IgAN. Individuals with the CC genotype of rs3795299 had about three fold reduced risk of IgAN compared with those with the GG genotype in the codominant model (p=0.0028) and those with the genotypes containing G allele (GG or GC) in the recessive model (p=0.002).After Bonferroni correction, the association between rs3795299 CC genotype and reduced risk of developing IgAN remained significant. Furthermore, the renal expression of IL-22R1 was significantly higher in healthy controls compared with subjects with IgAN. Conclusion: Our data suggest that the CC genotype of rs3795299 polymorphism in IL-22R1 gene is associated with the reduced risk of IgAN, and this genetic association was supported by the higher renal expression of IL-22R1 in healthy controls compared with patients with IgAN.

Abstract# P-SAT168 Toll-like receptor 9 gene polymorphisms contribute to development of proteinuria in childhood IgA nephropathy Henoch-Schoenlein purpura (HSP) is the most common form of immune-mediated systemic vasculitis in children, which mainly affects skin, joints, gastrointestinal tract and kidney. The overall prognosis of HSP is favorable, but the long-term outcome is dependent on the degree of renal involvement. The incidence of renal involvement varies from 20 to 60% and there have been some reports showing that nephritis might be related to an older age at onset, persistent purpura (> 1 month), severe abdominal pain, and relapsing disease.Recently, several studies have shown that galactose-deficient IgA1 (Gd-IgA1) is recognized by anti-glycan antibodies, resulting in the formation of the circulating immune complexes and their mesangial deposition causing renal injury in HSP nephritis and serum galactose-deficient IgA1 levels were highly inherited in children with HSP nephritis.Regarding the treatment of HSP, one randomized double-blinded controlled study recently showed that patients with abdiminal pain or arthralgia may benefit from early treatment with prednisone, but the drug has not been proven to be capable of preventing the development of renal symptoms. However, it was effective in altering the course of renal involvementMild Henoch-Schoenlein purpura nephritis (HSPN) generally does not require aggressive treatment due to a favorable course of the disease, but severe nephritis has a high risk of progression to end stage renal failure. Although several intensive therapies, such as intravenous high-dose methylprednisolone pulse, immunosuppressive/cytotoxic drugs, fibrinolytic therapy, anticoagulants, antiplatelet agent and plasma exchange, have been used in children with severe HSPN, treatment of HSPN still remains controversial due to the rarity of randomized controlled studies in this field.

Expressions and significance of mTOR, p70S6K1 and E-cadherin in glomeruli of children with IgA nephropathy Objective: To investigated the expressions of mTOR, p70S6K1, and E-cadherin in glomeruli of children with IgAN to explore the role of mTOR/p70S6K1 signaling pathway in the progressive renal glomeruli fibrosis of IgAN.

Methods: Seventy-two children with IgAN were divided into three groups according to their clinical features: isolated hematuria group (IH group, 24 patients), hematuria and proteinuria(HP group, 26 patients), and nephritic syndrome group (NS group, 22 patients).

Patients were also also divided into three groups according to their pathologic grades: grade II(33 patients), grade III(25 patients) and grade IV(14 patients). Six normal renal specimens were used as control group. The expressions of mTOR, p70S6K1, and E-cadherin in glomeruli were determined by immunohistochemistry method and the relationship between these indexes and the clinical pathology indexes were analyzed. Results: The highest expressions of mTOR and p70S6K1 inglomeruli were found in the NS group, both of which in tubulointerstitium were higher in grade III and IV group than that in grade II group and control group while the lowest expressions of E-cadherin was found in the grade IV group. The glomerular lesion level degree was positively correlated with expressions of mTOR and p70S6K1 inglomeruli. The quantitative urinary protein of 24 h was positively correlated with expressionsss of mTOR in glomeruli. The concentration of serum IgA was negatively correlated with the expressionss level of mTOR in glomeruli. There was no significant correlation between the expressions of Ang II, mTOR, p70S6K1, E-cadherin and α-SMA in IgAN glomeruli and their courses from duration of illness as well as IgA depositional strength.

Conclusion: The mTOR/p70S6K1 signaling pathway was actived in the children with IgAN and it may play an important role in the progress of renal glomeruli fibrosis in children with IgAN.

Abstract# P-SAT171 IgA1 from HSP patients trigger apoptosis and inhibit cytoskeletal proteins in HUVEC Objective: Henoch-Schonlein purpura (HSP) is the most common form of small-vessel vasculitis in children, and its etiology is believed to be associated with immune injury. A polymorphism in the gene encoding heat shock protein 70-2 (HSP70-2) is known to be associated with immune diseases. The purpose of this study was to investigate the correlation between the HSP70-2gene polymorphism (+ 1267 A/G) and HSP in children.

The polymerase chain reaction-restriction fragment length polymorphism (PCR-RFLP) method was used to detect the HSP70-2 polymorphism in 205 cases of children with HSP and 53 controls, and the association of this polymorphism with HSP and HSP nephritis (HSPN) was analyzed.

Results: In patients with HSP, the A/A, A/G, and G/G genotypic frequencies at the +1267A/G position of HSP70-2 were 25.9%, 51.2%, and 22.9%, respectively. In the control group, the A/A, A/G, and G/G genotypic frequencies at the +1267A/G position of HSP70-2 were 30.2%, 60.4%, and 9.4%, respectively. Thus, the G/G genotypic frequency in the HSP group was significantly higher than that in the healthy control group (χ2=4.764, p<0.05). The frequencies of the A and the G allele were 51.5% and 48.5%, respectively, in the HSP group and were 60.4% and 39.6%, respectively, in the control group.

The frequencies of the A/A, A/G, and G/G genotypes in patients with HSP were 24.8%, 52.9%, and 22.3%, respectively. In HSPN, the frequencies of the A/A, A/G, and G/G genotype were 27.4%, 48.8%, and 23.8%, respectively. Conclusion: The +1267A/G polymorphism in the HSP70-2 gene is associated with HSP in children. The G/G homozygous genotype may be a genetic factor that predisposes individuals to HSP, but it is not significantly associated with the development of renal impairment.

Abstract# P-SAT174 Investigation of the correlation between tumor necrosis factoralpha gene polymorphism and Henoch-Schonlein purpura in children

Fei Zhao 1,2 , Songming Huang 1, 2 , Huaying Bao 1 , Guixia Ding 1 , Ying Chen 1 , Hongmei Wu 1 , Aihua Zhang 1 1 Nephrology, Nanjing Children's Hospital, Nanjing, China 2 Institute of Pediatrics, Nanjing Medical University, Nanjing, China

Objective: To investigate the association between serum tumor necrosis factor-α (TNF-α) gene polymorphism and the occurrence and development of Henoch-Schoenlein purpura (HSP) in children.

The polymerase chain reaction-restriction fragment length polymorphism (PCR-RFLP) method was utilized to detect and compare the genotype and allele frequencies at the TNF-α -308 locus in 205 HSP child patients and 53 non-HSP child patients. Furthermore, the relationship between the genotypic and allelic frequencies at the TNF-α -308 locus and the susceptibility to HSP and Henoch-Schonlein purpura nephritis (HSPN) was analyzed.

Results: 1. The G/G, G/A, and A/A genotype frequencies at the TNF-α gene (−308G/A) locus in the HSP group and the control group were 59.5%, 32.2%, and 8.3% and 77.4%, 20.8%, and 1.9%, respectively; among them, the A/A genotype frequency in the HSP group was higher than that in the healthy control group, with a statistically significant difference (x 2 = 6.447, P < 0.05). The G and A allele frequencies in the HSP group and the control group were 75.6% and 24.4% and 87.7% and 12.3%, respectively. The A allele frequency in the HSP group was higher than that in the healthy control group, with a statistically significant difference (x 2 = 7.241, P < 0.05). 2. The G/G, G/A, and A/A genotype frequencies in the HSP non-renal impairment group and the nephritis group were 64.5%, 26.4%, and 9.1% and 52.4%, 40.5%, and 7.1%, respectively, but these differences were not statistically significant (x 2 = 4.474, P > 0.05). The G and A allele frequencies in the HSP non-renal impairment group and the nephritis group were 77.7% and 22.3% and 72.6% and 27.4%, respectively; the difference in the frequency of the A allele in the HSP non-renal-damage group and the nephritis group was not statistically significant (x 2 = 0.240, P > 0.05).

Conclusion: The TNF-α (−308G/A) gene polymorphism was associated with HSP in children. A/A homozygosity may be a genetic predisposing factor for HSP; however, this factor was not significantly correlated with the development of kidney damage.

Abstract# P-SAT175 Objective: The nephrotic syndrome (NS) is characterized by proteinuria, hypoalbuminemia and generalized edema. Although the pathophysiological mechanisms of NS remain unknown, studies with animal models and patients have associated the NS with changes in immune response. The present study investigated the expression of molecules related to cell activation, such as the beta-2 integrin (CD18) and CD80 on peripheral blood leukocytes and renal production of reactive oxygen species in rats with NS induced by Doxorubicin. Meathods: Male Wistar rats, 250-300g, were divided into two groups: animals receiving intravenous injection of doxorubicin (7.5 mg/kg) (DOX, n=32) and control animals that received saline (CON, n=32). The animals were sacrificed at days 7, 14, 21 and 28 after injection, and 24 hour urine and blood samples were collected for biochemical and immunological analyzes. The phenotypic analysis of leukocytes was performed by flow cytometry. The expression of CD18 in monocytes, CD4+, CD8+ and NK cells and the expression of CD80 in monocytes were measured. In renal tissue samples, the oxidative activity was evaluated by TBARS production and antioxidant activity of SOD and catalase.

Results: The DOX group animals showed significant increase in cellular CD18 expression and in the percentage of cytotoxic T lymphocytes, NK cells and monocytes that expressed CD18 as well as raised CD80 expression on peripheral blood monocytes when compared to the CON group. The increased production of reactive oxygen species in renal tissue of DOX group animals was positively correlated with the CD80 expression on monocytes and serum levels of creatinine. Conclusion: These findings indicate a potential link between increased activation of peripheral monocytes and kidney damage in animals with NS. Additional studies analyzing the effects of the blockade of integrins and co-stimulatory molecules may offer new therapeutic opportunities to treat human NS.

Abstract# P-SAT182 Cyclosporine A protects podocyte via upregulating the expression of cofilin-1

Li xiaoyan, Zhang xiaoyan, Li xuejuan, Wang xuejing, Wang suxia, Ding jie Pediatric, Peking University First Hospital, Beijing, China

Objective: Podocyte foot process is dysregulated in nephrotic syndrome. The effacement of podocyte foot processes typically arises owing to perturbations in the actin cytoskeleton. Calcineurin inhibitor Cyclosporien A (CsA) is currently used in the treatment of nephrotic syndrome. Recent data suggest that the effects of CsA on nephrotic syndrome are independent of its effects on the immune system. They identified that CsA can stabilize the actin cytoskeleton through stabilizing synaptopodin in podocytes and thereby reduce proteinuria directly. Other studies also showed that CsA induced cofilin phosphorylation and promoted stress fiber generation in proximal tubular cells. However, whether the antiproteinuric role of CsA is played by regulating cofilin-1 in podocyte has not been studied. Methods: Acute podocyte injury and nephrotic syndrome were induced by puromycin aminonucleoside (PAN) injection in rats with or without CsA. Cultured podocytes were exposed to PAN with or without CsA treatment. Cofilin-1, nephrin, synaptopodin expression were determined by western blot or immunofluorescence. The cofilin-1 specific effect was determined using cofilin-1 siRNA.

Results: CsA reduced proteinuria, restored expression of cofilin-1, nephrin, syanptopodin and repaired foot process effacement of PAN induced nephropathy in vivo. In vitro studies showed that exposure of CsA restored the expression of cofilin-1 and nephrin which decreased by PAN. CsA also repaired actin cytoskeleton impaired by PAN. The protective effect of CsA was disappeared partially when cultured podocytes were exposed to cofilin-1 siRNA. Objective: To find a probably mechanism of immune disorder in RSV nephropathy in rats which resembled the change of minimal change nephrotic syndrome. Methods: 1. RSV mRNAs (G, F, M2, NS1, NS2) and proteins (G and F) were detected with fluorogenic quantitative RT-PCR and indirect immunofluorescence assay (IFA) to find the evidence of viral persistence. 2. DC-SIGN mRNA and protein were detected with RT-PCR and IFA to find the trend of its change. 3. The levels of IL-12/IL-10 and CD4/CD8 were measured by ELISA and flowcytometry.

Results: The course could be divided into two stages: 1. First stage (4d-14d) The mRNA of RSV F, G, M2, NS-1 and NS-2 in kidneys, lungs and spleens expressed and arrived at the highest on 14d, which accorded with the expression of RSV F and G proteins in IFA. The positively fluorescent luminance and mRNA of DC-SIGN expressed stronger than that in the control, especially from 8d (Ct kidney =18.50±3.12) to 14d (Ct kidney =11.40±2.07). Compared with the control, the secretion of IL-12 in renal tissues was rising from 4d (1205.46±262.07pg/ml) to 14d (1054.68±154.18pg/ml). The expression of CD4 and CD8 in peripheral blood was lower than that in normal control. 2. Second stage (30d-120d) RSV mRNA and viral proteins in the tissues were persistently expressed; it showed that partial RSV survived through immune escape. After 30d, the fluorescent luminance of DC-SIGN was gradually reduced in the three tissues. The expression of DC-SIGN mRNA in kidneys, lungs and spleens on 60d (Ct kidney =14.10±8.32) and 120d (Ct kidney =14.92±1.80) was higher, except that in spleens on 30d (Ct= 21.90±1.43) was significantly lower than the control. The secretion of IL-12 in tissues decreased after 30d (583.40±199.26pg/ml), but that of IL-10 gradully increased after 30d (3832.38±88.20pg/ml). Although the expression of CD4 and CD8 in peripheral blood was increased gradually after inoculation, the level was still lower than that in normal control.

Conclusion: There was RSV persistence in rats which likely attributed to the RSV-induced immune escape. RSV interacts with DC-SIGN to make DCs inhibit the immune ability of cytotoxic T cells, caused T cell immune dysfunction and finally induced the immune tolerance.

Abstract# P-SAT185 Evaluation of the migratory and regulatory profile of leukocytes from peripheral blood in pediatric patients with idiopathic nephrotic syndrome Objective: Dynamic regulation of the podocyte cytoskeleton plays an important role in maintaining the glomerular filtration barrier. Severe glomerular disorders are associated with perturbed organization of the podocyte actin cytoskeleton. The therapy is based on immunosuppressive agents, in particular cyclosporine A, whose beneficial effect seems also related to the stabilization of the actin cytoskeleton in podocytes. Previous studies of our group demonstrated, that the mTOR inhibitor everolimus (EV) might similarly involve the recovery of the actin cytoskeleton in a puromycin (PAN) experimental model of proteinuric disease.

Methods: In the present study, inhibitory effects of EV on mTOR complex 1 (mTORC1) and −2 (mTORC2) were analyzed by western blotting in human differentiated podocytes. Downstream of mTORC2 the activity of RhoA (GTPase pull-down assay) and myosin light chain (MLC) was studied. To identify further signaling pathways affected by EV, we performed Affymetrix microarray expression analysis followed by verification using real-time RT-PCR.

Results: Biochemical studies revealed a substantially decreased phosphorylation level of both mTOR effector proteins mTORC1 (reduced p-Akt) and mTORC2 (reduced p-p70S6K) by EV. Objective: Notch constitutes an evolutionarily conserved intercellular signaling pathway that determines cell fate in various organs. Activation of Notch1 and Notch2 has been implicated in human glomerular diseases. Notch1 was reported to play a critical role in development of glomerular diseases; however, a function of Notch2 remains unclear. Our aim of the study is to clarify Notch2 pathway's contribution to developing proteinuria and glomerulosclerosis. Methods: We injected either Jagged1 antagonistic antibody (mAb) or Notch1 agonistic mAb or Notch2 agonistic mAb intraperitoneally to mice with adriamycin (ADR) nephropathy, a model of nephrotic syndrome and focal segmental glomerulosclerosis, and evaluated the levels of proteinuria and the ratios of sclerotic glomeruli. Next, we treated cultured podocytes with ADR in the presence or absence of Notch2 agonistic mAb, and we assessed the effect on cell survival and examined the pathways involved. Then, we evaluated a correlation between Notch2 activation and podocyte loss in human kidney specimen of nephrosis, minimal change disease (MCNS) and focal segmental glomerulosclerosis (FSGS).

Results: Administration of Notch2 agonistic mAb ameliorated nephrosis and glomerulosclerosis in mice with ADR nephropathy, even when Notch2 agonistic mAb was administered therapeutically after the onset of nephrosis. In vitro, Notch2 agonistic mAb protected ADR-damaged podocytes from apoptotic cell death. The specific knockdown of Notch2 led to increased apoptosis in damaged podocytes. Notch2 rescued damaged podocytes from apoptosis through Akt pathway. Human kidney specimens of nephrosis showed a positive linear correlation between the number of podocytes expressing activated Notch2 and the number of residual podocytes. The glomeruli with MCNS showed more activated Notch2 and more podocytes, Conclusions: whereas those with FSGS showed less activated Notch2 and less podocytes. Notch2 pathway has a pivotal role in preventing apoptosis of damaged podocytes. Specific activation of Notch2 may represent a novel clinical strategy for the amelioration of nephrosis and glomerulosclerosis.

Abstract# P-SAT191 Proliferation and apoptosis of tubular cells in initial and advanced stages of focal segmental glomerulosclerosis (FSGS) Objective: To analyze processes of proliferation, apoptosis and ciliogenesis in proximal tubular cells during initial and advanced stages of FSGS associated with cystogenesis. Methods: Normal kidney tissues and tissues of FSGS kidneys were immunohistochemically analyzed using Ki-67 proliferation marker and caspase-3 apoptotic marker. Diameters of dilated and cystic proximal tubules were measured and correlated with proliferation index of tubular cells. Data were analysed by the Kruskal-Wallis and Dunn's post hoc test and expressed as mean+/−SD. Significance was accepted at p<0.05. Results: Normal kidney tissue showed absence of proliferation in proximal tubules. Initial stages of FSGS were characterized by intermingling areas of healthy non-proliferating proximal tubules, and pathologically changes, mildly dilated (15um diameter) proximal tubules showing proliferation index of 15.92%. Advances stages of FSGS displayed different stages of cystogenesis: while mildly dilated tubules (diameter 12+/−1.4um) contained 36,18% of proliferating cells, their number increased to 45,23% in cysts with diameters from 21+/−1.8um to 54+/−1.1um, and then decreased to 13,76% in cysts with diameter 73+/−2.1um. While in the largest cysts proliferation index was lowest and the primary cilia short and distorted, in distal tubules and collecting ducts, primary cilia were extremely long and branching. Apoptotic caspase-3 positive cells were observed within the tubular and interstitial cells.

Conclusion: Deterioration of proliferation and apoptosis, and primary cilia formation characterizes cystogenesis in FSGS kidneys. We suggest that changes in primary cilia length might cause alterations in transfer of signaling pathways which control proliferation, differentiation and apoptosis of proximal tubules cells. Advanced stages of FSGS are associated with cyst formation and increased proteinuria leading to nephrotic syndorme Objective: To investigate effect of prednisone on the expression of Ezrin and NEPH1 in rats with adriamycin-induced nephrosis. Methods: ADR model was induced by a tail intravenous injection of ADR.The rats were divided into three groups, which were control group, model group and prednisone group. Serum index and 24h urinary protein were measured at 4 and 8 weeks. Observe pathologic changes of renal tissues at 8 weeks. The expression of Ezrin and NEPH1 in glomerulus was evaluated by immunohistochemistry respectively.

Results: Compared with control group, at 4 weeks, 24h urinary protein and total cholesterol in model group and prednisone group were significantly increased(P<0.05),albumin and total protein were significantly decreased(P<0.01),which indicated that the model was successfully established;Compared with control group, at 8 weeks, the expression of Ezrin and NEPH1 in the model group were significantly decreased(P<0.01).Compared with the model group, The expression of Ezrin and NEPH1 in prednisone group were significantly increased(P<0.01),24h urinary protein and total cholesterol were significantly decreased(P<0.01),albumin and total protein were significantly increased(P<0.05), renal pathology and ECM/GA were significantly increased(P<0.01).

Conclusion: The expression of Ezrin and NEPH1 were reduced in rats with adriamycin-induced nephrosis which were negatively related to proteinuria and renal pathological damage. Prednisone can reduce the proteinuria and relieve the renal pathological damage by improving the expression of Ezrin and NEPH1.

Objective: The activation of the complement system plays an important role in various kidney diseases, such as antibody-mediated rejection or membranous glomerulopathy. The majority of the circulating complement components are produced in the liver. But in the last decade the local production of complement components by other cells is highly debated. The aim of our study was to proof the ability of human podocytes to produce and secrete complement components.

Methods: Immortalized human podocytes were analyzed with Western Blot (WB), immunofluorescence (IF) or/and PCR for their ability to produce components (C1/ C1q, C2, C3, C4, C5) inhibitors (factor H, MCP, CD 55, CD59) and activators (factor B, properdin) of the complement system. Secretion of components was measured in the medium, and functionality of C3 was tested in a specific C3convertase assay. Stimulation of the cells was done with interferon-γ, interleukin 6 and human albumin.

Results: PCR-studies revealed that human podocytes express on mRNA level the components C2, C3, C4, C5, the inhibitors factor H, MCP, CD 55 and CD59, and the activators properdin and factor B. On protein levels C2, C3, factor B, properdin, factor H, CD 55 and CD59 were detected. In immunofluorescence all the components showed either intra-plasmatic, perinuclear or peri-membranous distribution. In addition, we could show that podocytes secrete the factors C2, C3, C4, C5, factor B and factor H into the medium. The secreted C3 was clearly functionally active and could further enhanced by stimulation with interferon-γ. Objective: To construct the eukaryotic expression plasmid and its shRNA plasmids of IL-17 of mice, and to investigate their ability of expression or inhibitory effects on IL-17.

Methods: The RNA from spleen cells of Bal b/c mouse was reversed to cDNA, and the full-length CDS fragment of IL-17 was amplified.

The target segment was cloned into the expression vector pLVX-IRES-ZsGreen1, then, the recombinant plasmid pLVX-IL-17-IRES-ZsGreen1 was obtained. Three pairs of shRNA chains targeting IL-17 gene and one pair control shRNA chain were designed and synthesized, then annealed to form double strand ,and inserted into the expression vector pLVX-shRNA2. three shRNA plasmids and one control plasmid were constructed ,which were called ShRNA1/ShRNA2/ShRNA3/ShRNAC, respectively. These plasmids were identified by restriction analysis and sequencing, and then they were transfected or co-transfected into 293T cells in mediation of liposome. The transcription levels of IL-17 mRNA were detected by fluorescent quantitative PCR, while expression levels of IL-17 protein were detected by ELISA.

Results: Restriction analysis and sequencing proved that the recombinant plasmid were constructed correctly. The plasmid of pLVX-IL-17-IRES-ZsG reen1 could expression IL-17; when cotransfected with expression plasmid, IL-17 expression levels were significantly reduced in the group of ShRNA1/ShRNA2/ShRNA3 as compared with the ShRNAC group, and the group of ShRNA1 had the least expression. Conclusion: IL-17 eukaryotic expression plasmid and its specific ShRNA plasmids were constructed successfully, and plasmid ShRNA1 had the best inhibitory effects.

Abstract# P-SAT195 Reno-protective Effect of All-trans Retinoic Acid on Adriamycininduced Nephropathy Mice

Xiaoli Wang, Jingjing Cui, Qiu Li Nephrology and immunology department, children's hospital of Chongqing medical university, Chongqing, China

Objective: To investigate the reno-protective effect of All-trans Retinoic Acid on Adriamycin-induced Nephropathy mice. Methods: BALB/C mice were randomly divided into nephrosis(a single adriamycin 10.5mg/kg,n=20) and control(tail intravenous injection the same amount of saline,n=10). After two weeks nephrosis divided into ATRA-treated(intraperitoneal injection All-trans Retinoic Acid 20mg/kg, three times a week,n=10) and ADR nephrosis. Then, we chose five mice each group randomly after treatment 6 weeks and 10 weeks to collect 24-hours-urine, blood, kidney for detection. Results: All-trans Retinoic Acid significantly decreased 24-h urinary protein excretion, serum TC and TG(P<0.05). Fas/FasL shows by Immunohistochemistry and Fluorescence quantitative PCR is decreased sifnificantly(P<0.01). Conclusion: All-trans Retinoic Acid has anti-apoptosis on adriamycin induced nephropathy mice to protect kidney damage and delay fibrosis process.

Abstract# P-SAT196 Molecular mechanism of ERS induced by lipid accumulation in human mesangial cells (HMCs) Objective: Dehydroxymethylepoxyquinomicin (DHMEQ) is a novel NF-κB inhibitor that potently inhibits the DNA-binding activity of NF-κB, resulting in therapeutic effects for various pathological conditions. To elucidate the pathogenetic role of NF-κB in minimalchange nephrotic syndrome (MCNS), we examined whether DHMEQ could ameliorate the nephrosis in mice induced by puromycin aminonucleoside (PAN), which is considered to be an animal model for MCNS. Methods: Mice were injected with DHMEQ or only vehicle 2 hours before PAN injection on day 0. Mice without PAN injection served as controls. The DHMEQ or vehicle was injected on 5 consecutive days. The daily urine was collected from day 0 to day 30, and the urinary concentrations of albumin and creatinine were measured. Some mice were sacrificed 7 days after PAN-injection, and their serum concentrations of cholesterol, total protein, albumin and IL-6 were measured and the pathological changes of the kidneys were observed. Results: PAN injection without DHMEQ in the mice induced albuminuria which gradually increased up to day 10 and gradually decreased thereafter. In contrast, pre-treatment with DHMEQ was associated with no significant increase in the amount of albuminuria during the first 2 weeks. In the serological tests, PAN-injection induced an increase in the cholesterol level, a decrease in the total protein, a decrease in the albumin and an increase in the IL-6 in the serum compared to controls 7 days after injection, but DHMEQ ameliorated these changes. An electron microscopic analysis indicated an effacement of the foot processes of the podocytes in the PAN-injected mice, but this was rarely observed in the PAN-injected mice pre-treated with DHMEQ. Further immunohistochemical analysis showed that DHMEQ can inhibit the PAN-induced translocation of NF-κB from cytoplasm into nucleus. Conclusion: These results suggest that DHMEQ can be a candidate therapeutic agent for MCNS, because the activation of NF-κB of podocytes may be associated with PAN-induced nephrosis.

Knocking-down STIM1 gene expression inhibits some podocyte molecules jingjing yan, meigui wang, siguang lu The First People's Hospital, Lianyungang, Jiangsu, China

Objective: To study the effect of knocking-down STIM1 on nephrin,podocin,CD-2AP and α-actinin-4 in murine podocytes in vitro.

Methods: Conditionally immortalized murine podocyte cells were cultured in RPMI1640 medium at 33°C permissive condition.Then the cells were shifted to non-permissive condition at37°C and cultured for ten-fourteen days, and were transfected with STIM1 small interfering RNA(siRNA) using transfection reagent lipofectamine 2000.Transfection efficiency was measured by flow cytometer and inhibitory effect of STIM1 siRNA was determined by RT-PCR and western blot at fourty-eight, senventy-two hours after transfection respectively.

Results: (1)The transfection efficiency of FAM-siRNA was about 75.5%.

(2)After transfection with specific siRNA,the expression levels of STIM1 mRNA and protein were down-regulated by 80.7% and 49.8% respectively. (3) The expression levels of nephrin,podocin and α-actinin-4 mRNA were decreased by 62%, 35%, 60% respectively, whereas CD-2AP showed no-change.Both podocin and α-actinin-4 protein were decreased by 45%, 20% respectively. Objective: One of the most common causes of nephrotic syndromes in children is the Minimal-Change-Glomerulonephritis (MCGN). Typically, MCGN results in foot processes effacement of podocytes. Their plate-shaped flattening very likely results in dysfunction of the filtration barrier and may underlie the increase in urine albumin concentrations. Notably, MCGN often disappears at the onset of puberty, thus in parallel with increasing peripheral hormone levels. This is particularly true in girls.

Methods: Podocytes were studied by Western Blot analysis, Immunofluorescence and by radioimmunoassay for their ability to secrete and bind estrogens. Results: Podocytes express estrogen receptors both, estrogen receptor alpha and beta, suggesting that increasing levels of estradiol during puberty potentially maintains and restores morphological integrity of podocytes. This protective function could be mediated by stabilization of the podocyte cytoskeleton by estradiol, as evidenced in vitro by the increase in phosphorylation of cofilin, very similar to the effects of estradiol in neurons. Furthermore, we show that dissociated podocytes express aromatase, the final enzyme of estrogen synthesis, and that podocytes in fact synthesize estradiol, as evidenced by measuring estradiol content in the supernatant. Conclusion: As it was shown that estrogen synthesis in cells other than those in gonads becomes stimulated during puberty, an autocrine mechanism of estrogen action could underlie decreasing frequency of MCGN at the onset of puberty.

Overexpression of PGC-1α inhibits aldosterone-induced podocyte phenotypic changes and detachment by blocking mitochondrial dysfunction Objective: To explore the role of heparanase in the pathogenesis of rat respiratory syncytial virus (RSV) nephropathy. Methods: 150~200g Sprague-Dawley(SD) rats (n=5 per group) were inoculated with 6×10 6 (plaque-forming units, PFU) RSV and sacrificed on days 4,8,14 and 28 postinoculation (RSV 4 ,RSV 8 ,RSV l4 and RSV 28 ). Five normal SD rats inoculated with Dulbecco's minimum essential medium were served as normal control. The expression level of heparanase protein and mRNA in kidney of each group was determined by immunohistochemical staining and real-time quantitative RT-PCR respectively. The proteinurina was measured and the relationship between the expression level of heparanase and the 24hour urinary protein was studied.

Results: Rats with RSV nephropathy exhibited higher proteinuria by comparison with normal rats. There was a significant difference between each group(RSV 14 >RSV 8 >RSV 28 >RSV 4 ). Compared with normal control, Rats with RSV nephropathy showed up-regulated expression of heparanase protein in glomeruli. The expression level of heparanase protein in RSV 8 and RSV 14 group was higher than that in RSV 4 and RSV 28 group. There was a linear positive correlation between the expression level of glomerular heparanase protein and the quantity of urinary protein(r =0.783,P<0.05).Compared with normal control group, the expression level of heparanase mRNA in kidney f r o m R S V 4 , RSV 8 , RSV 1 4 a n d R S V 2 8 g r o u p w a s elevated(RSV 14 >RSV 8 >RSV 4 >RSV 28 ).There was a linear positive correlation between the expression level of renal heparanase mRNA and the 24-hour urinary protein(r=0.725,P<0.05).

The increased heparanase in kidney may be important to the loss of glomerular negative charge in glomerular basement membrane and is involved in the pathogenesis of RSV nephropathy. Podocyte morphology andautophagosomes were viewed using electron microscopy. Podocyte numerical density was estimated by Weibel-Gomez method. Expressions of autophagy markers and ERSassociated proteins were analyzed by Western blot.

Results: The expression level of ERS-associated protein GRP78 was up-regulated from day 4 to day 21 post-injection. The results also showed that autophagosomes were massively accumulated and the autophagy marker LC3 was up-regulated in the models on day 7 and 14. Furthermore, rapamycin was given to PHN rats to further explore the role of autophagy in the process of complement-dependent podocyte injury. Results revealed that rapamycin, which enhanced autophagy in podocyte, could reduce proteinuria, lighten podocyte lesionsand prevent podocyte loss on day 21. Conclusion: Taken together, our results demonstrated that ERS plays an important role in completment-dependent podocyte damage, and in another aspect, autophagy induced byERS can alleviate injury as a protective mechanism. This provides an important basis for a thorough understanding of the role of autophagy in the process of podocyte damage and the pathogenesis of MN.

Abstract# P-SAT209

The cytoprotective role of autophagy under oxidative stress in human podocyte Objective: Autophagy is a ubiquitous catabolic process involving selective degradation of cellular components. It shows cytoprotective effects in different cell types and helps to maintain cell homeostasis. Some studies demonstrated that cell stress, generated by starvation or some chemical reagents, can initiate autophagy which responsively resists harmful stimuli at the early stage. However, little is known about autophagy in human podocytes under oxidative stress. Since the apoptosis and autophagy pathways share some common molecules, we investigated the role of autophagy induced by puromycin aminonucleoside (PAN) in human podocytes. Methods: Human conditional immortalized podocytes were treated with PAN, the generation of reactive oxygen species (ROS) was measured by immunofluorescence. Then, autophagy was assayed by immunofluorecence staining for LC3 puncta and western blotting for LC3. In addition, the mammalian target of rapamycin (mTOR) and its substrates which play critical roles in autophagy inhibition were investigated for elucidating the mechanism of PAN induced autophagy. Podocyte apoptosis was assessed by flow cytometry (YO-PRO-1/PI and active caspase 3 assays). To study the effects of autophagy on podocyte apoptosis, 3-Methyladenine (3-MA) and chloroquine were used to inhibit autophagy. Results: Autophagy was detected in PAN-treated podocytes in a dose and time dependent manner and prior to apoptosis which was accompanied with increased ROS generation. LC3 aggregates were observed in the cytoplasm and the expression of LC3 II was significantly elevated. Intriguingly, Phospho-mTOR and its substrates (Phospho-p70 S6 Kinase and Phospho-4E-BP1) increased when the autophagy was activated. When autophagy was inhibited by 3MA and chloroquine, podocyte apoptosis increased significantly.

Conclusion: PAN induced both apoptosis and autophagy in human podocytes. Our results suggested that this in vitro model will be useful for the study of crosstalk between apoptosis and autophagy in podocytes. Autophagy may be the adaptive cytoprotective mechanism for podocytes under oxidative stress. Further studies directed at identifying the role of mTOR are essential.

Abstract# P-SAT210 Impact of Angptl3 knock-out on adriamycin-induced nephropathy mice Objective: Idiopathic focal segmental glomerulosclerosis (FSGS) is associated with recurrence after transplantation due to a circulating permeability factor or factors (N Engl J Med, 334:878-883, 1996) . We have shown the effects of FSGS plasma and its fractions on glomerular permeability in vitro and in vivo and have used state-of-the-art proteomics to identify cardiotrophin-like cytokine-1 (CLCF1), a member of the IL-6 family, as a candidate for the active substance.

We hypothesize that a new model of FSGS can be based on the effects of CLCF1 in mice.

Methods: rCLCF1 (R&D Systems) was injected intraperitoneally (IP), one dose, 10 μg/kg, or infused by minipump for 28 days, 40 μg /kg/day. A construct containing CLCF-1 was administered by electroporation. All studies were done in C57BL6 mice. Urinary albumin/creatinine, pJAK1, pSTAT3, pATK, and pERK in peripheral blood cells (PBC) and in kidney homogenate were measured and glomerular histology was assessed.

Results: Albuminuria was induced promptly by rCLCF1 after either injection or electroporation. Peak albuminuria occurred by 7 days of expression and was 3-5 fold increased vs. baseline. IP administration of rCLCF-1 increased pJAK2 and pSTAT3 of PBC within 15 min. and renal pJAK1 and pSTAT3 remained upregulated for at least 72 hours after injection. Kidney pJAK2 and pSTAT3 as well as pERK/12 and pAKT were markedly increased after 28 days of infusion. Mesangial matrix was increased at that time.

Conclusion: We conclude that CLCF1 mimics many of the renal effects of the active fraction of plasma from patients with idiopathic FSGS and may play an important role in its etiology. Its relationship to other candidates such as circulating urokinase receptor (suPAR) is not known. A murine model based on administration or overexpression of CLCF1 may permit us to define mechanisms of injury and to test potential therapeutic agents prior to use in clinical trials.

Over-expression of Myo1e in mouse podocytes enhance cellular endocytosis, migration, and adhesion Objective: To investigate the effects of tacrolimus (FK506) on hepatocyte growth factor and transforming growth factor β1 in kidneys of FSGS rats. Methods: Establish unilateral nephrectomy combined adriamycin double tail vein injection of FSGS model in rats divided to model group and treatment group ,then treatment group rats were treated withtacrolimus 8 weeks. The protein expression and site of HGF and TGF-β1 in renal of each group were assayed by Western blot and immunohistochemistry after animals were sacrificed. Urine protein, serum albumin, blood lipoids and kidney function were tested automatic biochemical analysis system. Results Compared with the normal control group , model group and treatment group rats 24h urinary protein excretion , serum creatinine , blood urea nitrogen , cholesterol significantly increased serum albumin significantly reduced severe renal pathological changes and TGF-β1 protein expression was significantly increased , and the differences were statistically significant ( P <0.05 ) ; compared with the model group , in experiments 9 weekend , the treated rats 24h urinary protein excretion , relevant serum biochemical indicators of renal pathological changes varying degree of improvement in renal tissue HGF protein expression was increased and TGF-β1 protein expression was decreased , the above differences were statistically significant ( P < 0.05 ). Objective: The cellular mechanisms of kidney injury caused by obstructive nephropathy are interstitial inflammation, fibrosis, and apoptosis. Rodent models can be used to simulate obstructive nephropathy in the human kidney. We developed shear-thinning hydrogels for the local delivery of IL-10 to abate the progression of inflammation and fibrosis that leads to CKD. Methods: Injectable Dock-n-Lock gels were developed as previously reported 1 (Fig. 1) . IL-10 was added to the gel or phosphate buffered saline solution at a concentration of 0.33 ug/uL. Study Design: Eight cohorts were studied (7, 21, 35 days, n=4): healthy, sham operation, healthy injected with MSA, healthy + gel, unilateral ureteral obstruction (UUO), UUO + IL-10, UUO + gel, UUO + gel/IL-10. 15 uL of IL-10 solution, gel, or gel/IL-10 was injected into the left kidney via retroperitoneal approach 3 days after the initial UUO or sham operation. Histology: Immunohistochemistry (IHC) was performed on paraffin sections to identify macrophages and apoptotic cells, and trichrome stain was used to evaluate fibrosis. Cells and total area were quantified for IHC and total fibrotic area was quantified for trichrome.

Results: Comparing the treatment groups to the untreated UUO, macrophage infiltration and apoptosis were significantly reduced at day 21 and 35. By day 35, adding the IL-10 via gel injection reduced macrophage infiltration more than IL-10 alone and IL-10 alone did not reduce apoptosis. Fibrosis was decreased by day 35 in all three treatment groups (Fig. 2) . Conclusion: Injectable hydrogels were synthesized that permit facile local delivery of immunotherapy to both healthy and obstructed kidneys. Renal inflammation and scarring was reduced in an animal model of CKD by using an injectable hydrogel for drug delivery. Logistic regression reveled that APD was the main parameter significantly associated with surgery treated UPJO cases (ROC plot was 0.79). A possible threshold of 14mm APD may be used as a cut-off value of surgery treated UPJO group with a sensitivity of 77% and a specificity of 69%. Conclusion: APD dilatation was the strongest predictor of surgery treated UPJO. PT and renal length also significantly discriminate the two groups and correlate with APD, only with lower predictive power. Our findings expand the clinical knowledge in the field of prenatal consult by highlighting a threshold of APD, which predicts the need for surgery in prenatally detected HN cases.

Abstract# P-SAT221 Survival and renal outcome in fetuses with Lower Urinary Obstruction (LUTO) with and without intra-uterine vesicoamniotic shunting.A ten years experience of a cohort. Objective: Ureteropelvic junction obstruction (UPJO) is the most common cause of hydronephrosis in children. The significant role of the surgical relieving of high grade obstruction is indisputable. Nevertheless, the effect of pyeloplasty on the function of the involved kidney remains controversial, especially in UPJO with significantly reduced relative renal function (RRF) before surgery. To evaluate the effect of pyeloplasty on the relative function of kidneys in children with UPJO and decreased RRF.

Methods: The records of children who underwent pyeloplasty for UPJO during a 10 year period in Schneider Children's Medical Center of Israel were reviewed. The study group included 40 children who underwent pyeloplasty for UPJO and had an initial RRF <40%. Children with bilateral UPJO, solitary kidney or other genito urinary abnormalities were excluded. The control group included 38 children with non obstructive hydronephrosis and an initial RRF<40%. Results: The average initial RRF in the study group was 26% (range 8-39%), and the final renal function was 32% (1-55%). In the control group, the initial RRF was 23% (2-40%), and the final function was 22% (0-44%). The final RRF of the study group was significantly higher comparing to the control group (P value<0.05). In the subgroup of patients with UPJO and initial RRF<30% (24 patients) the average initial function was 20% (8-30%) and the final function was 27% (3-55%). In the control subgroup with initial RRF<30% (26 patients) the initial average function was 17% (2-30%) and the final function was 17% (0-44%).

Conclusion: Pyeloplasty is associated with an improvement of renal function in children with UPJO and an initial RRF <40%. This is also true for patients with RRF<30% before surgery. Our results support the need for pyeloplasty in children with UPJO and reduced RRF. Objective: common cause of chronic kidney disease (CKD) in children. Kidney damage occurs in utero, and kidney disease progresses postnatally. The objective of this project was to identify clinical biomarkers, in particular antenatal variables, which predicted long-term renal outcome in boys with PUV.

Methods: This was a retrospective cohort analysis. Primary outcome was the development of end stage renal disease (ESRD) as defined as starting dialysis or preemptive transplantation. Clinical variables studied included antenatal factors, postnatal renal function, and modifiable variables. Continuous data for the two outcome groups were compared, receiver-operating characteristic (ROC), Kaplan-Meier, and logistic regression analyses were conducted to assess the robustness of each candidate biomarker as a predictor of outcome.

Results: In this cohort 15 cases reached the primary outcome of end stage renal disease at a mean age of 7.0 ± 6.7 yrs. Compared to those who didn't, those who progressed to ESRD had younger age at diagnosis (0.6 ± 1.5 vs 3.3 ± 7.3 yrs, p<0.05), valve ablation ( . FSGS rate was found as 60% in biopsied subjects. Nephrotic syndrome was defined by edema, massive proteinuria (>40 mg/m 2 per hour or a protein/creatinine ratio >2.0 mg/mg), hypoalbuminemia (<2.5 g/dl), and hyperlipidemia. Remission was defined as a urinary protein excretion below 4 mg/m 2 per hour or a protein/creatinine ratio below 0.2 mg/mg for three consecutive days. Steroid resistance was accepted as no achievement of remission in spite of treatment with prednisolone, 2 mg/kg per day for 4 weeks. If steroid resistance was seen, patients were also treated with cyclosporine A (CsA) (3-5mg/kg per day for least 6 months) and, thereafter, if required, with cyclophosphamide(CP) (2.5-3.0 mg/kg per day for 10-12 weeks). Renal failure was defined as a glomerular filtration rate (GFR) below 80 ml/min per 1.73 m 2 body surface area, and ESRD was defined as a GFR below 10 ml/min per 1.73 m 2 or the necessity for any renal replacement therapy. Bidirectional DNA cycle sequencing analysis of entire coding exons and adjacent intronic segments of NPHS2 gene was performed.NPHS2 gene mutation analysis has been also performed in 100 healthy children.

Results: Pathogenic NPHS2 mutations were found in 127 patients (18%) from the totally 700 SRNS children group. Mutation rate was 29% in familial group, and 16.5% in sporadic group. A total of 53 mutations were determined in the NPHS2 gene, and 37 of which were characterized as a novel mutation presented at HGMD databank. In the mutation positive NPHS2 group, most of the mutations were found out to harbor in exons 1, 4, and 5 while no mutation were found in exon 6 of the respective gene. Patients with P118L, R138Q, R138X, R168H, S211A, A212T, V218G, H228D, IVS7+5G>A, c.460-467insT, c.503delG mutations were progressed to end-stage renal disease (ESRD). Also, age at onset of proteinuria (years) was 4.2+/-0.5 in the mutation (-) (n=573) group; 3.6+/-3.08 in the mutation (+) (n=127) group. For the mutation negative patients, effects of other disease causing genes involving NPHS1, WT1, TRPC6, CD2AP, and ACTN4 for different molecular subtypes of SRNS may be considered. NPHS1 mutation screening was performed for all the patients who had proteinuria up to two years of age. As a causative SRNS gene, we should consider NPHS2 gene mutation screening in early diagnosis and the follow-up of the clinical course. Conclusion: In relation to homozygous or compound heterozygous NPHS2 mutated patients who have the lack of response to standard steroid therapy we suggest to perform NPHS2 gene mutation analysis for every child (if consent can be obtained) soon after the first episode of NS. For the newly diagnosed patients, the crucial certain determination of the causative disease gene mutation will enable clinicians to avoid redundant immunosuppressive therapeutic trials. We then performed Next Generation Sequencing in the 5 affected patients and in one of their unaffected relatives. Exome enrichment was conducted using the Agilent SureSelect Human All Exon V4+ UTRs capture kit. The multiplex libraries were then sequenced on an Illumina HiSeq2000 instrument with a 2x76 bp read length. Results: We unexpectedly identified a novel LMX1B mutation segregating with the disease in the family. Subsequently, we screened 74 additional unrelated families from our international cohort of autosomal dominant FSGS and found mutations of the same residue in 2 families. None of the probands had any sign of dysplasia of nails, patellae or elbows, iliac horns or glaucoma, or any ultrastructural changes suggestive of Nail-Patella-like renal lesions. LMX1B encodes a homeodomain-containing transcription factor that is essential during development. A LMX1B in silicohomology model suggests the mutated residue plays an important role in strengthening the interaction between the LMX1B homeodomain and DNA. Both mutations are expected to diminish such interactions.

Conclusions: Our data demonstrate that isolated FSGS could be due to mutations in genes also involved in syndromic forms and highlights the need to include these genes in all next-generation sequencing diagnosis approaches in FSGS. .In 24/37 patients mutations were identified by NGS (detection rate 65%). In 8/24 patients sequence variants were found in two different genes (33%) suggesting oligogenic inheritance. In 6 of these 8 patients the causative mutation was located in a gene following an autosomal dominant inheritance pattern. These patients showed a more severe form of the disease compared to affected family members who showed only the causative mutation without additional modifier variants. One additional patient of this cohort with Pierson syndrome had two single heterozygous mutations in NPHS1 and LAMB2, respectively (both genes following an autosomal recessive pattern of inheritance). A further patient showed two mutations in NPHS1 and the non-neutral polymorphism p.Arg229Gln in NPHS2. Structural and/or functional analysis of all mutations identified in patients with mutations in two genes suggested impaired protein function of the particular gene products.

Conclusion: The influence of modifier genes or digenic inheritance seems to play an important role in the pathogenicity of SRNS. The application of NGS is therefore of special interest and highly efficient in the diagnostics of patients with SRNS.

Abstract# P-SAT232 Mutation analysis in Japanese patients with congenital and infantile nephrotic syndrome Objective: Mutations in podocyte genes (NPHS1, NPHS2, WT1, and LAMB2) are associated with congenital (<3 months) and infantile (3-12 months) nephrotic syndrome (NS). The purpose of this study is to investigate the frequency of causative mutations in these genes in NS manifesting in the first year of life in Japan.

Methods: All exons and exon-intron boundaries were investigated in consecutive, unrelated 37 patients from regional pediatric kidney disease centers, by PCR-direct sequencing. Results: We detected disease-causing mutations in 64.9% (24 of 37) patients (75% in congenital and 25% in infantile) (Table) . Objective: Hypercoagulability along with thrombosis are prevalent complications of nephrotic syndrome. In cases of refractory nephrotic syndrome, intracardiac thrombus, although rare, is a serious complication because of its association with morbidity and high mortality Herein, we report a case of a patient with a right atrial thrombus associated with nephrotic syndrome who responded well to corticosteroid therapy and attained quick remission. Case Presentation: A 12-year-old Japanese boy was referred to our hospital for a right atrial thrombus associated with idiopathic nephrotic syndrome diagnosed 1 month ago. The patient had attained the partial remission within the 10 days of the corticosteroid therapy initiation. He was asymptomatic otherwise at the time of admission. The patient's vitals were stable and laboratory values were almost within the normal range. An echocardiogram showed a large isolated hyperechoic mass in the right atrium, which originated from the superior right atrial wall. The mass measured 31 × 26 mm in diameter and traversed through the tricuspid valve in a to-and-fro motion. The patient underwent emergency surgical thrombectomy on day 1 and anticoagulation therapy was started 7 days after the operation. The subsequent hospital stay was uneventful, and the patient was discharged on day 87. Objective: Nephrotic syndrome features proteinuria and severe sodium retention which is implicated in ascites and edema formation. Previous works realized in nephrotic rats (PAN) clearly highlighted that, the increase of sodium absorption in the CCD is associated with an increase activity of the Na/K ATPase; sodium absorption is independent of aldosterone, activation of epithelial sodium channel ENaC is not necessary and sodium absorption is inhibited by amiloride. Our hypothesis is that, in nephrotic syndrome, another apical sodium channel sensitive to amiloride and independent of SAAR pathway is implicated in sodium absorption in the CCD. Methods: Transcriptome of CCDs from control rats (CT) and PAN treated clamped rats (ADX PAN) was analyzed using a high-resolution quantitative and comparative analysis of gene expression. Subsequently, transcriptional (RT-qPCR) and proteic expression (western blot and immunohistochemistry) of channel were analyzed. Sodium handling in vivo and in vitro microperfused collecting ducts in different conditions (pH 6 and 7.4, apical zinc 0.3mM and amiloride 0.1mM) was analyzed.

Results: Metabolic study confirmed previous results: Sodium absorption and ascites were similar in PAN and ADXPAN rats suggesting that sodium absorption in nephrotic syndrome was independent of SAAR. Transcriptome analyses highlighted an increased expression of ACCN1 mRNA only in ADX PAN at day 6. RT-qPCR confirmed an increased expression of ACCN1 only in the CCD of ADX PAN (about eight time compared to ADX CT). Subsequently, protein shows an increase expression of ACCN1 (ADX PAN 0.72+/-0.12 vs ADX CT 0.31+/-0.03, p 0.03) and immunohistochemistry on microdissecated CCD highlighted a strong apical expression of ACCN1 only in ADX PAN rats. Schimke immuno-osseous dysplasia (SIOD) is a rare autosomalrecessive multisystem disorder, characterised by: disproportionate growth deficiency, defective cellular immunity, nephrotic syndrome and progressive renal disease.SIOD is caused by bi-allelic mutations of SMARCAL1 gene, which encodes the HepA-related protein (HARP), a member of the SNF2 family of ATPases, acting as chromatin remodelers within multi-protein complexes. A 5-year-old patient was hospitalized in November 2011, after detection of proteinuria (1.5 g/day). At our first clinical examination: height-weightgrowth retardation, thinning hair, thin and hypopigmented skin, arched palate and normal neuro-psychological development.Serological examination showed proteinuria (1.7 g/day), hyperchol. (Col tot 242 mg/dl, LDL-Col 165 mg/dl) and lymphocyte deficiency (17.6%).Renal function and complement levels were normal. Lymphocyte subpopulations showed a decrease of T lymphocytes and an increase of NK cells(CD16 + CD56 +).The patient had also a detour back-lumbar scoliosis and normal kidneys in size, but with reduced corticomedullarydifferentiation. We started therapy with ramipril 2.5 mg/day. The 24 hrs urine exams performed monthly showed persistent proteinuria (500-800 mg/day). At the end of April 2012, for the persistence of proteinuria, we performed renal biopsy, which showed focal and segmental glomerulosclerosis. At discharge, she started therapy with Irbesartan 75 mg/day with significant reduction of proteinuria (140 mg/day). After 6 months of therapy, thepatient did not present proteinuria and her renal function was normal. The molecular genetic study of SMARCAL1 gene revealed that patient was compound heterozygous for two mutations: a novel missense mutation in exon 3, inherited by mother, and a nonsense paternally-derived mutation in exon 17, leading to a truncated SMARCAL1 protein. Our data allow us to diagnose a Schimke immuno-osseous dysplasia (SIOD). In most patients, life expectancy is limited to childhood or early adolescence, due to the onset of stroke, infections,hematopoietic bone marrow failure and renal failure. Only patients with milder and late onset forms can survive until adulthood.

Gain of glycosylation in integrin-alpha-3 causes nephrotic syndrome and lung disease Objective: Congenital nephrotic syndrome and interstitial lung disease is a rare multiorgan disorder, characterized by disrupted basement membrane structures. ITGA3 gene mutations were recently identified as the genetic cause of this disorder, but the disease mechanism remains poorly understood. Methods: We describe a patient who presented with neonatal respiratory distress, glomerulosclerosis, proteinuria, pulmonary hypoplasia and alveolar glycogenosis, who died 7 months after birth due to respiratory insufficiency. A genome-wide screening for deletions and duplications revealed a large homozygous region that included the ITGA3 gene. We sequenced the gene and then conductedin vitro characterization studies to investigate the effect of the variant on the protein function.

Results: A novel homozygous missense mutation was identified in the coding region of the ITGA3 gene, which introduces an N-glycosylation motif to the protein sequence. Thereby, the mutant integrin alpha-3 protein becomes hyperglycosylated. Functional studies demonstrated that the conformation of integrin alpha-3 is affected and the mutant alpha-3 precursor is targeted for degradation. Consistent with these findings, alpha-3 integrin was not detected in the patient glomeruli. Furthermore, integrin alpha-3 protein expression was absent in murine podocytes that lack endogenous integrin alpha-3 and transfected with mutant ITGA3. Conclusion: Our findings underscore the role of the integrin alpha-3beta-1 complex as the main regulator of podocyte basement membrane integrity. Here, we show that hyperglycosylation of the integrin alpha-3 subunit, causing the complete lack of alpha-3-beta-1 expression on the basement membrane, is a new pathogenic mechanism underlying congenital nephrotic syndrome and interstitial lung disease. ITGA3 mutation screening was directly implemented in DNA diagnostics, facilitating early diagnosis, recurrence risk estimation, and genetic counselling.

The Clinical Study on PKHD1 Gene-based Testing for the diagnosis of ARPKD Objective: In order to study the implications of PKHD1 gene-based testing for ARPKD diagnosis in clinical practice, we performed a prospective study to apply PKHD1-based genetic testing to the suspected ARPKD patients. Methods: 12 suspected ARPKD patients from 10 unrelated families were detected on mutations of PKHD1 gene by PCR direct sequencing. These patients were evaluated by combining the testing results and their clinical materials. Results: In the 6 detected children, 4 mutations (P137S, V836A, Q1574H, L2658X) were novel and 7 mutations (T36M, R559W, R760C, N830S, A1262V, Q3899R, Q4048R) were previously described. 4 of the 11 variants were definite pathogenic mutations. Two pathogenic mutations in both chromosomes were found in 2 out of 6 families (30%), which greatly aid to making definite diagnoses. The 4 families with perinatal presentation consisted of 2 fetuses and 2 couples. One definite pathogenic mutation (S3457C) was found in the wife of family No.9, however, it was still difficult in conforming the diagnosis owing to lack of the pathogenic mutation from the husband. For the other families, only some potential mutations were found.Two in four children with congenital hepatic fibrosis were detected two pathogenic mutations in both chromosomes, and their liver functions were obviously lower than the other two children without pathogenic mutations.

Conclusion: PKHD1 gene-based testing is an effective means for the diagnosis of ARPKD, at the same time it could improve the understanding of this disease by analysing the testing results and their clinical materials. Objective: To present a case of Schimke Immuno-Osseous Dysplasia (SIOD) which is the first to be reported from Egypt. Methods and Results: This article presents a case from Egypt with mild form of SIOD presented at the age of 14.5 years with disproportionate short stature, SRNS (focal segmental glomerulosclerosis), laboratory evidence of cellular immune deficiency and radiologic characteristics of spondyloepiphyseal dysplasia and died at the age of 16.5 years with bone marrow failure and severe pneumonia. Conclusion: We emphasize that SIOD is to be considered in children with growth retardation, SRNS and bone abnormalities and that inherited nephrotic syndrome may be presented in late childhood or even adolescence and SIOD suspected patients even before full picture development should be closely monitored for proteinuria, hypertension, cellular immunity and opportunistic infections especially with the need to start immune suppressive therapy for nephrotic syndrome.

Abstract# P-SAT244 Denys-Drash syndrome presenting with polycystic kidneys Objective: Denys-Drash syndrome (DDS) is characterized by a congenital or infantile nephrotic syndrome due to diffuse mesangial sclerosis, male pseudohermaphroditism and a strong predisposition to develop Wilm's tumors and gonadoblastoma's. The syndrome is caused by a dominant mutation in the WT1 gene. We report a case of DDS associated with renal cysts, which has never been described so far.

We report a case of DDS in a 3 months old girl presenting with proteinuria and bilateral cortical cysts on renal ultrasound. Autosomal recessive polycystic kidney disease was excluded by a normal liver biopsy. A kidney biopsy showed diffuse mesangial sclerosis. At the age of 7 months, she developed a unilateral Wilm's tumor, which was treated by heminephrectomy and chemotherapy. The renal cysts were still present on ultrasound. Histological examination of a cyst (located in the resected specimen, but separate from the Wilm's tumor) showed one layer of flattened epithelial cells, without any evidence of malignancy. The combination of diffuse mesangial sclerosis and a Wilm's tumor pointed to the diagnosis of DDS. Genetic analysis showed a de novo heterozygous missense mutation c.1186G>A (p.Asp396Asn) in the WT1 gene, previously described in patients with DDS, confirming the diagnosis. Because polycystic kidneys have never been reported in DDS, we explored several genes responsible for these renal manifestations, such as HNF-1β, PAX2, PKD1 and PKD2. Remarkably, we identified a heterozygous missense variant c.12439A>G (p.Lys4147Glu) in the PKD1 gene. Mutations in PKD1 lead to autosomal dominant polycystic kidney disease (ADPKD). The pathogenicity of the newly identified missense variant in our case was evaluated by different mutation prediction software programs and was classified as being 'likely pathogenic'. The same variant was found in the patient's mother, having no renal cysts on ultrasound and in the grandfather, having bilaterally renal cysts.

Conclusion: This is the first case of DDS in combination with polycystic kidneys. We hypothesize that PKD1 c.12439A>G variant might be an incompletely penetrant allele that can cause a severe phenotype of ADPKD in association with a WT1 gene mutation. The phosphatase and tensine homolog (PTEN) gene is a tumor suppressor gene located on the long arm of chromosome 10 (10q22-23). The PTEN protein is broadly expressed in cells throughout the body, acting as both lipid and protein phosphatases, and regulates intracellular signaling via various pathways. Reduced PTEN protein resulting from a PTEN gene mutation may enhance cell proliferation, resist from apoptosis. In addition, it has became clear that, in vascular endothelial cells, PTEN gene abnormalities lead to increased expressions of vascular growth factors, such as angiotensin II (Ang II), that are essential to angiogenesis. Recent reports revealed a germline mutation of the PTEN gene to cause several syndromes with generalized, multiple hamartomatous lesions of tridermic origin. Thus, the new concept of PTEN hamartoma tumor syndrome (PHTS) has been proposed. To the best of our knowledge, we describe here for the first time a case of focal segmental glomerulosclerosis (FSGS) with PHTS. A 3-year-old girl was found to have proteinuria and hematuria on health examination and referred to us for persistent urinary abnormalities. IgM nephropathy were diagnosed by renal biopsy, and she started taking an angiotensin-converting enzyme inhibitor. However, proteinuria was not reduced, and an oral angiotensin II receptor blocker (ARB) was administered. Proteinuria persisted and she underwent renal biopsy again, yielding a diagnosis of FSGS. Despite continued oral ARB treatment, proteinuria is ongoing with a urine protein-to-creatinine ratio of approximately 0.5, and mild renal impairment. At age 13 years, 2 years ago, a neck mass prompted a detailed examination which revealed multiple thyroid nodules, ovarian cysts (mucinous cystadenoma), hemangiomas in the plantar and femoral regions, spinal cord lipoma, and renal nodular lesions, necessitating follow-up. Subsequently, additional workup detected a PTEN gene heterozygous mutation (exon 8 codon 335 CGA(Arg) TGA(STOP)) and she was diagnosed with PHTS. Methods: A 7-day-old girl, the first child of non-consanguineous Chinese parents, was hospitalized due to edema, gross proteinuria, and progressive renal failure. She was delivered spontaneously at 36 weeks of gestation with a birth weight of 2700gand a body length of48cm. During the delivery, she was experienced mild asphyxia. Placental weight was large, and the amniotic fluid was meconium-stained.

Pregnancy was unremarkable and had included routine prenatal ultrasound evaluation. The family history of proteinuria or renal failure was negative. On admission, the findings from physical examination showed the patient had bilateral microcoria and Limb hypotonia. Results of serological testing for TORCH infections were negative. Since Pierson syndrome was suspected, a kidney biopsy was performed when she was aged 10 days. Meanwhile, all coding exons of LAMB2 were analyzed by using PCR and direct sequencing. Results: Electron microscopy revealed some glomerular basement membranes were thick and thinning, with splitting of the lamina densa. In addition, the foot processes of podocytes were diffusely effaced, and the number of podocytes increased. Two different novel nonsense mutations (Trp16X and Gln748X) of LAMB2 were detected in the patient. These mutations were inherited from her parents respectively. Conclusions: Newborn with unexplained renal insufficiency or nephrotic syndrome should consider Pierson syndrome. Our report extends the genotypic spectrum of Pierson syndrome.

A familial WT1 mutation associated with incomplete Denys-Drash syndrome We report a familial WT1 missense mutation in exon 9 (1180C>T, R394W) in three members of one family. Patient 1, a 2 years old boy, was born at ambiguous genitalia (46, XY karyotype), penoscrotal hypospadias and bilateral inguinal hernias. He was found proteinuria on the preoperative examination and renal biopsy showed diffuse mesangial sclerosis. Patient 2, a 3 years old, is the older sister of Patient 1, who has normal genitalia. She was found proteinuria and renal biopsy showed focal mesangial sclerosis. Wilms's tumor was not found in both of them. A WT1 mutation was detected in both the two patients and their farther. The patients are considered as incomplete DDS and they can inherit mutations from their father. So, this finding will provide new evidence for a better understanding of DDS and further familial studies in patients of DDS are need.

Ying Chen 1 , Songming Huang 1, 2 , Guixia Ding 1, 2 , Hongmei Wu 1 , Aihua Zhang 1, 2 1 Nephrology, Nanjing Children's Hospital, Nanjing, China 2 Institute of Pediatrics, Nanjing Medical University, Nanjing, China

Objective: To investigate the correlations between Peroxisome proliferator-activated receptor (PPAR)-α, PPAR-γ, and PPAR-γ coactivator-1α (PGC-1α) gene polymorphisms and susceptibility to primary nephrotic syndrome (PNS).

Methods: Patient genotypes were determined using the polymerase chain reaction-restriction fragment length polymorphism (PCR-RFLP) technique for the Pro12AlA and Val290Met polymorphisms of the PPAR-γ gene, the Gly482Ser polymorphism of the PGC-1α gene, and the Leu62Val polymorphism of the PPAR-α gene. The gene polymorphisms in 111 cases of PNS in children and 111 normal controls (NC) were analyzed and compared to examine the differences in the clinical metabolism index, proteinuria, renal pathological types, and hormone treatment responses among PNS children with different genotypes.

Results: The PPAR-γ Pro12Ala and PGC-1α Gly482Ser mutations were not associated with the occurrence, blood pressure, total cholesterol, a decrease in glomerular filtration rate (GFR), urine protein excretion at the onset of disease, renal pathological type, or hormone treatment response in children with PNS. Although the Homa-IR values for children with the A allele did not differ significantly from those of children with the PP genotype of the PPAR-γ gene, their insulin levels were decreased and their ISI values were significantly increased (P=0.012 and 0.006, respectively). The triglycerides (TG) levels of children with the AA genotype of the PGC-1α gene were significantly increased (P=0.026). In addition, the PPAR-γ (Val290Met) and PPAR-α (Leu162Val) gene polymorphisms did not show any mutations in the 111 cases of PNS in children or the 111 NC children.

Conclusion: The Pro12Ala mutation of the PPAR-γ gene may be correlated with a decrease in insulin secretion and an increase in insulin sensitivity and the PGC-1α (Gly482Ser) gene polymorphism may be a causative genetic factor for the triglyceride abnormalities in children with PNS.

The relationship between endothelin-1 gene polymorphisms and primary nephrotic syndrome in children Objective: NPHS1 mutations have been reported in CNS. However, no hot mutation has been described in Chinese family. In this study, NPHS1 mutations were analyzed in a Chinese family with two siblings died of CNS. Methods: Genomic DNA samples were extracted from peripheral blood of the proband, her parents, and 150 unrelated normal individuals. All 29 exons of NPHS1 were detected by polymerase chain reaction(PCR) and direct DNA sequencing.

Results: The proband, a 15-day-old girl, weighed 3500g at birth. The placenta was one time heavier than usual. She was hospitalized with edema of legs, heavy proteinuria(+++), and hematuresis(+++). Two compound heterozygous mutations were identified in exon 15 (c.2020C>T, p.P674S), which was detected in her father, and in exon 16(c.2207T>C, p.V736A), which was also detected in her mother. These two new mutations were not found in the 150 Chinese controls. Conclusion: New compound heterozygous NPHS1 mutations (c.2020C>T and c.2207T>C) were identified in a Chinese family with two siblings of congenital nephrotic syndrome, which were suggested to be the causative mutations in this family. The compound heterozygous mutations (c.2020C>T and c.2207T>C) lie between Ig6 and Ig7 domain which has a free cysteine residues. These mutations might cause misfolding and defective intracellular transport, with consequent absence of the mutant nephrin on the plasma membrane.

Abstract# P-SAT255 Sporadic MYH9-related disease: a case report and mutational analysis of MYH9 gene .Genetic analysis confirmed that these three cases had intron 9 (+KTS) mutation.After confirmation of FS, Cytogenetic analysis showed XY in girls though phenotypically there were females.Abdominal ultrasound in them showed normal uterus with streak gonads and gonadectomy was done for both and histologically reported as Dysgerminoma stage I. Familial girl aged 12 entered ESRD and received live related renal transplant from her mother. The sporadic girl aged 9 years also entered end stage renal disease rapidly and died. The third was a nephrotic boy aged 9 years with FS. He had bilateral hypoplastic testes. His chromosome was XY. Renal biopsy showed MCD with IgM deposits and partially responding to therapy. 

A six year old girl presented at the emergency department with a history of 3 episodes of vomiting in 24 hours. She had a history of polyuria and polydipsia with a current daily intake of 3 liters. Physical examination revealed signs of mild dehydration. Plasma electrolytes revealed hyponatremia (128 mmol/l), hypokaliemia (1.6 mmol/l), metabolic acidosis with normal anion gap (14 mmol/l), hypophosphatemia (0.86 mmol/l), hypouricemia (90 umol/l) and normal serum creatinine (29 umol/l). Urinalysis confirmed the Na, K, Ph and acid uric losses: Na (67 mmol/l, fractional excretion 2%), K (83 mmol/l, TTKG 30), tubular phosphate reabsorption of 18%, and fractional excretion of uric acid of 30%. Cystine level in leucocytes was normal. She was discharged six days later with oral supplementation of potassium and phosphorus. Laboratory studies 3 weeks later revealed surprising findings with persistent hypokaliemia (3 mmol/l), metabolic alcalosis (HCO3 30 mmol/l), hypomagnesemia (0.65 mmol/l), and hypocalciuria (calcium/creatinine 0.06 mmol/mmol). We therefore sequenced the SLC12A3 gene and we found that she is compound heterozygous for 2 known missense mutations (p.Thr304Met and p.Gly439Ser).

Conclusion: GS can have atypical presentations including severe polyuria; it should be suspected in case of severe hypokaliemia even in the absence of the other classical features. This case report emphasizes the need for repeated laboratory tests in unclear tubular disorders, especially outside of confusing intercurrent illness.

A single basepair mutation causes cystinosis in the majority of Western Cape patients. Conclusion: RenalTube is being used by pediatric nephrologists over the world for the study of their patients with primary tubular disorders. RenalTube will likely contribute to a better care of these children and to a better scientific understanding of primary tubular diseases. The creation and development of this sort of international collaborative efforts must be encouraged within the Pediatric Nephrology community.

Objective: To study molecular mechanism of hypercalcemia-induced nephrogenic diabetes insipidus (NDI) by identification of proteins in inner medullary collecting duct (IMCD) responsible for this syndrome.

Methods: The effect on rat kidney medullary collecting duct of early onset NDI resulting from parathyroid hormone-induced hypercalcemia was studied using proteomics and phosphoproteomics in native inner medullary collecting duct (IMCD) cells. IMCD tryptic peptides and phosphopeptides were identified and quantified by mass spectrometry using a label-free methodology. The major findings were confirmed by selected reaction monitoring study, immunoblot, and immunohistochemistry.

Results: A total of 5,866 peptides corresponding to 1,107 proteins and 1,388 phosphopeptides of 580 proteins were identified, with significant changes in abundance of 69 proteins and 49 phosphopeptides in early onset NDI versus vehicle controls. Gene Ontology terms and pathway analysis revealed that hypercalcemiaaffected proteins and phosphoproteins are associated with integrin signaling, and actin cytoskeleton organization. Immunoblot and selected reaction monitoring LC-MS/MS studies confirmed the hypercalcemia-regulated proteins (Agrin, Arcp1b, Capg, ERM, Itgb1, Lamb2, and Lamc1) and phosphoproteins (Myh9, Add1, Dync1li1, Cgnl1, Aqp2). Hypercalcemia induced changes in abundance of vasopressin-regulated phosphorylation of aquaporin-2 (Aqp2) at Ser256 and Ser261, but not total Aqp2, and decreased abundances of Slc14a2 urea transporters UT-A1, UT-A3, and phospho-Ser486-UT-A1. The major findings were also identified in IMCD of rats with vitamin D-induced hypercalcemia. Filamentous actin aggregation was firstly demonstrated in IMCD of rats with hypercalcemia-induced NDI from both PTH and vitamin D.

Conclusions: Early increasing in water excretion in response to hypercalcemia are dependent of changes in abundances of Aqp2 phosphorylations, UT-A and its phosphorylations are consistent with prior literature pointing to key roles of the integrin signaling and the actin cytoskeleton in maintenance of collecting duct function. Objective: To study molecular mechanism of hypokalemia-induced nephrogenic diabetes insipidus (NDI) by identification of proteins in inner medullary collecting duct (IMCD) responsible for this syndrome.

Methods: The effect on rat kidney medullary collecting duct of early onset NDI resulting from hypokalemia was studied using proteomics in native inner medullary collecting duct (IMCD) cells. IMCD tryptic peptides were identified and quantified by mass spectrometry using a label-free methodology. The major findings were confirmed by selected reaction monitoring study, immunoblot and immunohistochemistry.

Results: A total of 2,477 peptides corresponding to 821 proteins were identified with significant changes in abundance of 189 proteins in early onset NDI versus vehicle controls.Gene Ontology terms and pathway analysis revealed that hypokalemia-affected proteins are associated with generation of precursor metabolite and energy and regulation of actin cytoskeleton. Immunoblot and selected reaction Objective: Inherited distal RTA (dRTA) is a rare condition and is almost always observed in children as a primary entity. Mutations in genes encoding transporter or channel proteins operating along the renal tubule may result in a variety of functional defects. The identification of the molecular defects in dRTA may provide a basis for future design of targeted therapeutic interventions and, possibly, strategies for gene therapy of these complex disorders. For this reason, we evaluated mutations in SLC4A1 and ATP6V1B1 genes. Methods Genomic DNA from 20 pediatric patients diagnosed with dRTA was extracted from peripheral blood samples. Sequencing was performed for five exons of SLC4A1 (14, 15, 17, 19, 20) and two exons of ATP6V1B1 (1, 7). All the selected exons are considered hotspots for mutations associated to this tubular disorder.

Results Silent mutations were identified in both studied genes. The mutation c.2688T>C was identified in exon 20 of SLC4A1 in one patient and the second variation (c.27T>C) was found in exon 1 of ATP6V1B1 in two patients. Although previously described, these silent variations are not highly frequent in the overall population. Also in SLC4A1, exon 19, two nonsynonymous variations were identified in the same patient (p.Pro854Leu and p.Val862Ile). The p.Val862Ile variation was also found in three other patients. It has been shown that these variations are rare polymorphisms in the population, but they have already been associated to Diego group erythrocyte antigen. In exon 1 of ATP6V1B1 the variation p.Met2? was presented in twelve patients and the mutation p.Thr30Ile was found in six patients. Although they occur in the coding region of ATP6V1B1, these variations have been described as highly frequent in the population. Dent's disease is an X -linked renal proximal tubulopathy associated with mutations in CLCN5 (Dent's type 1) and OCRL1 (Dent's type 2).OCRL1 mutations also cause the oculocerebrorenal syndrome of Lowe. We report the case of a 5 year old boy with Dent's disease associated with a novel hemizygous change, c.2T>C p.(Met1) in exon 1 of OCRL. Our patient presented at 3 years of age with incidental finding of non-nephrotic range proteinuria. He had elevated urinary beta -2 microglobulin, retinol binding protein and hypercalciuria. He was developmentally normal and is otherwise of good general health. Slit lamp examination and vision were normal. He had normal renal function but had very poor renal accumulation of 99mTc-DMSA on renoscintigraphy. Genetic testing identified a hemizygous change, c.2T>C p.(Met1), in exon 1 of OCRL, which affects the translation initiation codon. His healthy 8 year old brother who was also found to have low -molecular weight proteinuria and hypercalciuria, is currently being investigated. To the best of our knowledge, the hemizygous change, c.2T>C p.(Met1?) in exon 1 of the OCRL gene which was identified in our patient with Dent's disease represents a novel variant which has not been previously reported in a Dent's disease patient.

Abstract# P-SAT274 Enamel-renal syndrome associated to splenic and ovarian calcifications: a case report Sameh Mabrouk, Noura Zouari, Houda Ajmi, Jalel Chemli, Menair Tfifha, Saida Hassayoun, Saoussen Abroug , Abdelaziz Harbi Pediatrics, university hospital of Sahloul, Sousse, Tunisia Enamel-renal syndrome (OMIM204690) is a rare condition characterized by amelogenosis imperfecta and nephrocalcinosis.We report a new pediatric case of this rare association with the particularity of associated splenic and ovarian calcifications. This report concerns a 13-year-old girl, born to consanguineous parents (first degree cousins). She has no family history of nephrocalcinosis or kidneydisease. Her unique brother, aged 8 years, is in good health and has no dental or renal problems. She was first seen at the age of 12 years in dentistrydepartment because of dental abnormalities, the diagnosis of amelogenosis imperfecta was made and the patient was further investigated. A renal ultrasound showed a bilateral medullary nephrocalcinosis. CT confirmed bilateral nephrocalcinosis which was associated to ovarian and splenic calcifications. Besides this young girl has a normal growth, her puberty began at the age of 12 years. Laboratory findings including serum electrolytes, urea, creatinine, calcium, phosphate, parathormone and alkaline phosphatase were within normal ranges. On urine examination we found a hypocalciuria, with normal sodium and phosphate excretion. The patient is regularly seen in both dentistry department and pediatrics. The aim of this report is to highlight the important role of pediatricians as well as dentists in recognizing this rare and uncommon syndrome. Objective: IPEX syndrome, a hereditary (X-linked) immune dysregulation with autoimmune polyendocrinopathy and enteropathy, as the basic manifestations, presents a rare and severe disease. The objective of this case report is to highlight the pleomorphism of the syndrome.

Methods: The authors report the case of a male infant, with a family history of three male siblings affected by IPEX syndrome. The patients' medical records were reviewed in order to describe the case of the youngest one.

Results: During the follow-up of the youngest of three siblings, who presented eczema and intestinal manifestation, without compromised pancreatic and thyroid function, different from other two siblings, it was noticed the pattern of Dent's disease. We registered hypophosphatemia, hypercalciuria, glycosuria, low molecular weight proteinuria and ultrasound revealed second stage bilateral nephrocalcinosis. In this child there was no apparent glomerular involvement, as it was seen in the eldest sibling. Conclusion: Dent's disease is an X-linked renal proximal tubulopathy associated with mutations in the chloride channel gene CLCN5 (Xp11.22), which is next to FOXP3 gene on the X chromosome (Xp11.23-q13.3). It seems that in this sibling mutations occurred in exons of both of these genes. This case is to remind on pleomorphic potential of mutations that occur near the coding regions of the FOXP3 gene. Objective: multiple target organ defects (MTOD) is a subgroup of pseudohypoaldosteronism (PHA) type I and salt wasting is more severe in this form of PHA I. It has a poorer outcome than the renal form, and therapy must be maintained throughout life. Here, we reported an infant with MTOD who had severe hyperkalemia resistant to classical treatment of PHA. Hyperkalemia was normalized only by peritoneal dialysis (PD). Case: A 4 month-old boy was admitted to our hospital with persistent vomiting, metabolic acidosis, and severe hyperkalemia. There was consanguinity between parents and he had a healthy sister and brother. There was no history of medication. Physical examination revealed the signs of volume depletion and acidosis. Results: In laboratory examination, blood urea nitrogen was 71 mg/dl, creatinine 0.43 mg/dl, sodium 114mEq/L, potassium 9.5mEq/L, and chloride 97mEq/L. Blood pH was 7.33, base excess -12mEq/L, and HCO3 11mEq/L. Urinary analysis revealed low specific gravity (1003) with pH 8.0, and normal urinary sediment. Urinary sodium was 68mEq/L, potassium 1mEq/L, and plasma renin activity 80.2ng/ml (2.7-37.0), aldosterone 2800pg/ml (50-900), cortisol 11.1 mg/dl (7-17.5), and ACTH 5.5pg/ml (10-469). Renal ultrasonography did not show any abnormalities. Sweet-test showed high sodium waste from sweet glands (144mEq/L). The patient diagnosed as MTOD. Severe hyperkalemia persisted and was resistant to all treatment options for PHA, including high-sodium and low-potassium diet, fluid resuscitation, potassiumbinding resins, indomethacin, fludrocortisone, and hydrochlorothiazide. Nutritional status of the patient was also not good, and parents did not accept gastrostomy. Hyperkalemia was corrected only after PD treatment, and his nutritional status improved dramatically.

Conclusion: In the case of type IV renal tubular acidosis resistant to therapy, MTOD could be considered as a cause of acidosis. If the classical treatment options fail to correct hyperkalemia in these patients, PD may be a reasonable choice to normalize severe hyperkalemia.

Gülsün Gülay Yılmaz 1 Erta Yilmaz 1, 2 1 Pediatric Nephrology, MD, Antalya, Turkey 2 Akdeniz Univercity, Prof. Dr, Antalya, Turkey Tuberous sclerosis, which has an autosomal dominant hereditary disease with a high rate of spotaneous mutation, especially occupies the nervous system and many other organs like kidney, heart and lung. The case presented here was initially diagnosed as congenital heart and polycycystic kidney diasease, because of its characteristics which were quite different than expected for tuberous sclerosis. Finally, it was evaluated at our pediatric department together with its dermatological and radiological aspects and defined as a tuberous sclerosis of delateddiagnosis. The case was discussed with its former and actual findings and presented as a warning for early diagnosis.

Abstract# P-SAT285

The effect of bone marrow stem cells mobilization on expression of HIF-1α and EGF in ischemia/reperfusion-induced renal injury Objective: To investigate the therapeutic effects of bone marrow stem cells which has been mobilized by G-CSF and stem SCF on expression of acute tubular necrosis hypoxia inducible factor-1a (HIF-1α) and epidermal growth factor (EGF), and to investigate the mechanism of SCF and G-CSF on the treatment of ischemia/reperfusion-induced renal injury. Methods: 160 male Sprague-Dawley rats that are 8-10 weeks old were randomly allocated into 4 groups (n =40 ineach group): control group (group A), ischemic-reperfusion group (group B), SCF + G-CSF + ischemic-reperfusion group (group C) and SCF + G-CSF + control group (group D). Detection index: HIF-1α was measured by immunohistochemistry technique, the expression of EGF and CD34 + cells in kidney was measured by strept avidin-biotin-peroxidase(SABC) and EGF mRNA by RT-PCR .

Results: (1) At 5 days postoperative, the CD34 + cells of group B and C was conspicuous higher than group A and D(P<0.05), group C was higher than group B(P<0.05). They gradually descended from 5 days with the time prolonged.

(2)At 5 days postoperative, there was significant difference of the expression of HIF-1α between group A and D (P<0.05). The expression of HIF-1α of group B and C showed higher positive reaction at 5 days postoperative then decent to normal. At each time the expression of HIF-1α of group C was significant higher than other groups (P<0.05).

(3)The EGF expression of group B and C showed higher positive reaction at 5 days postoperative and decent to normal with the time prolonged, group B peaks at 17 days and higher than group A, group C peaks at 10 days, and it is the highest (P<0.05 Results: In contrast to model group, ATN treated with ADSCs displayed: cells expressing green fluorescent protein were dectected in injured tubule in kidney . treatment group were significantly higher than model group, P<0.05; Renal damage is lighter; kidney damages were relative gently and histopathologic lesion scores was relative lower; while Ki-67 positivece cells in treatment group were more than those in model group (P<0.05); The expression of Bax, Bcl-2 and cell apoptotic index(AI) in treatment group was lower than those in model group , P<0.05, but Bax/Bcl-2 ratio was higher than those in model group.

Conclusion: Allogenic ADSCs transplantation can accelerate proliferation of renal tubular epithelial cell and suppress apeptosis injury through up-regulating the ratio of Bcl-2/ Bax and down-regulating the expression of Bax protein in acute kidney injury.

Objective: Previously we showed that pretreatment with the antidepressant fluvoxamine (FLU) improves postischemic survival and ameliorates functional and structural kidney damage after renal ischemia/reperfusion (IR). In heart IR injury FLU is protective through activating the Sigma-1 receptor (S1R) -nitric-oxide synthase (NOS) system. Here we studied the intrarenal vasoregulatory effect of FLU and analyzed the renal S1R-NOS cascade. Methods: Male Wistar rats were were treated i.p. either with (1) vehiculum (VEH); (2) FLU (20 mg/bwkg; FLU); (3) FLU+ S1R antagonist NE-100 (1mg/bwkg; FN); (4) FLU+ non-selective NOS blocker L-NAME (10mg/bwkg); (5) FLU + selective endothelial (e) NOS blocker L-NIO (20mg/bwkg); (6) FLU and selective neuronal (n) NOS blocker 7-NI (25mg/bwkg). 30 minutes later rats were either harvested or subjected to 50 minutes of ischemia followed by 24 hours of reperfusion. Sham-operated animals served as controls (n=10/group). Renal S1R, Akt, eNOS and nNOS protein levels were measured, the alteration of renal capillary diameters was determined in vivo using muliphoton microscopy. Results: IR induced renal vasoconstriction, which was ameliorated by FLU. This increase was neutralized by all NOS blockers, mostly by 7-NI. After IR all measured protein levels increased. S1R expression was similar in all treatment groups. Akt and eNOS levels were lower, while nNOS levels were higher in the FLU treated group compared to VEH and FN. The acute vasodilatative effect of FLU 30 minutes after treatment was suspended by L-NAME and 7-NI and even reversed by L-NIO. S1R, Akt and eNOS protein levels were elevated 30min after FLU treatment, while nNOS levels remained unchanged. Discussion: Pretreatment with the S1R agonist FLU -used chronically without notable side-effects -improves postischemic renal perfusion through the activation of S1R -NOS system in a time and NOS isoform specific manner. Based on this data one can hope to find a new therapeutic target in the treatment of renal IR damage through the modulation of the S1R. Methods: 36 SD rats were randomly divided into model group, intervention group and normal group, 12 rats in each group. Intraperitoneal injection with gentamicin for 7 days to establish renal tubular injury rat model. Then the intervention group rats were transplanted with 4×10 6 /ml BM-MSCs via tail vein. 7 days after transplanted with BM-MSCs, took blood and kidney specimen of rats for testing. Serum creatinine (SCr), urea (BUN), Malondialdehyde (MDA), Superoxide dismutase (SOD), Glutathione peroxidase (Gpx), Heme oxygenase -1 (HO-1) were detected by spectrophotometry. HE staining was used to evaluate the change of renal tissue pathology, then score the renal tubular injury according to the Nangaku semiquantitative scoring method. TUNEL method was used to detect the apoptosis of epithelial cell rate in renal tubular, while immunohistochemistry was used to detect the proliferation of renal tubular epithelial cells (PCNA labeling index). Results: 1. SCr and BUN of intervention group rats were lower than those in the model group, the difference was statistically significant (P <0.01); 2. Renal tubule pathological score of intervention group rats were lower than those in the model group, the difference was statistically significant (P< 0.01); 3. Apoptosis ratio of renal tubular epithelial cell in intervention group rats were lower than the model group, the difference was statistically significant (P<0.01); 4. The PCNA labeling index of intervention group rats were higher than those in the model group, the difference was significant (P<0.05); 5. The MDA level was significantly reduced in intervention group rats than in the model group (P < 0.01); 6. The SOD, Gpx and HO-1 inintervention group rats were significantly increased than those in the model group (P < 0.01).

Conclusion: Antioxidant factors such as SOD, Gpx and HO-1 were significant increased after BM-MSCs transplanted into rats. It means that BM-MSCs may have antioxidant effect to heal the renal tubular damage.

The effect on regeneration and repair in renal tubular epithelial cell after injury deal with AT2R antagonist PD123319

Objective: To study the effect of proliferation of renal tubular epithelial cell after injury deal with AT2R antagonist PD123319,And to explore the function and mechanism of AT2R in the regeneration and repair after acute kidney injury. Methods: To establish the human renal proximal tubule cells (HK-2 cells) hypoxia /reoxygenation model. HK-2 cells were divided into two groups. (1) (1) These results suggest that the renal proximal tubule epithelial cells after hypoxia/reoxygenation can be simulated tubular epithelial cell process damage; (2) The proliferation of renal tubular epithelial cell after injury was inhibited by PD123319.AT2R may play an important role in the regeneration and repair in the kidney by means of promoting the proliferation of renal tubular epithelial cells.

(3) ARB have no significantly inhibit the proliferation of renal tubular epithelial cell after injury, suggesting that AT1R may not have major role in regeneration and repair after acute kidney injury.

The effect and mechanism on regeneration and repair in renal tubular epithelial cell after injury by the inhibitory proliferation effect of ACEI and ARB

Objective: To study the effect of regeneration and repair of renal tubular epithelial cell after injury deal with fosinopril and valsartan, And to explore the function and mechanism of ACEI and ARB induced acute kidney injury by suppressing proliferation. Objective: Ischemia-reperfusion injury (IRI) is a major cause of acute kidney injury (AKI) and both innate and adaptive immunity contribute to the pathogenesis. T cell immunoglobulin-3 (Tim-3) has been reported as an important regulatory molecule and plays a pivotal role in several inflammatory diseases. However, it keeps unknown whether Tim-3 is involved in renal IRI. To investigate the expression of Tim-3 on kidney mononuclear cells (KMNCs) from mice with renal IRI and explored its role in the pathogenesis of renal IRI. Methods: The left renal pedicle was clamped in C57BL6 mice for 45 min, followed by reperfusion. Animals were sacrificed at baseline, day 1,3. Flow cytometry was used to quantify Tim-3 expression on KMNCs ,CD4 + T cell,CD8 + T cell,Foxp3 + Tregs and CD19 + B cell. The levels of TNF-α, IFN-γ, IL-4 and IL-10 in kidney tissue were measured using ELISA. Results: At day 1, the increased expression of Tim-3 on KMNCs ,CD4 + T cell,CD8 + T cell,Foxp3 + Tregs in the injured kidney from mice with renal IRI compared to those from uninjured kidney tissues and baseline (P<0. 05). Percentage of Tim-3 + cells in KMNCs showed an inverse correlation with kidney injury score and kidney TNF-α level. Similar negative correlations were found between kidney injury score and Tim-3 levels on CD4 + T, CD8 + T cells. Consistently, Tim-3 expression on CD3 + T cells was further increased in mice at day 3. Tim-3 expression on Foxp3 + Tregs negatively correlates with kindey TNF-α. Conclusion: Tim-3 might participate in the proceeding of renal IRI by regulation on various CD4 + T cell subsets. Tim-3 might be a potential new marker for assessing severity of renal IRI.

Expression of Galectin-9 and Tim-3 in kidney of mice with renal ischemia reperfusion injury Objective: T cell immunoglobulin-3 (Tim-3) is a surface molecule expressed on various immune cells which plays a central role in immune regulation. Identification of galectin-9 (Gal-9) as a ligand for Tim-3 has established that the Tim-3/Gal-9 pathway has been linked to several inflammatory diseases by regulating adaptive and innate immunity. To study the expression of Gal-9 and Tim-3 in kidney of mice with renal ischemia reperfusion injury (IRI). Methods: Thirty C57BL6 male mice were randomized into renal IRI groups with and without recombinant Gal-9. The left renal pedicle was clamped in C57BL6 mice for 45 min, followed by reperfusion. Animals were sacrificed at baseline, day 1,3,10, 21 after IRI. Gal-9 and Tim-3 mRNA levels in kidney tissues were determined using real-time RT-PCR. Expression of Gal-9 in kidney were detected by immunohistochemistry staining .The levels of TNF-α, IFN-γ,IL-4 and IL-10 in kidney tissue were measured using ELISA.

Results: The expression of Gal-9 and Tim-3 mRNA in the injured kidney tissues increased significantly compared with uninjured kidney tissues and baseline (P<0. 05). Compared with the uninjured control and baseline control, the expression of TNF-α and IFN-γ increased significantly in the injured kidney. The expression of Gal-9 and Tim-3 mRNA was positively correlated with renal IL-4 and IL-10 level (r=0.792, r=0.79 respectively; P<0. 05), but negatively correlated with kidney TNF-α and IFN-γ level (r=-0.69,r=-0.75 respectively,P<0.05). After recombinant Gal-9 treatment for three days, the kidney injury ameliorated and inflammatory cytokines (TNF-α and IFN-γ) decreased.

The expression of Gal-9 and Tim-3 in kidney tissues increase in mice with kidney IRI. Tim-3/Gal-9 pathway are closely related to inflammatory process in renal IRI. Objective: We previously found that RGC-32(Response Gene to Complement-32), a key factor in regulating cell cycle, plays an important role in dealing with epithelial-mesenchymal transition (EMT). This study aimed to evaluate the effects of RGC-32 regulating cell cycle in renal tubular epithelial cells injury and repair. Methods: (1) Objective: Autophagy is a lysosomal degradation pathway that is essential for cellular stress adaptation and normal homeostasis. Increased level of autophagy has been reported in the post-ischemic kidneys by static analysis. This study aimed to understand the dynamics of epithelial autophagy in kidneys following acute ischemic injury and during renal repair. Methods: Taking the advantage of differential pH sensitivity of RFP (pKa 4.5) and EGFP (pKa 5.9) fluorescence, we generated a new strain of CAG-RFP-EGFP-LC3 mice to distinguish early autophagic vacuoles from autolysosomes and to monitor autophagic process in the kidneys with ischemia-reperfusion injury (IRI). Results: Renal epithelial cells responded to nutrient deprivation with easily detectable fluorescent puncta that represented autophagic vacuoles and corresponded to LC3-II and Atg5 protein levels. The majority of the EGFP lost its fluorescence in the acidic environment of the autolysosomes where bright RFP signals remained. In normal kidneys, few EGFP and RFP puncta were present in the nephron. IRI led to dynamic changes in autophagic process in the proximal tubules with the number of EGFP puncta reaching the peak at 1 day and returning to the control level at 3 days whereas RFP puncta persisted at a high level through 3 days post injury, indicating autophagy initiation at 1 day but autophagosome clearance at 3 days as kidneys were recovering. Since RFP puncta persisted in cells with recent autophagy, we examined Ki67 expression and found significantly lower proliferation in cells that contained RFP puncta, suggesting that autophagic cells were less likely to divide for tubular repair. Furthermore, 87% proximal tubular cells with mTOR activation indicated by p-S6 kinase expression contained no RFP puncta. Inhibition of mTORC1 activity with rapamycin caused a 2-fold decrease in cell proliferation. Conclusion: Our results highlight the dynamic regulation of autophagy in post-ischemic kidneys and suggest a role of mTOR in autophagy resolution during renal repair.

Abstract# P-SAT297 Novel mechanisms by which Heparin can regulate the vascular activity of Angiotensin II (Ang-II) and Fibroblast Growth Factor-2 (FGF-2) and affect the outcome of acute kidney injury (AKI) Objective: Critically ill children treated with extracorporeal membrane oxygenation (ECMO) and/or cardiopulmonary bypass (CPB) frequently develop hypertension, endothelial dysfunction, and acute kidney injury (AKI). These patients show high serum levels of Ang-II and FGF-2, and are treated with heparin to prevent clotting disorders. However, the mechanisms by which heparin may affect the vascular activity of Ang-II and FGF-2 are not clearly understood. We carried out this study to determine whether heparin can modulate the vascular activity of Ang-II and FGF-2 by affecting the Rho-A, Rac-1, Src, and PKC signaling pathways. Methods: Normal FVB/N mice were injected with adenoviral vectors carrying a secreted form of human FGF-2 or Lac Z vectors, and treated with heparin (5,000 U/kg) or control buffer (n=5 per group). Vascular contractility was studied in pressurized isolated resistance-sized mouse mesenteric arteries, in the presence and absence of Ang-II and FGF-2. The activation of the Src, Rho-A, Rac-1, and PKC signaling pathways were assessed by pull-down assays and Western blots, both in vivo and in vitro. Permeability changes were explored in cultured human renal glomerular endothelial cells (HRGEc) using FITC-dextran. Results: Heparin significantly enhanced the FGF-2-induced activation of Rho-A and Rac-1 in the kidney and isolated mouse mesenteric vessels. In addition, heparin antagonized the Ang II-induced contractility of isolated mouse mesenteric vessels through Rho-A and PKC-dependent pathways. These changes were reverted by FGF-2, and abolished by the Rho kinase inhibitor Y27632. Both heparin and FGF-2, acting in a synergistic manner, increased the permeability of HREC by activating the Rho-A and Src signaling pathways. Conclusion: We conclude that heparin and FGF-2, acting in a synergistic manner, can modulate the activity of Ang-II in the kidney, isolated resistance vessels, and cultured HRGEc. These findings clearly identify novel mechanisms by which heparin, acting alone or in combination with FGF-2, may affect the control of blood pressure, renal perfussion, and capillary permeability in critically ill children treated with ECMO and CPB.

Abstract# P-SAT298 Cisplatinum (Cis) toxicity in immortalized human kidney (HK-2) proximal tubular epithelial cells is independent of DNA strand breaks and has implications for biology and therapy of renal cell carcinoma (RCC) Results: Viability of HuH-7 and HK-2 cells decreased similarly after IC50 doses of Cis with morphology and MTT assays. Many DNA damage genes, including ATM-related genes, were expressed less at mRNA and protein levels in both cell types. However, p53 expression decreased in HK-2 cells. Comet assays showed extensive DNA strand breaks in HuH-7 but not in HK-2 cells. ATM promoter activity increased in only HuH-7 cells. Also, S and G2/M populations were depleted by FACS in HuH-7 but not HK-2 cells. Conclusion: HK-2 cells transformed by HPV E6/7 oncogenes displayed independence from Cis-induced DNA breaks and ATMmediated cell cycle arrest. Such independence from genotoxicity will help explain mechanisms imparting resistance to chemo-or radiotherapy and invasiveness in RCC.

Abstract# P-SAT299 Renal neutrophil gelatinase-associated lipocalin and kidney injury molecule-1 expression in children with acute kidney injury on Henoch-Schonlein purpura nephritis Yue Du, Jinjie Guo, Ling Hou, Yubin wu, Tingting Sun Paediatrics, Post Graduate Trainee, China

Objective: To evaluate serum, urinary and renal neutrophil gelatinaseassociated lipocalin(NGAL) and kidney injury molecule-1(KIM-1) in children with AKI on HSPN by pRIFLE or HSPN with nephroticrange proteinuria.

Methods: We performed a prospective single-center evaluation of serum, urinary and renal NGAL and KIM-1 in a cohort of children. We recorded any relevant data including age, gender, weight, hemoglobin(Hb), serum creatinine(SCr), Cystatine C(CysC), serum beta2-MG, Albumine, urine beta2-MG, urine protein in all patients.

Each patient's estimated creatinine clearance was calculated using the original Schwartz formula. Blood sample and Five ml of urine sample from each participating patient were collected for NGAL and KIM-1 using an enzyme-linked immunosorbent assay. Analysis of proteins NGAL and KIM-1 using SABC immunohistochemical assay. Results: Twenty five patients were enrolled in the study. Nine patients with AKI-on-HSPN(A-on-C) and six were done renal biopsy, Sixteen patients with HSPN with nephrotic-range proteinuria and ten were done renal biopsy. Blood cystatinC, beta2-MG , SCr, NGAL and KIM-1 were increased significantly in patients with A-on-C than those with HSPN, and so do urine NGAL, KIM-1 and beta2-MG. There was no significant difference of proteinuria between the patient with A-on-C and the patients with HSPN. Immunohistochemical results showed that NGAL and KIM-1 were expressed in proximal tubule and their expression were significantly higher in A-on-C group than in HSPN group. The correlation analysis showed that urine NGAL and KIM-1 were negative correlated with GFR and they were uncorrelated with proteinuria.

Conclusion: Blood, urine and renal NGAL and KIM-1 were significantly increased in patients with A-on-C than those with HSPN, and they were negative correlated with GFR. We may conclude that NGAL and KIM-1 may diagnose A-on-C patients more sensitive than SCr.

Abstract# P-SAT300 Objective: Neonatal period is an important stage in the development of renal function at the children. The frequency of renal damage in infants undergoing resuscitation is high, but non-specific clinical symptoms and low information existing survey methods impede their timely diagnosis. Purpose -evaluation of diagnostic importance of determining the level of carbonic anhydrase IX (CA9) in the urine of newborns in critical states. Methods: We evaluated Human carbonic anhydrase IX in the urine of 40 newborns who had asphyxia at birth and receiving treatment in the intensive care unit -the main group. The control group consisted from healthy newborns (13 people). For the study used a sample of newborn urine collected at 1-2 days of age. Carbonic anhydrase was determined using enzyme immunoassay kit. Results: Human carbonic anhydrase IX (CA9) -trans membrane protein, the main physiological function of which is to regulate the pH by the reversible hydration of carbon dioxide. The main pathogenic factor causing kidney damage of fetus is chronic fetal hypoxia. There is a single study HIF-I alpha (hypoxia inducible factor-I alpha) in the blood, increased expression of which occurs during hypoxia. This is connected with damage of tubules and interstitial cells of the kidneys, their proliferation, synthesis of cytokines and extracellular matrix. In this case, activity of CA9 is reduced in the blood. Normally, CA9 is missing in the urine, so its definition can be a marker of early renal dysfunction. The authentic increase of CA9 level in the main group was revealed in comparison with the control group ( and β2-MG, 59,723/ 32,616/ 1511/ 996/ 1084/ 600 μg/g Cr. Levels of each marker were very high in NB and 1M children, and subsequently decreased gradually. Urinary α1-MG levels reduced the quickest, and became the same as at >=3 years old by 6 months after birth. Conclusion: Renal tubular function can be evaluated in children <3 years old using these normal values. The most stable and useful marker from early infancy seems to be urinary α1-MG.

Urinary biomarkers for gentamicin-induced acute kidney injury in the neonatal intensive care unit Objective: Gentamicin (GM) is an aminoglycoside frequently used in the neonatal intensive care unit (NICU). GM is nephrotoxic and may cause acute kidney injury (AKI). Serum creatinine (sCr) appears to be an insensitive and unreliable marker for detecting AKI. To determine whether urine biomarkers are useful for early detection of GM-induced AKI in neonates in the NICU. Methods: Prospective, clinical, observational study. Forty-six neonates (32M/14F) without pre-existent kidney disease were divided in a GM group (n=26) and a reference group (n=20). Demographics, vital signs and clinical conditions were recorded. Only neonates with a bladder catheter in place were included. Urine samples were collected every two hours. Biomarkers (GSTA1-1, GSTP1-1, KIM-1, NAG, NGAL) were determined. Residual blood samples were used to measure sCr. Results: The GM and reference group were comparable for gestational age, weight and mortality. Neonates treated with GM are admitted longer than neonates in the reference group. Treatment with GM resulted in higher sCr compared to the reference group (58. 5 [44.8-58.5] vs. 34.0 [28.3-58.8]; p<0.05). Higher levels of sCr correspond with higher urinary excretion of all biomarkers, especially in neonates treated with GM. The average time until the highest peak was shorter for all biomarkers compared with sCr (p<0.05). There was no difference in produced urine volume between the GM and the reference group. Conclusion: Higher sCr levels correspond with higher urinary excretion of all biomarkers, especially in neonates treated with GM. GSTP1-1 seems the most useful marker for early detection of AKI in neonates.

Seong Heon Kim, Sang Wook Mun, Su Young Kim 1 1. Pediatrics, Pusan National University Children's Hospital, Yangsan, Korea

Objective: Spontaneous tumor lysis syndrome (STLS) before cancer treatment is rare and develop mostly in Burkitt lymphoma and non-Hodgkin lymphoma. Here we report a case of STLS secondary to Tcell acute lymphoblastic leukemia(ALL) which presented with renal stone and subsequent AKI.

Methods and Results: A 6-year-old boy was admitted to our hospital for generalized tonic clonic seizure. One month ago, He visited other hospital for episodic right-sided flank pain and evaluations revealed microscopic hematuria, hyperuricemia and stone on right uretero-pelvic junction. His blood pressure was 160/90 mmHg and he did not have any dysmorphic features and he had developed normally. Physical examination showed tender hepatomegaly (2 cm below costal margin) and no splenomegaly.

His initial laboratory findings were as follows : BUN 119.3 mg/dL, creatinine 4.77 mg/dL, uric acid 47.6 mg/dL, LDH 460 IU/L, WBC 11,900 /uL, Hb 9.9 g/d L, platelet 242K /u L. There was no blast on his peripheral blood smear test. Emergent hemodialysis was started because of AKI, hypertension and seizure. His renal function, blood pressure, uric acid and electrolyte abnormalities gradually improved with appropriate therapy after 2 times of hemodialysis. But serum uric acid level increased again (from 6.1 mg/dL to 12.9 mg/dL), so allopurinol was added on. The cause of AKI was unclear and we thought that AKI presumed to be secondary to acute uric acid nephropathy caused by STLS. After few days, bone marrow biopsy was done and demonstrated normocellular marrow without evidence of malignancy. After day 15 of hospitalization, abdominal pain with hepatomegaly was getting worse than before and uncontrolled high fever occurred. 20% of blasts were seen on his peripheral blood smear and subsequent second bone marrow biopsy demonstrated acute T cell lymphoblastic leukemia. Then, he was referred to division of pediatric hemato-oncology on our hospital and induction chemotherapy was started.

Conclusion: STLS with AKI is very uncommon initial presentation of leukemia and STLS presenting with renal stone is extremely rare. In a clinical situation of marked hyperuricemia with acute kidney injury, we need to consider occult malignancy and STLS.

The application of Serum beta-2-microglobulin (beta-2-MG) and cystatin C(CysC)concentration to evaluation of renal function impairment in patients withneonatal jaundice

Yanan Xin, Cairong Jiang, Junfeng Yang, Hui Xu, Yuan Zhang The Fourth Hospital of Baotou, Baotou, China

Objective: To explore the application of Serum beta-2-microglobulin (beta-2-MG) and cystatin C(CysC)concentration to evaluation of renal function impairment in patients with neonatal jaundice. Methods: 60 neonates with hyperbilirubinemia from Jan. of 2012 to dec.of 2012 in pediatric department and 34 full term infants were chosen as research object, hyperbilirubinemia divided into mild group (bilirubin<256.5umol/L) and moderate severe (bilirubin> 256.5umol/L) group according to the level of bilirubin. The different of sex and age were not significant between neonates with hyperbilirubinemia and control group. Serum beta-2-MG, CysC, Cr and BUN were measured in the two groups.

Results: Serum beta-2-MG and CysC in 60 neonates of hyperbilirubinemia were significantly higher (P<0.01) than healthy neonates, meanwhile, Serum Cr and BUN were significantly lower (P<0.01) than healthy neonates. After treatment, Serum beta-2-MG and CysC of neonates with hyperbilirubinemia were significantly lower (P<0.01) than pretreatment. However, Serum beta-2-MG were higher (P>0.05) abeta-d CysC were significantly higher (P<0.01) than normal group. But there was no significant difference between mild and moderate severe group in the level of Serum beta-2-MG, CysC, Cr and BUN(P>0.05).

The result implied that neonates with hyperbilirubinemia suffered for renal function to varying degrees. Howerer, the damage can partially recovered after energetic treatment.

Clinical and pathological analysis of IgA nephropathy with acute kidney injury

Minguang Chen, Xiaohua Ye, Qing Yang Nephrology, Yuying children's Hospital, Wenzhou, China

Objective: To investigate the incidence, etiology, clinical pathological characteristics and prognosis in primary IgA nephropathy(IgAN) children with acute kidney injury(AKI).

Methods: Retrospectively analysis the clinical and pathological manifestations and follow-up results of Chlidren with primary IgAN and AKI in our department from January,1996 to Jun, 2012.

Results: There were 19 cases with AKI in 196 Chlidren with IgAN(9.7%), The peak serum creatinine were from 94.5umol/ to 282 umol/L. Histological changes: with the formation of crescent in 10 cases, diffuse endocapillary proliferation in 5 example, 15 cases of renal tubular injury, 10 cases of red blood cell and protein cast, 1 cases with acute interstitial nephritis. Multivariate logistic regression analysis showed: with massive proteinuria were independent risk factors of IgAN in children with AKI (OR = 27.370, 95% confidence interval was 3.151-237.740, P<0.01). The etiology of AKI except with massive proteinuria, include: 1. IgA nephropathy with severe glomerular damage, including crescentic glomerulonephritis and diffuse endocapillary proliferation; 2 complicated acute interstitial nephritis; 3 drugs causing decreased glomerular filtration rate; 4. renal tubular injury induced by gross hematuria. All of the patients were not on dialysis, hormone therapy in 13 cases (including 7 cases of methylprednisolone pulse therapy), 6 cases combined with cyclophosphamide treatment. Except 1 cases no significant improvement, the renal functiones of all patients recovered or improved within 1-2 months after treatment. Follow-up from 1 month to 7 years, 3 cases had renal function improved, but 2 were lost to follow-up and after 3 years enter to the chronic renal failure, 1 case with renal function loss after 32 months and repeated renal biopsy showed glomerular sclerosis of 31.6% during the follow-up period. Conclusion: AKI is not uncommon in children with IgAN, the causes are varied and massive proteinuria is independent risk factor among them. We should adopt different treatment strategy according to different causes and the short term prognosis is good.

Abstract# P-SAT307 Biopsy proven acute interstitial nephritis in children Objective: Biopsy proven acute interstitial nephritis (AIN) is an uncommon cause of acute renal failure in children. The cause of AIN most is commonly due to medications such as antibiotics or infections. We reviewed our experience of biopsy proven AIN in children.

The biopsy database of all native renal biopsies over a 15 year period was reviewed. All biopsies which listed acute interstitial nephritis were selected and clinical and laboratory data were extracted from clinical records.

Results: 16 cases of AIN from 540 biopsies,(2.9%) was identified. Median age 11.5 years, range 1-14 12/16 cases presented with acute renal failure without an obvious cause. 13/16 cases had non specific constitutional symptoms and oliguria was reported by 6 patients. Antibiotics were the most common medications implicated (5/16 patients) Three patients were on multiple medications with one having chronic epilepsy and the other Crohn's disease. Five patients required short term dialysis ranging from 2 to 7 days.13 patients were treated with corticosteroids and at last follow up, 4 had reduced GFR 45-74ml/min/1.73m 2 .

Conclusion: Acute interstitial nephritis is uncommon in childhood but when it occurs, antibiotics are the single commonest cause. There is significant morbidity associated with the illness with not all patients making a full recovery.

Abstract# P-SAT308 Acute kidney injury as presentation of Burkitt's lymphoma Eva Greta ter Haar 1 , Anne Uyttebroeck 2 , Marleen Renard 2 , Veerle Labarque 2 , Djalila Mekahli 1 1 Pediatric Nephorlogy, University Hospital Leuven, Leuven, Belgium 2 Pediatric Hemato-Oncology, University Hospitals Leuven, Belgium

Objective: Acute kidney injury (AKI) has become increasingly prevalent. Approximately 2-3% of children admitted to pediatric tertiary care centers present with this life-threatening condition. The most common causes are post-operative septic shock, organ or bone marrow transplantations and intrinsic renal disease. The latter comprises multiple disorders, but tumor invasion due to a lymphoproliferative malignancy is very exceptional. Methods and Results: We report two cases of AKI caused by infiltration of Burkitt's lymphoma. Both four-year-old male patients presented with abdominal pain, nausea, vomiting, weight loss and overall weakness since a few weeks. Clinical examination showed hepatosplenomegaly and impressive bilateral nephromegaly. Both had malignant hypertension. Blood analysis showed severe renal impairment in both patients. The first had creatinine of 2.9 mg/dL (eGFR 21 ml/min/1.73m 2 ), urea of 97 mg/dL and uric acid of 8.9mg/dL. The second had creatinine of 5.97mg/dL (eGFR 10 ml/min/1.73m 2 ), urea of 215mg/dL and uric acid of 12mg/dL. Lactate dehydrogenase was extremely elevated in both cases (5860 U/L; 758 U/L respectively). Urinary sediment was normal. Ultrasound showed bilateral nephromegaly in both patients (Patient 1:+12SD and +11SD; Patient 2: +10SD and +9SD, for right and left kidney respectively). MRI demonstrated a homogenous renal enlargement with features of an infiltrative lesion. Bone marrow was inconclusive and diagnosis of Burkitt's lymphoma was confirmed by a renal biopsy. After starting chemotherapy according to the Inter-B-NHL Ritux 2010 protocol, both children developed a tumor lysis syndrome and required hemodialysis. At last follow-up both children still had hypertension. Nevertheless, renal size and function were normalized. Conclusion: Lymphomatous infiltration due to Burkitt's lymphoma is a rare cause of AKI. However, it should be considered in a patient presenting with unexplained renal failure and bilateral nephromegaly with normal urinary sediment. Renal biopsy may be needed to confirm diagnosis.

Abstract# P-SAT309 Does malnutrition interact with acute kidney injury in children?

Objective: To investigate the interaction between malnutrition and acute kidney injury (AKI) assessed by pRIFLE criteria and to assess the effects of these factors on the outcomes of pediatric intensive care unit (pICU) patients. Methods: Prospective cohort study conducted on children. Outcome variables: mortality, need for dialysis, pICU free days (the number of days alive from ICU discharge to day 28) and ventilator-free days (the number of days alive and breathing without assistance from admission to day 28). Exposure variables: Malnutrition on admission (WHO growth standards) and any change in pRIFLE criteria during the first ICU 14 days. Results: Of 98 patients (median age =19 IQR =50 months, 35 girls) enrolled, 39 (40%) were malnourished and 34 (35%) developed AKI. Among the malnourished, 19/39 cases (49%) had AKI, while this complication occurred in 15/59 (25%) of patients without malnutrition resulting in a risk ratio of 1.9 (95%CI 1.1 -3.3). The 28 days mortality rate was 10/98 cases (10%) and AKI was associated with higher risk (8/34 versus 2/64 cases -RR=7.5 95%CI 1.7-33.5). Concurrent malnutrition and AKI was present in 19/98 cases (19%) producing a tendency (p=0.2) to a further increased risk of death (RR=9.4 95%CI 1.5-58.0). 9/98 (9%) patients needed dialysis and malnutrition increased the risk for this outcome (RR=5.3 95%CI 1.2-24.2). AKI was associated with significantly higher ICU free days (19±7 vs. 15±10) and ventilator-free days (22±9 vs. 13±12) but concomitant malnutrition did not reduce either of these outcomes. Conclusion: Malnutrition and AKI are common in children admitted to the ICU and the presence of malnutrition is associated with increased risk of developing AKI. The need for dialysis was increased in malnourished patients and more powered studies are demanded to test whether the tendency to higher mortality observed in patients with concomitant AKI and malnutrition is confirmed.

Urine erythropoietin level is associated with kidney and brain injury in critically ill neonates Yanhong Li, Jie Yan, XiaoZhong Li Department of nephrology, Children's Hospital affiliated to Soochow University, SuZhou, China

Objective: Erythropoietin (EPO) is a glycoprotein hormone produced predominantly in the kidneys. The protective effect of exogenous EPO in hypoxic-ischemic brain injury has been thoroughly examined in neonates. However, the metabolism of endogenous EPO in neonates remains unclear. We aimed to evaluate the concentration of urinary EPO (uEPO) in critically ill neonates and to identify possible clinical and laboratory variables that may be associated with uEPO levels.

Methods: The concentrations of EPO, cystatin-C, microalbumin, and α 1 -microglobulin in the first available urine sample during the initial 72 hours of life were measured in 103 critically ill neonates. Clinical and laboratory data were collected for each neonate.

Results: There was a positive correlation between uEPO levels and urinary levels of cystatin-C (r =0.265, p =0.008), microalbumin (r =0.422, p <0.001), andα 1 -microglobulin (r =0.421, p <0.001). The concentration of uEPO was elevated in neonates who developed acute kidney injury (AKI) during the first week of life compared with those without AKI (P =0.002) and was also elevated in neonates with brain injury, as demonstrated by ultrasound or magnetic resonance imaging, compared to neonates without brain injury (P =0.008). An increased log 10 uEPO level was associated with the occurrence of AKI (odds ratio of 2.70, p =0.007) and brain injury (odds ratio of 2.33, p =0.016).

Conclusion: An increased urinary EPO level in the early postnatal period is significantly associated with kidney and brain injury in critically ill neonates.

Abstract# P-SAT311 Acute kidney injury following extracorporeal membrane oxygenation support and concomitant hemofiltration -the role of diuretics Objective: Fluid overload (FO) is common during extracorporeal membrane oxygenation (ECMO) and can be managed by continuous hemofiltration (HF) and/or diuretic therapy. Although combination therapy of HF and diuretics in particular can be most effectively for FO removal during ECMO, it may also increase the risk for prerenal acute kidney injury (AKI) post-ECMO. Our objective was to describe the incidence of AKI post-ECMO in patients treated with ECMO and HF who received concurrent diuretics.

Methods: In this cohort study all neonates (≤28 days after birth) treated with ECMO and concomitant HF between 2007 and 2011 were included. Patients were divided into two groups based on diuretic regimen: Group-1, patients who received no or one single diuretic dose during ECMO and Group-2, patients who repeatedly received diuretics. Both groups were compared using Mann-Whitney U test for nonparametric data and Chisquare test for categorical data. AKI was defined as the highest serum creatinine (SCr)-based RIFLE class reached on post-ECMO day 1 up to post-ECMO day 4 (Risk, Injury, or Failure being 150%, 200% or 300% of median SCr reference values for age).

Results: 56 neonates received ECMO support with HF, 6 died immediately following decannulation and 6 others were lost to followup since they were transferred back to their referring hospitals within 3 days post-ECMO. Of the 18 patients in Group-1, 3 (17%) qualified as Risk and 1 (6%) as Injury. Of the 26 patients in Group-2, 3 (12%) qualified as Risk, 4 (15%) as Injury and 9 (35%) as Failure (Group-1 vs. Group-2, p=0.039). Patients in Group-2 had an increased ECMO duration (p=0.001) and number of ventilator days (p=0.002). No differences were observed in baseline characteristics (e.g. age at the start of ECMO, underlying diagnosis, severity of illness scoring using the PIM and PRISM risk adjustment systems), HF flow rate, and fluid balance between both study groups. Conclusion: The incidence and severity of AKI immediately post-ECMO was significantly higher in patients who repeatedly received diuretics in addition to HF during ECMO.

Valentina Sitnikova, Yuliya Pashkova, Tatiana Zvyagina, Elena Kulakova, Alexandra Nastausheva Voronezh state medical academy, Voronezh, Russia

Objective: It is actually to find the new markers of kidney damage and spread them in practice. Cystatine C (Cys) is one of these, but there are only few data about it in children with urinary tract infection (UTI) according to the age. The aim of our study was to evaluate serum concentration of Cys in children with UTI of different age. We investigated 83 patients (47 girls and 36 boys) with UTI from 1 month to 17 years among them 32 children were under 1 years old. Methods: Concentration of Cys were measured by immunoenzime method (ELISA) with test-system BioVendor (Check Republic). Results: The mean Cys concentration in children with UTI under 1 year was 1.43± 0.37 mg/l, in older children it was lower: 1.23 ± 0.36 mg/l, p= 0.025 (Mann-Whitney). In 16 girls before 1 year Cys concentration was 1.47± 0.32 mg/l and in 16 boys of the same age it was 1.40±0.43 mg/l, p>0.05. In 31 girls over 1 year Cys concentration in serum was 1.31± 0.41 mg/l, in 20 boys -1.3± 0.33 mg/l, p>0.05.

The results of our study showed that among children with UTI serum concentration of Cys was higher in children of the first year of life and did not depend on sex. Probably, higher level of Cys in infants connects with low glomerular filtration rate in children of this age.

Microalbuminuria Can Prognosticate Outcome In The Critically Ill Children

Biplab Maji, Surupa Basu, Rajiv Sinha Paediatrics, Post Graduate Trainee, Kolkata, India

Objective: Microalbuminuria [albumin creatinine ratio (ACR) >30 mg/g] increases in acute inflammatory conditions as a result of glomerular endothelial dysfunction. The study evaluated its prognostic potential in the critically ill child. Methods: An ongoing prospective observational study (Oct 2012-Mar 2012) of random urine ACR (mg/g) estimated on day1 and day 3 of pediatric intensive care unit (PICU) admission, of a tertiary care pediatric hospital. Demographic, laboratory data, vasopressor use, mechanical ventilation, length of stay, outcome and PELOD scores were recorded. Results: Of 113 children, 72 children (median age 4.5years, 68% male) recruited. The commonest cause of admission was pneumonia, infection associated hemophagocytic lymphohistiocytosis followed by meningitis. Day1 ACR (median 70.8 mg/g) significantly correlated with day 3 total leukocyte count (P=0.016). Both day 1 and day 3 ACR correlated with PELOD scores [median 8] (P=0.002 & p= 0.003 respectively) and duration of mechanical ventilation (p=0.003 and p=0.001 respectively). Both median day 1 ACR, and day 3 ACR were significantly different between survivors (n= 29) and non-survivors (n=11) (p=0.0001 and p<0.0001, respectively). ROC curve analysis for mortality prediction revealed the highest area under curve (AuC) of 0.963 for ACR3, followed by 0.928 for ACR1 and 0.867 for PELOD scoring (p>0.05, non-significant for AuC comparisions) (Fig. 1.) . A cut-off value of day 3 ACR of 102.4mg/g had a positive predictive value of 95.8% for negative outcome Conclusion: Microalbuminuria is an early inflammatory marker that correlates with organ dysfunction. Significant levels reliably predict negative outcome early into intensive care admission, as accurately as PELOD scores, which can help counsel patients, plan treatment and triage, and allocate resources judiciously.

Abstract# P-SAT314 An unusual presentation of congenital nephrotic syndrome caused by WT1 mutation Ann Raes, Sofie Maebe, Joke Dehoorne, Bert Callewaert, Johan Vande Walle Pediatric Nephrology, University Hospital, Gent, Belgium

Objective, Methods and Results: A Caucasian girl was admitted at the age of 28 days because of progressive lethargy, poor feeding, oliguria en peri-orbital edema. extreme hyponatremia (89 mmol/L), severe renal insufficiency (creat 1.9 mg/dl, P 10.7 mg/dl), low plasma albumin (1.5 g/dl), respiratory compensated metabolic acidosis (arterial pH 7.31 pCO 2 27 mmHg, bicarbonate 13.5 mmol/L, BE -10.9 mmol/L) and massive proteinuria (12 g/L). Clinical examination was normal without anomalies of the external genitals or other dysmorphic features. Despite supportive therapy (sodium, bicarbonate, albumin and furosemide) she progressed to respiratory failure and renal failure with need for mechanical ventilation and peritoneal dialysis. Congenital infections and thrombosis was ruled out. Histological examination showed diffuse mesangial sclerosis (DMS). The combination of congenital nephritic syndrome and DMS on biopsy was suggestive for mutations in the Wilms' Tumor 1 (WT1) gene. Genetic analysis showed normal female karyotype and conformed a heterozygosity for the c.1223c>G or p.His441Gln mutation within the Zinc finger 2 domain. After a long and complicated ICU episode, a stable situation with home choice peritoneal dialysis was achieved. Due to the important risk for comparision of AuC of PELOD with ACR 3, p= 0.766 for comparison with ACR 1) for the prediction of mortality in the pediatric intensive care unit. development of Wilms' tumor, bilateral nefrectomy was performed and kidney transplantation will be planned.

We present an unusual case of congenital nephrotic syndrome with extreme hyponatremia at presentation and fast progression to ESRD, due to mutation in WT1 gene. Several genes have been implicated in congenital nephrotic syndrome. Genetic testing is mandatory and can add important information such as risk of malignancy. Renal histology can play an important role in the approach for appropriate mutational screening. Mutations in the WT1 gene are associated with Denish Drash syndrome and Frasier syndrome, but also with isolated and sporadic steroid resistant nephrotic syndrome. In the last category the majority of patients is female, the age of onset and time to ESRD is variable, although the vast majority of cases are older at presentation and time to ESRD is longer than in our case. Objective, Methods and Results: A 8-year-old girl, affected by b thalassemia major, was admitted for proteinuria detected at the emergency unit. Fever, hyporexia and diarrhea had been present in the last three days. On physical examination she presented with decreased skin turgor and hypertension. Laboratory examinations showed: hyponatremia (124 mEq/l), hypokalaemia (2.9 mEq/l), hypophosphatemia (1.1 mg/dl), hyperazotemia (60 mg/dl), reduced creatinine clearance (CrCl = 68 ml/min/1.73 m 2 ) and metabolic acidosis (HCO 3 -12.8 mmol/l). Urine analysis revealed: proteinuria, glycosuria, hypercalciuria, hyperuricuria, hyperphosphaturia and aminoaciduria. She had been under treatment for five years with Deferasirox (DFX) 26 mg/kg/die. We stopped DFX and gave supplementation of potassium, phosphate, sodium bicarbonate and sodium chloride with electrolyte and renal function (CrCl 130 ml/min/1.73 m 2 ) normalization in 6 days. After one month only minimal proteinuria persisted. Conclusion: In so far a Fanconi Syndrome (FS) has been reported in 11 patients (6 children), while mild renal insufficiency (RI) has been documented only in 2 children. In our case dehydration seems to be responsible for the RI, while an interstitial nephritis cannot be excluded. Close monitoring of renal function should be done in children on DFX. Objective: To test the hypothesis that neutrophil gelatinase-associated lipocalin (NGAL) and interleukin-18 (IL-18) are early biomarkers for AKI in critically ill patients and evaluate the predictive value of them in patients with established AKI at inception of CRRT. Methods: Children from PICU and health examination center in Guangzhou Women's and Children's Medical Center were divided into four groups: critically ill patients with AKI recevied CRRT group (group1 ), critically ill patients with non-AKI recevied CRRT group(group2) , critically ill patients with AKI don't recevie CRRT group (group 3),healthy control group (group4). 1.5 mL venous blood and Urine specimens were collected and was kept under -70°C.from each patient in PICU untill they were discharged, transferred or die . serum creatinine (Scr) and urine NGAL and urine IL-18 were analyzed . Results: Compared with group2 and group4, the urine NGAL and urine IL-18 increases obviously in group1 and group3 (P<0.05). There is no significance of urine NGAL and urine IL-18 between group2 and group 4.(P> 0.05).The concentration of urine NGAL increased more than10 times obviously 2 days before diagnosed of AKI under the AKIN standard with AUC 0.841(P <0.05) , and the concentration of urine IL-18 increased more than 5 times 2 day before AKI with AUC 0.808(P <0.05). The AUC was 0.943(P <0.01) when they were combined. The level of urine NGALand IL-18 at initiation of RRT were higher in in non-survivors compared to survivors. Conclusion: Urine NGAL and urine IL-18 are useful indicators to predict and early diagnose AKI. The level of urine NGAL and IL-18 at initiation of RRT.were negative correlation with the outcome of renal prognosis and survival rate . Objective: The aim of this study was to evaluate the rate of early kidney injury in infants with congenital heart disease (CHD). Neutrophil gelatinase associated with lipocalin (NGAL) which can be measured both in serum (sNGAL) and in urine (uNGAL), is at the moment the most promising marker directed to discover early kidney injury within the glomeruli and distal and proximal tubules. Less known is cathepsine L as a marker of tubule necrosis. Methods: The study group consisted of patients (22 boys and 12 girls) with congenital heart disease. All patients were under 3 years of age (mean 8.2 months). Blood and urine samples were obtained during routine checkups. All patients had normal serum creatinine level at the day of collecting samples. The control group (n = 20) was age-and gender-matched. Levels of sNGAL, uNGAL and cathepsine were compared in whole group. The study group was divided depending on the nature of the defect (cyanotic/acyanotic) and the treatment (surgical/conservative treatment). Results: Chlidren with congenital heart disease had significantly higher concentration of sNGAL, uNGAL and urine cathepsine than the control group. No significant difference was observed in the urine cathepsine and urine and serum NGAL level between patients with acyanotic and cyanotic CHD. There were also no significant differences in patients who underwent surgery and on conservative treatment in NGAL level (both in urine and serum). Cathepsine level was significantly higher in group after surgical treatment. Conclusion: Patients with CHD are at higher risk of early kidney damage. This process is independent on the etiology and nature of the defect. NGAL determined in the serum and urine of these patients may be used to detect kidney injury or monitoring disease progression. Urine cathepsine is more efficient marker of early kidney injury in patients after cardiac surgery.

Abstract# P-SAT318 Tim-3 expression in kidney mononuclear cells and its relationship with Foxp3+ Tregs from mice with renal ischemia reperfusion injury Yamei Wang, Yuhong Tao, Li Ye Department of Pediatrics, West China Second University Hospital, Chengdu, China Objective: Foxp3 + Tregs participate in the repair of renal ischemia reperfusion injury. T cell immunoglobulin-3 (Tim-3) plays a pivotal role in several inflammatory diseases by modulation of Foxp3 + regulatory T (Treg) cells. To detect the expression of Tim-3 in kidney mononuclear cells (KMNCs) from mice with renal IRI and analyze the relationship with Foxp3 + Tregs, to explore the role of Tim-3 in repair of renal IRI. Methods: The left renal pedicle was clamped in C57BL6 mice for 45 min, followed by reperfusion. Animals were sacrificed at baseline, day 1,3,10, 21 after IRI. Tim-3 expression in KMNCs were determined using real-time RT-PCR and flow cytometry. The percentage of Foxp3 + Treg in CD4 + T cells was quantified by flow cytometry. The levels of TNF-α、IFN-γ、IL-4 and IL-10 in kidney tissue were measured using ELISA. The correlation among Tim-3 expression, Foxp3 + Treg and cytokine level was analyzed.

Results: The expression of Tim-3 expression in KMNCs from injured kidney at acute stage(day 1) was significantly higher than those from uninjured kidney at acute stage and lower than those from injured kidney at repair stage (day 3,10, 21). The percentage of Foxp3 + Treg in CD4 + T cells was up-regulated with time. Compared with the uninjured kidney, the expression of TNF-α and IFN-γ increased significantly in the injured kidney at acute stage and the expression of IL-4 and IL-10 increased significantly in the injured kidney at repair stage. The Tim-3 expression in KMNCs at IRI repair stage was positively related with the level of Foxp3 + Treg, IL-10 (r=0.81, r=0.79 respectively; P<0. 05), but negatively related with the level of TNF-α and IFN-γ. Conclusion: The increased expression of Tim-3 in KMNCs may take part in the repair of renal IRI and contribute to the development of Foxp3 + Treg. Objective: (D+)HUS is a critical health problem in Argentina since it is the main cause of acute renal failure in children and the second cause of chronic renal failure. Fecal contamination of food and drinking water by asymptomatic cattle is often the source although secondary infection through personto-person contact may also occur. Occasionally in (D+) HUS, the onset among affected siblings occurred within a short time of each other. To evaluate the risk and clinical severity of illness for childhood (D+)HUS in siblings. Methods: We retrospectively analyzed the clinical records of 133 children with D+HUS that were admitted in our pediatric department between March 1997 and December 2012. Results: (D+) HUS occurred in 2 siblings in 4 of the 129 families studied (3%). 16 patients had an affected sibling with diarrhea and 4 progressed to HUS (25%). Family cases: Mean age: 28 months, 5 were girls. The mean duration of interval between HUS episodes was 4 days. Second family members had prolonged oligoanuria (3 vs 12 days) and a most of them developed neurological complications. Long-term renal complications were more frequent in this group, but differences were not statistically significant. Conclusion: These findings emphasize the potential for extensive intra-familiar transmission of STEC, especially between siblings.

A second family member might also develop an even more severe HUS episode, so siblings should be kept under close surveillance.

Objective: Kawasaki disease (KD) is a common cause of systemic vasculitis in children. Other than well known complications like coronary artery aneurysm, there have been few reports of this disease involvement in renal system. According to the study from Wang JN et.al, 52% (26 out of 50) of the patients who suffered from KD showed renal inflammatory foci in DMSA renal SPECT which suggested a possibility of renal scar formation subsequent to KD. Therefore, this study was performed to verify the renal inflammation following KD. Methods: From March 2011 to October 2011, 15 patients who were diagnosed as KD at National Health Insurance Service Ilsan Hospital were enrolled to the study. All of the patients underwent DMSA renal SPECT to evaluate renal involvement during their acute phase of KD. Echocardiography was performed to assess cardiac involvement such as coronary artery aneurysm. Complete blood cell counts, aspartate amino-transferase, alanin amino-transferase, albumin, c-reactive protein, and BUN/Creatinine were measured. Also Urine β2microglobulin was measured to assess renal tubular function. Addition to fever of more than five days of duration, 4 of the following symptoms, rash, conjunctival injection, changes of lips or oral mucosa, erythema and swelling of hands and feet and cervical lymphadenopathy, was the diagnostic criteria for KD. Results: Among the 15 patients, 80% showed increased white blood cell s (WBC) and 47% showed elevated AST/ALT level. Serum albumin was below 4.0 g/dl in 93% of the patients. All of the patients presented normal renal function test. In urinalysis, hematuria and pyuria were observed in 13% and 33% respectively. Echocardiography revealed coronary artery aneurysm in 33% of the patients. Urine β2-microglobulin was elevated in 46%. Regardless abnormal findings in urinalysis and elevated β2microglobulin, no significant findings were observed in DMSA renal SPECT. Conclusion: According to the study, mild abnormality in the urinalysis and elevated β2-microglobulin were the only findings of renal involvement in KD. However there was no aggressive renal manifestation which could be detected in DMSA renal SPECT.

Objective: In this study, the question remains if NGAL is a culprit or only bystander in AKI due to sepsis in children. Methods: Twenty seven children, (M-17, F-10) admitted to Intensive Care diagnosed with sepsis, severe sepsis or septic shock were enrolled in this study. The concentration of NGAL, protein C and S, Antithrombin III and basic parameters were measured in the plasma at diagnosis and after 10 days of treatment. Results: Mean creatinine concentration in septic children at admission was higher than in reference group (p <0.05). There was statistically significant difference in APTT, prothrombin time, prothrombin ratio, thrombocytes count, heart rate, and systolic blood pressure between study groups in first and after 10 days (p<0.005, p<0.001, p<0.001, p<0.01, p<0.05, p<0.05 respectively). The PC concentration in the septic patients was significantly lower than those of the references (p<0.0001), and during intensive 10 days treatment raised (p<0.01). Also protein S concentration rose during the treatment (p<0.005), and was lower in septic children then reference group (p<0.0001). NGAL concentration was significantly higher in study group on the admission day comparing with reference group (p<0.05). Plasma NGAL was correlated at admission and after 10 days of treatment with protein C (respectively: r=-0.48, p<0.00005; r=-0.53, p<0.05). Multivariate regression revealed that NGAL was significantly predicted by creatinine (β=0.39, p<0.01) and Protein C (β=-0.38; p<0.01), yielding a model R2 =0.34 (p<0.001). Conclusions: It is possible that kidney binding of pathogenic antibodies stimulates local expression of NGAL, which plays a crucial role in the pathogenesis of AKI. Objective: The aim of the study was to assess the impact of perinatal risk factors (PRFs) on serum NGAL level in term neonates. Methods: The study group consist of 62 term neonates with PRFs, and 14 healthy neonatescontrol group (CG). Serum NGAL (ELISA) was measured in samples of cord blood (cNGAL) and peripheral blood (pNGAL) taken within first 5 days of life, and stored in -80°C until ELISA procedure was performed. PRFs were divided into 3 groups: 1. pathology of pregnancy or mother: diabetes (D) n=23, infection (I) -elevated CRP or WBC n=20, positive vaginal culture (VC) n=21, hypertension (HT) n=7, others n=5; 2. labor pathology: instrumental delivery (ID) n=41, premature rupture of membranes, >6 hours (PROM) n=26, fetal distress (FD) n=26; 3. neonatal pathology: intrauterine infection (II) n=10, CAKUT n=5, perinatal asphyxia (PA) n=4. The results were shown as median; values of cNGAL, pNGAL were logtransformed before analysis. Statistical analysis was performed with t-student, MANOVA tests with help of Statistica 10. Statistical significance: p<0.05. Results: In 62 term neonates, median cNGAL was 117.69ng/mL vs 64.37ng/mL in CG. Mean log cNGAL was significantly higher (p<0.01) vs CG, pNGAL was NS. Mean log cNGAL correlated positively with mean log pNGAL (r=0.36, p<0.01). Univariate analyzes of log NGAL in neonates with chosen PRFs vs neonates without particular factor showed significantly higher (p<0.05) mean log cNGAL in ID and FD group (median cNGAL: 126.47 vs 67.50 and 122.30 vs 77.01ng/mL respectively), whereas in II group significantly higher were mean log cNGAL and mean log pNGAL (median cNGAL 209.69 vs 96.52ng/mL, pNGAL 457.35 vs 156.37ng/mL). Multivariate analysis showed that the impact of ID was significant in groups: D (F=9.84; p<0.002), I (F=4.56; p<0.04), PROM (F=6.01; p<0.02) and FD (F=5.36; p<0.02). Conclusion: 1. Intrauterine infection may have a significant influence on NGAL in cord and peripheral blood whereas fetal distress and instrumental delivery only in cord blood. 2. The instrumental termination of pregnancy has an import impact on NGAL level in cord blood of neonates whose mothers had diabetes or infection, with PROM or fetal distress. Objective: Acute kidney injury (AKI) is a common problem and associated with significant morbidity and mortality in neonates. Pediatric-modified RIFLE (pRIFLE) classification system was developed to standardize the definition of AKI in children. We aimed to evaluate the performance of pRIFLE score diagnosis, severity and prognosis of AKI in term and preterm neonates. Methods: In this retrospective study, charts of 820 patients who were admitted to neonatal intensive care unit (NICU) over a 4 year period were reviewed for development of AKI. A diagnosis of AKI was determined for 254 patients (30.9 %) according to the pRIFLE criteria Results: Of the 254patients included in this study, 101 of them were girls and 153-were boys. Mean age was 4.12days (min-max: 0-49), mean birth week was 33.6 ±5.1 weeks (min-max: 24-42), mean birth weight was 2166 ± 1045 gr (min-max: 580-4600 gr), length of NICU stay was 37.3 ± 33.5(min-max: 2-165days). Two hundred fifty four patients with AKI were classified according to pRIFLE criteria. Ninetyeight patients (38.5%) achieved an 'R' level of AKI severity, 86 patients (33.5%) an "I" level, 66 patients (26%) an "F" level, 4 patients (1,5%) an "L" level. The most common etiologies of AKI -were prematurity, congenital heart disease, hypoxic ischemic injury, sepsis and usage of nephrotoxic agents. Patients classified with a higher pRIFLE score had a longer NICU stay and required a greater number of mechanical ventilation (p<0.05). Renal replacement therapy (RRT) was needed for 30 patients (11.8%). Mortality rate was 26% and all deceased patients were classified as pRIFLE 'I','F' or 'L',p < 0.05) Conclusion: Using pRIFLE criteria in neonates with AKI is an efficient way to assess the severity and prognosis of the disease. Objective, Methods and Results: I present a case of a 11 years albanian old boy, 25th centile of development. Presented with abdominal pain, vomiting. Four days after, was addmited in the surgery clinic for uregent apendicitectomy. WBC: 23.0, SE: 64/, BUN: 6.1, Cre: 60, urine: normal. After the intervention medications that were used were: Gentamycin, Metronidazol, Cephtriaxon, H2 blocators. Two days after he was better. Thereafter he had polyuria and frequent urinating. The fifth day he had oliguria to seventh day, when he couldn't urinate. Nativ Rtg of abdomen resulted normal. He was transferede to Pediatric clinic-Nephrology department as an Akute kidney injory. He looked very sick, consious, stratified tongue, ECG: 50b/min, BP: 115/60 mmHg, breathing sounds were normal, periferal edemas and cold extremities. Lab resultrs: Cre: 624, BUN: 16.6, K: 5.1, Na: 131, eGFR: 8.8 ml/min oer 1.73m2. Ultrasound resulterd with urinary stasis: hydronephrosis, huge bladder. First was suspected for acute obstruction and AKI couse of a medication. We changed a bladder cathether which we find out that was blocked and took out 2700 ml urine/24h. We Dialysed him for three days and cre was stabilized to 266, when we stoped dialyse. BP was stabile, his weight droped from 32kg to 27.5 kg. He had proteinuria 2 g/24 h. Schintigraphy was done (biopsy we couldn't do) and resulted: eGFR was 12% less than adecuat for age, suspected for Nephritis tubulointestialis. He had some toxoalergic exanthema spread to the body. Repeted US resulted with ascaridosis in the gallbladder, which was treated with Mebendazole and in the feces were found the parasites. Imune Ab were negativ, IgE: positive, periferal blood smear had eosinophils, wastage of Na and Mg with urine. Cre after four weeks was 58, BUN:5, negative proteinuria. No changes of uveitis in the eyes. Importance of this case is cause this is the first case in Kosova that was dialysed and has recovered and now is free of dialyse (all other cases have traveled abroad until then). Conclusion: We have to think for parasites as a cause of a Tubulointesticial Nephritis but also of a abdominal pain or fatigue. Surgery team should have a close cooperation with pediatricians, in order to identifye in time complications of the surgery.

Urine output in first 48 hours after birth in relation to AKIN criteria Saroj Kumar Patnaik, Gaurav Aggarwal, Uday Kumar, Vempati Venkateshwar, Shamsher Singh Dalal Pediatrics, Command Hospital Air Force, Bangalore, India

Objective: Applicability of AKIN urinary output (UO) criteria in neonates during first 48 hours of life remains contentious since nonpassage of urine during this time has traditionally been considered 'normal'. We hypothesize that 'physiological' oliguria in first 48 hours of life is mostly prerenal in origin related to poor fluid intake during transition and UO criteria should be applicable in the 1st 48 hours of life too. We aimed a) to investigate pattern of UO in babies admitted to NICU with underlying morbidity but regulated fluid intake versus roomed-in babies in postnatal ward on breastfeeds in first 48 hours of life; b) to relate UO till 48 hrs of life with development of AKI Methods: Prospectively timing of first void and 6 hourly UO from birth till 48 hours age was compared between consecutive postnatal ward and NICU admissions. Neonates with structural malformations and requiring ventilatory/vasopressor support were excluded. Outcomes-Primary : time of first void after birth Secondary: UO in 1st 48 hrs in relation to subsequent AKI Results: Amongst eligible 87 postnatal breastfed babies (mean bwt 2.86 Kg ; SGA 18; 27 LSCS born) and 110 NICU admissions (median birthweight 2.01 kg,mean GA 34 wks (26-43 wks); 67 prematures (10 < 30 wks); 82 SGA (6 ELBW) recruited after informed consent, first voiding was significantly earlier for NICU babies (Mean(95%CI) (hrs) Postnatal 4.12 (3.29, 4 .93) vs NICU 2.31 (1.50,3.12) hrs). Nonvaginal delivery(LSCS 3.00 (1.89,4.11) vs vaginal 4.68 (3.60,5.75 ) and normal birthweight (AGA 4.08(3.10, 5.06) vs SGA 4.22(2.81, 5.63) ) had a trend for earlier voiding. 80% babies voided within 6 hrs of birth -more in NICU babies (90.4% NICU vs 76.7% postnatal (p<0.004) (Fig) .Asphyxia with MAS in NICU and IUGR and poor feeding in postnatal ward had delayed first void beyond 12 hours. 6 postnatal and 7 NICU neonates anuric in 1st 6hrs met stage I AKIN creatinine criteria. 2 asphyxiated babies anuric beyond 12 hours in NICU developed Stage III AKI. Conclusion: 'Physiological' anuria in 1st 48 hrs of life is misnomer and is related to fluid intake. Most babies are nonoliguric by 6 h; anuria >12 h needs investigation. AKIN urinary criteria should be applicable in neonates.

Odillha Morales Maglalang-Reed Nephrology, Philippine Children's Medical Center, Quezon City, Philippines

Objective: The precise mechanism of renal injury among Dengue patients is not known. Patients who have ATN will usually require early dialysis. However, on admission to the hospital, it is difficult to distinguish DSS patients with ATN from patients with reversible prerenal causes that will respond to simple hydration. Our understanding of the complex pathogenesis of tubular injury in Dengue AKI is very limited that until it is sufficiently increased, therapeutic strategies will continue to fail.Therefore we sought to explore the limitations of serum creatinine in this setting. General objective is to determine the clinical and diagnostic factors which are predictive for the need for dialysis among DSS patients at PCMC. Specific objective is to determine if the following factors are predictive of the need for dialysis: decrease in estimated creatinine clearance by 75% or <35 ml/min/BSA with urine output of <0.3ml/kg/hr. X 24 hours or anuria of 12 hours, assess the usefulness of urinary sediment scoring (USS) in predicting the need for dialysis, Methods: This retrospective study covered 60 newly admitted cases of Dengue Shock Syndrome lll and lV at the Philippine Children's Medical Center between January 2010 to December 2011. Results: Data from 60 patients were available for analysis. Comparison of the demographic characteristics between patients who required dialysis and those who did not showed no significant difference as proven by all p values >0.05. Of the differentclinical and laboratory parameters, there was a significant difference in the HR, RR, O 2 saturation, bicarbonate and base excess as proven by all p values <0.05. The HR an RR were significantly higher among those who needed dialysis than those who did not O 2 saturation, bicarbonate and base excess were significantly lower among those who needed dialysis than those who did not. The estimated creatinine clearance was significantly lower among those who needed dialysis than those who did not. Urine sediment score (USS) ≥3 was significantly higher among those who needed dialysis than those who did not. Conclusion: Our data indicate that a decrease in estimated creatinine clearance by 75% or <35 ml/min/BSA with urine output of <0.3ml/kg/hr. X 24 hours or anuria of 12 hours is not predictive of dialysis among DSS-induced AKI as well as other clinical and laboratory indices. But rather, relatively lower serum creatinine level among DSSinduced AKI who needed dialysis is associated with greater urine sediment scorecompared with those who did not. A USS ≥ 3 correlated with ATN and was predictive of early dialysis.However, estimated crea < 50 is the only factor predictive of dialysis, since this is the only one that was positive after logistic regression.

Abstract# P-SAT327 Pre-operative FGF23 predicts acute kidney injury in pediatric cardiac surgery patients: a prospective study Mark R Hanudel, Myke Federman, Barbara Gales, Georgina Ramos, Vicky Campbell, Kristen Ethridge, Mary Scotti, Brian Reemtsen, Isidro B Salusky, Katherine Wesseling-Perry Pediatrics, UCLA, Los Angeles, USA Objective: Fibroblast growth factor 23 (FGF23) is a phosphaturic hormone that predicts renal disease progression in CKD. However, in the setting of AKI, there is a paucity of prospective data on FGF23, especially in the pediatric population. Thus, we prospectively measured FGF23 levels in pediatric patients undergoing cardiopulmonary bypass (CPB) and assessed the ability of FGF23 to predict AKI. Methods: Pediatric patients, age newborn to 21 years, without underlying CKD, undergoing cardiac surgery requiring CPB were eligible for the study. Plasma FGF23 levels (2nd generation Cterminal, Immunotopics) were measured pre-operatively and at 2, 6, 12, 24, 48, and 96 hours post-reperfusion. Serum creatinine was obtained at baseline and daily post-reperfusion. AKI was defined by the AKIN criteria, estimated GFR was calculated using the Schwartz formula, and cardiac surgery complexity was classified via RACHS-1 score. Results: Of the 20 enrolled patients, 13 developed at least Stage 1 AKI. Gender, weight SDS, height SDS, baseline eGFR, cardiac surgery complexity, and CPB duration did not differ between the AKI group and the non-AKI group. Patients who developed postoperative AKI were younger than those who did not. Preoperative FGF23 levels were inversely related to age (r = -0.67, p = 0.001). Pre-operative FGF23 levels were significantly higher in patients who developed AKI than in those who did not; this remained significant upon correcting for age. Post-operatively, FGF23 levels increased in all patients. Conclusion: Pre-operative FGF23 levels predict the development of post-operative CPB-associated AKI. FGF23 levels increase in non-CKD patients undergoing CPB, even in patients without significant changes in serum creatinine, suggesting that FGF23 may be a more sensitive marker of AKI than serum creatinine.

Non-AKI Pateints p value Objective: Improved perinatal care increased not only the survival rate but also the frequency of acute kidney injury (AKI) in newborns. We aimed to determine the frequency, etiology, clinical course and mortality of AKI in a third level neonatal intensive care unit (NICU). Methods: Medical records of all patients admitted to a NICU in western Turkey during 2007-2011 were evaluated and those having AKI within 0-30 days of life were determined. Birth weight, gestational age, mode of delivery, gender, maternal morbidity, hospitalization period, accompanying morbidities and mortality of all patients; and primary disease causing AKI, highest serum creatinine and prognosis of patients with AKI were recorded. Results: There were 677 patients (M/F:392/285) and 94 (13,9%) had AKI of which 80% developed during 0-7 days of life mostly due to birth asphyxia, hypovolemia, cardiac disease, sepsis and urinary system anomalies. AKI incidence and total mortality rate were higher in patients with birth weight <1000 g and with gestational age <28

week. However, mortality in patients with AKI was increased independent on birth weight (42,1% in <1000 g vs 32,1% in >1000 g; p=0,324) and gestational age (44,1% in <28 week vs 31,7% in >28 week; p=0,227). Mortality tended to increase in the presence of AKI independent on the underlying cause, but this was significant only for sepsis (33, 3% vs 5, 9%, p<0, 001; OR 7, 9) and cardiac diseases (50,0 %vs 15,0%, p=0,001; OR 5, 6) . Hospitalization period was increased if AKI was present (39,1 vs 20,5 days, p<0,001). Serum creatinine in deceased patients with AKI was higher than those who survived (2,0 vs 1,6 mg/dL, p<0,05). Chronic kidney disease was developed in 6 (10%) patients of whom 3 had urinary tract anomalies and 3 had birth asphyxia. Conclusion: AKI incidence in NICU was 14%, and 80% of AKI developed during the first week of life. AKI is associated with low birth weight, prematurity, birth asphyxia, sepsis, hypovolemia, cardiac diseases and urinary tract anomalies. Mortality in patients with AKI is increased independent on the birth weight, gestational age and underlying etiology. Objective: Cisplatin (CDDP) is one of the antineoplastic agents widely used for solid tumors in adults and children. An excessive dose due to medical error causes severe nephrotoxicity, ototoxicity and myelosuppression. Previous reports described the effects of plasma exchange, sodium thiosulfate, N-acetylcysteine and other interventions. However, the strategy for over-dose patients, as regards the selection or intensity of therapies and the target of cisplatin removal, remains uncertain in children.

A 12 year-old girl was admitted for headache and vomiting. She was found to have a right cerebellar tumor and underwent total extirpation of this medulloblastoma. Next, we administered chemotherapy mainly using CDDP. However, she developed renal insufficiency and hearing loss on the fourth day. We then realized that we had been administering CDDP 90 mg/m 2 for 4 days, instead of the intended 1 day. We promptly discontinued the CDDP and began plasma exchange and sodium thiosulfate administration. Nonoligiric renal failure was confirmed by a urea nitrogen value of 63.6 mg/dL and creatinine level of 4.4 mg/dL. With 4 cycles of plasma exchange and 2 weeks of sodium thiosulfate, renal function improved and the CDDP concentration decreased from 6.04 to 0.99 mg/mL. After 1 month, the concentration was 0.34 mg/mL. We switched from CDDP to carboplatin, and chemotherapy was continued for 4 courses and then radiation therapy was added. Ultimately, she had mild renal insufficiency and moderate hearing loss. We evaluated the relationship between renal function and the CDDP concentration. Severe renal failure and hyponatremia were observed at a CDDP concentration of 1 mg/mL or more. Creatinine clearance of 60 mL/min/1.73 m 2 was maintained at a CDDP concentration below 0.5 mg/mL. Conclusion: In children with a CDDP over-dose, plasma exchange and sodium thiosulfate administration are effective. Also, monitoring of CDDP concentrations is recommended in patients with acute renal failure. In over-dose patients, early severe renal failure can be prevented when the CDDP concentration is reduced to less than 0.5 mg/mL with these therapies.

Abstract# P-SAT331 Peritoneal Dialysis in Children with Acute Kidney Injury: A Developing country experience

Om P Mishra 1 , Aditya K Gupta 1 , Vishal Pooniya 1 , Rajniti Prasad 1 , Narendra K Tiwary 1 , Franz Schaefer 2 1 Pediatrics and Medicine, Institute of Medical Sciences, Varanasi, India 2 Division of Pediatric Nephrology, Heidelberg University Medical Centre, Heidelberg, Germany

Objective: Peritoneal dialysis (PD) is the preferred and convenient treatment modality for acute kidney injury (AKI) in children and hemodynamically unstable patients. The present study analyzed the efficacy of PD in patients with AKI and factors contributing to mortality.

Methods: The outcome of acute PD was studied in 57 children (39 males) with AKI, aged 1 month to 12 years, at a tertiary care centre of a teaching hospital in India.

Results: There were 14 patients less than 1 year of age, 23 patients 1-5 years of age, and 20 patients more than 5 years of age. Per the RIFLE criteria, 5 patients were classified at the Risk stage; 13, at the Injury stage; and 39, at the Failure stage at the time of the decision to start PD. Hemolytic uremic syndrome was the most common cause of AKI (36.8%), followed by septicemia (24.6%) and acute tubular necrosis (19.3%). Treatment with PD was highly effective in lowering retention markers (blood urea decreased by 40% and serum creatinine by 34% during the course of dialysis therapy, both trends significant at p < 0.001). Overall mortality was 36.8%. Deaths occurred 2-7 days after hospitalization. Significantly higher proportion of non-survivors had fluid overload (66.7% vs 25%, p= 0.002) and septicemia (47.6% vs 11.1% , p< 0.001) than survivors at presentation. The risk of mortality by multivariate analysis was higher when patients were anuric [Odds ratio (OR) 8.2, 95% confidence interval (CI) 1.3-49, p<0.05), had septicemia (OR 3.79, 95% CI 1.55-25.8, p<0.05), or severe infectious complications (OR 8.2, 95% CI 1.5-42.9, p<001). Conclusion: Because of its simplicity and feasibility, acute PD is still an appropriate treatment choice for children with AKI in resource-poor settings. Septicemia and severity of AKI are contributory factors to high mortality in pediatric acute kidney injury.

Outcome of Acute Kidney Injury managed in a regional paediatric nephrology centre

Shivaram Hegde, Sabina Pahari Paediatric nephrology, University Hospital of Wales, Cardiff, UK

Objective: This study reviewed the aetiology, treatment modalities and outcome of children with AKI managed in our tertiary paediatric nephrology unit.

Method: Retrospective analysis of referral practices, aetiology, and management of 38 children treated for AKI over the last 5 years. Children primarily treated in intensive care units were excluded.

Outcomes noted as complete recovery, residual renal injury, renal replacement therapy (RRT) dependency or death. They were followed up until their renal function normalised and any proteinuria or hypertension resolved. Result: Out of the total 38 children aged 5 months to 16 years, 34% were under 5 years. Haemolytic Uremic syndrome (HUS) was the commonest aetiology in 18 cases (47%), 15 of them secondary to Ecoli 0157 and 3 with atypical HUS. Obstruction was second most common (5) and renal function improved following relief of obstruction. Supportive management sufficed in 23 (60%) cases and 15 (37.5%) received renal replacement therapy (RRT); peritoneal dialysis being the commonest mode. Most children needing dialysis were oliguric (14). At discharge there were no deaths, 5 patients showed complete recovery of renal function, one was dialysis dependant and renal function was improving in the rest. At 3 months we found normal renal function in 26 (68%) children and chronic kidney disease (CKD) stage II in 7 (18%) and CKD stage III in 4. The dialysis dependent child underwent renal transplantation. Based on the data from 12 patients currently under follow up (for 12-62 months, mean 41 months), 32 (84%) children have recovered completely and 5 have developed CKD; stage I in 2 and stage II in 3. Conclusion: Prognosis following AKI was excellent in our patients, probably because of lack of multiorgan dysfunction. HUS was the commonest cause and urgent renal imaging needed when obstruction is suspected. Oliguric patients are more likely to require dialysis and need early referral to the regional unit. All cases should have long-term follow up to ensure renal recovery and detect delayed complications.

Clinical course and outcome of Acute kidney injury (AKI) due to childhood Haemolytic Uremic syndrome (HUS): A single centre experience Shivaram Hegde, Sabina Pahari Paediatric nephrology, University Hospital of Wales, Cardiff, UK

Objective: Clinical data, along with the management and outcome of 15 children with AKI due to HUS, caused by Shigatoxin-producing Ecoli (Stx-HUS) are described. Method: We analysed the data of children with Stx-HUS induced AKI, managed in our unit over the last 5 years. Outcomes noted as complete recovery, residual renal injury, dialysis dependency or death. All patients were followed up at least for a year and further monitoring continued until their renal function normalised and any proteinuria or hypertension resolved. Methods and Results: Stx-HUS was the commonest cause of AKI in our unit, accounting for 15 of the total 38 cases (39.5 %) treated for AKI during this period. 11 patients were less than 10 years of age. All children presented with blood in stool and 11 with oligoanuria. All showed microangiopathic haemolytic anaemia and thrombocytopenia. Positive stool cultures (for Ecoli 0157) were obtained in 11 and 4 had Ecoli 0157 lipopolysaccharide serum antibodies. 10 patients required dialysis (peritoneal dialysis in 7, haemodialysis in 1 and both modes in 2) and one child needed plasma exchange. The remaining responded to supportive management. Morbidities encountered included bowel perforation (1), hypertension (2), seizures (2) and diabetes mellitus (1) and three of these children needed intensive care management. At discharge there were no deaths, none with dialysis dependency or complete renal recovery but all showing improving renal function. At 3 months we found normal renal function in 12 (80%) and chronic kidney disease (CKD) stage II in 2 and CKD stage III in 1. A 13 year old girl with an unknown myopathy presented for the third time with clinical and laboratory features of rhabdomyolysis. On day two of admission, her renal function deteriorated with decreased urine output, increasing creatinine of 380 μmol/l and CK of 240,220 IU/l. She was commenced on haemodialysis and started on calcium supplements, as her calcium levels were 1.6 mmol/l. Three week into her illness, she was symptomatic with high blood pressure of 170 mm of Hg leading to a generalised seizure. Her serum calcium was 4.01 mmol/l. The CT brain did not reveal any evidence of haemorrhage or infarction. There were no other trigger factors identified for the seizure and the hypertension, apart from the hypercalcaemia. The hypercalcaemia was managed with low calcium dialysate, calcitonin and Sevalamer. Despite the above treatments, she continued to be hypercalcemic and developed erythematous palms and soles and an injected conjunctiva. In view of the refractory hypercalcaemia, She received two doses of intravenous Pamidronate (0.5 -1 mg/kg) when the creatinine was 317 mmol/l. Following this, her calcium levels normalised to 2.59 mmol/l. Her renal function improved and was discharged a week later. There was no nephrocalcinosis seen on follow up. Bisphosphonates are used in children with caution, as there is little evidence on its safety and efficacy. They act by binding to the surface of calcium phosphate crystals and inhibiting osteoclast formation, aggregation and dissolution. It is nephrotoxic causing acute tubular necrosis and collapsing focal segmental glomerulosclerosis. Pamidronate is usually used in children with chronic kidney disease but this was the first time it was used in a child recovering from acute kidney injury secondary to rhabdomyolysis. There were no complications with the use of pamidronate in our patient.

Chronic kidney disease during long-term follow-up in children treated with neonatal extracorporeal membrane oxygenation: do we need to worry? Objective: Acute kidney injury (AKI) is a common complication in children receiving extracorporeal membrane oxygenation (ECMO) support. As AKI may cause loss of a significant number of functioning nephrons, these children are at risk of developing Chronic Kidney Disease (CKD) post-ECMO. Therapeutic interventions might be needed to prevent further renal function deterioration or comorbidity of CKD in these patients. The objective of our study was to determine the prevalence of CKD during long-term follow-up (FUP) of children treated with ECMO.

Methods: This was a cross-sectional study performed between 2010 and 2013. All children previously treated with neonatal ECMO who visited our FUP clinic at the age of 1, 2, 5, 8, 12 and 18 years were screened for CKD. If more check-ups were available per patient, only the latter one was used for the study. CKD screening included height (Ht) and blood pressure measurements (BP), and laboratory parameters including serum creatinine (SCr) (Schwartz formula [0.413*Ht (cm)/SCr (mg/dl)] or MDRD formula were used to estimate GFR) and urinary protein/creatinine (uP/C) ratio. CKD was suspected in patients with hypertension (>95th percentile of reference values according to height and age), abnormal eGFR (<90 ml/min/1.73m 2 ) or proteinuria (uP/C ratio >30 mg/mmol creatinine). Patients were excluded if SCr was lacking.

Results: To date, 132 children visited the FUP clinic. Of these, 4 (3%) were excluded because of pre-existent kidney disease and 6 (5%) because of missing SCr data. Hence, 122 children (44% female) were screened for CKD. The number of patients per FUP age category was 17 <5 years, 56 between 5-12 years, and 49 >12 years. BP was within normal ranges in all children. In 13 (11%) children either an abnormal uP/C ratio or eGFR was observed. uP/C ratio was increased (median 36 [IQR 32-38 mg/mmol creatinine]) in 8 (7%) children, of which 1 was explained by low muscle mass. Only 5 (4%) children had an abnormal eGFR but all >60 ml/min/1.73m 2 .

Conclusion: The prevalence of CKD and its clinical implication in children previously treated with neonatal ECMO seems to be limited. Future research will focus on identifying risk factors for CKD following ECMO support.

Abstract# P-SAT336 10 years clinical retrospective analysis in children of acute poisoning inpatient

Man Jiang, Qiu Li Nephrology and immunology department, Children's hospital of Chongqing medical university, Chongqing, China

Objective: Acute poisoning is the common critical and emergency disease in children. Since childhood is the special life stage with continuous growth, clinical features are different from the adult and changes were happened in resent years. The change rules, clinical features, treatments and the outcomes of acute pediatric poisonings inpatients were investigated in this article. Methods: Retrospective evaluated the 1005 cases of pediatric acute poisonings admitted to the Children's Hospital of Chongqing Medical University. Cases were divided into 5 groups by ages, or divided into 2 groups based on different origins (urban or suburban), different causes and routes of poisonings in different groups were calculated. The clinical manifestations, treatments and prognosis in acute intoxication were also studied. Results and Conclusion: Pediatric acute poisoning mainly happened in 1-4 years old children (50.02%), there was no statistical difference between genders. The common causes of poisonings were pharmacological poisoning (26.07%), food poisoning (19.50%), animal bites and stings (16.92%) and pesticide poisoning (14.73%). In the resent five years, Pharmacological poisoning rose to be the top reason of poisonings, and was mainly composed by children≤3 years old (P < 0.001),and urban children were more than children from suburban(P < 0.001). Neurological and psychiatric drugs were the most common (32.06%) in drug poisoning. Rural children with animal bites and stings, pesticide poisonings and rodenticide poisonings were more than the urban children (P < 0.001, P < 0.001, P < 0.05). The main poisoning causes in 6-12 years old group and >12 years old group children were food poisoning (29.20%) and pesticide poisoning ( Objective: Little is known about cardiac surgery-associated acute kidney injury (CS-AKI) in children in developing regions of the world.

The study aimed to determine the prevalence of CS-AKI, associated factors and its impact on mortality and utilization of hospital services.

Methods: Hospital records of children aged 0-17 years that underwent cardiac surgery (other than device closure procedures and pacemaker insertion only) at an Indian hospital between 2011 and 2012 were reviewed. CS-AKI was defined as a rise in serum creatinine of ≥0.3 mg/dl in any 48 hours and or by urine output less than 0.5ml/kg/hr for an 8-hour period in the first 5 days after cardiac surgery.

Results: The study included 323 children with a median age of 1 year (0.04-17), of which 22 (6.8%) were neonates and 18.3% had single ventricle. About 60% of the children had RACHS-1 1 or 2 interventions. CS-AKI occurred in 39 children (12.1%), most often in the first 48 hours after cardiac surgery. On univariate analysis CS-AKI was associated with sepsis and intra and post-operative hypotension. In-hospital mortality was 6-fold higher in children who developed CS-AKI. CS-AKI was associated with 2-3 days longer duration of mechanical ventilation, inotropic support and ICU stay. Conclusion: CS-AKI occurs in children in developing countries but at a lower frequency mainly due to older children with less complex congenital heart disease undergoing cardiac surgery. CS-AKI was associated with higher in-hospital mortality and increased utilization of hospital services. Factors associated with CS-AKI included intra and post-operative hypotension and sepsis.

Objective: Data on long term effects of newborns after acute kidney injury is limited. The aim of the study was to evaluate long term effects of acute kidney injury (AKI) in neonatal period.

Methods: Inclusion criteria were as follows: oligo-anuria or plasma creatinine >1.5 mg/dl during first three postnatal days or >0.7 mg/dl after third postnatal day or ≥25% increase in plasma creatinine within 48 hours during hospitalization in neonatal period. Three hundred sixty children who had AKI during neonatal period (between January 2000 and December 2009) and survived to hospital discharge were invited; 106 of them accepted to participate. Patients' characteristics during AKI and during follow-up were recorded; a physical examination and laboratory studies, including ACR (urine microalbumin/creatinine ratio) were performed. Schwartz formula was used to estimate GFR; hyperfiltration was defined as a GFR >160 ml/min/1.73 m 2 . Hypertension was defined as blood pressure ≥95 th percentile for age, gender and length.

Results: The mean age on evaluation was 6.78±2.9 years. 15.8-15.8-12 .2% of patients. All these children were dialyzed for more than 7 days on acute phase of the disease. At time of discharge from hospital proteinuria remained in 53 (85.5%), hypertension -in 47 (75.8%) patients. After 1 year, 5years and >10 years period proteinuria was detected accordingly to 26.3-7.9-33.3%, hypertension to 11.3-6.5-12.9% of patients. After >10 years from the onset of disease hypertension was detected more often in the group of children who were < 1 years old on acute phase of the disease (40% vs 11.1%, p=0.05). In the group of patients who hadn't proteinuria on the acute phase, after >10 years period proteinuria was observed to 25% of them. After 1 year period renal impairment of various degree was observed to 26.3%, after 5 years -to 39.5 %, after >10 years-to 33. . On admission his mean arterial pressure was 35 mmHg, his creatinine was 5.8, potassium 6.5 and sodium 121. Usual medical management for AKI was initiated but even by late D5 he continued to be anuric with a rising creatinine. His uric acid was grossly elevated at 14 mg/dl. Dialysis was considered but as the parents were not in favour of it they were offered the option of rasburicase. Rasburicase was given on late D5 and within 12 hrs he started to produce urine which peaked at 4 ml/kg/hr by D9. The creatinine also showed a concomitant fall and by discharge (D10) it was 2.1 mg/dl and normalized at 0.5 mg/dl by D30. Uric acid was 0.5 mg/dl on D7 and 2.8 mg/dl on D9. Case 2: 6 years old boy with multiple special needs was transferred from another hospital intubated, anuric and in multi organ failure (MOF). Creatinine was elevated at 3.7 mg/dl and uric acid was also found to be raised at 9.1 mg/dl. In view of MOF along with Disseminated Intravascular Coagulopathy (DIC) dialysis was considered a risky process and parents were offered the option of rasburicase. Rasburicase was given on D2 of admission and although the repeat uric acid was 0.3 mg/dl and he did produce 5 ml of urine within 6 hours no further improvement in renal parameters was noticed. Dialysis (SLED) was initiated on D3. Although the creatinine improved with SLED he continued to be oligo-anuric and died of MOF by D5. Conclusion: Although Rasburicase seemed to work in case1, it was not beneficial in Case 2. The exact mechanism for rasburicase is still hypothetical and the few case reports have all documented positive results. Although it seemed to have worked in Case 1, to the best of our knowledge case 2 is the first documented case wherein it did not work. Proper randomized control trial is needed before advocating rasburicase as a novel therapy for AKI. Results: Age, sex, diagnosis, baseline and post-surgery hemoglobin, total leukocyte count, platelet count and biochemistry were recorded. Baseline and post-operative, urea (mg/dl), creatinine (mg/dl), urine output (ml/kg/hr) and inotrope dose were also recorded daily. The duration of CPB was noted. Post-operatively cardiac, renal, hepatic, neurological and respiratory dysfunctions were recorded. Conclusion: Fifteen (7.2%) children developed AKI stage I, one child (0.5%) developed AKI stage II and four children developed AKI stage III (2%). All patients with AKI had a longer stay in hospital. Eight children required dialysis for AKI; two required dialysis to maintain the fluid balance post operatively. None developed chronic renal impairment. Using stepwise regression, younger age (<1 year), weight < 10Kg, pump failure, sepsis and duration of CPB more than 60 minutes were significant risk factor for developing AKI. Results: most of the patients were male, with less than 28 days old and weighing less than 5kg.In the majority of cases (77%), ARF was diagnosed in the first 6 days after surgery, demonstrating influence of the procedure itself. All of them were treated by peritoneal dialysis(PD) for a mean time of 255 hours; 58.3% of them died. There was no statistical significant difference between age, time on extracorporeal circulation and duration of dialysis, comparing the patients who survived and the deceased ones. Conclusion: Acute renal failure is a common complication in children with congenital heart disease and PD is a safe dialytic method. The mortality rate is high and influenced by aspects related to the child, underlying disease, type of surgery and many other associated aspects.

Abstract# P-SAT344 Acute Kidney Injury epidemiology and associated factors in a pediatric intensive care unit. Conclusion: Acute kidney injury (AKI) is associated with significant morbidity and mortality in pediatric patients with critical illness. The main causes associated to AKI were sepsis and shock. We found no association with cardiac surgery, probably explained by the low level of complexity that is performed in our center. Almost half of our patients with AKI were in Failure and the mortality was high as previously reported. Objective: Fibroblast growth factor-23 (FGF-23), a phosphaturic hormone involved in calcium phosphate homeostasis, appears to predict renal disease progression in adults with non-diabetic CKD (Fliser, JASN 2007) . This study aimed to determine renal survival according to FGF-23 serum levels in children with CKD stage II-IV. Methods: 232 children participating in the ESCAPE trial (age 11.5±4 yrs, GFR 45±18 ml/min/1.73m 2 ; underlying renal disease: hypo/dysplasia (71%), glomerulopathies (12%), hereditary or other (17%)) were analyzed. All patients received fixed dose ACE inhibition and were followed prospectively by 2-monthly examinations for up to 5 years. The study endpoint was defined by eGFR loss >50% from baseline, GFR <10ml or start of renal replacement therapy. FGF-23 levels were determined at baseline (C-terminal human FGF-23 ELISA (Immutopics, San Clemente,CA,USA Objective: The long-term outcome of patients born with unilateral renal agenesis (URA) and of those who underwent therapeutic unilateral nephrectomy (UN) remains a topic of concern and debate. Children with a solitary functioning kidney (SFK) have an increased risk of developing hypertension, albuminuria and chronic kidney disease in later life. The purpose of this study was to identify whether plasma symmetric dimethylarginine (pSDMA) is an useful biomarker reflecting the level of renal injury in children with solitary functioning kidney (SFK). Methods: We measured circulating pSDMA in 51 patients with SFK and no other urinary defects. Patients were subdivided for two groups: primary SFK (pSFK)-unilateral renal agenesis (URA) and secondary SFK (sSFK) after unilateral nephrectomy. The control group (C) consisted of 21 healthy children, aged mean 9.92 ± 4.85 yrs. Immunoenzymatic ELISA commercial kits was used to measure pSDMA concentration. Data analysis was performed using computer program Statistica 9.0. Results: The age and sex of studied children did not differ from healthy controls (p> 0.05). Plasma SDMA levels in SFK children were higher than in healthy participants (p< 0.05). There was no difference in pSDMA concentrations between pSFK and sSFK patients (p> 0.05). SDMA plasma levels correlated significantly with C cr (r= -0.32, p< 0.01) in all participants. ROC analyses performed in order to define the diagnostic efficiency of serum creatinine and pSDMA in identifying children with C cr < 90ml/ min/ 1.73m 2 among SFK and healthy participants revealed no difference between all two AUCs (p> 0.05).

Conclusion: In children with a solitary functioning kidney increased pSDMA levels were observed, however the sensitivity and specificity of this marker in detecting the decrease in C cr was not better than creatinine. Objective: Renal osteodystrophy encompasses a variety of skeletal disorder ranging from high turnover lesions of secondary hyperparathyroidism (SHPT), to low turnover lesionsof diverse etiology that are usually associated with normal or reduced PTH levels. So our aim was to assess by DEXA which is a precise , rapid and noninvasive procedure the degree of osteopenia in patients with CRF. Methods: 53 children aged (11-3.84) years, 17 on conservative treatment and 36 on hemodialysis were included in the study BMD of lumbar spine and wrist were measured by (DEXA) and compared with age and sex matched controls.

Results: shows that out of 53 patients, 25 (47%) are osteopenic 22 (88%) on hemodialysis and 3 (12%) on conservative treatment, of these 13 (24.4%) had severe osteopenia as regard BMD of the spine, while DEXAwrist shows 11 (21%) are osteopenic, 7 (64%) on regular hemodialysis and 4 (36%) on conservative III, of these 3 (5.66%) had severe osteopenia ,correlation between Z-score spine in the osteopenic group and different biochemical parameters shows non-significant correlation except -ve correlation with duration and age of the patients, while Z-score wrist of the same group shows +ve correlation with bicarbonate. our result shows that group with IPTH >= 200 pg/ml are more osteopenic than those with lower IPHT levels, although difference did not reach level of statistical significance P > 0.05. Conclusion: We can conclude that osteopenia is frequent in patients with CRF more in the dialyzed group, with longer duration of the disease, older age and severe acidosis, irrespective of the severity of the disease. Although, degree of osteopenia is not correlated with biochemical findings of (SHPT) but still patients with (SHPT) are more osteopenic and have lower cortical bone density. Mineral and bone disorders were defined according to K/DOQI guidelines.

Results: During the study period 95% of stage I-IV CKD patients and all ESRD patients and renal allograft recipients had at least one type of MBD. High turnover bone disease with hyperphosphatemia, hyperparathyroidism and hypocalcemia was the most frequent type of MBD in stage I-IV CKD and ESRD patient. Adynamic bone disease was seen in only one ESRD patient. Hypophasphatemia was the most common disorder in renal allograft recipients. Vitamin D deficiency/insufficiency was seen in 55% of stage I-IV CKD, 65% of ESRD patients and all renal allograft recipients. All patients with stage I-IV CKD and ESRD were treated with calsitriol and renal allograft recipients were treated with vitamin D3 supplementation. Conclusion: Mineral and bone disorders especially high turnerover type is common in children with CKD and ESRD. Although successful kidney transplantation corrects many of the metabolic abnormalities associated with the development of MBDs, renal allograft recipients had increased risk of vitamin D deficiency/insufficiency. Objective: Calciphylaxis is a complication of chronic kidney disease that characterized by necrotic lesion in the skin and in histological examination reveals microcalcification of medium sized blood vessels. Materials and Methods: We report on a 21-month old girl with end stage renal disease due to diffuse mesangial sclerosis referred for Tenckhoff catheter implantation. Laboratory tests on admission were: BUN = 150 mg/dl, Cr= 5 mg/dl, uric acid = 10 mg/dl, calcium= 4 mg/dl, phosphate = 40 mg/dl, alkaline phosphatase= 1017, PTH= 700 pm/l,VITD= 21 mg/ml. Calciphylaxis process started when she received calcium gluconate IV the day before surgery and continued progressively when oral calcium carbonate and oral calcium gluconate were given. Hourly peritoneal dialysis was started and all calcium containing medications were stopped. But the process continued progressively. Surgical detriment of the necrotic tissue of right wrist was done. Comfeel dressing had no effect and the circulation of tissue was poor. Therefore the left wrist was kept as control. Pamidronate 0.5 mg/kg/day prescribed for 6 days and then once a week for 5 weeks. After one week of starting pamidronate skin lesion began to heal, circulation improved and after six weeks all skin lesions completely recovered. As shown in figure one debridement cause skin scar on right wrist. Conclusion: Pamidronate is effective to stop calciphylaxis in children with advance renal insufficiency and severe calcium-phosphate imbalance. Medical or surgical debridement are not suggested and lesions will recover without scar by pamidronate Objective: Fibroblast growth factor 23 (FGF23) is a key player in kidney-bone axis and regulation of calcium and phosphate homeostasis. Most of the recent research has focused on FGF23 in chronic kidney disease and there is paucity of data in healthy children. Hence we initiated a study on the various biomarkers of calcium-phosphate metabolism including FGF23 in healthy children and present here the interim report of our findings as of now.

Methods: A total 100 healthy school children and adolescents aged 6-16 years, were enrolled in this cross-sectional study, after a written consent from their parents. Height, weight and pubertal staging were assessed. Blood was collected and calcium, phosphate, PTH and vitamin D were estimated by standard methods. Plasma C-terminal fragment of FGF23 was quantitated using a commercial ELISA kit (Immutopics, USA). Three day dietary recall was done to calculate mean daily calcium, phosphate and protein intake.

Results: Of the 100 children enrolled 60% were males. The mean age was 12.3 yrs, mean height was144 cms and mean weight was 34kgs.

The mean blood biochemistry values of the children tested so far for Ca, P, PTH and vitamin D shown in table 1. The parameters of the children were normal for their respective ages however FGF23 levels were found to be lower compared to reports in literature.

Conclusions: From the results available as of now all of the children had normal biochemistry for their ages. The FGF23 levels were found to be lower than reports in the literature. Whether it can be attributed to the dietary differences from the children of the west needs further investigation. Testing of the entire cohort will reveal if this lower FGF23 is a universal phenomenon among all children in our study. On the contrary, all DXA parameters showed a decreased in their mean Zscore BMD values that reached statistically significance. Finally, multivariate stepwise regression analyses showed that estimated glomerular filtration rate at the beginning of the study was the best predictor of the difference in BMD Z-scores measured at lumbar spine. Additionally, values of iPTH at the beginning of the study and the change of iPTH throughout the study predicted the 72.3% of the difference in Z-score of SOS measured at radius with an inverse relationship. Conclusion: The use of two different techniques have shown bipolar changes of bone properties after renal transplantation. Reversal of renal osteodystrophy and consequently correction of many of the underlying metabolic abnormalities, mainly normalization of iPTH, strengths cortical bone as this is best illustrated by QUS assessment. However, reduction in BMD, is predominantly considered as an adverse effect of steroid immunosuppression. age 3.3 yr) (group 1), 33 patients with CKD stage 5 on PD or Hemo (mean age 9 yr, mean time on dialysis 2 yr) (group 2) and 29 patients with CKD stages 2-4 (mean age 7 yr, mean GFR 37 ml/min/1.73 m 2 ) (group 3). 25OHD levels were deficient in 20% and insufficient in 35% of the group 1 patients, 48% were deficient and 15% were insufficient in group 2, and 7 % were deficient and 17% were insufficient in group 3. Results: Intact PTH (iPTH) was above the target level by different CKD stages in 52 % of the group 1 patients, in 58% of the group 2 and in 52% of the group 3 and showed an inverse correlation with 25OHD levels (p<0.05, r=−0.25). There was inverse correlation with age in the complete cohort of patients (p=0.0005, r =−0.33) Height standard deviation score (SDS) was associated with 25OHD only in group 2 (p=0.025, r=0.45). There was direct correlation between 25OHD levels and GFR in group 1 and 2. Conclusion: Our data suggest that 25OHD deficiency is common in pediatric renal transplant and CKD children of any stage even in an area with year-long sunshine, especially in adolescents. Hyperparathyroidism was also frequent in all groups. It would be advisable to monitor vitamin D status in these patients. 3) years, 28 with CKD 5 (2 on HD, 9 on PD and 17 Tx) mean age 13 (5.4-17.5) and 113 healthy children, mean age 11.5 (5-17.9 Objective: The aim of our study was to analyze twelve single nucleotide polymorphisms (SNP) of gene LRP5 potentially associated with osteoporosis risk in children with routine steroidotherapy in the course of the idiopathic nephrotic syndrome. Methods and Results: Glucocorticosteroids are important risk factors for drug induced osteoporosis. Decrease in bone mineral density (BMD) and increased risk for pathological fractures are caused by direct division of steroids at the cellular level by inhibiting the replication of osteoblasts and stimulation of its apoptosis. Also proven its effects on the inhibition of type I collagen synthesis. LRP5 is one of the Wnt signaling pathway proteins coreceptors involved through the RANK-RANKL system in regulation of the osteoblasts function. LRP5 is included to the osteoporosis phenotype genes group and its selected single nucleotide polymorphisms can be responsible for BMD decrease in patients with glucocorticoid therapy. The study group was composed of 38 children with idiopathic nephrotic syndrome, 21 boys and 17 girls beetwen the age of five to twelve years old , 14 with osteoporosis and 24 with normal bone mass density. Study also included the control group consists of 102 healthy individuals at the same age not treated with glucocorticoids. The analysis was carried out with polimerase chain reaction (PCR) and TaqMan molecular probe designed for 12 single nucleotide polymorphisms (SNPs) nearby investigated mutations in LRP5 gene that can be connected with osteoporosis fenotype. Odds ratio value (OR) was based on frequencies of single nucleotide polymorphisms in LRP5 gene and its haplotype analysis.

The results showed significant differences in OR value among the three groups. It was also found the differences in the LRP5 gene structure. Based on Gabriel algorytm single nucleotide polymorphisms pairs analysis in children with osteoporosis and nephrotic syndrome we proved the correlation between selected SNPs pairs presence and decretion of bone mineral density in studied group. Objective: Growth hormone (GH) and insulin-like growth factors are essential for normal growth and development. Chronic renal failure (CRF) results in major changes in the circulating growth hormone /insulin-like growth factor (IGF) system. Our aim isto study: To assess clinical and laboratory parameters of growth and osteodystrophy including IGF1 and IGFBP2 as part of the somatotropic hormone axis in Egyptian children suffering from CRF on conservative therapy. Methods: 62 Egyptian children (47 boys and 15 girls) with a mean age of 9.7y (0.47 to 21.12y) suffering from CRF on conservative therapy and 21 controls were included in the study. Ht, wt and TSF were measured ,pubertal staging was assessed and followed up for a period of 6 months. At the end of the follow up period serum for IGF1 and IGFBP2, renal function, electrolytes, Ca, P ,and alkaline phosphatase and acid base balance were measured and an X-ray of the left hand and wrist was done to determine their bone age by Tanner and Whitehouse. Results: Our study shows that Children suffering from CRF in Egyptian conservative therapy have growth retardation with a mean ht of -3.7 SDS, a mean wt of -2.24 SDS. TSF mean was -1.3 SDS. On the average the patients had a delay of 2.95y (+/-2.0) in their bone age. Their height was retarded more than their bone age with a height age/bone age of 0.8 (+/-0.18). Alkaline phosphatase as a markers of renal osteodystrophy is significantly correlated to the height, height age , bone age and to the pH. The mean IGF1SDS (-0.6 +/-1.8) did not differ from that of controls while the mean IGFBP2SDS (2.4 +/-4.6) was significantly higher in patients with CRF than in controls. Height and weight were significantly correlated to IGF1 but not IGFBP2. There is a significant correlation between IGFBP2 level and the glomerular filtration rate. Conclusion: The imbalance between normal insulin-like growth factor-I (IGF-I) and markedly increased IGFBP2 plasma levels plays a pathogenic role for growth retardation in children with chronic renal failure. The lower the GFR the higher the IGFBP2 level. The latters inhibitory action may provide hope for improving growth in cases of CRF by reducing the level of IGFBP2 or displacing IGF1 from it. Objective: Anemia is one of the most common complications of chronic kidney disease (CKD) and renal transplantation. This study evaluated the prevelance and the etiology of anemia in children with stage I-IV CKD, end-stage renal disease (ESRD) and renal allograft recipients.

Methods: Between 2010 and 2012, we prospectively followed 21 pediatric stage I-IV CKD, 20 ESRD patients and 27 renal allograft recipients. Anemia was defined according to K/DOQI guidelines as Hb is less than the fifth percentile of the normal, adjusted for age and sex. Results: During the study period52.3% of stage I-IV CKD, 100% of ESRD patients and 48.1% of renal allograft recipients developed anemia. The mean Hb levels were 11.30+/-1.72 g/dl, 9.62+/-1.94 g/dl and 11.95+/-1.88 g/dl in stage I-IV CKD, ESRD patients and renal allograft recipients, respectively. The mean Hb levels were significantly lower in ESRD patients as compared to stage I-IV CKD patients and renal allograft recipients (p<0.0081, p<0.002). Erythropoetine (EPO) deficiency was the most frequent cause of anemia in stage I-IV CKD and ESRD patients, followed by iron deficiency. In addition to EPO and iron deficiency, bone marrow suppression and parvovirus infection were the causes of anemia in renal allograft recipients. Erythropoietic stimulating agents were used in 27% of stage I-IV CKD, 100% of ESRD patients and 23% of the renal allograft recipients. Conclusion: Although anemia is one the most common problems seen in children with CKD and ESRD, the prevelance is also high in renal allograft recipients. These patients should be evaluated and treated accordingly to the underlying causes.

Abstract# P-SAT375 Determinants and prevalence of anaemia among preschool children in highly focussed states of India Results: About more than 60 % child is anaemic among all EAG States. The prevalence of severe anaemia is 6.7 % in Rajasthan followed by Uttar Pradesh (3.6 %) and Madhya Pradesh (3.4 %). Those children aged 12 to 17 months are 7 times significantly more likely to be severe anaemic as compare to the 36 to 59 months aged children. Those mothers have severe anaemia, their children are also found to be severe anaemic (16 times more than not anaemic mothers). Mothers who are highly educated and belong to richest quintile their children are less likely to be anaemic.

The finding of the study shows that demographic and socio-economic indicators play significant role in determining the prevalence of anaemia in EAG states which needs focused programme to reduce the prevalence of anaemia in preschool children.

Abstract# P-SAT376 Impaired renal growth hormone mediated JAK/STAT5 signaling in juvenile rats with chronic kidney disease Objective: Linear growth retardation is a major problem in children with chronic kidney disease (CKD) and is ascribed to GH insensitivity. Treatment with exogenous GH has been accepted as standard therapy in children with CKD and short stature. However, concerns have been raised in the past on the potential fibrogenic effects of GH. There is no information regarding renal GHR signaling pathway in CKD. Methods and Results: To investigate this pathway, surgically 5/6 nephrectomized (CKD) and pair-fed control (C) juvenile (3 wk old) rats were sacrificed after 2 weeks of CKD. Serum creatinine and albumin excretion were significantly elevated (associated with glomerulomegaly and early fibrosis) while body weight and length gain were reduced in CKD rats. Serum IGF-I levels were decreased in CKD, even though serum GH levels were unchanged. Kidney GHR mRNA and protein levels were reduced and phosphorylation of JAK2 and STAT5 was significantly impaired. Supressor of cytokine signaling (SOCS3) mRNA was increased in association of increase in renal IL6 mRNA. Renal IGF-1 mRNA was unaltered in CKD. Thus, in the remnant kidney of CKD growth retarded juvenile rats there is impaired GH mediated JAK2/STAT5 signaling. This defect may be due to a reduction in GH receptor expression and signaling together with an increase in SOCS3 expression. Conclusion: We suggest that the insensitivity of the remnant kidney to GH may serve to protect against the potential adverse renal effects of exogenous GH in CKD patients. Objective: Concerns have been raised about possible adverse effects of growth hormone(GH) in short children with chronic renal diseases. Six cases of short children with chronic renal diseases CKD were studied in our hospital, the microalbuminuria as a sensitive parameter of early glomerular damage was detected during the treatment of recombinant human growth hormone. The individual growth response to GH was also observed. Methods: Of the 6 cases, male were 4 , female 2, the onset age ranged from 4 to 14 years old, all were diagnosed as growth retarded children with various chronic renal diseases. The dosage of GH was 0.1 IU/kg/d. The follow-up period was from 3 months to 1years. Microalbuminuria was measured by a commercially available ELISA kit, as a sensitive parameter of early glomerular damage in children being treated with recombinant human growth hormone. Serum insulin-like growth factor IGF-I concentration and IGFBP3 were also measured before and during the treatment with GH.

Results: There was no statistically difference on the level of microalbuminuria compared with that of pretreatment. Linear growth were 1.0 cm/month with an increment in height SDS by 1.5 at the end of the first year of GH treatment. Growth retardation in children with CKD is associated with normal to slightly low concentrations of (IGF)-I and IGFBP3. Objective: Iron deficiency anaemia is common in chronic haemodialysis patients and is the most common factor of resistance to erythropoeitin treatment. KDOQI Clinical Practice Guidelines and Recommendations for Anaemia in CKD suggest maintaining Hb levels between 11-12 g/dL, TSATS >20% and serum ferritin above 100 ng/ml. Our unit's previous practice provided 1-2 mg/kg/week of elemental iron intravenously to our haemodialysis population and monitored blood parameters monthly. From July 2012, we changed our practice to withhold routine administration of IV iron, unless blood parameters (monitored fortnightly) suggested iron deficiency. We assessed the change in our practice in safely reducing the frequency of IV iron administration in our haemodialysis population.

Methods: We included all 9 children attending our haemodialysis unit (July 2012). We used %hypochromia of >4%, correlating with Hb level of <11 g/dL as a marker for initiating IV iron sucrose (3mg/kg/week) until improvement of the above parameters. Serum Hb and %hypochromia were monitored at the start of change-over and fortnightly thereafter. All patients received routine erythropoietin at recommended doses of between 50-300 units/kg/week. Results: At initiation of change-over, there were 9 patients with mean Hb 11.3 g/dL, mean %hypochromia 1.2% and mean TSATS 27.7%. From July 2012 -Mar 2013, four patients required IV iron therapy (mean Hb 9.2 g/dL, mean %hypochromia 6.2%, mean TSATS 16.3%). Three patients had 4 weeks and one patient had 7 weeks of IV iron sucrose 3 mg/kg/week to achieve normalisation of serum Hb (mean 11.5 g/dL), %hypochromia (mean 2.73%) and TSATS (mean 26%). At time of reporting (Mar 2013), there were 7 patients with mean Hb 11.22 g/dL, mean %hypochromia 2.07% and mean TSATS 28.2%. Two patients received transplants.

Conclusion: A novel change in our practice has resulted in a safe reduction of IV iron therapy in our population of children undergoing haemodialysis. From our findings, we suggest avoiding routine iron infusions in paediatrichaemodialysis, which is unlikely to have an adverse effect on the incidence of anaemia and will reduce the risk of inadvertent iron overload.

Abstract# P-SAT380 Reversible portal ascitis after bilateral nephrectomy in infant with polycystic kidney disease We report here on a newborn with moderate renal failure and severe hypertension due to autosomal recessive polycystic kidney disease (ARPKD). Antihypertensive therapy associating minoxidil, labetalol, amlodipine and lasilix were not able to control adequately blood pressure. Due to massive kidney enlargement, inadequate respiratory function and inability to tolerate enteral nutrition, parenteral nutrition was initiated at 2 months to maintain adequate calories intake. At 4 months of life, the infant presented severe viral pneumonia complicated by cardio respiratory arrest . He required cardio respiratory resuscitation and hospitalization in ICU for several days. Unilateral right nephrectomy was decided after this severe complication in order to avoid similar accidents due to massive kidney volume and to improve respiratory and nutrition status. One week after unilateral nephrectomy, we noted rapid increase volume of the left kidney and important ascitis having the biological characteristics of portal hypertension. Repeated aspiration of more than 100 ml of portal ascitis was performed every day. In parallel daily perfusion of albumin for more than 3 weeks and complete parenteral nutrition did not succeed in treating ascitis. Faced to resistant ascitis and persisting difficulties in maintaining enteral nutrition and adequate respiratory ventilation , left nepherctomy was decided and performed. Automated peritoneal dialysis was rapidly initiated in our anuric infant (weight after binephrectomy4kgs). Portal ascitis disappeared in 48 hours with rapid normalization of respiratory ventilation and return to normal and well tolerated enteral nutrition. Conclusion: To our knowledge, this is the first case reported of portal ascitis noted after palliative unilateral nephrectomy to facilitate pulmonary expansion and gastrointestinal function in ARPKD.

Ascitis was resistant to daily albumin perfusion and repeated aspirations but was completely reversible after bilateral nephrectomy and initiation of peritoneal dialysis. Early bilateral nephrectomy at 2 months of life was probably the ideal choice to avoid many complications in our infant due to abdominal and thoracic compression by massive kidney compression. Ethical considerations, therapeutic possibilities, and parent consent should be discussed in such challenging and very difficult cases.

Abstract# P-SAT381 Serum hepcidin 25 levels and anemia in pediatric chronic kidney disease Objective: Hepcidin restricts the availability of iron from its stores for erythropoiesis resulting in a functional deficiency of iron and hyporesponsiveness to erythropoiesis stimulating agents. The objectives were to estimate the serum hepcidin 25 in children with chronic kidney disease stage II to IV and correlate it with hemoglobin, iron status (serum ferritin and transferrin saturation), inflammation (C reactive protein) and estimated glomerular filtration rate (eGFR). Methods: It was a cross sectional study. Children aged 2 to 18 years with CKD stage 2-4 on stable dose of iron and or erythropoetin for the last 4 weeks were included. Children with anemia due to other causes, severe acute infection, chronic cardiac or respiratory conditions and those who received parenteral iron and or blood transfusion in last 4 weeks were excluded. Hemoglobin, serum ferritin, transferrin saturation, C reactive protein were assessed. Serum hepcidin 25 levels were estimated using a ELISA based kit in the cohort as well as in 15 age and sex matched normal children to estimate the levels in normal population. Multivariate regression analysis was used to assess the relationship between hepcidin and hemoglobin, iron status, inflammation and eGFR. Results: Forty children were recruited into the study. The mean age of children was 9.1+ 4.5 years. Around 30%, 28% and 42% of children had CKD stage II, III and IV respectively. The mean serum hepcidinlevels in children with CKD (55.87+14.27ng/ml) was significantly higher than controls (11.17+4.38ng/ml). Conclusion: Serum hepcidin levels showed an increasing trend with decrease in eGFR, but this was not statistically significant. The mean haemoglobin was 10.76+1.59g/dL, the mean ferritin was 43.27+38.3ng/ml , the mean transferrin saturation was 21.68+11.5%. Anemia and absolute iron deficiency was seen in 100% of our cohort and worsened with increasing stages of CKD. serumhepcidin levels did not correlate significantly with haemoglobin, ferritin or with C-reactive protein. Objectives, Methods and Results: Presenting a 16-year old Armenian patient who developed a secondary amyloidosis on top of a familial Mediterranean fever (FMF) and moved to Germany 4 years ago. At the time of the move the patient had already a terminal renal insufficiency and received intermittent hemodialysis. Despite the therapy with colchicine moderate attacks of the FMF occurred with an interval of 4 weeks and inflammatory parameters were permanently elevated. The dosage of colchicine was titrated up to 3 mg per day. As a consequence diarrhea occurred and the dosage had to be decreased. Over the course the amyloidosis progressed and affected all internal organs including heart and bowel. The combination of inflammation, uremia and amyloidosis led to malnutrition (BMI 14 kg/m2). At the age of 16 years the most severe colitis with peritonitis and paralytic ileus occurred. The treatment with colchicine was paused in assumption of a toxic effect. After improvement of the diarrhea but still persisting abdominal pain the colchicine treatment was slowly started again. Shortly afterwards a very severe attack of the FMF developed with an accompanied cardiovascular insufficiency. The treatment with a single high-dose of methylprednisolone and the inhibition of interleukin 1 with anakinra (1 mg/kg, given after dialysis) led only to a short remission. The high-dose methylprednisolone treatment was therefore repeated three times and anakinra was given on a daily basis. Since then no new attack of the FMF occurred and the combination of lowdose colchicine and anakinra improved the general status of the patient The elevated inflammatory parameters including serum amyloid A have almost normalized. Objectives: Invasive aspergillosis is almost exclusively occurs in immunocompromised hosts. The central nervous system (CNS) is one of the most frequent sites of invaziveaspergillozis after the lungs. We report a case of invasive Aspergillosis in a boy with end stage renal failure. Methods and Results: A 15-year-old boy was admitted to the hospital for abdominal pain, fewer and cough. He suffered from chronic kidney failure (CRF) known for a year and he was on peritoneal dialysis for seven months. He was initially treated with iv. ceftriaxone for lobar pneumonia, intraperitonealceftazidime and cefazolin for peritonitis. The peritoneal fluid was also cultured for fungus and mycobacteria and all cultures were negative. On the seventh day, the patient complained for lower extremity weakness, and bilateral hyperactive deep tendon reflexes and positive babinski reflex were found. Cranial magnetic resonance imaging (MRI) was normal, but the spinal cord was compressed between C7-T5 by a solid mass which was originated from right lung on spinal MRI. Computed tomography-guided tru-cut lung biopsy was performed for the solid mass. Repeated peritoneal fluid culteres were also negative. Two days later, when unconsiousness and convulsions were seen, intraparenchymal multiple hemorrhagic abscesses were found on his second MRI. Typical hyphae of aspergillusfumigatus was present in pathologic specimens and galactomannan test was positive. Cerebral abscesses were evaluated as CNS involvement of invasive aspergillosis. Anti fungal therapy was began immediately as amphotericin B and caspofungin. The peritoneal catheter was removed and continuous veno-venous hemodiafiltration was performed. However his condition kept deteriorating and death ensued on the fourth day of anti fungal therapy. Conclusion: Invasive Aspergillosis may rarely occur in immunocompetent patients and diagnosis may be missed or delayed due to lack of the particular clinical signs. We should be careful about diagnosis of fungal infections in patients with CRF since early treatment before the invasion and dissemination of aspergillosis to adjacent tissues and/or organs offers a higher survival chance for the patient.

questionnaire and to find a way to increase reliability for healthy children aged 8-12 years especially, according to Iranian culture. Objective: We lunch this study to compare the parent and children's behavior problem in pediatric patients suffering from CKD referring Arak Amir Kabir Hospital. Methods: To perform this case control study, we recruit 116 children with CKD and compared them with other 116 non affected children age between 5 to 16 years old. The child behavior checklist CBCL4/18 for child behavior assessment and general assessment function GAF for the evaluation of their parent's behavior were completed by the parent's data was analyzed using qualitative variables and chi-square formula.

Results: Among 116 patients with CKD, 10 case 8.6% showed behavioral problem while this figure was 3 case 2.6% in the control group, denoting a significant difference P-0.04. Moreover 20 children 17/2% in the case group and 9 children 7.8% in the control group had internalizing problem P-0.02. 22 children 19% with CKD and 8 children 6.9% in the healthy group had externalizing problem which was also a significant difference P-0.0003. As a significant P-0.0001 the parent's average stress and behavior scores in case and control were 3.65 & 3.76, respectively. Conclusion: The higher prevalence of behavioral problem in the children suffering from CKD and their parent's functional impairment highlights the important of early treatment and subsequently prevention of future behavioral problem in their sibling.

Abstract# P-SAT391 Development in 6-year-old children with and without CKD

Objective: CKD is a pathophysiologic process with multiple etiologies, resulting in the inexorable attrition of nephron number and function and frequently leading to ESRD. A number of studies have suggested that children with CKD have problem in development than other children our goal was to compare development of children with CKD with normal children within the common age range for the disease.

Methods: In this descriptive-analytical study, we selected 30 children with 6-year-old children whom were diagnosed with CKD as our case group and studied their developmental status by ages and stages questionnaire (ASQ). The control group was selected from 6-year-old children who attended the clinic for reasons other than CKD. Conclusion & Application to Practice: The eleven key areas of responsibility used to measure SC in a periodic evaluation demonstrated a strong correlation to the increasing extent of QPDO. Additionally, as the nurses progressed to becoming expert a direct correlation to the QPDO was notable. The study became the foundation for staff training and developing a competency appraisal framework in renal nursing practice thereby promoting quality assurance procedures while attaining QPDO.

Objective: Urinary incontinence is a child health problem that affect both the child and the family in psychological and social aspect and that leads to a decrease in quality of life of the child and his caregiver. Patients with higher CKD stage had significantly lower QOL score in all domains in the child-self reports, but not parent-proxy reports. Whereas there was no difference between patents with stage I and stage II. According to gender, boys had a tendency to present better QOL than girls, but there was no significant difference between these two groups. Age discrepancy was not a significant factor to decide QOL in children with CKD. In addition, there was significant difference between parent-proxy reports and child-self reports and QOL scores in the child-self reports was significantly higher than in the parent-proxy reports, especially in the domains of emotional, school functioning and psychosocial health score. Conclusion: Residual renal function in children with CKD is a important factor to decide QOL. In addition, emotional and psychosocial support for their parents can be necessary to improve QOL in children with CKD.

Abstract# P-SAT398 Results: The mean period of post-transplant follow-up was 4.7+/-2.1years(range: 0.8-9 years). In the first year of transplantation, 14 (15.2%) recipients had obesity Obese patients had higher serum GGT levels than non-obese patients (21.2+/-6.2 u/L vs 14.9+/-3.8u/L, p=0.01). There was a positive correlation between BMI and GGT and TG levels in obese patients (r=0.61, and r=0.5, p<0.01 respectively). Patients with dyslipidemia were found to have significantly higher mean GGT level (14.2+/-3.6 u/L vs. 19.2+/-6.2 u/L, p=0.016) and BMI (18.6+/-3.4 vs. 29.4+/-6.3 p=0.04). We could not find any correlation between BMI, and gender, post-transplant follow-up, duration of CRF, primary disease, donor status and graft loss. In the first year of transplantation, GFR was significantly lower in obese patients than non-obese recipients (p<0.05) Conclusion: Our data has suggested that, even within its normal range, elevated serum GGT concentrations and hyperlipidemia are closely associated with obesity in transplanted patients and obesity has negative effects on graft functions.

The relationship between the graft function and the serum and urine NGAL in children with renal transplantation

Tehran university of medical science, associated professor, Tehran, Iran

Objective: The evaluation of graft function long term after transplantation is important. Serum creatinine is the most common used marker for graft function in renal transplantation. The level of serum and urine NGAL may predict graft function in renal transplant recipients. Methods: We evaluated 21 pediatric renal transplant recipients by measurement of serum and urine NGAL, serum creatinine concurrently. The GFR of the patients was also estimated by Schwartz formula.

Results and Conclusion: The time after transplantation was 3-12 years (mean: 6.8+/-2.47 years). The mean serum NGAL was 140+/-94.2 ng/ml (15.4-324) . The mean urine NGAL was 17.8+/-20 ng/ml (32-68). We didn't find any association between serum creatinine, GFR estimated by Schwarz and serum NGAL. We also didn'y find any association between urine creatinine, GFR estimated by Schwarz and serum NGAL. Glomerular filtration rate measured by DTPA scan of fourteen patients were available. We also didn't any association between GFR measured by DTPA scan and serum and urine NGAL.

Abstract# P-SAT409 Racial disparities in paediatric kidney transplantation in Australia Glomerulonephritis and late referral were more common in non-Caucasian patients. Among patients who did receive a transplant, Caucasians had overall better HLA matches to their graft and were less sensitised. Conclusion: Caucasian Australian paediatric patients have greater access to kidney transplantation, particularly from living donors, and are likely to have the better graft survival. Some racial disparities may be inevitable due to differences in kidney diseases, however further work is required to understand barriers to live donation and address disparities.

Abstract# P-SAT410 Cost-effectiveness of renal transplantation in paediatric and adult transplant recipients. [1.4-4.8] ), were associated with a lower inscription probability 6 months after reaching ESRD. The national inscription rate was 69% with a significant center variability (median 69% [IQR 51-80]) that remained after adjustment on patient characteristics (p<0.0001). Preemptive transplantation rate explained 19% of the inter-center variability (p=0.06). Probability of transplantation 12 months after inscription decreased with time of waiting list inactivity (p<0.0001) and with the probability of finding a compatible transplant considering patient's HLA and ABO groups. Underweight (BMI <-2SD) was associated with lower transplant probability (OR 0.3 [0.1-0.9]). At 12 months, 81% of patients were transplanted with a significant center variability (median 80% [IQR 72-88] that remained after adjustment (p=0.02). Conclusion: As expected patients' characteristics contribute to intercenter variability as well as centers' attitude towards the inscription of younger children, management of specific primary renal diseases and preemptive transplantation. Despite national allocation rules we found a significant inter-center variability in the probability of being transplanted after inscription on the list, which was not explained by either the patient or center characteristics of our study.

Abstract# P-SAT414 Ten year outcomes of paediatric renal transplantation: the Irish experience Objective: Focal segmental glomerulosclerosis is the most common cause of steroid-resistant nephrotic syndrome in children and adolescents. Within 10 years of initial presentation, 50-70% of patients will progress to end stage kidney disease requiring kidney transplant. Recurrence rate after transplantation is high and may be associated with early graft loss. Methods: We analysed demographic and transplant data collected by the Australian and New Zealand Dialysis and Transplant Registry (ANZDATA) on children and adolescents with a diagnosis of primary FSGS who were 20 years of age or younger at the time of transplant. Kaplan-Meier analysis was performed to compare graft survival according to recurrence of FSGS and donor characteristics.

Results: During the 20-year period from 1st January 1992 to 31st December 2011, 80 transplants were performed in 74 patients (age range 3-20 years, median 15 years). Twenty seven patients developed recurrent FSGS, with one child developing recurrent disease in 2 grafts (overall recurrence rate 36%). The median time to recurrence was 15 days, with loss of graft function in 12/28 transplants (43%). Median graft survival in patients with recurrence was significantly shorter than in patients with no recurrence (2.3 years vs >10 years, respectively; p<0.01). There was no significant difference in recurrence rate in recipients receiving deceased donor (DD) versus live donor (LD) transplants (p=0.24) and median graft survival was significantly better in recipients with live donors (11.7 yrs vs 1.6 yrs for DD, p<0.01)

Conclusion: The rate of recurrent FSGS and graft loss in this retrospective cohort study of children and adolescents transplanted within the Australasian region, was similar to previously reported studies. However, in contrast to previous studies, there was no significant difference in rate of recurrence for live versus deceased donors. Additionally, live donor recipients had longer median graft survival compared to deceased donor recipients.

Outcomes of calcineurin inhibitors conversion to mammalian target of rapamycin inhibitors in children with renal transplantation Nuntawan Piyaphanee, Suroj Supavekin, Anirut Pattaragarn Pediatrics, Siriaj Hospital, Bangkok, Thailand

Objective: To study outcomes of pediatric renal transplant recipients who underwent CNI elimination and conversion to mTOR inhibitors. Methods: We performed retrospective analysis of all pediatric renal transplant recipients who underwent CNI elimination and conversion to mTOR inhibitor at Siriraj Hospital, a tertiary care center in Thailand. Indications for conversion were defined. Graft function prior and post conversion, history of acute rejection, and CNI elimination failure were reviewed. Results: Nine of 32 pediatric renal transplant recipients underwent complete CNI conversion to mTOR inhibitor. Triple-drug regimen including prednisolone, CNI and MMF/MPA was basically prescribed to the recipients. Eight with tacrolimus and 1 with cyclosporine were conversed to everolimus. Mean ages at transplantation, conversion and last follow up were 12.1±2.4, 13.6±2.1 and 14.5±1.8 years, respectively. Everolimus was initiated within 3 months in 4 patients.

Each patient due to CNI induced hemolytic uremic syndrome, posttransplant diabetes mellitus, graft impairment with early onset nephrocalcinosis and graft impairment with renal artery stenosis. Everolimus was initiated after 3 months in 5 patients due to 1 chronic CNI nephrotoxicity, 2 chronic allograft nephropathy (CAN) and 2 unfavorable graft functions with history of delay graft function. Median duration from transplantation to CNI conversion was 4.5 months (range 0.8-88) and median time from conversion to the last follow-up was 8 months (range 2-26). Mean creatinine clearance (CrCl) prior to the CNI elimination was 33.0±13.2, as compared to mean CrCl of 49.0±16.3 ml/min/1.73m 2 at last follow up (p =0.069). Three patients had acute rejection (AR) before the conversion, but only 1 patient had AR post CNI elimination. There were no patients with graft loss or CNI-elimination failure. Conclusion: Conversion from CNI to everolimus was safe without increasing risk of acute rejection in pediatric kidney transplant patients who experienced early and late CNI associated complications. Objective: Due to a severe shortage of suitable deceased-donor kidneys for children awaiting kidney transplants (KT), we have performed a series of ABO-incompatible (ABO-i) living KT since 1989. Historically, ABO-i KT was performed using several session of plasmapheresis (PP) to remove existing anti-A or anti-B antibodies, followed by splenectomy to prevent rebound of antibodies. Because splenectomy had risks of surgical complications including bleeding and pancreatic pseudocyst, we introduced a new protocol, for ABO-i KT, in January 2006. The new protocol without splenectomy utilizes the anti-CD20 monoclonal antibody (rituximab) and PP. This study retrospectivery examined the efficacy and safety of this protocol. Methods: Eight de novo ABO-i KT (5 males and 3 females) were performed between January 2006 and December 2012. The mean age at transplantation was 15.3±4.0 years (range 9.9-20.5 years). The immunosuppressive protocol consisted of cyclospolin or tacrolimus, mycophenolate mofetil, and methylprednisolone. All patients received induction therapy with basiliximab. The preconditioning protocol included PP or double filtration plasmapheresis (DFPP) and a single dose of rituximab (average dosage 179 mg/m 2 ). All patients who underwent KT achieved a isoagglutinin titer less than 1:16 with 1-4 sessions of PP/DFPP treatment before transplant.

Results: The patients were followed for 5 to 82 months with a mean follow-up of 36 months. Patient and graft survival rates were 100%. One non-adherent patient experienced antibody mediated rejection. Figure1 ). In the RTR who had graft loss, there was no significant difference in graft survival time (6.0, 3.1 years, DSA negative vs DSA positive), Grade 1 rejection (0.5, 0.7 episodes per patient), Grade 2 rejection (0.5, 0.7 episodes per patient) or C4d staining (100%, 57% patients). One patient in each group had BK virus associated nephropathy. Two patients had plasma cell infiltrates on biopsy and lost their grafts within 6 months of DSA detection. Results: Fifty-one kidney recipients (male 32 cases; female 19cases) were enrolled in this study. The median age was 15 years, the youngest kidney recipient was 3years old with a body weight of 10.0kg). There were 11 recipients less than equal 10 years, including one children with congenital renal dysplasia and one with congenital renal artery malformation. About 73.7% kidney recipients came from GuangdongProvince. About 1 to 7 pediatric patients accepted renal transplatation each year from 2003 to 2011, but there were13 in2012, which showed a significant increase than in each previous sigle year.The policy that children had a kidney recipients priority may contribute to this increase. The kidney donors were cadaveric kidneys(72.5%) and living kidneys(27.4%), only one patient accepted auto-renal-transplatation. All kidney recipients were survival to present with the treatment of glucocorticoid and or immunosuppressants,such as CsA, FK506, MMF,ect. Only two patients accepted retransplatation due to losing function of the renal grafts. Conclusion: Renal transplantation in children increased significantly in 2012 in our hospital, and it seemed an effective treatment to children with ESRD. Recent adult studies have shown that low levels of immunosuppression (IS) are associated with dnDSA. However, limited data is available on significance of dnDSA and its management in paediatric population. To assess relationship between dnDSA with renal function, histological findings, immunosuppression levels and graft outcome in paediatric renal transplant recipients (RTR). In our centre, at the time of the study, DSA were tested if deterioration in graft function was found necessitating renal biopsy. Methods: Retrospective review of all RTRs in a single tertiary nephrology centre tested for dnDSA. Data collected included dnDSA, histological findings, eGFR as marker of renal function, baseline immunosuppression, subsequent treatment and outcome. Results: 27/88 patients had DSA tested; 12/27 had dnDSA detected. Median time for measured of dnDSA was 7.6 years post-transplant (range 1.07-13.02).Histology: 8/12 biopsies in dnDSA+ve subgroup had antibody mediated rejection (ABMR) of which 6 were active ABMR (3 were C4d positive) compared to 1/16 in dnDSA-ve subgroup. The following treatments were used in the dnDSA+ve group: increase in IS+ pulsed methylprednisolone (MP) (5 patients), increase in existing IS (2), rituximab (2), pulsed MP (1), sirolimus added+pulsed MP (1) . In one patient no change was made. At last follow up, in 9/12 patients with dnDSA an improvement in eGFR was observed. On re-testing for dnDSAs, 3/12 patients tested negative, 2/12 had lower and 3/12 increased levels. 3/12 had not yet had repeat dnDSA testing. Conclusion: Despite previous reports of dnDSA conferring poor prognosis, in our cohort with allograft dysfunction and positive dnDSA an improvement in renal function was observed in 75%. However, larger prospective studies are required to further evaluate these findings.

Abstract# P-SAT425 Anuria Since Birth: Does It Impact Outcome Of Kidney Transplant In Infants?

Shefali Vyas, Maria Isabel Roberti Pediatric Nephrology, Saint Barnabas Medical Center, Livingston, USA

Objective: Scarcity of data exists for allograft outcomes and urological problems in children with long term de functionalized bladders. However, even less is known about the outcome of infants anuric since birth and whose bladders underwent "forced rehabilitation" after a successful renal transplant.

Methods: In this retrospective study we compared urological events and allograft outcome in infants with ESRD mainly due to urological problems. They were grouped according to the history of pre-txp urine out put: Group A with urine output prior to txp and Group B anuric since birth (all had visible small bladders by ultra sonogram).

Results: There were no significant differences regarding birth history or ethnicity . Group A had 13 boys (87%) and group B had all girls; All but one child in group B received LRD Txp. All received induction followed by triple therapy (tacrolimus, steroids and MMF). Group A: 8/15 had V-U reflux and 13/15 were on PD prior to txp (mean time= 7 mos). All patients in Group B were started on PD in the neonatal period (mean =9.8 days). Pre txp native nephrectomies were done in 3 patients (2 in Group A and 1 in Group B). Group A had 2 acute rejections and none in Group B.

Conclusion: Anuric young infants had higher rates of post txp UTIs and V-U Reflux, a direct consequence of their inherent small bladders. However, the 3 year graft survival, patient survival and GFR remained excellent in anuric infants (Group B )compared to young infants transplanted with higher rates of V-U Reflux and dysfunctional bladders pre txp.

Abstract# P-SAT426 Kidney transplantation in a child with bladder dysfunction who underwent prior bladder augmentation: A case report 1 Tepecik Training and Research Hospital, Pediatric Nephrology, They were divided into 2 groups; 12pts with ESRD on dialysis (D), 4/12 with history of kidney transplant, and 9 kidney transplant recipients with good graft function (T). All subjects were tested for anti-HLA antibody 1 month prior to and 1 month and 6 months after administration of the combination vaccine of Influenza A/ H1N1. Results: Among the T group, no pt tested positive for either anti-HLA class I or class II antibodies before or after Influenza A/ H1N1 vaccine. In the D group, of the 4 pts with a history of graft failure who were sensitized before immunization; 2 showed no change in class I & II, one patient had mild increase in class I after vaccination, and one patient had an increase in class I by 24%. [figure] Conclusion: None of the T pts had clinical evidence of either cell mediated or humoral rejection after the Influenza A/H1N1 vaccine. In the D group, no pt had any statistically significant increase in anti-HLA antibody following vaccination. Our study suggests that Influenza A/H1N1 vaccination may be safe and tolerable in pediatric dialysis pts with or without a failed kidney allograft.

The effect of dipping blood pressure status on structural and diastolic heart function in renal transplant recipients Mitra Basiratnia 1 , GholamHossein Ajami 2 1 Shiraz nephrology urology research center, Shiraz university of medical sciences, Shiraz, Iran 2 Department of pediatric cardiology, Shiraz university of medical sciences, Shiraz, Iran

Objectives: Non dipping has been linked to cardiovascular disease in adults, however the impact of non-dipping on cardiovascular status of the adolescents with renal transplantation has not been well established. The aim of this study was to evaluate the influence of non dipping status on left ventricular mass index and diastolic function in subjects with renal transplantation. Methods: Sixty six stable renal transplant patients (34 females, 32 males), aged 7 to 25 years (mean 17.4±4.3 years) were enrolled in this study. Cardiac function assessed by tissue Doppler echocardiography and blood pressure measurement performed using ambulatory method. Dipping was defined as at least 10% BP decline during the night and was calculated as (mean daytime -mean night time/mean daytime)*% 100. Left ventricular mass (LVM) was calculated by standard 2dimensional directed M-mode echocardiography according to the formula of Devereux and was indexed to height in meters to the 2.7 power to allow the comparison between recipients of different sizes. Left ventricular hypertrophy was defined as LVMI>38.6g/m 2.7 in patients younger than 18 years and 51g/m 2.7 in patients > 18 years. Data analysis was performed by SPSS-15. A P<0.05 was considered statistically significant. Results: Non-dipping was identified in 48 (73%) patients. Five recipients were systolic non-dippers, 1 diastolic non-dipper, and 42 both systolic and diastolic non-dippers.

Left ventricular hypertrophy (LVH) was found in 37.1% of the renal allograft recipients. LVH was present in 41% of the systolic nondippers and 27.8% of the systolic dippers (p=0.33) . Forty five percent of the diastolic non dippers and 22.7% of the diastolic dippers had LVH (p=0.08) . There was no correlation between systolic and diastolic dipping status and LVH, respectively (p=0.33, p= 0.08). There were no significant differences in terms of diastolic function [measured by Early diastolic inflow velocity (E), E/A ratio, and early diastolic mitral inflow velocity to earlydiastolic annular velocity (E/Ea)] between dipper and non dippergroups(p= >0.05). Conclusion: Non dipping is common among renal transplant recipients, but is not always related to diastolic dysfunction and LVH. Prospective longitudinal studies are required to determine the impact of dipping status on diastolic and structural heart function in renal transplant recipients.

Parsa Yousefi Chaijan, Parvin Soltani, Farshid Haghverdi, Masood FazeliMoslehabadee Nephrology, Arak University of medical sciences, Arak, Iran

Objective: Nephrolithiasis in renal grafts is a relatively common phenomenon which can induce organ damage; hence early diagnosis and management of predisposing factors can preclude subsequent complications. The aim of this analytic cross-sectional study is to determine the contributory factors to nephrolithiasis after renal transplantation.

Method: 56 renal-transplanted patients (10-40 years old) were enrolled in the study, being divided into two groups of 28 transplanted patients suffering from nephrolithiasis (within first 5 years after surgery) and 28 transplanted patients free from renal stone with the same age and gender and similar GFR. Data were collected and arranged in Excellmicrosoft program and the statistic analysis was carried by SPSS V17. Pvalue < 0.05 were considered statistically significant.

Result: The studied showed that male gender (P = 0.048), age group of 15-25 years (P=0.021), hyper cholesterolemia (P=0.007), hyper triglyceridemia (P=0.031), 3 rd year after surgery (P=0.023), hyperuricosuria (P=0.012) , hypocitraturia (P=0.001) and Anemia (P=0.006) were significantly more common in patients with nephrolithiasis.

Conclusion: It is better to evaluate hyperuricosuria and hypocitraturia in kidney donors, moreover all patients had better undergo serial sonography for early screen and management of renal stone as well as treatment for hyperlipidemia and anemia. It also seems prudent to further assess the donors of transplanted patients suffering from renal stone and possbile relation between CNI & nephrolithiasis ( regardinghyperuricusuria) Objective: Epstein-Barr virus-associated smooth muscle tumor (EBV-SMT) in immuno-compromised patients has recently been reported. But there were no evidence about the treatment of EBV-SMT. We report a 6-year-old girl treated for multiple EBV-SMT by using rituximab.

Methods: Case report Results: She was suffering from end stage renal disease due to congenital nephrotic syndrome induced by WT1 mutation. Renal transplantation (TX) was performed at the age of 2 years, and immunosuppressive therapy was performed her. Soon after performing TX, she was infected cytomegalovirus(CMV), BKV and EBV. She was reduced in amount of immunosuppresive therapy, and she recovered from viremia of CMV and BKV. Her EBV titer did not become negative, but we did not perform any medication because of no symptom. Four years after TX, she was pointed out 1cm of cholecystic polyp in a protocol abdomen ultrasonic examination. We soon performed cholecystectomy and extirpation of swelling mesenteric lymph nodes. The pathological finding of cholecystic polyp was EBV encoded small RNA (EBER) positive smooth muscle tumor but there was no finding about post-transplant lymphoproliferative disorder in mesenteric lymph nodes. Positron-emission tomography (PET) showed accumulation of 1cm mass around 12th of thoracic vertebra and CT scan showed three small masses in the lung and one in the liver. We diagnosed her suffering multiple infection of EBV-SMT. Immunosuppression therapy was reduced, but there was no change about size of her tumor. Rituximab was administered for keep away from repetition of tumor after proving infection of EBV only in B cells by using flow cytometry. After medication of rituximab, the EBV DNA counts were normalized. Ultrasonic examination detected three small masses in liver and one lymph node around main artery. But her mass around 12th of thoracic vertebra was disappeared and whole body CT scan detected only one small residual mass in the lung.

We report a case of an EBV-SMT presenting multiple infections, which was treated with rituximab. Rituximab may be an effective treatment for multiple EBV-SMT but careful observation is also needed for the reappearence of EBV-SMT.

The usefulness of monitoring of Epstein-Barr viral load after renal transplantation in pediatric recipients with EBV seronegative Objective: The purpose of this study are to establish a protocol for monitoring Epstein-Barr virus (EBV) infection for identification of pediatric renal transplant recipients with a high risk of developing posttransplantlymphoproliferative disorder (PTLD) and to predict the development of PTLD. Methods: Peripheral blood mononuclear cells (PBMCs) and plasma EBV loads were measured by nested PCR (n-PCR) and real-time PCR (r-PCR) every 1-3 months after grafting in 17 pediatric recipients who were seronegative for EBV before grafting (4 with EBV-associated symptoms, including 2 with PTLD (group A); 6 with asymptomatic persistent high EBV loads in PBMCs of >1,000 copies/ug DNA for over 6 months (group B); and 7 with neither EBV-associated symptoms nor persistent high EBV loads in PBMCs (group C). EBV-CTLs were also measured in 13 patients without EBV-associated symptoms.

Results: The EBV genome detected by n-PCR was present in plasma in 3 (75%), 1 (17%), and 0 (0%) in groups A, B and C (p<0.01 for A vs. B and A vs. C). EBV loads detected by r-PCR in PBMCs were significantly higher in groups A (p<0.05) and B (p<0.01) compared to group C. EBV genomes in plasma were detected by n-and r-PCR in only the 2 cases with PTLD. One patient with lymphadenitis in group A and 1 patient in group B had EBV-DNA in plasma based on n-PCR, but the viral loads using r-PCR were <250 copies/ml. The CTLs' percentage was significantly lower in Group B when EBV loads first rose above 100 copies/ugDNA. Conclusion: Plasma EBV loads (over 250 copies/ml) estimated by r-PCR and CTLs' monitoring may be useful to distinguish PTLD from other EBV-associated diseases or asymptomatic viremia, and to avoid PTLD as patients with asymptomatic persistent high EBV loads had higher EBV loads and lower percentages of CTLs.

Abstract# P-SAT437 Risk factors for post-transplant lymphoproliferative disorder (PTLD) in children with kidney transplantation (KTx) -A single center survey since the introduction of tacrolimus (Tac) Since pre-transplant evaluation showed complete obstruction of IVC/ iliac vein below diaphragm, she had been managed with peritoneal dialysis for 7 years until January 2012 when she received a living donor kidney allograft from her mother. The graft was transplanted in a left orthotopic position. Venous drainage was to the left ascending lumber vein. In addition, a venous bypass was made using donor ovarian vein between graft vein and splenc vein after splenectomy. Such double venous drainage was working very well after transplantation. There was no surgical complication and the serum creatinine (s-Cr) during the hospital stay was 0.54mg/dl. At second month after the transplantation, she developed an increase of s-Cr with edema and hypertention. However, biopsies of kidney allograft performed at 2 nd and 4 th month after the transplantation revealed no evidence of acute rejection. Hypertention was difficult to control even with a maximum dose of calcium blocker, and a temporary increase of the s-Cr up to 1.30 mg/dl was noted when angiotensin II receptor blocker was administrated. By the ultrasonography, the maximum arterial blood flow of the kidney allograft was high enough (459cm/s) to suggest renal arterial stenosis. Moreover, severe renal arterial stenosis was clearly shown for about 10mm length from the anastomosed site of Aorta by the contrasting CT.For the treatment of artery stenosis of the kidney allograft, percutaneous transluminal renal angioplasty (PTRA) was performed at 8 th month after the transplantation. The stenotic lesion was expanded using the special dilatation balloon from 1.7mm through 2.0mm in diameter. Because intravascular ultrasonography showed no intimal thickening of the blood vessel, a vascular stent was not placed. After the PTRA, s-Cr was decreased to 0.5mg/dl, and the blood pressure became controllable by antihypertensive agents. Moreover, the angiography performed 4 months after the PTRA revealed no progression of arterial stenosis. The s-Cr of this patient is now stable in 0.7 mg/dl.

Post-transplant encapsulating peritoneal sclerosis in children: a single center experience

Kei Nishiyama Tokyo women's mdical university, pediatric nephrology, Tokyo, Japan

Objective: A substantial proportion of encapsulating peritoneal sclerosis (EPS) cases develop after renal transplantation (RT), an entity known as post-transplant EPS. Although risk factors for EPS include prolonged PD, recurrent peritonitis, decreased ultrafiltration and prolonged administration of hypertonic glucose or icodextrin, the pathophysiology of post-transplant EPS is largely unknown. However it has been postulated that the use of calcineurin inhibitors (CNIs) after transplantation may promote EPS, as these drugs are considered profibrotic. A recent Scottish study showed that the contribution of post-transplant EPS might be even as much as 50% of the total EPS patients. By contrast pediatric cases are very rare. Therefore we examined the incidence of post-transplant EPS in pediatric renal transplant recipients. Methods: In this study, we retrospectively investigated clinical records from 52 consecutive pediatric renal transplant recipients in our center between 2007 to 2012, who performed PD before transplantation. Clinical parameters and PD-related risk factors of EPS were collected at the time of RT. Transplant-related variables were also collected. EPS cases who met ISPD diagnostic criteria including clinical feature and either radiologic and/or histopathological confirmation were examined. Results: The median duration of PD was 2.0 yr (range 0.5 to 7.9). Twelve patients (23.1%) had at least one episode of peritonitis. Ten patients (19.2%) were administered hypertonic glucose and/or icodextrin. The median follow-up period after transplantation was 2.3 yr (range 0.3 to 5.8). All patients were administered CNI, and 2 patients discontinued corticosteroid during the follow-up period. Although 5 patients had ultrafiltration failure at the time of RT, there was no case who developed post-transplant EPS.

Conclusion: The case of EPS after RT was not seen in this study. Possible explanation of this result might be associated with shorter durations of PD and small sample size. Since post-transplant EPS is rare but carries a high mortality, caution should be paid to developing post-transplant EPS even in pediatric patients with a relatively long pre-transplant duration of PD.

Asli Kantar, Kaan Gulleroglu, Esra Baskin, Umut Bayrakci, Zafer Ecevit, Hande Arslan, Aydincan Akdur, Gokhan Moray, Mehmet Haberal Pediatric Nephrology, Baskent University, Ankara, Turkey

Objective: Viral infections remain a significant cause of morbidity and mortality following renal transplantation. Although cytomegalovirus is the most common opportunisticpathogenesis in transplant recipients, numerous other viruses may affect clinical outcome. Viral infections are potentially severe complications of transplantation, as they not only induce specific diseases, but they also favor the development of allograft damage, opportunistic infections and acute rejection. We evaluated the major viral infections seen following kidney transplantation and allograft outcomes in our pediatric patients. Methods: We evaluated retrospectively 94 pediatric renal transplant recipients for the occurrence of viral infections and compared outcomes among these patients. Patients were divided in to two groups those who developed an infection and those who did not. Inthese groups, we recorded induction therapy used at transplantation, immunosuppressive therapy given at discharge, acute rejection rate, patient and graft survival rates.

Results: The mean age of the patients was15.5±5.3 years. Viral infection was found in 32 patients. Cytomegalovirus infections were the leading causes; EBV and BK virus were following causes in our study. Any significant correlation could not be shown between viral infections and immunosuppressive therapy. We did not observe any correlation between acute rejection and viral infections. Lowest GFR at 6 th month was shown in patients with BK virus infection. A significant correlation was shown between viral infection and graftloss (r: 0.22 p: 0.028).

Conclusion: Viral infections are common after kidney transplantation. Patients should be monitored more carefully for provide against damage in transplanted kidney. Objective: Anemia is a frequent condition in kidney transplant recipients and it has a negative long term impact on graft and patient outcomes. Recently it has been shown that treatment with angiotensinconverting enzyme inhibitors (ACEI), angiotensin II receptor blockers (ARB) and mTOR inhibitors could be the leading causes of anemia in renal transplant recipients.To study the association of hemoglobin (Hb) and ferric parameters with GFR, immunosuppressive drugs, ACEI, ARB and clinical features of kidney recipients. Method: Hospital records of 94 (F/M:48/46) kidney recipients were reviewed retrospectively. The mean age of the study group was 15.5±5.3 years. The mean follow-up was 52.6±36.31 months.

Results: Thirty six (38%) patients were found to be anemic. Mean Hb levels of anemic and non anemic patients were 10.2±1.15 mg/dl vs 13.87±3.41 mg/dl respectively. Ferritin and iron levels as well as transferrin saturation index, RDW and MCV did not differ among the groups. Anemia was not found to be correlated with immunosuppressive or antihypertensive drugs including ACEI and ARB. Donor status did not also have any influence on anemia. Mean GFR of patients at posttransplant 6 months and 1 year follow-up was found to be significantly lover than patients without anemia (74.4±34.1 vs 99.1±28.9 ml/min/1.73m2 respectively). Graft loss was also found to be significantly higher in anemic patients (16% vs 6%, p<0.018).

Conclusion: In this study we examined the prevalence of anemia and its risk factors in kidney transplanted patients. Its incidence is quite high in our patients. We found that anemia in kidney recipients is neither related to iron status nor medications like ACEI, ARB or immunosuppressive drugs. The major determinant of Hb level, especially during the first year of transplantation is the graft function.

The most important conclusion of this study is the considerable controversial impact of anemia on graft survival. There was no significant effect on blood pressure ; 4 patients 7±2 Modigraf® administration days in the converted group. In the whole series, mean creatinine level was 0.7±0.5 mg/dl throughout the observational period, and no rejection episodes were detected. Blood pressure was well controlled and no proteinuria was seen. The equivalent dose ratio between Modigraf® and liquid tacrolimus was 1.28.

Conclusion: Modigraf® appears to be a safe and sustainable way to administer tacrolimus in kidney transplanted infants. In our experience required Modigraf® dose was 1.28 times the oral liquid tacrolimus one, and therapeutic levels were attained in one week. Objective: Prescribing of medications in paediatric practice is problematic as many drugs remain unlicensed. This is especially true of immunosuppressive medications. However, there is now a licensed product for Tacrolimus called Modigraf® with data available on bioavailability. We undertook a single centre prospective study of conversion to Modigraf®, an oral liquid available in granule formulation (1mg and 0.2mg) that allows for dosing according to body weight. Methods: All paediatric renal transplant recipients (RTR) under the care of a single centre were considered for conversion to Modigraf® from their current Tacrolimus regimen. Inclusion criteria included all RTR under 18 years of age who were on Tacrolimus suspension. Exclusion criteria included recipients of multi-organ grafts, patients with lactose intolerance and patient choice to continue with their current suspension. Patients were then seen by the multi-disciplinary team. After equivalent dose conversion, patients were monitored with blood tests one week after conversion with subsequent doses adjusted accordingly. Patients continue to be monitored with blood levels over a 3 month period after conversion with renal allograft function, renal allograft loss and side-effect profiles recorded. Results: Forty-three (27% of 158) RTR were considered for conversion to Modigraf®. The families of four patients requested to stay on their current Tacrolimus suspension, three patients were deemed unsuitable due to lactose intolerance and three patients were excluded due to low doses (incompatibility with the granule dosing). Thirty-three patients were then converted to Modigraf® and closely monitored. After blood level monitoring one week after conversion, 12% (4) patients had their doses increased due to lower than anticipated 12-hour trough tacrolimus levels. There was stable renal allograft function without renal allograft loss after conversion. Conclusion: Conversion to Modigraf® immunosuppression can be safely undertaken in paediatric RTR, although regular monitoring in the immediate period of conversion is required to provide accurate immunosuppression dosing.

ESCORT trial -Effects of strict control of blood pressure in pediatric renal transplant recipients -Baseline characteristics of patients from a randomized controlled trial Tomas Seeman, Jiri Dusek, Nadezda Simankova, Karel Vondrak, Jakub Zieg Dpt. of Pediatrics, University Hospital Motol, Prague, Czech Republic

Objective: Arterial hypertension is a known risk factor for impaired graft survival in patients after renal transplantation (RTx). Strict control of blood pressure (BP <50 th percentile) delays progression of chronic kidney diseases in children (ESCAPE trial). It is not known whether strict BP control has renoprotective effect also in children after RTx. The aim of this randomized controlled trial was to investigate whether strict BP control can protect kidney graft in children after RTx. Methods: All children from our pediatric renal transplantation center were screened for eligibility for the study (children 3-16 years at least 1 year after RTx, no acute rejection in the last 3 months, eGFR>15 ml/min/1.73m2, 24hr mean BP >50 th percentile using ambulatory blood pressure monitoring ABPM). Altogether 23 children fulfilled the inclusion criteria. They were randomized to intensified BP control group (INTENS, target 24hr MAP <50 th percentile, n=12) or standard BP control group (STAND, target 24hr MAP 50-95 th percentile, n=11). All antihypertensive drugs are allowed to reach the target BP. The study period is 3 years. The primary endpoint is the yearly change in eGFR (Schwartz formula, ml/min/1.73m2/year), the secondary endpoints are graft failure, change in proteinuria, left ventricular mass and safety of strict control of BP.

Results: The baseline characteristics of the patients are given in the allelic variants. TAC dose (mg/kg/day) and TAC exposure normalized for dose (TAC Co/D) (ng/ml/mg/kg/day) were analyzed with respect to CYP3A5 genotype and for interaction with azoles and corticosteroids, for a period of one year post-transplant. Over time, TAC Co/D was significantly lower in recipients with a CYP3A5*1/*3 genotype compared to those being homozygous for the CYP3A5*3 allele (55.33 +/-21.30 versus 83.07 +/-9.9 ng/ml/mg/kg/day, P= 0.0068). The dose requirement was significantly higher in children with a CYP3A5*1/*3 genotype compared to those being CYP3*3/*3 (0.21 +/-0.02 versus 0.17 +/-0.01 mg/kg day, p=0.0079). The TAC Co/D was significantly higher for patients receiving azoles (n=17) than those not receiving azoles (n=40) (117.21 +/-1.06 and 62.01 +/-0.52 ng/ml/mg/kg/day, P= 0.002) and consequently the required TAC dose was lower in patients receiving azoles overtime (0.08 +/-0.00 versus 0.22 +/-0.01 mg/kg/day, P < 0.001). In children receiving steroids without azoles the TAC Co/D was significantly different between the CYP3A5*1/*3and CYP3A5*3/*3 genotypes, respectively (52.86 +/-1.71 vs. 58.68 +/-0.85 ng/ml/mg/kg/day, P=0.0044) but the TAC Co/D was not different for those receiving steroid versus those not receiving steroid with CYP3A5*3/*3 genotype ( 84.91 +/-12.7 and 84.21 +/-1.41 ng/ml/mg/kg/day, P=0.16). Conclusion: In conclusion, the TAC dose is influenced by the CYP3A5 genotype and drugs such as azoles, while steroids may not impact TAC dose. While therapeutic drug monitoring of TAC remains necessary, integrated knowledge of patient genotype and comedication use provides the opportunity to refine TAC dosing in children receiving kidney transplant.

Abstract# P-SAT458 Basiliximab induction therapy in pediatric renal transplantation, A double blind clinical trial

Hasan Otukesh Tehran university of medical science, associated professor, Tehran, Iran

This is an open, single center, randomized study to compare induction therapy with basilixamb with no induction therapy in children with renal living transplantation. In this trial 20 pediatric renal transplant recipients enrolled randomly to one group with basiliximab as induction therapy and another group without basiliximab induction therapy. Both group received prednisolone, cyclosporine and cellcept. We assessed graft function at 3 months after transplant and compared this item between these two groups. In the congress the data and results of this randomized trial will be presented.

Abstract# P-SAT459 Legalization of the organ donation and optimal utilization of young pediatric donor kidneys into pediatric recipients in China Objective: China has started to establish a new national system for organ donation and transplantation since March 2010 by the Ministry of Health and the Red Cross Society of China. The aim of this study was to describe our initial experience of pediatric renal transplantation using organ donations from pediatric patients no more than 6 years old.

Methods: The procedure of organ donation includes: 1. Judgment of brain death, or circulatory death, or brain death followed by circulatory death by doctors; 2. Organ donation informed consent form signed by family (children's parents); 3. Approval by the hospital Ethics Committee; 4.Organ donation to the Red Cross Society and allocation by the China Organ Transplant Response System (COTRS). The Red Cross Society has been commissioned by the Ministry of Health to run this system. Clinical data of 8 children who underwent renal transplantation using organ donations from pediatric patients no more than 6 years between September 2011 and March 2013 were retrospectively analyzed. Results: The age at transplantation for these 8 patients was 4.5 years to 14 years and the weight was 14 kg to 35 kg. Among these 8 cases, 7 donors were used aged from 33 days to 6 years and all diagnosed with circulatory death. 5 recipients received en bloc kidney transplantation and the other 3 recipients received one single kidney according to the size of both recipient and donor. The duration of follow-up after the transplantation was 1 month to 18 months. Patient survival rate was 100% and graft survival rate was 7/9 (77.8%, one graft loss due to the hemorrhagic complication and the other one due to the thrombosis). At latest follow-up, the median serum creatinine level was 92umol/L and the median eGFR was 80ml/(min·1.73m 2 ). Conclusion: Organ transplantation legislation is necessary to ensure the rights and obligations of donors, recipients and medical institutions. We believe young pediatric donors can be expanded further to increase the number of pediatric renal transplants. This pediatric to pediatric combination on the one hand efficiently lower the discarding ratio of the kidneys from small donor, and give more chances to the younger recipient on the other.

To assess the normalization of serum 25 (OH)D level (>30 ng/ml) after standard treatment dosing among Primary Hypertension (PH) and Chronic Kidney disease (CKD)

Methods: We enrolled 144 patients aged 2-19 yrs

We collected retrospective data on age, sex,race, cause of kidney disease, eGFR, Ht, Wt, BMI, BP z-scores, lipid panel, 25(OH) D level, PTH, Calcium, Phosphorus, Magnesium, medications, type and dosing of Vitamin D Supplements and follow up serum vitamin D level three months post treatment. Results: Mean age (yrs) was (12.99±5.05). white(2)

Prevalence of Vitamin D deficiency was (88%); 60% had level <20 ng/ml. Mean pretreatment 25(OH) D (ng/ml) was (19.25±10.69)

After completion of standard treatment almost 64 % patients had 25 (OH) D level <30 ng/ml; PH(28.02±9.22) and CKD(25.38±9.8). None were in toxic range. Conclusion: The standard treatment dose of Vitamin D doesn

Abstract# P-SAT360 Mineral metabolism in European children with end-stage renal disease

Marjolein Bonthuis 1

The Netherlands 2 Pediatrics, Nephrology and Dialysis Unit

Results: Hypocalcaemia was found in 22% of HD, 18% of PD, and 48% of transplanted patients, with a mean time on transplantation of 4.3 years. Hyperphosphataemia was found in 47% of HD, 37% of PD, and 5% of transplanted patients. PTH was outside target in 56% of HD, 58% of PD and 14% of transplanted patients. In dialysis patients, calcium and PTH were inversely associated with age; 53% of adolescents were hyperphosphataemic resulting in a significantly higher risk compared to 3-5 year olds (OR:1.77, 95%CI:1.43-2.19). Patients transplanted pre-emptively had a lower risk of hypocalcaemia compared

FGF23 Is Modulated By Calcium In Children Under Chronic Peritoneal Dialysis

Azocar 1 , Maria L. Ceballos 1 , Angelica M. Rojo 1

Luis Calvo Mackenna Children's Hospital

Human intact FGF-23 levels (pg/ml, Immutopics) were determined through a 2-site ELISA kit. Klotho levels were determined by a solid phase sandwich ELISA kit (pg/ml). Descriptive statistics, univariate and multivariate analysis were performed

Methods: We measured serum calcium, phosphorus, intact parathyroid hormone, alkaline phosphatase (ALP), 25-hydroxy vitamin D3 (25D3), 1, 25-hydroxy vitamin D3 (1,25D3), and FGF-23 from 233 children (Male:female = 157:76, Mean age 10.2 years) with CKD stage I-V predialysis in Korea with 0.5-1 year intervals since 2011. Results: Hypocalcemia was observed in 23.8%, 32.4%, 21.8%, 22.4% and 31.8% of patients with CDK I to V (The rest is the same as above)

3%, 8.3%, 47.1%, 68.1% and 40.9%. 25D3 level was below

FGF-23 (RU/mL) was 38.0, 44.9, 54.4, 67.3 and 119.6. Serum P had positive correlation with ALP (P < 0.0001), iPTH (P = 0.0489) and FGF-23 levels (P < 0.0001) and was inversely correlated with urine phosphorus

Conclusion: The prevalence of hypocalcemia, hyperphosphatemia and hyperparathyroidism increased as CKD progressed. Serum iPTH and FGF-23 increased and 1,25D3 level decreased in proportion to the progression of CKD

Malaysia 6 National Transplant Resource Centre, National Transplant Resource Centre, Kuala Lumpur, Malaysia Objective: To analyse the cost-effectiveness of paediatric and adult living related renal transplantation (LRT) and deceased donor renal transplantation (DRT)

The time horizon was the lifetime of transplant recipient from transplant to death. Data for survival analysis was obtained from National Renal Registry. Statistical analysis was performed using STATA SE version 11.2. The costs was discounted at 3% p.a. Results: We reviewed 206 medical records. There were 88 children (39 LRT, 49 DRT) and 118 adults (63 LRT, 55 DRT). The paediatric recipients mean age was 12.3 +/-3.4 (LRT) and 14.0 +/-2.4 years (DRT), whereas the adult recipients mean age was 33.4 +/-10.3 (LRT) and 41.8 ±8.9 years (DRT). The 5 and 10-year patient survival rates for both paediatric LRT and DRT were 95% and 85% respectively. The 5 and 10-year patient survival rate for adult LRT was 92.5% and 90% respectively, whereas adult DRT was 77.5% and 70% respectively. Mean cost per paediatric transplant at first year was RM 81,000 (LRT) and 90,000 (CRT), from second year onwards was at 20,000 per year (LRT) and 37,000 per year (DRT) . The total lifetime cost was RM 650,000 (LRT) and 630,000 (DRT)

Their serum creatinine, eGFR, cholesterol, LDL, proteinuria, full blood counts and liver function were profiled to compare the pre-/post-conversion changes. Common adverse effects, acute rejection, opportunistic infection and need of treatment for hypercholesterolaemia and proteinuria were investigated. Results: We have 15 eligible patients with M: F = 7 : 8. The mean age at renal transplantation was 12

Sirolimus was started at a mean age of 15.4 +/-5.0, at a mean time period of

+/-2.5 years after renal transplantation, and used for a mean duration of

We observed new onset hyperlipidaemia in 9 patients (60%) and needed statin treatment. We found significant proteinuria (spot Pr/Cr > 1.0 mg/mg) in 4 (27%) patients and needed ACEI treatment. There was no adverse effect on blood counts and liver function. There was no opportunistic infection observed after conversion to Sirolimus in the study period. There was one acute cellular rejection (Ia) one month after conversion to Sirolimus and responded to pulse methylprednisolone. Conclusion: Converting from a CNI based immunosuppressant protocol for Paediatric renal transplantation patients to a Sirolimus based one was effective and the benefit was shown up to 54 months post-transplantation

One patient received a desensitisation regime with Rituximab and plasmaexhange pre-transplant and IV immunoglobulin post-operatively. Surgical complications occurred in 7 patients; 2 lymphocoeles, 3 ureteric leaks and 2 renal vein thromboses. Early post-operative medical complications included hypertension (47), pulmonary oedema (13), seizures (9), reaction to Basiliximab (3), sepsis (5), acute tubular necrosis (8), delayed graft function (5), acute rejection (2) and diabetic ketoacidosis (1). Primary EBV infection occurred in 38 patients -1 developed PTLD. There were 9 (12%) graft failures; renal vein thrombosis (2), acute humoral rejection (1), acute tubulointerstitial nephritis and tacrolimus toxicity (1), de-novo acute glomerulonephritis (1), chronic rejection (2) and non-compliance (2). Graft survival rates were 95% at one year, 93% at 3 years, 89% at 5 years and 88% at 10 years. Patient survival was 100% at 10 years. Conclusion: Our outcomes compare well with international figures

A Desensitisation Program for Paediatric Renal Transplant Patients in NSW

One child received ATG in addition. Results: Complications/ Patient Outcomes:1: 2 yo boy, donation from mother. DSA antibodies MFI 658. No complications. No infections. Good renal function, creatinine 45 at 2yrs. 2: 14 yo boy, previous transplant from biological father. Donation from foster father. DSA antibodies MFI 2602. Post operative complication of pseudomonas pneumonia/bronchiectasis, CMV, Acute Cellular Rejection x2. Good renal function creatinine 120 at 1 yr. 3: 17 yo boy, donation from father. DSA antibodies MFI 724. Early e-coli urine infection. No other complications, creatinine 117 at 1 yr. 4: 14 yo girl, recipient of 3 liver transplants. Maternal donor with positive B cell CxM and DSA MFI 6488. No complications. Good renal function

Four of them had subsequently increasing needs in bicarbonate and/or sodium supplementation, and F was reintroduced in 3 of them. Conclusion: F is effective in most cases of severe tubulopathy after RTx. However, side-effects can occur. Further prospective studies are needed to validate this indication

Methods: Eighty-one (F/M: 41/40)pediatrictransplant patients were included to the study.Demographic characteristics and laboratory parameters were recorded.Risk factors for hyperuricemia and the effects of plasma uric acid levels at 3 rd and 6t h months, 1 st and 3 th years on allograft outcomes were evaluated. Results: Mean age was 16.9±5.6 years.Mean follow-up time after transplant was 3.5±0.47 years. Hyperuricemia was detected in 17.6% of patients. A significant negative correlation was found between 6 th month uric acid leveland 3 th year of GFR value (r = -0.33, p = 0.04 and r = -0.33, p = 0.017). A significant positive correlation between 3 th and 6 th months uric acid levels and3 th year plasma creatinine level was demonstrated

Conclusion: Uric acid levels may have predictive value in the long term assessment of renal function.Posttransplanthyperuricemia can be used as a long term prognostic marker of poor renal outcome.Patients with hyperuricemia should be monitored closely for renal functions

Abstract# P-SAT447 Tuberculosis in Paediatric Renal Transplant Patients -Single Centre Experience

Methods: Retrospective descriptive folder review of TB in Paediatric Renal Transplantation at Red Cross Children's Hospital, University of Cape Town from 1994 -2012. Results: 14 paediatric renal transplant with TB identified. Male 11: Female 3. Ages 3.4 -18.8yrs(mean 12.0; median 13.2). All patients screened for TB prior to transplant

Polycystic Kidney Disease, 2 Dysplasia, 2 Chronic Glomerular Nephritis, 2 unknown cause, 1 Systemic Lupus Erythematosis and 1

All patients were on steroids and 8 had recent intensification of immunosuppression. TB treatment included conventional drugs in all but 1 case. Levels of Calcineurin inhibitors were affected in 12/14 patients and required increased dosing(up to 3 times baseline) according to levels in all. Rejection was seen in 7 patients(all biopsy proven). Successful treatment of TB in all patients with retention of graft. Graft and patient survival 100% post treatment. Conclusion: TB is a significant problem in paediatric renal transplants in developing countries. Our series shows that with careful investigation and diagnosis of TB as well as careful monitoring of immunosuppressant levels

Objective: To evaluate the reliability, validity and feasibility of the Persian version of the Pediatric Quality of Life inventory (PedsQL TM 4.0TM 4.0) Generic Core Scales in Iranian healthy students ages 7-15 and chronically ill children ages 2-18. Methods: We followed the translation methodology proposed by developer to validate Persian version of PedsQL TM 4.0TM 4.0 Generic Core Scales for children. Six hundred and sixty children and adolescents and their parents were enrolled. Sample of 160 healthy students were chosen by random cluster method between 4 regions of Isfahan education offices and 60 chronically ill children were recruited from St. Alzahra hospital private clinics. The questionnaires were fulfilled by the participants. Results: The Persian version of PedsQL TM 4.0TM 4.0 Generic Core Scales discriminated between healthy and chronically ill children (healthy students mean score was 12.3 better than chronically ill children, P< 0.001). Cronbachs alpha internal consistency values exceeded 0.7 for children self reports and proxy reports of children 5-7 years old and 13-18 years old. Reliability of proxy reports for 2-4 years old was much lower than 0.7. Although, proxy reports for chronically ill children 8-12 years old was more than 0.7, these reports for healthy children with same age group was slightly lower than 0.7. Constructive, criterion face and content validity were acceptable. In addition, the Persian version of PedsQL TM 4.0TM 4.0 Generic Core Scales was feasible and easy to complete. Conclusion: Results showed that Persian version of PedsQL TM 4.0TM 4.0 Generic Core Scales is valid and acceptable for pediatric health researches. It is necessary to alternate scoring for 2-4 years old

Objective: Bisphosphonates are widely used in the management of children with steroid induced osteoporosis (SIO). With the increasing use of bisphosphonates, there have been reports of abnormal radiological findings in the growing skeleton. Therefore, their use in pediatric patients remains controversial. The present study was conducted to evaluate the long term follow-up results of radiographic features especially metaphyseal sclerotic lines, associated with pamidronate therapy in pediatric patients with nephropathy. Methods: Twenty two children with nephropathy receiving oral calcium and pamidronate (mean duration: 7.9 months, dose: 125mg daily) were evaluated restrospectively. All Patients had SOI because of chronic glucocorticoid therapy for the treatment of nephropathy. Biochemical tests, long bone radiography and bone mineral density (BMD) were performed before the treatment of pamidronate and followed up several years later. The physeal growth rates were estimated by measuring the distance that the sclerotic lines moved on the radiographs during the corresponding time intervals. Results: The mean follow-up period was 9.5 years. In all patients, the well-defined sclerotic lines at the metaphyseal ends were observed and progressively moved from physeal plate to diaphysis on the radiographs of long bones. The mean moving rates of the sclerotic lines was 7.95 mm per year and in twelve patients, the lines disappeared. And the mean growth rate of height was 4.50 cm per year. Conclusion: Our long-term follow-up results suggest that the metaphyseal sclerotic lines associated with pamidronate treatment tend to disappear without affecting the skeletal growth. Bisphosphonate treatment for SOI in pediatric patients with nephropathy seems to be safe although further studies for larger number of patients are needed Abstract# P-SAT366 Clinical effectiveness and safety of the high dose active vitamin D therapy on severe hyperparathyroidism in children with ESRD Eun Gu Kang, Su-Yon Kim, Joo Hoon Lee, Young Seo Park Department of Pediatrics, Asan Medical Center Children's Hospital, Seoul, Korea Objective: A potent inhibiting effect of active vitamin D on parathyroid hormone is well known, but there is uncertainty on the dose to be used in hyperparathyroidism. The aim of this study is to assess the effectiveness and safety of high dose active vitamin D treatment on severe hyperparathyroidism in children with end stage renal disease (ESRD). Methods: Fifty-four patients underwent dialysis for more than 1 year between May 2002 and Feb 2013 in Asan Medical Center. Among them, patients who were administered high dose of active vitamin D (dose of alfacalcidol 1mcg/day to 3mcg/day) with severe hyperparathyroidism(intact parathyroid hormone (iPTH) >800pg/mL) were selected. Changes of iPTH, plasma albumin-corrected calcium, phosphorus and 1,25 (OH)2 vitamin D were analysed. Results: Fourteen patients (10 boys and 4 girls) with median age of 12.5 years (6-19 years) were included. The mean duration of dialysis was 49±32 months and the median duration of the high dose alfacalcidol therapy was 5 months(3-22months). The iPTH level significantly decreased in 10 patients (71%) from 1206.3±387.6 to 215.2±117 during the high dose alfacalcidol therapy (p<0.001). Serum phosphorus (5.9±1.3mg/dL vs. 6.0±1.8mg/dL, p=0.88) and calciumphosphorus product (53.4±11.9mg2/dL2 vs. 59.8±18.4mg2/dL2, p=0.20) were not significantly changed and 1,25 (OH)2 vitamin D was low or normal after therapy. Plasma albumin-corrected calcium significantly increased (9.4±0.64mg/dL vs. 10.2±0.75mg/dL, p=0.016). Four patients (29%) had persistent severe hyperparathyroidism despite treatment with high dose of alfacalcidol, which was controlled after kidney transplantation (2 patients) or after parathyroidectomy (2 patients).Conclusion: High dose active vitamin D therapy controlled severe hyperparathyroidism in the most children with ESRD without significant adverse events.

Clinical features and treatment of CKD-MBD in children with CKD I-V predialysis Objective: Vitamin D is known to have multiple effects on the cardiovascular system, renal function, hyperparathyroidism and growth, its deficiency is common in adults and children with chronic renal disease (CKD), but data in kidney transplant children are scarce. The aim of the present study was to investigate the vitamin D status in 3 groups of children and adolescents with CKD and to establish the association between 25(OH) vitamin D (25OHD) levels, age, hyperparathyroidism, short stature, and renal function. Methods: We recruited 105 children: 44 renal transplant patients with a functioning graft for at least 6 months (mean age 12 yr, mean graft Abstract# P-SAT386 The expression and significance of IL-6 、IP-10 and IL-17 in serum and synovial fluid with juvenile idiopathic arthritis. Objective: To detect the disparity of three cytokines about Interleukin-6(IL-6),Interferon-inducible protein 10(IP-10) and in peripheral blood(PB) and synovial fluid(SF) of patients with juvenile idiopathic arthritis (JIA). Method: Serum concentrations of the three cytokines were measured in 27 patients with 13 systemic-onset JIA(sJIA), 14 polyarticular JIA(pJIA) and 28 healthy controls using enzyme-linked immunoabsorbent assays (ELISA). 19 patients being no marked arthritis symptom or only temporary arthralgia enrolled in probable sJIA group. SF from 18 patients with 7 sJIA,11pJIA were examined for cytokine levels. Objective: The aim of the present study is to evaluate serum concentrations of ghrelin and leptin, and their associations with fat mass and insulin homeostasis in children undergoing chronic dialysis. Methods: The study population consisted of 40 patients on maintenance dialysis (22 PD and 18 HD) aged between 5-19 years and 20 age-and sex-matched healthy children. Serum levels of total ghrelin and leptin were measured in all patients and controls. Fasting serum glucose and insulin levels were also measured in the patients; insulin resistance was estimated by the homeostasis model assessment of insulin resistance (HOMA-IR). Nutritional status was assessed by measuring body mass index (BMI), triceps skinfold thickness (TSF) and multi-frequency bioimpedance analysis (BIA). Body fat mass was estimated by the BIA method. Results: The mean total ghrelin level was significantly higher in the patients than the controls (1671±1317 vs. 543±365 pg/mL, p<0.001). Higher total ghrelin levels in dialysis patients were significantly associated with younger age (p=0.015), lower BMI-SDS (p=0.050) and lower BIA-based fat mass-z score (p=0.007). The mean leptin level was also higher in dialysis patients compared to the controls but the difference was not statistically significant (15.5±29.2 vs. 6.48±5.51 ng/mL). However, the ratio of leptin levels to fat mass was significantly higher in dialysis patients than the controls (1.16±1.31 vs. 0.50±0.12, p=0.041). Serum levels of leptin in dialysis patients positively correlated with BMI-SDS, TSF-z score and BIA-based fat mass-z score (p<0.001 for all). Serum leptin levels also had a positive correlation with serum insulin (p=0.001) and HOMA-IR (p<0.001), and an inverse correlation with total ghrelin level (p=0.005). Conclusion: Children on maintenance dialysis have high levels of total ghrelin that are closely related to decreased fat mass and poor nutritional status. In contrast to ghrelin, leptin is associated with increased fat mass and insulin resistance; however, these patients have inappropriately elevated leptin levels in relation to body fat mass that may be related to wasting. Objective: This study investigated the influence of social support and other psychosocial factors upon mortality, adherence to medical care recommendations, and physical QoL amongst hemodialysis patients. Method: 272 HD patients were examined using the QoL questionnaire to determine self-reported inclinations. Logistics regression through Weighted K was used to analyze data. Results: 53.5% of patients reported health had interfered with their social activities demonstrating a strong associated with risk towards All-Cause (sp=1.33) and Cause-Specific Mortality including cardiac diseases (sp=1.28). These patients had a greater risk of withdrawing (sp=1.67) from treatment, non-adherence to Phosphorus (sp=1.06) greater than 7.5 mg/dL and increased risk towards an albumin of less than 3.5 g/dL (sp=1.23). Patients reporting dissatisfied with family support (12.0%) were at highest risk to non-adherence to intra-dialytic weight gain (sp=1.27), shortening the dialysis session (sp=1.21) and increased risk of Potassium level greater than 6 mEq/L (sp=1.14). However, patients reporting dissatisfied with staff support (14.1%) revealed a higher risk of decreased physical QoL (sp=0.76). Conclusion: This study demonstrated that physical QoL was not only affected by medications and other laboratory work-ups but also with additional psychosocial support. The study led to the development of programs empowering patients and families to participate in their treatment plans. The program includes various counselling approaches directed to patient, families, and health team. Objective: The aim of the study was to analyze health-related quality of live (HRQoL) in children with chronic kidney disease (CKD) dependent on the CKD stage, treatment modality and selected social life elements in families of the patients. Furthermore, potential differences between self-and parent/proxy reports and the factors influencing them were assessed. Methods: 203 CKD children (on hemodialysis-HD, peritoneal dialysis-PD and conservative treatment-CT) and their 388 parentproxies were enrolled into a cross-sectional national study. A semistructured interview form was used to determine the demographic and social characteristics of the participants. We used the Pediatric Quality of Life Inventory (PedsQL) 4.0 Generic Core Scales to assess the HRQoL in children.

Results: HRQoL scores for all CKD groups were significantly lower in all domains compared with population norms, the lowest one being in the HD group. In CT children, HRQoL did not depend on the CKD stage. Children with CKD reported problems with education and emotional functioning. Both parents assessed the HRQoL of their children differently depending on their involvement in the care. There are differences between the HRQoL scores of the children and their parents. Conclusion: The HRQoL in children with CKD is lower than in healthy children. This is already observed in the early stages of the disease. The disease itself influences the child's mental state. Children on HD require special support on account of the lowest demonstrated overall HRQoL. Children's lower rating of the quality of life observed by their parents may render the patients unmotivated and adversely affect their adjustment to life in later years. It may also create conflicts between the parents and the children. Objective: Chronic medical illness is a significant risk factor for the development of psychiatric disorders. The aims of the study were: to investigate the level of anxiety in children with chronic kidney disease (CKD) and to identify factors associated with the presence of that emotional problem. Methods: CKD children on hemodialysis (HD, n=22), peritoneal dialysis (PD, n=20,) and on conservative treatment (CT, n=95), and healthy subjects (n=100) were enrolled in the study. We used State-Trait Anxiety Inventory (STAI) for adolescents and STAI-C for children. Socio-demographic and physical factors were assessed. Results: There was a significantly higher level of anxiety-state among HD children (8-12 years) compared with other groups of participants of the same age. The level of anxiety among adolescents (13-18 years), both anxiety-state and anxiety-trait, was significantly higher in the HD group compared with other groups, which did not differ among themselves. In the HD adolescents, there was a correlation between the anxiety-state and the duration of the disease as well as with the number of hospitalizations. PD adolescents in the mainstream education had higher levels of anxietystate and anxiety-trait compared with home schooled patients. Conclusion: Even though children and adolescents with CKD are at risk of developing a variety of emotional disorders, the level of anxiety among the researched group, with the exception of HD patients, was not significantly different than the level of anxiety among healthy subjects. Adolescents on HD who present a high level of anxiety should undergo long-term psychological treatment.

Ipek Akil Objective: Compare medication adherence and kidney graft loss rate before and after transition in a single-center cohort of pediatric patients. Methods: Records of the patients transplanted in our center between 1990 and 2011 were screened and patients who remained in the program by their 18th birthday were included in the analysis. Adherence and graft function were assessed for the period of 2 years prior and 2 years after transition. Undetectable and/or sub-therapeutic levels of calcineurin inhibitors and their level variability were used as measures of adherence. Graft survival analysis was performed for the period of 4 years before and 4 years after transition. Results: Out of 197 screened patients, 71 were eligible and 25 of them were transitioned (TG -transitioned group). The remaining 46 patients were used as a comparison group (CG), 27 of them did not reach the age of transition by the time of the analysis. The median age at transplantation was 17.7 [15.1-19.1] years in the TG and 15.8 [13.5-17.6] years in the CG, median age at transition was 22. 5 [21.7-23 .3] years. Overall, there was no significant difference in adherence within TG before and after transition (p=0.5 for low drug levels and p=0.6 for drug levels variability); however, patients with lower pre-transition adherence (tertile I) showed improvement of their adherence following transition when compared with those with high pre-transition adherence (tertile III), p=0.02. There were 4 graft losses per 67.9 patient-years in TG vs 19 losses per 102.4 person-years in CG (p=0.02). The peak graft loss in all pediatric transplant recipients in our center (n=105 losses) occurred 3 years (SD 5.7 years) prior to the transition. Conclusion: At our institution, transition was not associated with worsening adherence or graft loss. This may be in part do to the fact that the transition occurred at a later age than in other institutions.

A report on 7-years experience in the use of mTOR-inhibitor (Sirolimus) in Paediatric renal transplantation patients with Calcineurin inhibitor (CNI) toxicity The management of children with end-stage renal disease (ESRD) deu to congenital urological abnormalities is more problematic and difficult than in patients with ESRD due to other causes. Kidney transplantation in neurogenic bladder patients with small capacity and defunctionionalized urinary bladders is a challenging issue in the field of pediatric transplantation. In these patients with severe bladder dysfunction, augmentation cystoplasty can protect the transplanted kidney by reducing intravesical pressure and creating an appropriate capacity. 8-year-old boy who presented urinary tract infection with fever at 2 year-old and while his investigation, stage 3 chronic kidney disease secondary to bilateral grade 5 reflux disease was found (blood urea: 81 mg/dl, creatinin: 1.5 mg/dl, creatinin clerance: 32 ml/min/1.73m 2 ). His mixiocystoureterographic and urodynamic study were showed bladder wall irregularity and trabeculation; decreased bladder capacity and compliance, respectively. At 8-month of followup he was putted into dialysis programme about 6 years. Thereafter, normal bladder capacity was achieved after bladder augmentation implementation. He was achieved renal transplantation from his mother, after 6-month of augmentation operation. Now, he still got uneventfull follow-up period about 24 months. Consequently, bladder augmentation application with timely and rationally could be a chance to kidney transplantation in children with bladder dysfunction.

The Effect Of Anti-Hla Antibodies On Renal Graft Functions Esra Baskin 1 , Asli Kantar 1 Umut Bayrakci 1 , Kaan Gulleroglu 1 , Mahir Kirnap 1, 2 , Feza Karakayali 1, 2 , Aysegul Haberal 1, 3 , Gokhan Moray 1, 2 , Mehmet Haberal 1, 2 1 Pediatric Nephrology, Baskent University, Ankara, Turkey 2 General Surgery, Baskent University, Ankara, Turkey 3 Immunology, Baskent University, Ankara, TurkeyObjective: The identification of suitable donor kidneys for transplant candidates with high levels of circulating antibodies against human leukocyte antigen (HLA) is a major challenge and results in adverse graft outcome. Methods: Seventy four kidney transplanted children without any shown HLA antibody in the pre-transplant period were enrolled in the study. Their anti HLA antibody status was checked by Luminex during post transplant period and its relation with the graft function and prognosis of the patients is studied. Results: Mean age of the patients was 13.5±5.2 years. Mean follow-up time was 3.8±1.1 years. Pre-transplant cytotoxicity tests and PRA was negative in all patients. Nine (12.1%) patients were found to have anti HLA antibodies after kidney transplantation. Mean time for the detection of antibodies was found as 11±4.8 months. Patients with anti HLA antibodies were similar with patients without antibodies in the terms of age, sex, HLA mismatch, transfusions and immunosuppressive drugs as well as the presence of viral infections. Mean serum creatinine level was found to be higher in patients with anti HLA antibodies. The antibody mediated rejection rate was found to be 7.2% (5/65) in patients without anti HLA antibodies while it was 55.1% (5/9) and remained dialysis dependent. All 6 were dialysis dependent by 6 months post diagnosis. Time from ANCA GN diagnosis to kidney transplant (Mean±SD) was 31±12 months (range 17 -48 months). All patients received induction therapy and maintenance immunosuppression with prednisone, mycophenolate mofetil, and tacrolimus. Median duration of follow up post transplantation was 3.5 years (range 1.25 -6.9). eGFR at last follow up was 71.9 ± 34.7 ml/min/1.73m 2 (range 5.7 -100.5). 1 patient lost her transplant to biopsy-proven, severe acute cellular rejection due to complete non-adherence to medications after 21 months of stable transplant function. No patient had recurrence of vasculitis. Conclusion: Short-term patient and allograft survival in paediatric patients with ESKD secondary to ANCA GN is excellent despite aggressive disease, with no recurrence of vasculitis post transplant.Abstract# P-SAT429 En-bloc kidneys from infant donors less than 5 kg transplantation into pediatric recipients at school age Objective: Given the shortage of donor kidneys in China, the use of grafts from deceased infant donors (weight < 5kg) is a potential approach to expand the donor pool. In this study, we reviewed the results of the first cohort of en bloc kidney transplantation of infant donors to pediatric recipients at school age in our center.Methods: From February 2012 to March 2013, 4 infant en bloc kidney transplants in pediatric recipients were performed in our center. En bloc kidneys from 4 infant donors (Maastricht Category III) who died of severe congenital disease were recovered and donated to the Red Cross Society of Tianjin and allocated to our center by China Organ Transplant Response System (CORTS). Donor age ranged from 33 to 56 days with weight ranging from 2.5 to 5.0 kg. Recipients included 2 females and 2 males with age ranging from 5 to 11 yr. The en bloc graft was implanted extraperitoneally in the right iliac fossa. The distal end of the donor aorta was anastomosed end-to-end to the internal iliac artery, while the donor vena cava was anastomosed to the external iliac vein. The donor ureters were implanted separately onto the bladder with double-J stents placement. After the operation, the recipients received basiliximab as an induction therapy. Maintenance immunosuppression consisted of tacrolimus and Myfortic. Prophylactic anticoagulation with heparin was used for the first week after transplantation. Results: Patient survival was 100%. Complications included delayed graft function in 1 patient (managed by PD for one week), urine leak in 1, and anticoagulation-related hemorrhage in 1. Due to discontinued anticoagulation, one graft was lost early from vascular thrombosis. Of the remaining 3 recipients, all had immediate and excellent long-term function with average creatinine of 1.13±0.31 mg/dL at 5 months follow-up (range 1-12 months). Conclusion: This is the first report of en bloc kidney transplantation from infant donors into pediatric recipients in China. Many improvements by our transplant teams had improved the survival of patients and grafts. Based on our experience, albeit very limited, we concluded that favorable outcomes can be obtained from en bloc transplantation from infant donors. Objective: To describe the rates and outcomes of renal transplantation in children with intellectual disability (ID). Methods: We performed a retrospective analysis of all children receiving a first kidney alone transplant in the United Network for Organ Sharing (UNOS) dataset from January 1, 2008 to October 31, 2011. Recipients with definite, probable, and without ID were compared using chi-square and Fisher's exact tests. Kaplan Meier curves were constructed for patient and graft survival. Results: Over the study period, 218 children with definite (90) or probable (128) intellectual disability underwent first renal transplant accounting for 17% of all first pediatric renal transplants (total n=1280). Children with definite or probable ID did not significantly differ from other recipients on the basis of gender or ethnicity but tended to be younger. Children with definite ID had higher rates of structural kidney disease and lower rates of glomerulonephritis. Children with ID were not significantly different than children without ID with respect to rate of preemptive transplant, donor source, or number of episodes of acute rejection. Graft and patient survival were similar between children with definite or probable ID and without ID. In Cox regression, intellectual disability was not significantly associated with patient or graft survival. Conclusion: In this first large-scale study, 17% of all first pediatric renal transplants are performed in children identified as having intellectual disability. Early outcomes after transplant appear to be equivalent between children with and without ID. Further research is needed on long-term outcomes and quality of life effects of transplant in this population.

Mariana Guerra Duarte Rosa de Lima, Ana Cristina Simoes e Silva, Nadine Marcia de Faria, Eleonora Moreira Lima Pediatrics, Universidade Federal de Minas Gerais, Belo Horizonte, BrazilObjective: The aim of this study was to evaluate the clinical course of children and adolescents undergoing renal transplantation at Federal University of Minas Gerais between 2000 and 2011 and to identify possible factors that could interfere with graft survival. Methods: A retrospective observational cohort study through analysis of medical records of patients below 18 years submitted to renal transplant were performed. Data were analyzed in SPSS, version 19.0. The results were expressed by descriptive variables and survival analysis was performed using the Kaplan-Meier method. Comparisons between subgroups were made using the log-rank test. P level was set at below 5%. Results: We analyzed 64 patients who underwent 66 transplants. The mean age was 10.5 ± 3.9 years. The main causes of chronic kidney disease were CAKUT (32.8%) and glomerular diseases (26.6%). Most patients were submitted to dialysis before transplantation (81.9%) with a mean duration of 36.5 ± 25.5 months. Deceased donor was used in 57.6% and the average cold ischemia time was 22.2 ± 6.3 hours. Dialysis after renal transplantation was performed in 22.7% of cases. The median survival of patients 1, 5 and 10 years was 98.3%, 95.8% and 92.8%. There were four deaths in this population, two after graft loss. Twelve patients lost their grafts (19.7%). The median survival of the graft was 9.2 years and 1, 5 and 10 years survival was 91.7%, 81.2% and 75.6%, respectively. No statistical difference was detected in graft survival between the deceased versus. living donor recipients, the different age groups, the occurrence of hypertension or not, the preemptive transplantation vs. preceded by dialysis (p=0.97) and those with more than three versus less than three HLA mismatches. A significant reduction in graft survival was associated with cold ischemia time associated with 24h, need for dialysis after transplantation, early acute or late rejection and creatinine greater than 1mg per dl after the first year os transplantation. Conclusion: In our center, graft survival was similar to that described in the literature. However, further studies are needed to address the variables that negatively impacted the results of transplantation. Objective: The aim of this study is to assess whether there is an increase in anti-HLA antibodies after the Influenza A/H1N1 vaccine given to pediatric pts with ESRD on dialysis (D) and with kidney transplant (T). Methods: 21 pediatric pts were enrolled. Mean age was 15.5 years old, 29% female, 71% male, 57% African American, 29% Hispanic.Objective: PTLD is the most common malignancy and important complication of pediatric solid organ transplantation. The rates of PLTD have been increasing through 1990s in KTx recipients. We conducted a survey of PTLD in children who received KTx since the introduction of Tac. Methods: From 1975, KTx was performed in 463 recipients at our center. Among them, we analyzed data from children who had undergone KTx since the introduction of Tac and retrospectively studied incidence of PTLD and risk factors, including immunosuppression protocol and pre-KTx EBV serology. Results: We retrieved the data of 188 pediatric recipients since 1997 (110 boys, median age at KTx 8.2 years, living KTx 170, pre-emptive KTx 26, EBV-seronegative 82). Among them, 11 cases (10 EBVrelated, 1 non-EBV) of PTLD were diagnosed in 10 recipients (5.3%), and all cases were EBV-seronegative at KTx. The median duration from KTx to the onset of PTLD was 7 months (range: 4-101 months). The most common presenting symptom of PTLD was abdominal complaints in 9 cases, followed by fever in 3, superficial lymphadenopathy in 3 and nasal congestion in 1. Immunosuppressants were reduced in all cases; the subsequent treatment consisted of antiviral agents + IVIG (3/11), anti-CD20 monoclonal antibody (5/11), and anti-CD20 monoclonal antibody + chemotherapy (2/11). Only one recipient died due to PTLD. Assessing the correlation between PTLD and immunosuppressants in 82 EBV-seronegative recipients (median age at KTx 5.7 years, CyA 39, Tac 43, MZR 26, MMF 56, IL-2 receptor antibody 56), a significantly higher incidence of PTLD was associated with Tac (9/43) than CyA (1/39) [OR 18.3, p=0 .003] in multiple logistic regression analysis. We compared the incidence of PTLD, median age at KTx, and rate of EBV-seronegativity in 3 eras (A: 1997-2001, B: 2002-2006, C: 2007-2011) of the study period and found 4%/8.2 yrs/32% in A, 3%/7.1 yrs/43% in B, and 10%/9.0 yrs/55% in C. Thus the incidence of PTLD increased with the increase in EBV-seronegative recipients. Conclusion: In our study, maintenance treatment with Tac (compared with CyA) is associated with a higher risk for developing PTLD. Attention should be paid to the increase in the EBV-seronegative recipients.Abstract# P-SAT438 Acute graft dysfunction and encephalitis post renal transplant: the role of Epstein-Barr Virus Objective: Epstein-Barr Virus (EBV) has been described as a rare cause of acute kidney injury and encephalitis in children. Methods: We report a case of a 5 year old renal transplant recipient who developed acute graft dysfunction and a significant neurological insult with evidence of primary EBV infection. Results: The patient had been diagnosed with posterior urethral valves prenatally and received a live related renal transplant aged 4 years. Seven months post-transplant he presented fluid overloaded, oliguric, with significant uraemia and hyponatraemia. There was a history of lower urine output and sore throat 4 days before admission with evidence of anaemia with red blood cell fragments, thrombocytopenia, mild monocytosis, raised LDH, ALT and splenomegaly on presentation. Haemodialysis was commenced. Primary EBV infection was subsequently detected by serology and PCR.Renal biopsy showed a picture of acute glomerular thrombotic microangiopathy(figure above) and acute tubular injury secondary to haemoglobinuria.Scanty EBER+ lymphocytes containing EBVencoded small nuclear RNA were also present. Within 3 days of presentation patient deteriorated neurologically developing a reduced conscious level, dysarthria, decreased motor power peripherally and an inability to upward gaze. An MRI brain demonstrated increased T2 signal and mild diffusion restriction in in the caudate nuclei, putamina and ventro-lateral thalami. This picture has been previously described in EBV encephalitis. There was a gradual neurological improvement and by day 7 of admission, and 6 doses of i.v. methylprednisolone, the patient had a full neurological recovery. The number of EBV copies has subsequently decreased on lower dose of immunosuppression. By week 4 after presentation he was no longer dialysis dependent with creatinine 3-fold higher than the previous baseline. Conclusion: This is the first report of acute graft dysfunction and encephalitis due to primary EBV infection in renal transplant recipient.Abstract# P-SAT439 Indoleamine 2,3-Dioxygenase (Ido) As A New Immunological Marker In Kidney Elisa Loiacono 1 , Barbara Votta 2 , Alessandro Amore 1 , Licia Peruzzi 1 , Maria Paola Puccinelli 2 , Roberta Camilla 1 , Luca Vergano 1 , Giuliana Guido 3 , Maria Elena Donadi 1 , Rosanna Coppo 1 1 Nephrology, Cittàdella Salute e dellaScienza. Regina Margherita Children's Hospital, Turin, Italy 2 Nephrology, Cittàdella Salute e dellaScienza, Turin, Italy 3 Nephrology, Sapienza University of Rome, Rome, ItalyObjective: The enzyme indoleamine 2,3dioxygenase (IDO), induced by interferon-gamma (IFN-gamma) and Toll-like receptors (TLRs) ligands in dendritic cells, degrades the essential aminoacid tryptophan (Trp) to kinurenine (Kyn). Its activity is estimated by the ratio of Kyn to Trp concentration (Kyn/Trp).T-cell activation and proliferation are affected by Trp deprivation and accumulation of Kyn. Therefore, activating IDO during immune responses counter balances mechanism of negative feedback loop of IFN-gamma and acts as a tool to downregulate overwhelming immune activation. IDO activation has been reported to be increased in acute rejection and downregulated in vitro by the immunesuppressants adopted for organ transplantation. Objective: To determine the prevalence of mycophenolic acid (Myfortic) use as part of the immunosuppressant regime in our current paediatric renal transplant recipients in a single tertiary paediatric nephrology centre and examine the demography of these cases. Method: Case note and pharmacy record review of all paediatric renal transplant recipients' history, immunosuppressant medication and current renal allograft function in January 2013. Indication for medication switch from mycophenolatemofetil to enteric-coated mycophenolic acid tablets (Myfortic) was noted. Data presented as median (range). Results: 14 (10 male) out of a total of 88 patients with renal transplants are currently receiving Myfortic in our centre. Age and time from transplant was 12.3 (8. 1-17.4 ) years and 4.3 (0.68-10.6) years. The most common cause of end stage renal disease requiring transplantation in this subgroup was posterior urethral valves in 5/14 cases. Two of these recipients had ABO incompatible living related transplants. Current eGFR was 46.7 (20.9-82.3) ml/min/1.73m2. 13/14 patients receiving Myfortic were also receiving prednisolone and tacrolimus as part of their immunosuppression regime. One patient was on tacrolimus and Myforctic only (history of idiopathic intracranial hypertension). Dosage of Myfortic varied based on patient size from 180mg twice daily to 720mg twice daily. The indication for carrying out a medication switch from mycophenolatemofetil to Myfortic was related to gastrointestinal symptoms in all cases; specifically diarrhoea in 9 cases, abdominal pain in 2 cases and both symptoms in 3 cases. These symptoms improved on Myfortic with renal allograft function remaining stable, however in view of neutropenia in one patient her Myfortic is currently suspended and under review.

We have found that enteric-coated mycophenolic acid (Myfortic) was a well-tolerated alternative in paediatric renal allograft recipients who developed gastrointestinal symptoms whilst receiving mycophenolatemofetil. Its usage should be considered in such patients who are able to take tablet preparations.

Ferretti Alfonso, Ilaria Luongo, Bruno Minale, Gabriele Malgieri, Carmine Pecoraro Nephrology and Urology, Santobono Children' Hospital, Naples, ItalyObjective: UTI represent one of the main complications after KT and have an important role in graft funcion impairment. This retrospective report is aimed to evaluate the incidence of UTI in a group of patients attending our hospital for post transplant usual medical controls. Methods and Results: We incuded in our study 75 children who underwent KT between 2000 and 2011: 42 M and 33 F, mean age 11.6 +/-5.3 . Four children received a graft from a living related donor and 71 from a deceased donor. First morning sterile urine sample to the microbiology laboratory was obtained when attending the hospital for its usual medical control or immunesuppressive drug detection levels. Results: in 41 patients (54.7%) ESRD was secondary to CAKUT. Thirty-six patients (48%) presented at least one episode of UTI occurred in the first 6 months after KT, mainly in females (1.2:1) . UTI developed in the first period were caused mainly by Gram negative bacteria (91%). E. Coli was the main agent (66.1%), the other uropathogens involved were: Proteus (9%), Enterobacter Cloacae (9%), Pseudomonas Aeruginosa (6%), Candida Albicans (3.3%) and Klebsiella (3.3%). Later infections were caused mainly by Candida, Klebsiella, ProteusansdEnterobacterfaecalis. One patient at the 15th month post-transplantation manifested UTI caused by Corinebacterium Urealyticum associated with concretions of baldder mucosae. Eight patients (5M and 3 F) experienced the graft loss: 4 patients were affected by CAKUT and had febrile UTI postTx. One patient left the second graft by a an acute deterioration of renal function during an UTI by BKV. Conclusion: UTI in Kidney transplanted children represent a main complaint as demonstrated by the high incidence (48%) in total population mainly in females. A significant role is played by primary uropathy and immunosuppression as demonstrated by the high frequency also in non uropathic patients. The role of UTI in longterm outcome of KT remains controversial.

Factors associated with elevated pulse wave velocity in children after renal transplantation Objective: Even after successful transplantation children with underlying chronic kidney disease (CKD) still carry a high cardiovascular risk. This is also documented by cardiovascular death being the second leading cause of death after transplantation in this patient cohort.Methods: In a cross-sectional approach 109 renal transplant recipients at three German transplant centres were enrolled. We measured pulse wave velocity (PWV), a strong predictor for cardiovascular events, as our primary end point. In all participants we assessed classical (e.g. blood pressure, cholesterol) and non-classical (e.g. CRP, PTH) cardiovascular risk parameters. Results: Patients were 13 ± 3 years of age and had received their transplant 5 ± 4 years ago. PWV-SDS adjusted to height was 0.4 ± 1.5. In order to identify predictors of elevated PWV, we performed a univariate screen and introduced factors with a p-value below 0.2 into a stepwise forward linear regression analysis. We found that systolic blood pressure in the ambulatory blood pressure measurement and GFR independently predicted PWV-SDS (table) . Conclusion: Our results show a large variability in aortic stiffness in children after renal transplantation. We found elevated PWV to be associated with classical as well as non-classical risk factors. The wide range of cardiovascular comorbidity seen in these patients highlights the need for individualized risk factor monitoring and management. Objective: Renal transplantation is the optimal treatment for end-stage renal disease in children,. However, it remains technically challenging in small recipients, especially with adult-sized graft. We report on two 4-year-old children, undergoing living donor kidney transplantation, and describe our management after failure to close the abdominal wall. Method: Both children weighed < 15kg and received their father's kidney. The recipient/donor weight ratios were 1/5 and 1/6. Surgical procedure was an extra peritoneal approach to the iliac fossa, with vascular anastomosis on the aorta and vena cava. They recieved intensive fluid replacement during surgery to optimize hemodynamic status and graft perfusion. After initial successful reperfusion, all attempts to close the abdominal wall (even simple skin closure or superficial muscle layer closure) led to graft hypoperfusion, diagnosed on graft colour change and doppler ultrasound. Closure with resorbableVicryl?mesh gave the same results and failure. Finally, we had to close with a synthetic non resorbable plate (Goretex?), without skin closure.Results: The first patient presented acute tubular necrosis and required continuous hemofiltration from day 2 to 6. The second one had immediate diuresis and with a decrease of serum creatinine to 65μmol/l at day 2. In both cases, kidney was successfully replaced intra abdominally at day 6, with superficial closure of facia and skin in one child and with Vicryl? mesh and skin in the second one. No effect on clinical graft perfusion or Doppler parameters were observed after final closure. In patient 2, a limited necrotic area 3x3cm, on direct contact with the Goretex?plate, was noticed during the second surgery procedure but it remained very superficial. Finally, with a follow-up of respectively 1 year and 1 month, recipients and grafts are doing well without any other events and a serum creatinine of 40-50 μmol/l. Conclusion: In conclusion, in small patients kidney size discrepancy and tissue oedema can lead to renal allograft compartment syndrome. In this situation, Goretex? plate is an option for initial wound closure and graft salvaging. Objective: AMR in the transplant is one of the most complicated form of rejection, which do not respond to the standard therapy. Survival of the transplant exposed to AMR is still low. This is a clinical report of an AMR-treatment in children in our centre. Methods: 4 girls with AMR after the deceased donor renal transplantation were observed of age10, 13, 15, 16 from 2009 to 2012 in our centre. All had a negative "cross-match" at the time of transplantation, the quantity of discordant antigens varied from 3 to 6. For 1 girl it was the 2 transplantation. 3 children had an immediate function of the graft,1-delayed. Induction of the immunosuppressive therapy: polyclonal antibodies and methylprednisolone, maintenance: tacrolimus, prednisolone, MMF. 2 children had high level of preexisting antibodies (more than 30%) at the time of transplantation. There were morphological confirmation and decrease of the graft function for the moment of AMR. The treatment of AMR included: pulse therapy of methylprednisolone, polyclonal antibodies, rituximab, immunoglobulin iv. Function of the graft was evaluated by the Cr levels, GFR, the level of DSA.Results: The Cr of children was 483±256 umol/l for the moment of developing of AMR. Function of the graft has been restored to satisfied: Cr fall to 121±28 umol/l, there were no proteinuria. It was noted a significant reduction of DSA I and II class in 3 from 4 patients. Conclusion: Thus, treatment of an AMR of the graft with methylprednisolone, rituximab and immunoglobulin intravenously let us achieve 100% 1-year graft and recipient survival after deceased donor kidney transplantation in children. Since the risk of AMRdevelopment is high in children with high levels of preexisting antibodies, addition of one-time rituximab and immunoglobulin intraveneously in therapy seems reasonable.Abstract# P-SAT454 Tacrolimus granules use in kidney transplanted infants. Dose and safety.Ramon Vilalta, Enrique Lara, Alvaro Madrid, Marina Munoz, Sara Chocron, Gema Ariceta Paediatric Nephrology, HospValld'Hebron, Barcelona, SpainObjective: Tacrolimus in infants has been administered as an extemporaneously compounded oral liquid made by hospital pharmacy. Modigraf® is a new tacrolimus formulation available as sachets containing granules (0.2 mg and 1 mg), that are made up with water, prepared by parents or caregivers. Modigraf® is considered at least as safe and practical than the compounded oral tacrolimus liquid. Furthermore, it is easier to be stored and ready to use, and promotes patient's autonomy, preventing potential medication errors. 9 kidney transplanted infants, 3 de-novo and 6 converted from oral liquid to Modigraf® were evaluated to assess its safety and adequate dose. Methods: After induction with Basiliximab or ATG, de novo's3 patients were treated with Modigraf® associated with MMF and with tapered steroids. 6 infants with similar immunosuppression regimen were converted from oral tacrolimus to Modigraf®. Target tacrolimus through level was 8-10 ng/ml. Mean patients' age at transplantation was 2.2 years old, and mean follow-up period with Modigraf® was 10±8 months.Results: In the de-novogroup the stable Modigraf® dose needed to reach target levels was 0.28±0.12 mg/kg/day. In the converted group, the initial oral tacrolimus liquid dose was 0.20±0.12 mg/kg/day, whereas Modigraf® stable required dose was higher: 0.30±0.10 mg/kg/day (p =0.0003). Target levels of tacrolimus were reached after