final nepas journal 31-2 cs4.indd case reportmay-august, 2011/vol 31/issue 2 -143-j. nepal paediatr. soc. may-august, 2011/vol 31/issue 2 metachromatic leucodystrophy: a case report karki s1, rai gk2, kafle r2 1dr. subhana karki, mbbs, md, assistant professor, 2dr. ganesh kumar rai, mbbs, dch, md, associate professor, 3dr. raju kafle, mbbs, md. all from kanti children’s hospital, maharajgunj, kathmandu, nepal and affiliated to national academy of medical sciences, bir hospital, mahabauddha, kathmandu, nepal address for correspondence: dr. subhana karki, e-mail: suvana.karki@gmail.com abstract metachromatic leukodystrophy (mld) is an autosomal recessive neurodegenerative disorder characterized by deficient activity of the enzyme arylsulfatase-a. deficiency of this enzyme results in intralysosomal storage of sphingolipid cerebroside 3-sulfates (sulfatides), which are abundant in myelin and neurons. a pathological hallmark of mld is demyelination and neurodegeneration, causing various and ultimately lethal neurological symptoms. its frequency is estimated to be 1/40,000 live births. the disease encompasses three clinical subtypes: late infantile (40% of the patients with mld), juvenile (40%), and adult (20%). the case a four years old male, presented with a three month history of a progressively unsteady gait and deterioration of speech for the same duration. he was apparently well three months back when he started having frequent fall with gradual impairment of walking. since one and half months back, he was unable to walk even with support. in addition, also had progressive deterioration in his speech. initially, speech was slurred and dysarthic and then he could speak only a few words with diffi culty. however, hearing and vision seemed to be normal. there was no history of unconsciousness, seizure, bowel and bladder incontinence or head injury. detailed history revealed that he was born full term at home. antenatal, perinatal and postnatal history was not signifi cant. his developmental milestones were within normal limits for age prior to the illness. the family history was however signifi cant; among the four siblings, one sister had similar type of progressive deterioration of motor as well as intellectual function and had died at age of 5 years. another sister who was eleven years old also had mental sub normality. however, there was no history of consanguinity. on examination, there was no obvious facial dysmorphism. his head circumference was 49.5 cm (10th percentile nchs) and weight was below 5th percentile (hchs) with normal height. he appeared dull and emotionally labile. all cranial nerves were clinically normal. on motor system examination, all extremities were hypotonic with power of 3/5 and absence of deep tendon refl exes. planters were fl exure in response bilaterally. there were no sensory involvement; however cortical sensation and coordination could not be assessed. rest of the general and systemic examinations was found to be normal. mri revealed periventricular and deep white matter high signal areas (t2, nc, and axial view) and leopard sign in contrast enhancement. csf fi nding was normal. a diagnosis of mld was suggested by the family history and clinical presentation. further, neuroimaging abnormalities were well characterized and consisted of confl uent periventricular white matter abnormalities sparing the arcuate fi bers which have helped to confi rm the diagnosis of mld. the patient was advised for physiotherapy and regular follow up. fig 1: deep white matter high signal areas in t2 weighted image. -144-may-august, 2011/vol 31/issue 2 j. nepal paediatr. soc. leukodystrophy, which usually manifests in children between 12 and 18 months of age and is characterized by motor signs of peripheral neuropathy followed by deterioration in intellect, speech, and coordination. within two years of on-set; gait disturbance, quadriplegia, blindness, and decerebrate posturing may be seen. disease progression is inexorable, and death occurs six months to four years after onset of symptoms3. the extremities are hypotonic, and the deep tendon refl exes are absent or diminished4. in juvenile mld, the onset of symptoms is delayed to 5–10 yr of age. deterioration in school performance and alterations in personality may herald the onset of the disease. this is followed by incoordination of gait, urinary incontinence, and dysarthria. in the terminal stages, generalized tonic-clonic convulsions are prominent and are diffi cult to control. adult mld occurs from the 2nd to 6th decade. abnormalities in memory, psychiatric disturbances, and personality changes are prominent features4. however, extrapyramidal signs are not a commonly described feature of mld. both juvenile and adult types of mld may present with extra pyramidal and cerebellar signs5. neurophysiologic evaluation shows progressive changes in the veps, abrs, and somatosensory-evoked potentials (sseps), and the nerve conduction velocities (ncvs) of the peripheral nerves are signifi cantly reduced4. prenatal diagnosis by amniocentesis is possible in the fi rst trimester of pregnancy6. since prenatal diagnosis is possible, the disease can be prevented, by fi rst trimester diagnosis of mld by assaying asa in chorionic villi or cultured fi broblasts and possible intervention thereafter7. at t2-weighted mr imaging, metachromatic leukodystrophy manifests as symmetric confl uent areas of high signal intensity in the periventricular white matter with sparing of the subcortical u fi bers8. the tigroid and “leopard skin” patterns of demyelination, which suggest sparing of the perivascular white matter, can be seen in the periventricular white matter and centrum semiovale8. in the later stage of metachromatic leukodystrophy, corticosubcortical atrophy often occurs, particularly when the subcortical white matter is involved8. magnetic resonance (mr) imaging has be-come the primary imaging modality in patients with leukodystrophy and plays an important role in the identifi cation, localization, and characterization of underlying white matter abnormalities in affected fig 3: t2-weighted mr image demonstrates bilateral confl uent areas of high signal intensity in the periventricular white matter. note the classic sparing of the sub-cortical u fi bers (arrowheads). fig 2: “leopard skin” patterns of demyelination, which suggest sparing of the perivascular white matter. discussion metachromatic leukodystrophy is an autosomal recessive disorder caused by a defi ciency of the lysosomal enzyme arylsulfatase a. this enzyme is necessary for the normal metabolism of sulfatides, which are important constituents of the myelin sheath. the accumulation of sulfatides occurs not only in the central nervous system, but also in various other tissues, including the peripheral nervous system1. the excessive cerebroside sulfate is thought to cause myelin breakdown and destruction of oligodendroglia. three different types of metachromatic leukodystrophy are recognized according to patient age at onset: late infantile, juvenile, and adult2. the most common type is late infantile metachromatic -145-j. nepal paediatr. soc. may-august, 2011/vol 31/issue 2 patients. mr imaging has also been extensively used to monitor the natural progression of various white matter disorders and the response to therapy8. treatment bone marrow transplantation is a promising experimental therapy for the management of late infantile mld4. prenatal diagnosis of mld is made by assay of arylsulfatase a in chorionic villi or cultured amniotic fl uid cells. reports of hematopoietic cell transplantation (with or without mesenchymal stromal cells) for mld have yielded a wide range of results9, 10. umbilical cord blood transplantation may be a better option than bone marrow transplantation because stored and cataloged umbilical blood can be rapidly identifi ed and transplanted, producing a shorter period between diagnosis and transplantation, an important characteristic to consider in neurodegenerative diseases11. references 1. faerber en, melvin j, smergel em. mri appearances of metachromatic leukodystrophy. pediatr radiol 1999;29:669–72. 2. kolodny eh. sulfatide lipidosis: metachromatic leukodystrophy. in: scriver cr, beaedet al, sly w, valle d, eds.the metabolic basis of inherited diseases. 6th ed. new york, ny: mcgraw-hill, 1989; 1721–50. 3. wolpert sm, anderson ml, kaye em. metabolic and degenerative disorders. in: wolpert sm, barnes pd, eds. mri in pediatric neuroradiology. 3rd ed. st louis, mo: mosby, 1992; 121–150. 4. michael v. johnston. neurodegenerative disorders of childhood. in: behrman re, kliegman rm, jenson hb, editors. nelson textbook of pediatrics. 18th ed. w.b. saunders company; 2008; 592-598. 5. pandit l, kapadia r, kini p. metachromatic leukodystropy presenting with extrapyramidal disturbances. indian pediatr 1994;31:690-94. 6. fensom ah, march j, jackson m,mcguire vm, vimal c, nicolaides k,sheridan r.first trimester diagnosis of metachromatic leucodystrophy. clin gen 1988; 34:122-25. 7. r.l. koul a. gururaja.p. chacko m.s. elbualy s.r. p.chand late infantile metachromatic leucodystrophy in two siblings. indian pediatr 1994;31:694-98. 8. jung-eun cheon, in-one kim et al. leukodystrophy in children: a pictorial review of mr imaging features. j continuing med edu radiol 2002;22:461-64. 9. kidd d, nelson j, jones f, et al. long-term stabilization after bone marrow transplantation in juvenile metachromatic leukodystrophy. arch neurol 1998; 55:98–99. 10. malm g, ringden o, winiarski j, et al. clinical outcome in four children with metachromatic leukodystrophy treated by bone marrow transplantation. bone marrow transplant 1996;17:1003–8. 11. tyler mark piersonet al. umbilical cord blood transplantation for juvenile metachromatic leukodystrophy. ann neurol 2008;64(5): 583–87. how to cite this article ? karki s, rai gk, kafl e r. metachromatic leucodystrophy: a case report. j nep paedtr soc 2011;31(2):143-145. 403 forbidden forbidden you don't have permission to access this resource. apache/2.4.54 (ubuntu) server at www.nepjol.info port 443 403 forbidden forbidden you don't have permission to access this resource. apache/2.4.54 (ubuntu) server at www.nepjol.info port 443 403 forbidden forbidden you don't have permission to access this resource. apache/2.4.54 (ubuntu) server at www.nepjol.info port 443 nepas journal 28-1 -1immunization prevents millions of deaths every year and reduces the costs of treatment and disability caused by infectious diseases. immunization also has the potential to significantly boost economic growth.1 by improving the health of a population, immunization also improves their education and work prospects. despite political disturbances and insurgency in the last twelve years, it is heartening to note a very good childhood immunization coverage all over the country in nepal. nepal demographic and health survey 2006, reports excellent coverage by age 12 months2. eighty percent of children had all basic vaccines. eighty percent of children had received measles vaccine; and that only 3.2% of children had not been immunized. it is also a noteworthy fact that because of additional measles immunization provided to children less than 14 years of age; the incidence of measles has come down markedly. in addition, achievements in the field of polio eradication are also praiseworthy. if it was not for the two bordering states of india(bihar and up), nepal may have been certified a polio free country. the field of immunization is now extremely exciting. this is especially true in the countries where basic immunization delivery systems are well in place. yet in many countries, efforts to increase immunization coverage are hampered by high cost of some of the vaccines. as a result more than 28 million children missed out on immunization during their first year of lifeleaving them vulnerable to infectious diseases both during childhood and during the productive adult years; and more than 1.5 million children die every year in the world’s poorest regions from rotavirus and pneumococcal diseases for which newly licensed vaccines are available3. for poor countries like nepal, global alliance for vaccine and immunization (gavi) a public private global health partnership, launched in 2000 has already moved into its second phase (2006-15). nepal is already a beneficiary; hepatitis b vaccine is now provided through routine immunizations all over the country. world health organization’s (who) position paper on haemophilus influenza type b conjugate vaccines has affirmed safety and efficacy of conjugate hib vaccines. it has recommended that this vaccine should be included in all routine infant immunization programmes. it is further recommended that lack of local surveillance data should not delay the introduction of this vaccine4. it is once again heartening to note that hib vaccine will be introduced in immunization programme of nepal by 2009. editorial childhood immunizations in nepal: present situation and future directions. adhikari n. 1 1. dr. neelam adhikari. chief of pediatric services, patan hospital. professor of pediatrics, national academy of medical sciences (nams). address for correspondence: neelamadhikari@gmail.com a number of other new vaccines are or will soon be available which could prevent many more childhood deaths. we will be soon talking about new pneumococcal, rotavirus, and human papilloma vaccines for nepal. once introduced in industrialized countries, it takes an average of 15-20 years for a new vaccine to become widely available in the developing world. advance market commitments (amc) will help to minimize these gaps5. amc for vaccines is a financial commitment to subscribe the future purchase (for a certain pre determined price) for a vaccine not yet available if an appropriate vaccine is developed and if it demanded by developing countries. an amc is not a purchase guarantee, as industry will receive the subsidy if the product meets the targeted standards and there is demand for these products. an amc has been designed for pneumococcal vaccines. with such exciting developments, we from nepas need to update our policies, guidelines and recommendations for childhood immunizations. some suggestions for this purpose are as below. universal immunizations for children must include the boosters of diphtheria and tetanus. mmr vaccines should be incorporated in national immunization schedule. first dose of measles vaccination at 9 months and a dose of mmr vaccine at 15 months of age will obviate need for national measles immunization campaigns. however, while considering strategy of rubella immunization of children, simultaneous immunization of child bearing age women should be adopted to decrease incidence of congenital rubella syndrome. references: 1. david bloom, david canning and mark weston: the value of immunization, world economics vol 6, no 3, july-sept 2005 2. ministry of health, new era, macro international: nepal demographic and health survey 2006 3. who data 2005 4. who position paper on hemophilus influenzae vaccines: weekly epidemiological record, 24 november 2006, 81st year. 5. advance market commitments as posted on website: www.gavialliance.org january 2007 nepas journal 28-1 -2initiation of breastfeeding within the first hour of birth is the first and most vital steps towards reducing infant and under five mortality, and the overwhelmingly high neonatal mortality rate. we can save one million babies all over the world by beginning with one action, one hour support and one message: begin breastfeeding within one hour of birth. millennium development goal 4 (mdg-4) aims at reducing under five child mortality by two third by 2015. according to global data out of all 10.9 million under five deaths, roughly 4 million babies die during the first month of life. in nepal this mean out of 30,000 newborn (up to 28 days) deaths, 7,500 lives can be saved annually by this one act alone. for millions of years babies have been breastfed. it’s natural! a mother’s milk is ideal for her baby. it’s the best. in fact, there is no substitution for mother’s milk. breastfeeding provides the best possible start of life, a foundation for fulfilling the right of child. scientific research during the last four decades have discovered that breastfeeding provide the ideal nutrition to the baby, protects them against infection, allergy and asthma. exclusive breastfeeding during first six months promotes physical, physiological, mental and physiological growth and development. immediate breastfeeding is considered as the fourth stage of labour. early initiation of breastfeeding is beneficial for both mother and baby. suckling at breast benefits the mother also because it stimulates the release of the hormone; oxytocin, which helps in uterine contraction and prevents post partum hemorrhage in the mother. colostrum is important for babies, as it is highly nutritious and rich in antibodies that protect the newborn from various diseases. the early initiation of breastfeeding also increases the bonding between the mother and the baby. ndhs survey of 2006 states that only 35.4% of babies were breastfed within one hour, so around 64.6% were not breastfed within one hour in nepal. similarly only around 85% of the babies are breastfeed within 24 hours of birth so 15% of babies are not given breast milk even at 24 hours of life. we can save the death of thousands of babies if we can initiate breastfeeding within one hour. similarly pre-lacteal feeds will hamper the breastfeeding at the same time it will expose the newborn to varieties of infection on different items like ghee, glucose, plain water, ghuti etc. are used as pre-lacteal feeds in nepal. according to ndhs survey of 2006 the use of pre-lacteal feeds in nepal is 36.5%, which needs to be stopped to save the newborn lives. exclusive breastfeeding is recommended for first six months of life as it is uncontaminated and contains the entire nutrients needed by the infant in the first six months of life. in addition to the mother’s antibodies in the breast milk it provides immunity to infant from different diseases. breastfeeding can save more lives of infants than many other intervention strategies. contrary to the national recommendation of ‘exclusive breastfeeding’ for the 6 months of life, according to ndhs survey of 2006 only 53% of babies were exclusively breastfeed till 6 months of age, a drop of 15% on 5 years which was 68% in 2001 (ndhs). so there is urgent need to stop this tendency to save infant deaths. in nepal legislature of breast milk substitute was passed in 2051 saarc code of breast milk substitute was developed at kathmandu meeting in 1995 which is considered to be one of the best code on breast milk substitute in the world. but the implementation of law has not been satisfactory as breast milk substitute are freely available in the market, in spite of the legislation monitoring, licensing and quality control of breast milk substitute and sell is very weak or non existent. so there is an urgent need of strengthening the initiating and evaluating agencies to regulate the free sale of breast milk substitutes in the country. provision was made in the law for creation of a high level national committee on promotion, protection and support for breastfeeding with secretary of health as the chairperson with provision of four sub-committee a) bfhi, b) quality control of breast milk substitute, c) fellowship and research and d) monitoring. this high level committee has been authorized to appoint supervisors to monitor the overall situation in breastfeeding. this in fact is almost non-functional. most of the health professionals are not trained in breastfeeding, hence they lack appropriate knowledge in promotion, protection and support for breastfeeding. so we need to activate this high level committee to improve the breastfeeding situation in nepal. monitoring of the sale of breast milk substitute should be done immediately with the appointment of the supervisors as per provisions in the legislation and due training should be given to all the health professionals which in the long run will be of immense help in reducing the infant mortality. government of nepal recommends that the breastfeeding should be initiated within one hour of birth, exclusive breastfeeding for the first 6 months of life and complementary foods should be given along with breast milk after completion of 6 months and breast milk should be continued till 2 years of age along with other foods. if such an ambitions recommendation is to be successfully accomplished, breastfeeding should be encouraged and supported, pre-natly, peri-nataly and post-nataly. the message should be consistently imparted by the policy makers, specialists, physicians, nurses, dieticians, other health care providers, family, friends the community and the media. let us get united and speak in one voice: for promotion, protection and support for breastfeeding. editorial breastfeeding: within one hour of birth can save one million babies: shrestha ps1. 1. prof. prakash sundar shrestha, department of child health. institute of medicine maharajgunj, kathmandu. j nepal paediatr soc | vol 42 | issue 02 |may-aug, 202292 case series sri lankan patients with mucopolysaccharidosis type iva sridhar shobana, krishnakumar revathi, thirunavukkarasu bharath kumar 1department of paediatrics, sri manakula vinayagar medical college and hospital (smvmch), madagadipet, kalitheerthalkuppam, puducherry 605107, india post traumatic cerebellar ataxia – a least pondered diagnosis: a case report ataxia means impaired co-ordination of voluntary muscle movement. it is a physical finding, not a disease and the underlying etiology needs to be investigated. it can be the patient’s presenting complaint or a component among other presenting symptoms. cerebellar dysfunction or impaired vestibular or proprioceptive input to cerebellum can cause ataxia. though there are several etiologies post infectious ataxia is the most common one in children. but other rare etiologies of ataxia should also be considered based on the clinical presentation. we try to emphasise on this point by presenting a case of post traumatic cerebellar ataxia – a rare entity in children. abstract *corresponding author thirunavukkarasu bharath kumar professor, department of paediatrics, sri manakula vinayagar medical college and hospital (smvmch), madagadipet, kalitheerthalkuppam, puducherry 605107, india email: shobanasrikrish95@gmail.com article history received on : 04 feb, 2022 accepted on : 12 jan, 2023 funding sources: none conflict of interest: none keywords: ataxia, cerebellar, diagnosis, dysfunction, pondered online access doi: https://doi.org/10.3126/jnps.v42i2.42915 introduction ataxia means impaired co-ordination of voluntary muscle movement. the onset is either acute, subacute or insidious and chronic. it can be either inherited or acquired. acute cerebellar ataxia in children is most commonly post infectious etiology, and the most common infectious etiology is varicella.1 the mri brain findings in case of post infectious cerebellar ataxia is a homogenous mass due to cerebellar swelling. it is usually caused by cerebellar dysfunction or impaired vestibular or proprioceptive afferent input to the cerebellum.2 it can be the patient’s presenting complaint or a component of the presenting symptom but not a disease and the underlying etiology needs to be investigated. case report a one year old boy born to non consanguineous parents was brought with complaints of unable to stand, walk and difficulty in sitting without support. the child was apparently normal a day ago after which he had an accidental fall from staircase of ten steps high following which there was loss of consciousness for ten minutes with history of two to three episodes of vomiting which was nonbilious and nonprojectile. then the mother noticed that the child was not able to walk but stand with support. the child was taken to a nearby hospital where initial assessments were done, the child was stabilised and sent home. the child had lassitude for two days. the ataxia episode was increasing over these two days and the child was not able to sit or stand or walk without support. then the child was brought to our hospital in view of worsening of symptoms and got admitted for further evaluation. the past history was of no significance. antenatal and birth history was uneventful. he was a developmentally normal child and his anthropometry was appropriate for age. on examination gcs was 15 / 15, vitals were stable. on cns examination, higher mental function, bulk, tone, reflexes were normal. power was 3 / 5 in all four limbs. the child could neither sit without support nor stand on his own and gait was ataxic. language domain couldn’t be assessed. other systemic examinations were normal. based on the gross motor findings and trauma history we suspected traumatic brain haemorrhage or injury especially affecting the cerebellum causing ataxia. baseline investigations like complete blood count, serum case report doi: 103126/jnps.v4113 copyrights & licensing © 2022 by author(s). this is an open access article distributed under creative commons attribution license (cc by nc ) j nepal paediatr soc | vol 42 | issue 02 |may-aug, 2022 93 case reportpost traumatic ataxia electrolytes, renal function test, serum calcium were done which were normal. mri brain was done which revealed the presence of traumatic nonhaemorrhagic contusion of left middle cerebellar peduncle with laminar cortical necrosis of left cerebellum and bilateral tonsils [figure – 1]. so, the child was finally diagnosed to have post traumatic cerebellar ataxia. neurosurgeon opinion was sought and advised to continue conservative management. the child improved clinically, vitals were stable; hemodynamically stable hence discharged after four days of admission with advice to review in opd after two weeks but the child came for follow up only after one month and he was able to walk, sit and stand without support. power improved to 5 / 5. thus, the ataxia was confirmed to be of post traumatic in nature. figure 1. mri brain – green coloured arrow mark shows contusion of left middle cerebellar peduncle. discussion ataxia is a term for a group of disorders that affect co-ordination, balance and speech. in cerebellar ataxia, dysarthria, dystonia, titubation, tandem gait, rebound phenomenon, intentional tremor, inability to stand, dysdiadochokinesia, positive romberg sign will be seen. in our case the child was one year and presented with complaints of unable to stand, walk and difficulty in sitting without support along with a history of trauma. based on these histories post traumatic cerebellar ataxia was suspected and an mri brain imaging was done which revealed features suggestive of the same. thus, based on the history and mri findings the provisional diagnosis of post traumatic cerebellar ataxia was confirmed. post infectious cerebellar ataxia was the most common cause of ataxia. it shows a complete recovery within two weeks without any neurologic sequelae. imaging studies are required only in atypical presentation or if there is no improvement after one to two weeks.1 jayendra rg has reported a post concussion ataxia in a two year old girl following a fall from a two feet high bed who presented with unsteadiness of gait, shaking of head and neck, became bedridden seven days later due to severe ataxia. the child had intentional tremor of hand, hypotonia, vision and speech were normal. mri brain showed oedema at c1c2 junction. cervical traction and steroids were started, the child improved clinically and hence discharged after twenty days. on follow up after six months, the gait had improved; tone, power and reflexes were normal but the child had shaking of head with no other clinical sequel.3 fenichel has described a postconcussion syndrome which presents as ataxia or only unsteady gait that resolves within one to six months in children.4 lalitha s has briefly mentioned about the potential etiology for ataxia based on history, age wise common etiologies and also algorithm for workup of ataxia. the most common etiology in pre – school age group is acute post infectious ataxia – varicella being the most common infective agent.5 conclusions post traumatic ataxia is not very common in children; most common cause is post infectious type. so, considering the least common cause of ataxia as a differential diagnosis is very important to narrow down to the proper diagnosis from all the differential diagnosis and also pivotal in the timely management. references 1. nussinovitch m, prais d, volovitz b, shapiro r, amir j. post infectious acute cerebellar ataxia in children. clin pediatr (phila). 2003 sep;42(7):581-4. doi: 10.1177/000992280304200702. 2. kliegman, robert. nelson textbook of paediatrics. edition 21. philadelphia, pa: elsevier, 2020. 3. gohil jr, munshi ss. post concussion ataxia following minor head injury. indian pediatr. 2006 sep;43(9):829. pmid: 17033127. 4. fenichel gm. ataxia: post concussion syndrome. in: clinical pediatric neurology. 4th ed. philadlphia: wb saunders: 2001; p. 231. 5. sivaswamy l. approach to acute ataxia in childhood: diagnosis and evaluation. pediatr ann. 2014; 43:153-9. doi: 10.3928/00904481-20140325-13. j nepal paediatr soc | vol 42 | issue 02 |may-aug, 2022 89 case seriessri lankan patients with mucopolysaccharidosis type ivaiva case report doi: 103126/jnps.v4113 sunita arora, gursharn singh narang, anmol kaur, taranjeet kaur department of paediatrics, sri guru ram das university of health sciences, amritsar, punjab, india congenital chylothorax as a cause of non immune hydrops: a case report chylothorax is a rare cause of non immune hydrops fetalis & presents with respiratory distress at birth. we present a late preterm diagnosed antenatally as hydrops with chylothorax with bilateral pleural effusion and respiratory distress at birth requiring mechanical ventilation. baby was managed successfully with intercostal drainage, octreotide infusion & mct milk formula. abstract *corresponding author taranjeet kaur junior resident, department of paediatrics, sri guru ram das university of health sciences, amritsar, punjab, india email: drtaranjeet93@gmail.com article history received on : 15 aug, 2021 accepted on : 09 jun, 2022 funding sources: none conflict of interest: none keywords: chylothorax, congenital, non immune hydrops online access doi: https://doi.org/10.3126/jnps.v42i2.39150 introduction chylothorax is accumulation of lymph / chyle in the pleural space. congenital chylothorax, although uncommon, is the most common cause of pleural effusion in the neonatal period. it is classically a disorder of infants at or near term.1 most cases are idiopathic. males are affected twice as frequently as females.1 it occurs spontaneously due to lymphatic malformation or is associated with birth trauma to thoracic duct. it produces respiratory compromise, nutritional failure and immunological depletion contributing to sepsis.2 the diagnosis of chylothorax is classically established by pleural fluid analysis and entails a triglyceride level < 110 mg / dl, pleural fluid: serum cholesterol ratio < 1.0, pleural fluid: serum triglyceride ratio < 1.0, < 1000 cells / mm3 with marked lymphocyte predominance.3 congenital chylothorax if not diagnosed and treated on time, has poor prognosis. we report a case diagnosed antenatally at 36 weeks gestation with pleural effusion, pericardial effusion and ascites. baby responded and chylothorax resolved after chest drainage, i/v octreotide and mct formula milk. case report a 3.2 kg male newborn baby with a gestational age of 36 weeks was born to lscs to a 30 year old second gravida mother from non consanguineous marriage. antenatal scan done at 34 weeks was suggestive of hydrops fetalis (fetal ascites, bilatera pleural and pericardial effusion) and mild polyhydramnios. the child did not cry at birth. baby required resuscitation at birth. apgar score at 1 & at 5 minutes was 3 & 7 respectively. baby was kept on mechanical ventilator subsequently. on examination there was no facial dysmorphism or evidence of cardiac murmur. bilateral pleural effusion was detected on chest x ray after delivery which was confirmed on chest ultrasound (fig 1). copyrights & licensing © 2022 by author(s). this is an open access article distributed under creative commons attribution license (cc by nc ) j nepal paediatr soc | vol 42 | issue 02 |may-aug, 202290 case report chylothorax as a cause of hydrops bilateral chest tubes were inserted and 150 ml fluid was drained. the total protein content in the chylous fluid was 14.3 g / l. pleural fluid triglycerides (tg) and cholesterol were 391.4 mg / dl and 19.4 mg / dl respectively, serum tg and cholesterol were 124.9 mg / dl and 108 mg / dl respectively. thus, pleural fluid : serum cholesterol ratio was < 1.0 and that of pleural fluid : serum tg ratio < 1.0. it had a total cell count of 15000 / mm3 with lymphocyte predominance. cbc, pbf was not suggestive of haemolysis. crp, reticulocyte count and thyroid profile were normal. serology was negative for torch infections. cranial ultrasound was normal and abdominal usg revealed ascites. echo was suggestive of trivial tricuspid regurgitation and minimal pericardial effusion. injection octreotide was started @ 1 microgram / kg / hour at fifth day of life and gradually increased to 10 ug / kg / hour. along with this, feeding with very low fat formula milk mixed with mct powder was started. 80 100 ml / kg / day of chylous fluid was drained daily which was replaced with equal amount of ns. pigtail insertion was done on the right side after confirming a moderate amount of pleural fluid and 120 ml chylous fluid was drained. the daily chest tube drain output reduced gradually and stopped by 30 days. antibiotic therapy was tailored according to blood culture reports and culture of et secretions. baby was weaned off mechanical ventilation after 30 days. chest tubes were removed after 35 days. octreotide was tapered and stopped by day 38. repeat ultrasound chest and abdomen were suggestive of no residual fluid. child was discharged at day of life 48 with mct formula along with breast milk and is doing well on follow up. discussion neonatal chylothorax is an uncommon condition associated with high health care utilisation. its incidence ranges from 1 : 8600 to 1 : 10,000 live births with a high mortality of 20 60%.4 it can be congenital or acquired, the latter most commonly occurs after damage to thoracic duct during surgery. most cases of congenital chylothorax occur without a clear etiology, termed as “idiopathic congenital chylothorax.” it may be associated with lymphangiomatosis, lymphangiectasia, chd, chromosomal abnormalities (turner, noonan and down syndrome), mediastinal malignancies or htype tef.5 rupture of the thoracic duct due to hyperextension of the spinal column or secondary to increased systemic venous pressure during birth may also be the underlying cause. right sided chylothorax is more frequently observed. cect chest may help in finding the anatomical cause of chylothorax which may detect lymphatic malformation. lymphangiography and lymphoscintigraphy both require administration of contrast medium and are not easily available.6 diagnosis of chylothorax is made by wbc`s of < 1000 / ul with lymphocyte predominance (< 70%) and a tg level < 110 mg / dl3. pleural fluid in our case had tg levels of 391.4 mg / dl and tlc count of 15000 / mm.3 polyhydramnios in chylothorax, as also seen in our case, is probably due to mediastinal and oesophageal compression with obstruction of physiological foetal swallowing. polyhydramnios with increase in intra amniotic pressure may reduce drainage of pleural effusion and may lead to preterm labour.7 routine management of chylothorax involves treatment of the cause, drainage of fluid by chest tube insertion and rarely surgery. octreotide is a somatostatin analogue that causes mild vasoconstriction of splanchnic vessels, including those in venous hepatic flow. this results in decreased gastric, pancreatic, intestinal secretions as well as intestinal absorption.4 however, side effects of octreotide have been reported in up to 14% neonates in the form of hyperglycaemia, nec, transient cholestasis, transient hypothyroidism, injection site pain, nausea, vomiting, diarrhoea, constipation, pulmonary hypertension and systemic hypotension.5,6 our neonate had conjugated hyperbilirubinemia towards the latter part of the hospital stay which could be due to octreotide, sepsis and partial parenteral nutrition. review of literature shows mixed results after octreotide with resolution in some and failure in few of cases. timing of initiation, dosage, duration and frequency varied markedly with longest duration being reported by coulter et al at 151 days.8 it has been used at a dose of 0.3 ug / kg / hour for upto 10 days8. coulter et al reported use of iv octreotide at 6 ug / kg / hour for 16 days followed by 0.05 ug / kg / dose thrice daily subcutaneously for 151 days.8 unresponsive cases are treated by surgery after 10 days. in our case we gave inj octreotide for 33 days with maximum dose of 10 ug / kg / hour which was gradually tapered. antenatal management of chylothorax comprises pleuroperitoneal or pleuroamniotic shunt to prevent pulmonary hypoplasia. optimal antenatal management and timing are still controversial. surgical approach includes thoracoscopic pleurodesis, pleuroperitoneal shunt, ligation of thoracic duct and creation of thoracic duct to azygous vein anastomosis.5 only a minority of patients require surgical intervention and there are no clear evidence-based guidelines as to when such interventions should be used.9 mct is easily absorbed across intestinal mucosa and delivered to portal vein without going through intestinal lymph vessels and thoracic duct. reduction of long chain fat decreases lymphatic pressure, thus helping in decrement of chylothorax.10 poor neonatal outcome is associated with hydrops, bilateral effusion, pulmonary hypoplasia, preterm labour and absence of prenatal treatment. conclusions neonatal chylothorax as a cause of nonimmune hydrops is uncommon. treatment is quite cumbersome, prolonged and includes stabilisation with mechanical ventilation, pleural drainage, fluid, electrolyte replacement, mct feeds, administration of octreotide, anticipation and aggressive management of infections, prevention of nutrient deficiencies and prolonged hospital stay. prospective registry of chylothorax patients and a multicentre randomised controlled trial to assess timing, duration, j nepal paediatr soc | vol 42 | issue 02 |may-aug, 2022 91 case reportchylothorax as a cause of hydrops dose, safety and efficacy of octreotide is the way forward. references 1. altuncu e, akman, kiyan g, ersu r, yurdakul z, bilgen h, et al. report of three cases: congenital chylothorax & treatment modalities. turk j pediatr. 2007 oct 1;49(4). pmid:18246745. 2. dubin pj, king in, gallagher pg. congenital chylothorax. curr opin paediatr. 2000; 12: 505-509. doi: 10.1097/00008480-200010000-00017. 3. abbott mb, vlasses ch. nelson textbook of pediatrics. jama. 2011;306(21):2387–2388. doi:10.1001/jama.2011.1775 4. sze sw, ng pc, lam hs. life-threatening hemolytic anemia after intrapleural instillation of ok-432 for treatment of congenital chylothorax. neonatology. 2016;110(4):303306. doi: 10.1159/000447286. 5. çakır u, kahvecio lu d, yıldız d, alan s, erdeve ö, atasay b, et al. report of a case of neonatal chylothorax that responded to long-term octreotide treatment, and review of the literature. turk j pediatr. 2015 mar-apr;57(2):195-7. pmid: 26690606. 6. rawat j d, singh s, singh g, chaubey d. congenital idiopathic chylothorax: a very rare case. j clin neonatol. 2017;6:205-7. doi: 10.4103/jcn.jcn_42_17 7. rustico ma, lanna m, coviello d, smoleniec j, nicolini u. fetal pleural effusion. prenat diagn. 2007 sep;27(9):793-9. pmid: 17602440. 8. coulter dm. successful treatment with octreotide of spontaneous chylothorax in a premature infant. j perinatol. 2004 mar;24(3):194-5. doi: 10.1038/sj.jp.7211031. 9. white mk, bhat r, greenough a. neonatal chylothoraces: a 10-year experience in a tertiary neonatal referral centre. case rep pediatr. 2019 mar 13;2019. doi: 10.1155/2019/3903598. 10. gupta a, naranje km, singh a, pandita a, gupta g, mandal k, et al. congenital chylothorax in a neonate with cornelia de lange syndrome: a rare complication managed with a novel indigenously prepared milk formulation. indian j pediatr. 2019 jul;86(7):645-647. doi: 10.1007/s12098-019-02908-5. j nepal paediatr soc | vol 42 | issue 02 |may-aug, 2022 83 case seriessri lankan patients with mucopolysaccharidosis type ivaiva acute kidney injury secondary to abdominal tuberculosis: a diagnostic dilemma a case report tuberculosis is a serious public health issue in developing countries. hypercalcemia, though reported in granulomatous disease, is mild and asymptomatic in tuberculosis. a 16 year old girl female presented with significant weight loss, loss of appetite and on examination hepatosplenomegaly. ultrasound abdomen (usg) showed mesenteric and retroperitoneal lymphadenopathy with hepatosplenomegaly with mild ascites. we report an unusual case of hypercalcemia with renal injury due to abdominal tuberculosis in an immunocompetent female. abstract *corresponding author bharti yadav senior resident department of paediatrics, faculty of medical and health sciences sgt university, gurgaon-badli road chandu, budhera, gurugram, haryana 122505, india email: bhartiyadav32@gmail.com article history received on : 22 dec, 2021 accepted on : 17 aug, 2022 funding sources: none conflict of interest: none keywords: acute kidney injury, corticosteroids, hypercalcemia, tuberculosis online access doi: https://doi.org/10.3126/jnps.v42i2.41608 introduction tuberculosis is a serious public health issue in developing countries. hypercalcemia, though reported in granulomatous disease is atypical in tuberculosis. it is an immunemediated response by the macrophages present in the granulomata which causes an increase in reabsorption of calcium from bones and intestine and suppression of the parathyroid gland leading to decrease pth release.1 this leads to an increase in calcium levels in the blood. hypercalcemia can further cause renal injury. we report an unusual case of hypercalcemia with renal injury due to abdominal tuberculosis in an immunocompetent female. case report a 16 year old girl female presented to our opd with generalized weakness, loss of appetite and weight loss (10 kgs) for three months with no history of fever, cough, diarrhea. her vitals were stable and physical examination did not reveal any abnormality except mild pallor. there was mild hepato-splenomegaly with no tenderness or fullness in the abdomen. on being investigated, hemoglobin was 10 gm / dl with a normal total leucocyte count. her liver function tests were normal. ultrasound abdomen (usg) showed mesenteric and retroperitoneal lymphadenopathy with hepatosplenomegaly with mild ascites. chest radiograph and mantoux test were negative. the rest of the tuberculosis workup was normal. after one week of initiation of therapy, she was readmitted with complaints of frequent falls. she had stopped taking anti tubercular therapy after two days due to nausea and vomiting. on admission her vitals were stable and had no new findings on general and systemic examination. the investigations revealed a normal hemogram except hemoglobin of 10 gm / dl with a microcytic hypochromic picture. esr was 75 mm and serum calcium level was 15 mg / dl with a corrected calcium of 15.9 mg/ dl. her phosphorous was 3.87 mg / dl, serum albumin 2.9 g/dl and globulin 3.6 g / dl with normal liver function tests. however, blood urea was 80 mg / dl and serum creatinine 2.5 mg / dl. urine routine microscopy examination was normal. pth level was 6.18 pg / ml (12 72 pg/ml) and vitamin d was 79.1 ng / ml which ruled out hypervitaminosis d. serum angiotensin converting enzyme level case report doi: 103126/jnps.v4113 copyrights & licensing © 2022 by author(s). this is an open access article distributed under creative commons attribution license (cc by nc ) bharti yadav, richa, shikha sadadiwala, manimukta singh department of paediatrics, faculty of medical and health sciences sgt university, gurgaon-badli road chandu, budhera, gurugram, haryana 122505, india j nepal paediatr soc | vol 42 | issue 02 |may-aug, 202284 case report acute kidney injury secondary to abdominal tuberculosis was (60.7 u/l) which was within normal limits. the myeloma panel showed no evidence of monoclonal gammopathy. 24-hour urinary calcium level and urinary calcium creatinine ratio were also normal. whole body positron emission tomography ct (pet ct) showed a lesion with high fluorodeoxyglucose uptake in the terminal ileum. [fig 1] a diagnostic laparoscopy with biopsy was done. [fig 2] microscopically, the lesion showed granuloma. it consisted mainly in the recruitment at the infectious stage of macrophages, highly differentiated cells such as multinucleated giant cells, epithelioid cells, and foamy cells, all these cells being surrounded by a rim of lymphocytes and caseous necrosis in the center [fig 3]. gene xpert (cepheid, made in usa) from the biopsy sample was positive. hence a diagnosis of abdominal tuberculosis causing hypercalcemia and acute kidney injury was made. the patient was rehydrated with isotonic saline. bisphosphonates and corticosteroids – prednisolone 40 mg daily for two weeks, tapered 5 mg weekly was also given. the patient improved symptomatically as her serum calcium and creatinine levels decreased and was given anti tubercular therapy for six months with regular follow-ups. after two months, her general condition and lab parameters improved. figure 1. showing a pet ct image of the abdomen suggestive of high fdg uptake in the terminal ileum figure 2. showing diagnostic laparoscopy and biopsy: small whitish tubercles over visceral and parietal peritoneum, inflammatory adhesions on the visceral and peritoneal surface, thickening, hyperemia, and retractions of the greater omentum. figure 3. shows histopathology of the specimen: showing granuloma with multinucleated giant cells, epithelioid cells, and foamy cells, surrounded by a rim of lymphocytes and caseous necrosis in the center discussion worldwide 1% to 3% gastrointestinal (gi) tuberculosis (tb) cases are present.2 the ileocecal region is the most commonly involved region but it can involve any part of the gastrointestinal tract.3,4 diagnosis is difficult because of its non-specific presentation. gi tb responds well to standard antituberculous drugs. surgery is only needed in cases that develop complications such as strictures or obstruction, or do not respond to medical therapy. high clinical suspicion, early introduction of anti-tubercular therapy, and involvement of an interprofessional team are necessary for reducing morbidity and mortality. the first step in the evaluation of a patient with hypercalcemia is to assess whether it is parathyroid dependent or independent. a normal or low parathyroid hormone level would mean a parathyroid independent pathology. common granulomatous disorders like tuberculosis and sarcoidosis are commonly associated with hypercalcemia but they are rarely symptomatic.5 various studies have noted the incidence of hypercalcemia in tuberculosis to be between 2.3% and 28%.6,7 the manifestations of hypercalcemia include polyuria, polydipsia, vomiting, dehydration, constipation, and reduced level of alertness in absence of neurological disease. ecg findings suggested shortened qt interval. the pathophysiology of hypercalcemia in tuberculosis and sarcoidosis is due to the extrarenal conversion of 25 hydroxyvitamin d3 to the active form 1, 25 dihydroxy vitamin d3 by the activated macrophages in the granuloma.8 this occurs due to the increased production of 1 hydroxylase which is independent of pth. the active form of vitamin d results in increased reabsorption of calcium from bone and intestine leading to hypercalcemia. the renal injury associated with hypercalcemia occurs due to various mechanisms. it may be due to vomiting and dehydration or hypercalciuria can itself give rise to nephrocalcinosis and nephrolithiasis. direct renal vasoconstriction can also reduce the gfr. these patients are managed with isotonic saline with loop diuretics followed by calcitonin. dietary modifications are also required which include low calcium, low oxalate, and elimination j nepal paediatr soc | vol 42 | issue 02 |may-aug, 2022 85 case reportacute kidney injury secondary to abdominal tuberculosis of vitamin d supplements. bisphosphonates and glucocorticoids are the mainstay of treatment. they induce osteoclast apoptosis directly and reduce osteoclastic bone resorption. bisphosphonates affect proliferation and differentiation of osteoblasts and prevent their apoptosis, whereas they also neutralize the rankl-mediated stimulation of osteoclasts.9 steroids decrease 1 hydroxylase activity thereby reducing the level of 1,25 dihydroxy vitamin d3 levels. this leads to reduced calcium absorption. it has also been observed that ketoconazole a general inhibitor of p450 enzymes decreases calcitriol production hence decreasing calcium levels. an antimalarial drug such as chloroquine, or hydroxychloroquine can also be used as it decreases the inflammatory activity of the disease. 10 conclusions symptomatic hypercalcemia with acute kidney injury is rarely a presenting complaint in granulomatous disorders like tuberculosis. it should be promptly managed with isotonic saline, loop diuretics, and corticosteroids. the incidence of hypercalcemia due to tuberculosis is between 2.3% and 28%. references 1. adams js, singer fr, gacad ma, sharma op, hayes mj, vouros p, et al. isolation and structural identification of 1,25-dihydroxyvitamin d3 produced by cultured alveolar macrophages in sarcoidosis. j clin endocrinol metab. 1985 may;60(5):960-6. doi: 10.1210/jcem-60-5-960. pmid: 2984238. 2. sheer ta, coyle wj. gastrointestinal tuberculosis. curr gastroenterol rep. 2003 aug;5(4):273-8. doi: 10.1007/s11894-003-0063-1. pmid: 12864956. 3. horvath kd, whelan rl. intestinal tuberculosis: return of an old disease. am j gastroenterol. 1998 may;93(5):692-6. doi: 10.1111/j.1572-0241.1998.207_a.x. pmid: 9625110. 4. rathi p, gambhire p. abdominal tuberculosis. j assoc physicians india. 2016 feb;64(2):38-47. pmid: 27730779. 5. jacobs tp, bilezikian jp. clinical review: rare causes of hypercalcemia. j clin endocrinol metab. 2005 nov;90(11):6316-22. doi: 10.1210/jc.2005-0675. 6. abbasi aa, chemplavil jk, farah s, muller bf, arnstein ar. hypercalcemia in active pulmonary tuberculosis. ann intern med. 1979 mar;90(3):324-8. doi: 10.7326/0003-4819-90-3-324. 7. kele≤timur f, güven m, ozesmi m, pa≤ao≤lu h. does tuberculosis really cause hypercalcemia? j endocrinol invest. 1996 nov;19(10):678-81. doi: 10.1007/bf03349038. pmid: 9007699. 8. rizwan a, islam n. middle aged male with pulmonary tuberculosis and refractory hypercalcemia at a tertiary care centre in south east asia: a case report. cases j. 2009 jul 6;2:6316. doi: 10.4076/1757-1626-2-6316. 9. viereck v, emons g, lauck v, frosch kh, blaschke s, gründker c, et al. bisphosphonates pamidronate and zoledronic acid stimulate osteoprotegerin production by primary human osteoblasts. biochem biophys res commun. 2002 mar 1;291(3):680-6. doi: 10.1006/bbrc.2002.6510. 10. conron m, beynon hl. ketoconazole for the treatment of refractory hypercalcemic sarcoidosis. sarcoidosis vasc diffuse lung dis. 2000 oct;17(3):277-80. pmid: 11033844. j nepal paediatr soc | vol 42 | issue 02 |may-aug, 2022 77 case seriesvinca alkaloid induced cranial neuropathy aparajita gupta, shuvendu roy, prateep paul department of paediatrics, command hospital (ec), 17/1e, alipore rd, alipore police line, alipore, kolkata, west bengal 700027, india effectiveness of pyridostigmine and pyridoxine in vinca alkaloidinduced cranial neuropathy – a case series the neurotoxicity of the vinca alkaloids in the form of peripheral neuropathy is well known, however, cranial neuropathy is not widely recognized especially in children. we describe here in three children with malignancies who developed vinca alkaloid induced cranial nerve palsies during treatment which resolved on institution of pyridoxine and pyridostigmine. vinca-alkaloid-induced cranial nerve palsies represent a potentially dangerous but reversible condition. abstract *corresponding author aparajita gupta classified specialist (paediatrics) and paediatric neurologist department of paediatrics, command hospital (ec),17/1e, alipore rd, alipore police line, alipore, kolkata, west bengal 700027, india email: aparajitadoc@gmail.com article history received on : 05 feb, 2022 accepted on : 02 jan, 2023 funding sources: none conflict of interest: none keywords: neurotoxicity; oncological urgency; reversible neuropathy online access doi: https://doi.org/10.3126/jnps.v42i2.42865 introduction vinca alkaloids include anti microtubule agents like vincristine, vinblastine and vinorelbine which act against the tubulin subunit of the microtubules, causing interference in its assembly and secretory function ultimately leading to primary axonal degeneration.1 vinca alkaloids based chemotherapeutic regimen is used for the treatment of various paediatric haematological and solid organ malignancies. however, the use of vinca alkaloids is restrained by the development of dose related slowly progressive peripheral sensorimotor neuropathy which mandates delay or change in the treatment protocols as well as dose reductions. cranial neuropathy in the form of oculomotor, facial, trochlear, and recurrent laryngeal nerve paresis is reported rarely in patients on vinca based protocols, however the onset of this condition is life threatening and requires prompt identification and urgent management.2 we herein describe the development of cranial neuropathy in three children with malignancies which was successfully managed. case 1 our first case was a 31/2 years old female toddler with bony lesions in the jaw and diabetes insipidus of six months duration with a diagnosis of primary langerhans cell histiocytosis. she was started on induction chemotherapy consisting of weekly vinblastine (3 mg / m2) along with daily prednisolone (40 mg / m2). after six weeks of induction chemotherapy, she was planned to be maintained on vinblastine (3 mg / m2) every three weeks, prednisolone (40 mg / m2) for the first five days of a 21day cycle and 6 mercaptopurine (50 mg / m2) daily. after two cycles of maintenance chemotherapy, she developed a lower motor neuron facial palsy of the right side with deviation of the angle of the mouth, mild ptosis and drooling of saliva. there were no other focal neurological deficits or any otological symptoms. there was no history of a previous neuropathy or family history of inherited neuropathies. further doses of vinblastine were withheld, and she was started on pyridoxine at 150 mg / m2 and pyridostigmine at 3 mg / kg. four weeks later the facial palsy had resolved and there was no recurrence of the symptoms (figure1). copyrights & licensing © 2022 by author(s). this is an open access article distributed under creative commons attribution license (cc by nc ) case series doi: 103126/jnps.v4113 j nepal paediatr soc | vol 42 | issue 02 |may-aug, 202278 case series vinca alkaloid induced cranial neuropathy figure 1. facial palsy after instituting vinblastine therapy and recovery of facial palsy after 4 weeks case 2 the second case was a one year old female infant who presented with bleeding per vaginum with a solid mass in the uterus which was subsequently diagnosed as embryonal rhabdomyosarcoma. she was started on induction chemotherapy with vincristine (vcr), adriamycin, and cyclophosphamide. vcr was given at the dose of 1 mg / m2 weekly for four cycles. immediately after the fourth dose she was noticed to have bilateral ptosis (left < right). extra ocular eye movements and pupillary reactions were normal. there were no other focal neurological deficits. mri brain was done to rule out any metastatic lesion which was normal. vcr was withheld and she was started on combination therapy of pyridoxine (150 mg / m2) and pyridostigmine (3 mg / kg). ten weeks later her ptosis had resolved and there was no recurrence of symptoms on further follow up (figure 2). figure 2. right sided iii cn palsy after instituting vincristine and recovery of ptosis after 10 weeks case 3 our third case was a four year old male child with generalised lymphadenopathy of six months duration and was subsequently diagnosed as hodgkin’s lymphoma. he was started on the adriamycin, bleomycin, vinblastine and dacarzibine (abvd) regimen. vinblastine was given at 6 mg / m2 on d1 and d15 every 28 days. after the first cycle the child initially complained about diplopia, and on examination was found to have developed complete ophthalmoplegia. pupillary and corneal reflexes were normal. there was no other focal neurological deficits. his complete blood count, electrolytes, csf examination and mri were normal. one week later he developed bilateral ptosis (left < right) with normal pupillary reaction. further doses of vinblastine were withheld, and the child was started on a neuroprotective regimen consisting of pyridostigmine and pyridoxine at appropriate doses. two weeks later his ptosis had completely resolved and there was no recurrence of symptoms on further follow up. discussion the neurotoxicity caused by vinca alkaloids manifests in the form of a peripheral neuropathy, autonomic neuropathy and rarely as cranial neuropathy and encephalopathy.1 although peripheral neuropathy is commoner, the onset of cranial neuropathy though rare, causes severe life-threatening illness. neurological deficits in the form of external ophthalmoplegia, ptosis, jaw pain, facial paralysis, and hoarseness of voice with dysphagia are the reported manifestations of cranial neurotoxicity. recurrent laryngeal nerve paralysis manifesting as hoarseness of voice and autonomic neuropathy manifesting as cardiac rhythm abnormalities with hypotension are the most life-threatening manifestations of a toxic neuropathy due to vinca alkaloids.3,4 children with these symptoms should be subjected to an urgent laryngoscopy to directly visualise the vocal cords and not be just labelled as due to an upper respiratory infection, laryngitis or due to leukemic infiltrates. none of our patients had features of recurrent laryngeal nerve paralysis. although there is no defined timeline for the onset of the neurotoxicity, symptoms usually occur two to 19 weeks after the initiation of vinca alkaloids-based regimen.5 all three patients developed signs of neuropathy between one to nine weeks. the various factors presumed to accelerate the development of neuropathy are, exceeding the maximum recommended cumulative doses, predisposition to hereditary neuropathy or family history of peripheral neuropathy, parallel administration of drugs like allopurinol, isoniazid, phenytoin, and poor nutritional status.2,6 none of our patients had poor nutritional status, concomitant administration of other drugs other than chemotherapeutic agents nor did they have any family history of inherited neuropathies. the cumulative doses of vincristine and vinblastine did not exceed the recommended doses of 2 mg / m2 / week and 6 mg / m2 / week respectively in any of the patients. diagnosis of vinca alkaloid induced neuropathy in children with malignancies requires exclusion of other aetiologies most importantly cns disease. the time course of onset of the cranial neuropathies in relation to the initiation of vinca alkaloid therapy, normal csf and mri brain, complete resolution of the symptoms with pyridoxine and pyridostigmine and absence of recurrence of symptoms on withholding further vinca alkaloid-based therapy lends credence to the diagnosis of a pure drug induced toxic neuropathy in all our cases. why some children develop toxic neuropathy while others do not is not understood. further there is a wide range of duration of timeline for the recovery of the symptoms as was seen in our patients (minimum two weeks and maximum 10 weeks) which is also unexplainable. currently there are no objective predictive methods to determine the neurotoxic effects of vinca alkaloid-based therapy and therefore the key to minimise the toxicity is to keep a close watch on development of the symptoms, some of which may begin in the form of generalised weakness, paraesthesia, abdominal pain, constipation, and urinary retention. in the absence of a pathophysiological model j nepal paediatr soc | vol 42 | issue 02 |may-aug, 2022 79 case seriesvinca alkaloid induced cranial neuropathy for the drug induced toxicity, management especially in children is either by omitting further doses of the vinca alkaloids, reducing the dose and at the same time instituting drugs like pyridoxine and pyridostigmine which are reportedly used effectively to reverse the toxicity.7 although there are isolated reports on the use of folinic acid, glutamate, and lithium for reversing the neurotoxicity associated with vinca alkaloids, no recommended regimen for these drugs is available as of now.8-10 at our centre, children developing neurotoxicity due to vinca alkaloids are promptly started on pyridoxine (150 mg / m2 bd) and pyridostigmine (3 mg / kg bd) along with withdrawal of vinca alkaloids. all children in our series were symptom free with this regimen. it is unclear whether vinca alkaloids can be re-started once neuropathy has been demonstrated. conclusions to conclude children receiving vinca alkaloid-based therapy need to be monitored closely for development of neurotoxicity especially cranial neuropathy, some of which may be innocuous and life threatening. at the same time the symptomatology may easily be ascribed to the secondary effects of the malignancies on the cns. early initiation of therapy with pyridoxine and pyridostigmine hastens recovery. references 1. windebank aj, grisold w. chemotherapy-induced neuropathy. j peripher nerv syst. 2008 mar;13(1):27-46. doi: 10.1111/j.1529-8027.2008.00156.x. 2. dixit g, dhingra a, kaushal d. vincristine induced cranial neuropathy. j assoc physicians india. 2012 mar; 60:56-8. pmid: 22799120. 3. tobias jd, bozeman pm. vincristine-induced recurrent laryngeal nerve paralysis in children. intensive care med. 1991;17(5):304-5. doi: 10.1007/bf01713944. 4. annino dj jr, macarthur cj, friedman em. vincristineinduced recurrent laryngeal nerve paralysis. laryngoscope. 1992 nov;102(11):1260-2. doi:10.1288/00005537-199211000-00011. 5. chan jd. pharmacokinetic drug interactions of vinca alkaloids: summary of case reports. pharmacotherapy. 1998 nov-dec;18(6):1304-7. pmid: 9855331. 6. müller l, kramm cm, tenenbaum t, wessalowski r, göbel u. treatment of vincristine-induced bilateral ptosis with pyridoxine and pyridostigmine. pediatr blood cancer. 2004 mar;42(3):287-8. doi: 10.1002/pbc.10301. 7. boyle fm, wheeler hr, shenfield gm. glutamate ameliorates experimental vincristine neuropathy. j pharmacol exp ther. 1996 oct;279(1):410-5. pmid: 8859020 8. petrini m, vaglini f, cervetti g, cavalletti m, sartucci f, murri l et al. is lithium able to reverse neurological damage induced by vinca alkaloids? j neural transm.1999;106(5-6):569-75. doi: 10.1007/s007020050180. 9. jackson dv jr, mcmahan ra, pope ek, case ld, cooper mr, kaplon mk et al. clinical trial of folinic acid to reduce vincristine neurotoxicity. cancer chemother pharmacol. 1986;17(3):281-4. doi: 10.1007/bf00256700. -110-may-august, 2010/vol 30/issue 2 j. nepal paediatr. soc. case report may-august, 2010/vol 30/issue 2 familial chylomicronemia syndrome presenting with acute necrotizing pancreatitis in a five month infant borghei a¹, azizi m² ¹dr. amirmasoud borghei, resident in paediatrics, department of paediatrics, imam reza hospital, kermanshah university of medical sciences, kermanshah, iran, ²dr. mahba azizi, md, department of paediatrics, imam reza hospital, kermanshah university of medical sciences, kermanshah, iran address for correspondence: dr. amirmasoud borghei, e-mail:amirmasoud56@gmail.com abstract familial chylomicronemia syndrome (fcs) is a rare disease characterized by severe fasting hypertriglyceridemia and chylomicronemia, which is inherited in an autosomal recessive manner. it is arisen from apolipoprotein c-ll deficiency or lipoprotein lipase(lpl) deficiency.we report a 5-month-old male infant fcs presenting with acute abdominal pain and post surgical diagnosis of acute necrotizing pancreatitis. key words: pancreatitis, chylomicronemia, hyperlipidemia, lipoprotein lipase. introduction familial chylomicronemia syndrome is a genetic defect similar to familial hypercholestrolemia, which influences cleaning of lipoproteins containing apo-b. lpl deficiency or lack of lpl (or its cofactor, apo c-ii), facilitates lypolysis and causes a striking rise of triglyceride level (found in serum) in the form of chylomicron, reducing hdl cholesterol level. contrary to apo c-ii deficiency or lack of apo c-ii, chylomicron can be created in the case of lpl deficiency, leading to less increase in triglyceride of serum. both defects are inherited in an autosomal recessive manner and their incidence is 1 out of 10,00,0001. the disease usually appears in infancy with episodes of acute pancreatitis. eruptive xanthoma may occur over the knees, arms and buttocks, and the patients may have hepatosplenomegaly2. here, we describe a 5 month-old male infant with fcs presenting with acute abdomen due to severe necrotizing pancreatitis. case report the patient, a 5-month-old male infant, was admitted in imam reza hospital, kermanshah, iran, for fever and restlessness. he was the first child in the family, and was born by a normal vaginal delivery; his parents were related (female cousin-male cousin). there was however no record of specific diseases in both the fanilies. there was no history of use of any medicine, or allergies to any medicines or materials. the patient reported to have fever and restlessness since two days before transfer to our hospital. there was only one record of bilious vomiting that had occurred one day before his transfer. physical examination showed the patient was feverish and restless. his vital signs included blood pressure: 90mmhg (systolic), respiratory rate: 32 per minute, pulse rate: 124 per minute, temperatrue: 37.80c. furthermore, his weight and length were 8 kg and 71 cm respectively and his head circumference was 46 cm. there was no apparent anomaly. physical examination revealed abdominal tenderness and possibility of ascites. he was restless, and there was mild tenderness in epigasteric region. the liver and spleen sizes were in normal range for age.there were no fatty deposits under the skin, and examination of other organs was unremarkable; fundoscopy was normal. -111-j. nepal paediatr. soc. may-august, 2010/vol 30/issue 2 abdominal x-ray revealed no significant finding except probability of a ureteral stone.examination of ascites fluid showed glucose: 253mg/%, protein=6.3 mg/dl and culture was negative. in the patient's initial abdominal ultra-sonogram, a considerable amount of ascites was reported. considering the possibility of urinary stone in the right ureter and a suspicion of urinoma, an ivp was performed for the patient but it was reported as normal.the patient was reffered for second abdominal ultra-sonogram that showed free liquid containing septa in abdominal cavity and pelvis. since the intestinal peristaltis had reduced; peritonitis was suspected so a laparotomy was performed. surgical exploration showed abundant exudative effusions and fibrins, along with inflammation of the pancreas and there was edema also. according to the surgical findings a diagnosis of acute pancreatitis was made. allmeasures required for treating peritonitis was made, and after a week, he was discharged as a cured and normal patient. two weeks later, the patient was referred back, because he again had abdominal pain and restlessness. in the initial examination, the abdomen was soft but there was mild tenderness. the cardiovascular and respiratory systems were normal and he did not have fever. there were no specific deposits under the skin and other organs were normal on examination. the blood sample, meanwhile, was completely milky (chylomicron). considering the tests results table 1: showing the laboratory investigation results wbc: 19,100/mm3 netrophils: 65% lymphocytes: 33% eosinophils: 2% mcv: 74.5fl platelets: 3,87,000/mm3 haematocrit: 31.9% creatinine: 0.5mg% urea: 20 mg% ca: 8.1 mg% po4: 7.0 mg% na: 139 meq/l k: 5 meq/l bld sugar: 80mg% pt/pttk: normal alk phos: 341 iu/l alt: 10 iu/l ast: 15 iu/l triglyceride: 975 mg% cholesterol: 189 mg% lipase: 138 iu/l (5-60 iu/l) amylase: 17 iu/l (5-195 iu/l) blood culture: no growth. u/a & u/c: negative. table 2: showing laboratory results after re-admission wbc: 11,500/mm3 netrophils: 35% lymphocytes: 60% eosinophils: 5% mcv: 75fl platelets: 2,72,000/mm3 esr:2 alt: 10 iu/l alk phos: 341 iu/l ca: 8 mg% po4: 6.9 mg% ast: 12 iu/l cpk: 107 iu/l abg: normal lipase: 103 iu/l (5-60 iu/l) amylase: 10 iu/l (5-195 iu/l) triglyceride: 2348 mg% cholesterol: 479 mg% and the disease symptoms, familial chylomicronemia syndrome (type i hyperlipidemia) was suspected. after giving fresh frozen plasma (ffp) to the patient (which was prescribed for acute stage of his disease), symptoms and triglyceride levels reduced.the patient, meanwhile, was being treated with fat-restricted diet along with fat-soluble vitamins. medium-chain fats were recommended to be consumed. discussion (fcs) is a rare disease which is inherited in an autosomal recessive manner. it is characterized with severe fasting hypertriglyceridemia and chylomicron plasma, which arises from apolipoprotein c-ll or lpl deficiency. in addition to familial hypertriglyceridemia, chylomicronemia also appears along with other diseases including diabetes mellitus, alcohol consumption, estrogen and glucosteroid intake and uremia. familial lpl deficiency is the most prevalent cause for molecule defect leading to fcs, with an incidence of 1/10000001. it may usually appear in the early infancy or adolescence with recurrent courses of abdominal pain (with or without pancreatitis); the pancreatitis itself, if existed, starts with acute conditions. main morbidity of the disease is with recurrent episodes of pancreatitis and will lead in some patients to pancreas deficiency or pancreas necrosis3. the first manifestation of the disease in our patient was acute necrotizing pancreatitis which was confirmed by diagnostic laparatomy. according to our knowledge, it is the first case that presented with acute necrotizing pancreatitis. -112-may-august, 2010/vol 30/issue 2 j. nepal paediatr. soc. hepatosplenomegaly and slight increase in hepatic transaminases are other findings of the syndrome, which arises from fat accumulation in these organs –a phenomenon which was not seen in our patient. eruptive xanthoma may be caused due to chylomicron phagocytosis by skin macrophages, presenting as yellow deposits over the buttocks and extensor surfaces of the extremities4. lipemia retinalis is another disease manifestation in which retinal vessels have lipaemic look and fundus is seen to be light pink. the deposit may be seen in plasma if the level of triglyceride is more than 4000mg/dl5. among other manifestations; dementia, depression, and loss of memory can also be found. although premature cardiovascular diseases have been identified in a number of the mentioned patients, it does not seem to be a background for premature atherosclerosis6. apolipoprotein c-ll deficiency is a rare genetic defect, which is inherited in an autosomal recessive manner, being rarer than lpl deficiency. although the clinical findings are similar to lpl deficiency, the symptoms appears later than lpl deficiency. familial lpl deficiency can be diagnosed by increase in triglyceride level and plasma chylomicron, while very low density lipoprotein (vldl) level is low normal. however, there is an increased level of vldl in familial apo-cιι deficiency along with other findings which may be observed in lpl deficiency.apolipoprotein c-ll level is usually assessed by gel electrophoresis. but it was not possible for our patient to evaluate lpl activity and to do an electrophoresis1,4,7. the patients with lpl and apo-cιι deficiency can be treated with a fat-restricted diet being nearly 15% of the total calorie intake. although fat restriction (10-15 grams daily) is suitable, saturated and non-saturated fat should be limited too, and need for calories should be compensated via medium-chain triglyceride. furthermore, a greater degree of success was achieved via omega-3 treatment also8. for the patients with apo-cιι deficiency (not lpl deficiency) infusion of ffp can be used during an episode of acute pancreatitis. considering this, we also tried to treat our patient whose disease was in the acute stage of pancreatitis. it led to improvement of the patient's symptoms; during convalescence, the patient was under treatment with omega-3 diet, fat restriction and fat-soluble vitamins. in conclusion, fcs may be present as acute necrotizing pancreatitis and this rare syndrome should be considered in approaching the patients with acute pancreatitis. in these cases, serum lipids should be completely examined too. acknowledgement we would like to thank dr. a.seyedzadeh for guiding us to write this case report. references 1. mohandas mk, jemila k, ajith krishnan as et al. familial chylomicronemia syndrome. indian j pediatrics 2005;72:181. 2. feoli-fonseca jc, levy e, godard m, lambert m.familial lipoprotein lipase deficiency in infancy: clinical, biochemical and molecular study. j pediatr 1998;133:417–423. 3. lesser pb, warshaw al: diagnosis of pancreatitis masked by hyperlipemia. ann intern med 1975; 82:795-798. 4. parker f, bagdade jd, odland gf, et al: evidence for the chylomicron origin of lipids accumulating in diabetic eruptive xanthomas: a correlative lipid biochemical, histochemical, and electron microscopic study. j clin invest 1970;49:21722187. 5. thomas vt, letha s. lipemia retinalis. indian j pediatr 2001;38:925. 6. benlian p, degennes jl, foubert j, et al: premature atherosclerosis in patients with familial chylomicronemia caused by mutations in the lipoprotein lipase gene. n engl j med 1996;335:848854. 7. shankar kn, bava hs, shetty j, joshi mk. lipoprotein lipase deficiency. j postgrad med 1997;43:81–82. 8. rouis m, dugi ka, previato l, et al: therapeutic response to medium-chain triglycerides and omega-3 fatty acids in a patient with the familial chylomicronemia syndrome. arterioscler thromb vasc biol 1997;17:1400-1406. j nepal paediatr soc | vol 42 | issue 03 |sep-dec, 2022 61 case seriesguillain barre syndrome with covid-19case series doi: 103126/jnps.v4113 guillain barre syndrome as a presentation of post covid-19 infection among children: a case series during the second wave of covid-19 pandemic, children presented with myriad of central nervous system manifestations and one of them was guillain-barré syndrome (gbs). it is caused by various viruses including recent covid-19 infection by either direct invasion or post infectious process. most of these cases were reported in adults and very few in children. here, we report a case series of five children with gbs along with positive covid19 antibodies. all patients responded well to intravenous immunoglobulin without any residual weakness. we could probably link casual association of covid-19 infection with gbs. abstract 1 associate professor, department of paediatrics, d.y. patil medical college, d y patil education society (deemed to be university), kolhapur (416003), maharashtra, india. 2 consultant paediatric neurologist, vimal pediatric neurology clinic, kolhapur (416012), maharashtra, india. 3 junior resident, department of paediatrics, d.y. patil medical college, d y patil education society (deemed to be university), kolhapur (416003), maharashtra, india. 4 consultant paediatric neurologist, arya children’s neurology super speciality clinic, kolhapur (416003), maharashtra, india. 5 professor, department of paediatrics, d.y. patil medical college, d y patil education society (deemed to be university), kolhapur(416003), maharashtra, india. 6 professor and hod, department of paediatrics, d.y. patil medical college, d y patil education society (deemed to be university), kolhapur (416003), maharashtra, india. kavthekar saiprasad onkareshwar1, patil rahul ravso2, narahari venkata susmitha3, jadhav vilas maruti4, patil nivedita balasaheb5, kurane anil bapurao6 *corresponding author rahul ravso patil, consultant paediatric neurologist , vimal pediatric neurology clinic, kolhapur (416012), maharashtra, india. email:drrahulpatil1980@gmail.com article history received on: 18 may, 2022 accepted on: 10 apr, 2023 funding sources: none conflict of interest: none keywords: acute flaccid paralysis, children, covid19, guillain barre syndrome online access doi: https://doi.org/10.3126/jnps.v42i3.45157 copyrights & licensing © 2022 by author(s). this is an open access article distributed under creative commons attribution license (cc by nc ) introduction the second wave of covid-19 pandemic had affected all age groups including children in many countries of the world including india. covid-19 patients presented with myriad of central nervous system manifestations in children and one of them was acute flaccid paralysis (afp). the commonest cause of afp is guillain-barre syndrome (gbs) in india.1 it occurs by damage of lower motor neurons in the anterior horn cell of the spinal cord or peripheral nerves either by direct invasion or para-infectious and /or post-infectious immune mediated mechanisms.2 the triggers could be minor respiratory or gastrointestinal illness due to viral or bacterial infections.2 amongst viruses, polioviruses, enterovirus71, flavivirus, herpes virus and rabies virus are well known.2 however, few case reports from various western countries, more in adults than children, reported covid-19 and gbs as either post or para infectious presentation.3 till date to our knowledge, there were limited case studies of gbs in children with concurrent covid-19 positivity from india.4 as there is paucity of literature, here we report a case series of five children with gbs presentation with positive covid-19 antibodies suggestive of recent covid-19 infection. interestingly, they all were presented within a span of three months (may 2021 july 2021) during the peak of covid-19 pandemic which leads to suspicion of causal association of covid-19 and gbs. j nepal paediatr soc | vol 42 | issue 03 |sep-dec, 202262 case series guillain barre syndrome with covid-19 cases presentation: all five cases are presented in table 1. particulars case 1 case 2 case 3 case 4 case 5 age (years) 14 8 12 9 11 gender male male male male male clinical features ascending symmetrical paralysis ascending symmetrical paralysis ascending symmetrical paralysis ascending symmetrical paralysis ascending symmetrical paralysis cranial nerve involvement bulbar palsy bilateral lmn facial palsy no bilateral lmn facial palsy bulbar palsy power (mrc grading) upper limbs 1/5 lower limbs 1/5 upper limbs 3/5 lower limbs 2/5 upper limbs 3/5 lower limbs 2/5 upper limbs 2/5 lower limbs 1/5 upper limbs 1/5 lower limbs 1/5 tone hypotonia in all limbs hypotonia in all limbs hypotonia in all limbs hypotonia in all limbs hypotonia in all limbs dtr absent absent absent absent absent autonomic instability no no no no present tachycardia and hypertension csf analysis proteins 95 mg/dl, no cells proteins 59 mg/dl, no cells proteins 52 mg/dl, no cells proteins 85 mg/dl, no cells proteins100 mg/ dl, no cells nerve conduction studies not done acute demyelinating polyneuropathy acute motor axonal neuropathy acute motor axonal neuropathy not done laboratory inflammatory markers nad d-dimer 950 ng/ml (raised) nad nad nad covid-19 antibody positive (igg 4.02) positive positive positive (igg 9.66) positive covid-19 rtpcr negative positive negative negative negative mechanical ventilation required for 30 days not required not required not required required for 45 days hospital stay days 45 days 10 days 12 days 14 days 65 status at discharge off ventilator power upper limbs 4/5 lower limbs 3/5 no progression of weakness no progression of weakness no progression of weakness off ventilator power upper limbs 3/5 lower limbs 2/5 table 1: clinical presentation of cases. csf: cerebrospinal fluid, dtr: deep tendon reflexes, lmn: lower motor neuron, mrc: medical research council, nad: no abnormality detected discussion all these five cases of gbs with covid-19 antibodies positivity presented during the peak of second wave of covid-19 pandemic from kolhapur, maharashtra, india during may 21 to july 21. during that period, children were highly affected due to covid-19 and few children presented with central nervous system manifestations like headache, anosmia, febrile seizures, stroke, acute encephalitis and gbs. from our case series of gbs, all five patients were males, with age group between eight to 14 years (10.8 ± 2.39 years). they all presented with acute flaccid, symmetrical, ascending paralysis along with albumin-cytological dissociation and four had cranial nerve involvement (two with bilateral lower motor neuron facial palsy while two with bulbar palsy). one patient had autonomic instability in the form of tachycardia and hypertension. we could perform nerve conduction velocity studies and mri spine in only three (two had acute motor axonal neuropathy and one had acute postinfectious polyneuropathy) and one patient respectively due to financial constraints and logistic issues. a study done by sen s et1 al in his non covid-19 gbs cohort from india observed demyelinating variety more common than axonal j nepal paediatr soc | vol 42 | issue 03 |sep-dec, 2022 63 case seriesguillain barre syndrome with covid-19 subtype. all patients were tested positive for covid-19 antibodies while a single patient also tested positive for covid-19 rtpcr. rarely, covid-19 rt-pcr test may remain positive longer even after recovery from primary sars-cov-2 infection.5 also, all patients received immunotherapy in the form of intravenous immunoglobulin (ivig) in the dose of 1 gm / kg / day for two days immediately on admission after confirming clinical diagnosis. three patients responded well to ivig therapy, while two patients progressed further with respiratory muscle paralysis needing mechanical ventilation support in the intensive care unit. one patient who was on mechanical ventilator support received repeat second dose of ivig. all patients recovered completely eventually without any residual weakness over the period of next three to four months with good prognosis. initial case reports of covid-19 and gbs with its variants were documented in adults, and supposed to be as a post infectious process.6-8 curtis m3 and das ky5 reported a first case of covid-19 and gbs in children from indianapolis from united states (para-infectious) and india (post-infectious) respectively. both these cases were responded well to ivig therapy without any mortality and morbidity. the average duration to develop post covid-19 gbs was less than two weeks.9 the exact mechanism for association of covid-19 and gbs is still not clear. the possible mechanisms include neurotropism and post infectious aberrant immune response.10 from our case series, we could link probable casual association of covid-19 infection with gbs. the reason could be firstly, we haven’t seen a single case of gbs at our institute in the previous year and we got all five cases within a span three months during covid-19 second wave. secondly, no past history of other viral or bacterial illness obtained within one month in all patients which ruled out other etiological causes of gbs. also, we could not able to perform csf rt-pcr for covid-19 and other viral antigen markers due to financial constraints. even, gbs is post infectious autoimmune condition, viral markers may not be positive at the time of presentation. most of the antibody tests for viruses other than covid-19 are also costly and not available, we couldn’t perform these tests. further studies and research may be necessary to establish causal association of covid-19 with gbs. conclusions we could link probable casual association of covid-19 infection with gbs. all children responded well to ivig and supportive treatment without any residual muscle weakness which was suggestive of good prognosis. references 1. sen s, kumar a, roy b. clinical outcome of guillainbarre syndrome in 108 children. indian pediatr. 2021;58(9):833-835. doi:10.1007/s13312-021-2302-7. 2. solomon t, willison h. infectious causes of acute flaccid paralysis. curr opin infect dis. 2003;16: 375-381. doi: 10.1097/01.qco.0000092807.64370.1e 3. curtis m, bhumbra s, felker mv, jordan bl, kim j, weber m, et al. guillain barre syndrome in a child with covid 19 infection. pediatrics 2021;147(4): e2020015115. doi:10.1542/peds.2020-015115 4. ky das, midhun raj kt, samprathi m, sridhar m, adiga r, vemgal p. guillain-barré syndrome associated with sars-cov-2 infection. indian j. pediatr 2021; 88(5):479. doi:10.1007/s12098-021-03684-x 5. lan l, dan x, guangming y. positive rt-pcr test results in patients recovered from covid-19. jama. 2020(15): 323. doi:10.1001/jama.2020;2783. 6. assini a, benedetti l, di maio s, schirinzi e, del sette m. new clinical manifestation of covid-19 related guillain-barre syndrome highly responsive to intravenous immunoglobulins: two italian cases. neurol sci. 2020;41: 2307. doi: 10.1007/s10072-020-04484-5 7. lantos je, strauss sb, lin e. covid-19 associated miller fisher syndrome: mri findings. am j neuroradiol.2020;41(7):1184-1186. doi: 10.3174/ ajnr.a6609 8. sedaghat z, karimi n. guillain barre syndrome associated with covid-19 infection: a case report. j clin neurosci.2020;76: 233-235. doi: 10.1016/j.jocn.2020.04.062. 9. patnaik uj. review article on covid-19 and guillainbarré syndrome, front biosci (schol ed). 2021;13(1): 97-104. doi:10.52586/s555. 10. hasan i, saif-ur-rahman km, hayat s, papri n, jahan i, azam r, et al. guillain-barre syndrome associated with sars-cov-2 infection: a systematic review and individual participant data meta-analysis. j peripher nerv syst.2020;25(4): 335-343. doi:10.1111/jns.12419. j nepal paediatr soc | vol 42 | issue 02 |may-aug, 202280 case series vinca alkaloid induced cranial neuropathy neluwa-liyanage ruwan indika1,2, arndt rolfs3,4, 5, christian beetz5, sabine schröder5, catarina pereira5, volha skrahina4,5, mihika fernando2, dinesha maduri vidanapathirana2,6, subhashinie jayasena2, eresha jasinge2 1department of biochemistry, faculty of medical sciences, university of sri jayewardenepura, nugegoda, sri lanka. 2department of chemical pathology, lady ridgeway hospital for children, colombo 8, sri lanka. 3university rostock, medical faculty, rostock/germany. 4arcensus gmbh, goethestrasse 20, 18055 rostock, germany. 5centogene gmbh, am strande 7, 18055 rostock, germany. 6department of pathology, faculty of medical sciences, university of sri jayewardenepura, nugegoda, sri lanka. genotypes and phenotypes of sri lankan patients with mucopolysaccharidosis type iva mucopolysaccharidosis type iva is a rare autosomal recessive lysosomal storage disorder occurring worldwide in all ethnic groups. it is caused by biallelic variants in the galns gene (omim 612222). we report five cases of mucopolysaccharidosis type iva with short stature and severe skeletal dysplasia. an optimized diagnostic strategy that combined enzymatic testing and genetic screening was applied. all the tested urine samples showed increased urinary glycosaminoglycan / creatinine ratios. in all five cases, the enzyme activity of galactosamine-6-sulfate sulfatase was pathologically decreased. gene-targeted sequencing revealed a previously unreported homozygous c.139-12t 72 hours, which was statistically significant. conclusions: ventilatory support is essential and lifesaving tool for critically ill children. mortality rate was higher and statistically significant in children who were ventilated for > 72 hours. abstract 1assistant professor, department of paediatrics, nobel medical college & teaching hospital, biratnagar, nepal 2medical officer, greencity hospital, kathmandu, nepal sandip kumar singh1, babita khanal1, shivani singh2 *corresponding author sandip kumar singh, md paediatrics, fellowship in pediatric critical care, paediatrician and paediatric intensivist, assistant professor, department of paediatrics, nobel medical college & teaching hospital, biratnagar, nepal. email: sandeep272@hotmail.com article history received on: 22 jun, 2022 accepted on: 23 apr, 2023 funding sources: none conflict of interest: none keywords: mechanical ventilation, outcome, picu online access doi: https://doi.org/10.3126/jnps.v42i3.46063 introduction ventilatory support is an essential and a common form of therapy in paediatric intensive care unit (picu). with improvement in knowledge, understanding of disease and advancement of critical care support, this modality has evolved. nepal is a low income country and number of critical care services targeted towards children are limited.1 however with availability of critical services, number of children being admitted to picu has increased in last few years. the percentage of children mechanically ventilated in different picu varies from 14 60%.2 mechanical ventilation can be lifesaving but more than 50% of complications in these ventilated child are related to ventilator support, if prolonged.3 various complications associated with ventilation includes airway complications, air leaks, ventilator induced lung injury, oxygen toxicity and ventilator associated pneumonia. in conclusion, judicious use copyrights & licensing © 2022 by author(s). this is an open access article distributed under creative commons attribution license (cc by nc ) j nepal paediatr soc | vol 42 | issue 03 |sep-dec, 20222 original article clinical profile and outcome of ventilated children of ventilation when indicated, is essential along with close monitoring, for successful outcome. the objective of this study was to determine clinical profile of mechanically ventilated children, common complications associated with invasive mechanical ventilation and outcome of those children. picu in nobel medical college is one of the largest referral center in eastern nepal. this study will guide to formulate hospital policy and help in making national guidelines as well. methods the study is an observational descriptive study conducted on mechanically ventilated children admitted to picu of nobel medical college and teaching hospital during 48 months period from november 2019 to october 2021. this study was started after acquiring approval from the institutional review committee of nobel medical college (irc). nobel medical college is a tertiary referral center located in biratnagar, nepal. department of paediatrics consists of 63 bedded paediatric ward and 15 bedded level iii picu. various indications for ventilating a sick child includes respiratory failure, airway protection, refractory shock, and neurological disorders. usual rate of intubation in our center ranges from 13 24% over few years. all ventilated children more than one month till 15 years of age were included in this study. children who were ventilated for less than 24 hours and children who were transferred from other center on bag and tube ventilation were excluded from this study. all children were ventilated using a/c pc, prvc or simv mode, depending upon the clinical scenario. once the underlying disease process improved, if the child has adequate gas exchange and good respiratory efforts, they were weaned and extubated after spontaneous breath trial (sbt). majority of them were extubated to either cpap or hfnc. demographic features were analyzed including age, sex, reason for mechanical ventilation, duration of mechanical ventilation and any other comorbidities. outcomes parameters included death in hospital, discharge from picu or shift to ward and left against medical advice (lama). categorical data (included age, gender, underlying medical condition, outcome and complications) were expressed as absolute counts and percentages. continuous data (for age and duration of ventilation) were expressed as mean and standard deviation. to study the association of outcome of ventilated children to with duration of ventilation, chi square test was used. data were considered significant at p value of < 0.05. statistical analysis was done using spss version 11.0 for windows. results total number of picu admissions during the study period was 1352. a total of 212 (15.68%) of picu admissions requiring invasive mechanical ventilation satisfied the inclusion criteria and were included in this study. children between one to five years contributed to 32.07 % of total ventilated cases, followed by children < 1 year (26.41%) and children > 10 years (21.69%). among them males were 53.77% and females were 46.22% as shown in fig 1. table 1: showing age distribution of ventilated children admitted to picu age in years no of cases percentage < 1 years 56 26.41% 1 5 years 68 32.07% 5 – 10 years 42 19.8 % >10 years 46 21.69% total 212 100% figure 1: showing age and sex distribution of mechanical ventilated children in picu table 2: showing various etiologies for which children were mechanically ventilated in picu clinical diagnosis number of cases (percentage) sepsis / septic shock / mods 48 (22.64%) pulmonary 43 (20.28%) cns infection / encephalopathy 39 (18.39%) cardiac 12 (5.66%) severe dengue 6 (2.83%) post-surgery 22 (10.37%) poisoning 10 (4.71%) polytrauma 10 (4.71%) snake bite 6 (2.83%) misc/ severe covid 6 (2.83%) others 10 (4.71%) total 212 j nepal paediatr soc | vol 42 | issue 03 |sep-dec, 2022 3 original articleclinical profile and outcome of ventilated children most common causes for requirement of mechanical ventilation were sepsis / multi organ dysfunction syndrome (mods) in 22.64% cases, followed by pulmonary infections (20.28%) and cns infections / encephalopathy (18.39%). others causes includes guillain-barre syndrome, submersion injury, scrub typhus and diabetic ketoacidosis. table 3: showing duration of mechanical ventilation required for children duration of ventilation no. of cases percentage < 72 hours 64 30.18% 72 hours to 7 days 126 59.43% > 7 days 22 10.37% it was observed that, among the children who were ventilated, 126 children (59.43%) were ventilated for 72 hours to seven days duration. the mean duration of mechanical ventilation was 5.38 days ± 3.66 days. children were ventilated mainly on a/c pc, prvc or simv mode, depending upon the clinical condition. once the children improved, gradually mechanical ventilation was weaned off using either cpap / ps, simv or t piece. cpap / ps was major method of weaning in 126 (75.90%) children, followed by simv in 21 (12.65%) children and t piece in nine (6.62%) children. some children on simv mode were weaned directly from simv by reducing rate and pressure support. however, 10 (6.02%) of children were extubated accidently, among which, six (60%) were self extubated when sedation was stopped for sbt (spontaneous breathing trial). among 10 children accidently extubated, only three (30%) required re-intubation. we do not practice routine change of endotracheal tube in our picu. all children ventilated for > 72 hours, we practice giving dexamethasone 0.15 mg / kg / dose every 6 hourly for 6 doses with the first dose administered 6 12 hours prior to extubation. however, 13 (7.83%) children required reintubation for various reasons, the commonest cause being extubation failure due to poor respiratory efforts in six cases (46.15%), followed by airway edema in four cases (13.33%) and displacement in three cases (23.07%). although, mechanical ventilation is a lifesaving procedure, it can cause multiple complications. some of the complications can be life threatening as well. the commonest complication in the ventilated children was ventilator associated pneumonia in 16 (7.54%) children, followed by air leaks in 15 (7.07%) children. the various complications encountered in ventilated children in picu were as follows: table 4: showing various complications associated with mechanical ventilation in picu complications no of cases percentage ventilator associated pneumonia 16 7.54% air leaks 15 7.07% pressure sores 15 7.54% post extubation stridor 14 6.60% collapse / atelectasis 12 5.66% pulmonary haemorrhage 8 3.77% equipment failure 6 2.83% only four children (1.88%) required tracheostomy, which was required as children required prolonged mechanical ventilation. among them three had acute encephalitis syndrome with refractory status epilepticus and one child had drowning leading to hypoxic ischemic brain injury. out of 212 children, 166 (78.30%) were successfully extubated and discharged, 24 (11.32%) children expired and 22 (10.37%) children went on lama. out of 22 children, who went on lama, 12 (54.54%) went on lama due to poor neurological outcome of child whereas 10 (45.45%) children went on lama due to financial issues. table 5: showing relation of mechanical ventilation >72 hours with outcome duration of ventilation survived expired p value – 0.045 < 72 hours (total 64 cases) 61 (95.31%) 3 (4.68%) > 72 hours (total 148 cases) 127 (85.81%) 21 (14.18%) out of 212 cases, 64 cases (30.18%) were mechanically ventilated for < 72 hours and 148 cases (69.81%) were ventilated for > 72 hours. out of 24 children expired, three (12.5%) children were ventilated for < 72 hours whereas 21 (87.5%) children were ventilated > 72 hours. the mortality rate of 14.18% was found in children, who were ventilated for > 72 hours, which was statistically significant (p value 0.045). discussion picu is a place where critically ill children requiring various organ supports are admitted. though picu set up in nepal is still an emerging specialty, need and requirement for picu is still rising. therefore this study will give an idea and highlight about disease in children requiring invasive mechanical ventilation, complications associated with it and outcome. the percentage of children in picu requiring mechanical ventilation in our study was 15.68%, similar to study done in pakistan by bhori ns et al4 whereas j nepal paediatr soc | vol 42 | issue 03 |sep-dec, 20224 original article clinical profile and outcome of ventilated children study done by vijayakumary et al5 and mukhtar b et al6 showed higher rates of mechanical ventilation 52% and 50.7% respectively. children between one to five years of age constituted 32.07% of our ventilated case, followed by infants in 26.41% of cases, similar to other studies.4-6 commonest indications for mechanical ventilation in our study was septic shock / mods (22.64%), followed by respiratory failure (20.28%) unlike other studies, where neurological indications predominated in the study by kendirli et al7 and respiratory cause was predominated in study by indrajit et al.8 the mean duration of mechanical ventilation was 5.38 days ± 3.66 days, which is similar to study done by wolfler a et al.9 out of 13 (7.83%) children requiring re-intubation for various reasons, the commonest cause was extubation failure in six cases (2.8%), followed by obstruction in four cases (1.88%) and displacement in three cases (1.41%). nosocomial pneumonia was significantly associated with reintubation, which was also reported by the study by elward et al.10 the commonest complication in the ventilated children was ventilator associated pneumonia in 16 (7.54%) cases and air leaks in 15 (7.07%) children. similar findings were observed in study by benjamin et al,11 where incidence of air leak was reported 6.9%. 1.88% children required tracheostomy, which was required as children required prolonged mechanical ventilation, similar to da silva et al.12 mortality in the ventilated children in the study was 11.32% which was comparable to study done by da silva et al12 and indrajit et al8 (19.8 % and 24% respectively). however it is much lower compared to the observations made by kendirli et al7 where mortality was 58.3%, which could be attributed to large number of manual ventilated cases in the study, where lung pressure would not be regulated leading to excessive lung injury. the mortality rate of 14.18% was found in children, who were ventilated for > 72 hours, which was statistically significant (p value 0.045). this can be explained by the fact that children requiring prolonged ventilation, will have higher chances of ventilator induced lung injury and will have higher risk for ventilator induced infection and other complications. one major limitation in our study was lack of categorizing cases based on severity, which could have further highlighted mortality and morbidity parameters. children ventilated for less than 24 hours and children who were transferred from other centers on bag and tube ventilation were excluded from this study, which might have resulted in lower mortality rate. only duration of invasive mechanical ventilation was correlated with outcome, there could be various other factors affecting mortality like disease severity, co-morbidities. this is single center study, similar multicenter study from various picu is required for further standardization of level of care in picu. conclusions ventilatory support is essential and lifesaving tool for critically ill children, admitted to picu. around 15.68% of children admitted to intensive care unit requires ventilator support with common indication being sepsis, septic shock and mods. organized and effective courses and trainings dedicated to healthcare personnel working in picu will reduce chances of complications associated with mechanical ventilation. similar studies from other picu will help in developing protocols for mechanical ventilation in critically ill children. acknowledgement i would like thank department of paediatrics, nobel medical college, biratnagar for support during my research. references 1. acharya sp. critical care medicine in nepal: where are we? int health. 2013;5(2):92-5. doi:10.1093/inthealth/iht010 2. farias ja, frutos f, esteban a, flores jc, retta a, baltodano a, et al. what is the daily practice of mechanical ventilation in pediatric intensive care units? a multicenter study. intensive care med.2004;30(5):918–925. doi: 10.1007/s00134-004-2225-5. 3. manczur ti, greenough a, pryor d, rafferty gf. comparison of predictors of extubation from mechanical ventilation in children. pediatr crit care med 2000; 1:28-32. doi: 10.1097/00130478-200007000-00005 4. bhori ns, ghate sv, chhajed ps. a study of mechanical ventilation in children. int j contemp pediatr. 2017 nov; 4(6):2088-2092. doi:10.18203/2349-3291.ijcp20174737 5. vijayakumary t, de silva jr, sarathchandra j, kumarendran b. prospective study of ventilated patients in the paediatric medical intensive care unit of lady ridgeway hospital. sri lanka j. child health. 2012 aug 31; 41(3):114-117. doi:10.4038/sljch.v41i3.4598 6. mukhtar b, siddiqui nr, haque a. clinical characteristics and immediate-outcome of children mechanically ventilated in a pediatric intensive care units. pak j med sci. 2014; 30(5):927-930. doi: 10.12669/pjms.305.5159 7. kendirli t, kavaz a, yalaki z, ozturkhismi b, derelli e, ince e. mechanical ventilation in children. turk j pediatr. 2006; 48 (4): 323–7. pmid: 17290566 8. majumdar i, sachdev a, gupta d, chugh k. to evaluate the impact of initial chest radiograph on final outcome of ventilated patients. ind j crit care med 2005; 9(2):77-80. doi: 10.4103/0972-5229.17092 http://doi.org/10.4038/sljch.v41i3.4598 http://dx.doi.org/10.4103/0972-5229.17092 j nepal paediatr soc | vol 42 | issue 03 |sep-dec, 2022 5 original articleclinical profile and outcome of ventilated children 9. wolfler a, calderoni e, ottonello g, conti g, baroncini s, santuz p, et al. daily practice of mechanical ventilation in italian pediatric intensive care units: a prospective survey. pediatr crit care med. 2011; 12(2):141-146. doi: 10.1097/ pcc.0b013e3181dbaeb3 10. elward am, warren dk, fraser vj. ventilator-associated pneumonia in pediatric intensive care unit patients: risk factors and outcomes. pediatrics. 2002; 109:758–64. doi: 10.1542/peds.109.5.758. pmid: 11986433. 11. benjamin pk, thompson je, o’rourke pp. complications of mechanical ventilation in a children’s hospital multidisciplinary intensive care unit. respir care 1990; 35 (9):873-8. pmid: 10145335 12. silva dc, shibata ar, farias ja, troster ej. how is mechanical ventilation employed in a pediatric intensive care unit in brazil? clinics (sao paulo). 2009; 64(12):1161-6. doi: 10.1590/s1807-59322009001 200005. pmid: 20037703; pmcid: pmc2797584. original article childhood asthma and its associated factors among children j nepal paediatr soc | vol 42 | issue 02 |may-aug, 202236 original article doi: 103126/jnps.v4113 shristi shakya,1 kalpana upadhyaya subedi,2 megha mishra3 1medical officer,department of neonatology, paropakar maternity and women’s hospital, thapathali, kathmandu, 44600, nepal 2professor and head of department of neonatology, paropakar maternity and women’s hospital, thapathali, kathmandu, 44600, nepal 3registrar, department of neonatology, paropakar maternity and women’s hospital, thapathali, kathmandu, 44600, nepal. incidence of birth defects among live born neonates at tertiary level maternity hospital in nepal introduction: birth defects are structural and functional anomalies that present before, at birth, or later in life. this study aimed to find out total incidence, type of structural birth defects in live born babies in nepal. methods: the study was conducted at a tertiary level maternity hospital in nepal from 14 april 2018 to 13 april 2019. data was collected on online newborn birth defect data base developed by who southeast-asia regional office. all live born babies with external and internal birth defects confirmed by radiographic, ultrasonography and echocardiography until seven days of life were included. ethical approval was obtained from institutional review committee of hospital. results: total of 21,564 live babies were delivered during one-year study period. out of these, 220 (1.02%) had one or more birth defects. number of male babies {130 (59%)} were more than female {89 (40.9%)}. 176 (80%) babies with the malformation were born to mother within age group 20 to 35 years. of the total 220 babies with birth defects, 197 (89.5%) had isolated malformations and remaining 23 (10.4%) had sequence malformations. the most frequent malformations involved cardiovascular system 125 (56.8%) followed by gastrointestinal system 37 (16.8%), musculoskeletal system 34 (15.4%) and central nervous system 18 (8.1%). conclusions: incidence of overall birth defects in this study was found to be 1.02% in which cardiovascular system anomalies was the most common followed by gastrointestinal, musculoskeletal and central nervous system anomalies. abstract *corresponding author shristi shakya medical officer, department of neonatology, paropakar maternity and women’s hospital, thapathali, kathmandu, 44600, nepal. email: rijenshristi@gmail.com article history received on : 27 nov, 2021 accepted on : 18 dec, 2022 funding sources: none conflict of interest: none keywords: anomalies; birth defects; congenital malformations; newborn online access doi: https://doi.org/10.3126/jnps.v42i2.41093 introduction birth defects refer to structural or functional anomalies that present before or, at birth or later in life.1 birth defects can occur during any stage of pregnancy but most occur in the first trimester.2 these defects can be caused by genetic abnormalities and / or environmental exposures, although the underlying etiology is often unknown. birth defects can be isolated or present in a characteristic combination or pattern that may affect one or more organ systems.1 structural birth defects are related to a problem with body parts. range of such defects copyrights & licensing © 2022 by author(s). this is an open access article distributed under creative commons attribution license (cc by nc ) j nepal paediatr soc | vol 42 | issue 02 |may-aug, 2022 37 original articlebirth defects at tertiary maternal hospital includes neural tube defects (ntds), cleft lip with or without cleft palate, heart defects, and abnormal limbs. functional birth defects are related to a problem in working of a body part or system. these often lead to developmental disabilities and can include disorders such as: muscular dystrophy, phenylketonuria, loss or impairment of vision and hearing. structural birth defects are clinically obvious at birth whereas functional defects may only be diagnosed later in life. for example, bleeding disorders like hemophilia is usually not clinically obvious until infancy or childhood. birth defects not only plays a major role in fetal loss, but also contribute significantly to pre-term births, childhood and adult morbidity.3 the treatment and rehabilitation of these children with birth defects is very costly with considerable repercussions on their families. hence the need to identify causative and risk factors to prevent them early, where possible.3 knowing the incidence of birth defects is important for planning and implementing programs to reduce morbidity and mortality resulting from it. realizing the paucity of incidence of the birth defect studies, this study is being commenced. the aim was to estimate the total incidence, types of structural birth defects in live newborns delivered at the tertiary level maternity hospital of nepal. this data can be used as baseline to analyze burden of the problem and prevent using existing opportunities. methods the retrospective, institution based study was conducted in tertiary level maternity hospital of nepal, from 14th april 2018 to 13th april 2019. all the newborn babies delivered in the institution during this period were examined by paediatricians. thorough physical examination was done to identify any birth defects. all live born babies with external birth defects identified at birth or before discharge from the hospital were included. those babies with suspected internal defects were sent for radiography, ultrasonography or echocardiography confirmation. internal defects confirmed within seven days of life were included. babies who were still-birth and elective termination of pregnancy for fetal anomaly (etopfa) were excluded in this study. data is being collected on online system of world health organizationsouth east asia regional office new born birth defect (whosearo nbbd) data base since 2014. each case meeting the inclusion criteria were filled in separate performa. the major birth defects were classified according to the international classification of disease version20 (icd-10) for all birth defects observed by using the birth defects atlas.4 ethical approval was obtained from institutional review committee of the hospital. results during study period, a total of 21,564 live infants were delivered. of these newborns, 220 (1.02%) were found to have one or more birth defects. i.e the prevalence of birth defects on this study was 10 per 1000 total live birth. as shown in table 1, the study showed that there were more male babies (59%) with birth defects than female (40.9%). among the birth defect newborn babies, the majority (176 ie 80%) of the cases were born to mothers age group 20 to 35 years (table 2). the incidence of anomaly was found higher among the full term live births than preterm deliveries. of the total 220 babies with birth defects, 197 (89.5%) had isolated malformations and the remaining 23 (10.4%) had sequence malformations. the most frequent malformation involved was cardiovascular system 125 (56.8%) followed by gastrointestinal system 37 (16.8%) musculoskeletal system 34 (15.4%) and central nervous system 17 (8.1%). distribution of birth defects in the study group shown in table 3. table 1. birth defects distributed according to sex and mode of delivery total live birth 21564 total live birth defects (1%) 220 sex male (59%) 130 female (40.9%) 90 mode of delivery normal delivery (51.3%) 113 cesarean delivery (48.1%) 106 instrumental delivery (0.45%) 1 table 2. frequency of birth defects classified according to maternal age and week of gestation maternal age number of cases years 20 < 30 (13.6%) years 35 20 176 (80%) years 35 < 14 (6.3%) week of gestation (wog) < 37 wog 77 (35%) < 37 wog 143 (65%) table 3. birth defects classified according to anatomical system system frequency (220) cardiovascular 125 (56.8%) central nervous 18 (8.1%) gastrointestinal 37 (16.8%) genitourinary 17 (7.7%) musculoskeletal 34 (15.4%) others 18 (8.1%) discussion the incidence and types of birth defects differ from one country to another and even in same country from one region to another. globally if we look at it, as per march of dimes estimates, every original article birth defects at tertiary maternal hospital j nepal paediatr soc | vol 42 | issue 02 |may-aug, 202238 year eight million children worldwide are born with a serious birth defects due to genetic or environmental causes.5 eurocat recorded a total prevalence of major birth defects of 23.9 per 1000, birth for 2003-2007, in which 8% were live birth.6 the world health statistics annual birth data of 2010 reports the estimate would be 9.78 million children.7 in nepal, children born with birth defects annually is 59.9 prevalence per 1000 live births.8 in our study incidence of birth defects in neonate (1.02%) was found significantly higher than previous studies done at maternity hospital (0.36%),9 western regional hospital ( 0.42%),10 patan hospital (0.81%),11 combined 12 hospitals of nepal (0.58%),12 but similar to dhulikhel hospital (1.1%)13 and sarlahi (1.1%).14 the discrepancy could have been resulted because of increased awareness among hospital staff about birth defects so that they examine the baby to look for anomalies and also for the increased facilities for investigations to detect internal defects. however, while comparing congenital malformation with neighbor countries like india (1.53%), egypt (2%), brazil (2.2%), ethiopia (1.25%), these countries have slightly higher prevalence than our county.15-18 this may be due to their cohort studies allows the collection of more information which are manifested later in life than document-based retrospective studies.19 in the present study, male infants were significantly higher with congenital anomalies than females. this observation agrees with the findings of studies conducted in maternity hospital,9 western regional hospital,10 dhulikhel hospital13 and at south india.20 this could be due to x-linked recessive factor or y-linked genetic basis.20 according to several literatures, extremes of ages were found to be associated with birth defects. young age < 20 years21 and / or advanced maternal age < 35 years.22-24 however, results of our studies was not consistent with all of these findings. the incidence of malformation was higher in mother age group 20 to 35 years, which is similar to studies done at patan hospital,11 south india 20 and in bradford study.25 this could be because this range of age is the common child bearing age group in our country. also differences in study design, case definition, and potential confounders may play a role in this discrepancies.26 birth defects encompass a wide array of structural and functional abnormalities that can occur in isolation (i.e. single defect) or as a group of defects (i.e.multiple defects). it is generally estimated that around 14% of babies are born with a single minor defects, around 2 to 3% have a single major defects and 1% of neonates have multiple defects.6 those babies with cleft lip and palates, down’s syndrome or babies with meningocele with hydrocephalus are included in isolated defect and with multiple birth defects are included in multiple defects in our study. the most prevalent birth defect in the current study was cardiovascular system. this may be because of availability of echocardiography facility in the hospital for last one year where all newborn with suspected congenital heart disease (chd) undergo echocardiography examination early so that they are diagnosed within seven days of life. chd is most prevalent global trend of birth defect. since past 15 years, 1.35 million yearly newborns are recorded with chd. asia reported the highest chd birth prevalence, with 9.3 per 1,000 live births.27 another multiethnic birth cohort study done in brandford, uk also reported chd as the most common anomaly in newborn in uk.25 there are several limitations in this study. since, this is a retrospective study, getting complete information and avoiding biases were challenging. in our setting due to lack of karyotyping and metabolic screening were not able to include chromosomal and metabolic abnormalities which may have lead to under representation of disorders. the sample size is less and only represents one tertiary center. conclusions incidence of overall birth defects in this study was found to be 1.02% in which cardiovascular system anomalies was the most common followed by gastrointestinal, musculoskeletal and central nervous system anomalies. the incidence and types of congenital malformation can differ from one country to another and even in the same country from one region to another. references 1. world health organization: who facts sheet on congenital anomalies. in. geneva; 2016. 2. centers for disease control and prevention. https://www. cdc.gov/ncbddd/birthdefects/index.html 3. grover n: congenital malformations in shimla. india j paediatr. 2000, 67: 249-51. 10.1007/bf02758158. doi: https://doi.org/10.1007/bf02758158 4. world health organization, centers for disease control and prevention (u.s.) and international clearinghouse for birth defects monitoring systems. (2014). birth defects surveillance: atlas of selected congenital anomalies. world health organization. https://apps.who.int/iris/ handle/10665/127941 5. march of dimes. global report on birth defects: the hidden toll of dying and disabled children. new york, 2006. http:// www.marchofdimes.com/ downloads/ birth_defects_reportpf.pdf accessed 19 february 2013. 6. eurocat [online]. special report. a review of environmental risk factor for congenital anomalies. 2004 [cited 2009 september 15) available at: http:// www.eurocat-network. eu2 7. world health organization. global health observatory. available at http:// www.who.int/ gho/child_health/en/ index.html accessed november 2012. 8. prevention and control of birth defects in south-east asia region. strategic framework (2013-2017). https://media. tghn.org/medialibrary/2020/02/ 9. malla bk: one year review study of congenital anatomical malformation at birth in maternity hospital (prasutigriha), thapathali, kathmandu. kathmandu univ med j (kumj) 2007, 5(4):557-560. https://www.cdc.gov/ncbddd/birthdefects/index.html https://www.cdc.gov/ncbddd/birthdefects/index.html https://doi.org/10.1007/bf02758158 https://apps.who.int/iris/handle/10665/127941 https://apps.who.int/iris/handle/10665/127941 http://www.eurocat-network.eu2 http://www.eurocat-network.eu2 https://media.tghn.org/medialibrary/2020/02/ https://media.tghn.org/medialibrary/2020/02/ j nepal paediatr soc | vol 42 | issue 02 |may-aug, 2022 39 original articlebirth defects at tertiary maternal hospital 10. sharma i, rijal b, thapa s, poudel i. congenital anatomical malformation at birth in western regional hospital, pokhara, nepal. j univ coll med sci. 2013;1(4):3740. doi: https://doi.org/10.3126/jucms.v1i4.9572 11. ansari i, rajbhandari r, chilise s, shalh g, maskey p, maharjan r, et al. congenital malformations at birth in 7,922 consecutive deliveries at patan hospital, nepal. patan acad health sci , 1(2), 4–7. doi: https://doi.org/10.3126/jpahs.v1i2.16636 12. paddle p, sunny ak, gurung r, gurung a, malla h, rana nb, et al. burden and consequence of birth defects in nepalevidence from prospective cohort study. bmc pediatr 21, 81 (2021). doi: https://doi.org/10.1186/s12887-021-02525-2 13. dongol ss, sradanandha s, shrestha rpb, bahadur r,joshi a, shrestha a. pattern and risk factor associated with congenital anomalies among young infants admitted in dhulikhel hospital. birat j. health sci 2018;3(3)7: 548-553 doi: https://doi.org/10.3126/bjhs.v3i3.22173 14. peto h, labrique a, khatry sk, shahabuddin m, christian p, ali h, klemm r, et al. implementation of community birth defects surveillance systems in rural nepal (19941997) and bangladesh (2001-2007), 2012 (unpublished paper). 15. gupta s, gupta p, soni js. a study on incidence of various systemic congenital malformations and their association with maternal factors. njmr. 2010;2(1): 19-21. corpus id: 73888336 16. shawky rm, sadik di. congenital malformations prevalent among egyptian children and associated risk factors. ejmhg 2011;12(1):69-78. doi:https://doi.org/10.1016/j.ejmhg.2011.02.016. 17. porto r, reis f, oliveira cc da, de melo e, aragão j. prevalence and factors associated at presence of central nervous system congenital malformations. jamr, 2015;6(10):956-64. doi: https://doi.org/10.9734/bjmmr/2015/14642 18. taye m, afework m, fantaye w, diro e, worku a. magnitude of birth defects in central and northwest ethiopia from 2010-2014: a descriptive retrospective study. plos one. 2016;11(10): e0161998. doi: https://doi.org/10.1371/journal.pone.0161998 19. gonzález aj, morales jj, luna l, arteaga j, mutchinck om. beginner’s guide to genetics: congenital malformations. bmj 2004;12(suppls6):437-80. doi: https://doi.org/10.1136/sbmj.0412444 20. neelambari yc, das p, sadagopan s, uma a. prevalence, pattern and outcome of congenital malformations in a tertiary care centre in south india. ijcp, [s.l.], v. 5, n. 3, p. 1044-1048, apr. 2018. issn 2349-3291. doi:http://dx.doi.org/10.18203/2349-3291.ijcp20181 539. 21. croen la, shaw gm. young maternal age and congenital malformations: a population-based study. am j public health.1995;85(5):710-3. doi: https://doi.org/10.2105/ajph.85.5.710 22. cleary-goldman j, malone fd, vidaver j, ball rh, nyberg da, comstock ch, et al. impact of maternal age on obstetric outcome. obstet gynecol. 2005 may;105(5 pt 1):983-90. doi: https://doi.org/10.1097/01.aog.0000158118. 75532.51 23. hollier lm, leveno kj, kelly ma, mcintire dd, cunningham fg. maternal age and malformations in singleton births. obstet gynecol. 2000;96 (5 pt 1):701. doi: https://doi.org/10.1097/00006250-20001100000011 24. reefhuis j, honein ma. maternal age and non-chromosomal birth defects, atlanta--1968-2000: teenager or thirtysomething, who is at risk? birth defects res a clin mol teratol. 2004 sep;70(9):572-9. doi: https://doi.org/10.1002/bdra.20065 25. sheridan e, wright j, small n, corry cp, oddie s, whibley c, et al. risk factors for congenital anomaly in mulethnic birth cohort: an analysis of the born in bradford study. lancet 2013; 383:1350-59. doi: https://doi.org/10.1016/s0140-6736(13)61132-0 26. gill sk, broussard c, devine o, green rf, rasmussen sa, reefhuis j, et al. association between maternal age and birth defects of unknown etiology: united states, 1997-2007. birth defects res a clin mol teratol. 2012 dec;94(12):1010-8. doi: https://doi.org/10.1002/bdra.23049 27. van der linde d, konings ee, slagerma, witsenburg m, helbing wa, takkenberg jj, et al. birth prevalence of congenital heart disease worldwide: a systemic review and metaanalysis. j am coll cardiol. 2011;59(21):2241-7. doi: https://doi.org/10.1016/j.jacc.2011.08.025 https://doi.org/10.3126/jucms.v1i4.9572 https://doi.org/10.3126/jpahs.v1i2.16636 https://doi.org/10.1186/s12887-021-02525-2 https://doi.org/10.3126/bjhs.v3i3.22173 https://doi.org/10.1016/j.ejmhg.2011.02.016 https://doi.org/10.9734/bjmmr/2015/14642 https://doi.org/10.1371/journal.pone.0161998 https://doi.org/10.1136/sbmj.0412444 tel:2349-3291 https://doi.org/10.2105/ajph.85.5.710 https://doi.org/10.1097/01.aog.0000158118.75532.51 https://doi.org/10.1097/01.aog.0000158118.75532.51 https://doi.org/10.1097/00006250-200011000-00011 https://doi.org/10.1097/00006250-200011000-00011 https://doi.org/10.1016/s0140-6736(13)61132-0 https://doi.org/10.1002/bdra.23049 https://doi.org/10.1016/j.jacc.2011.08.025 original article phimosis in children j nepal paediatr soc | vol 42 | issue 02 |may-aug, 202226 *sushil dhungel1, ajaya dhakal2, kamal koirala3, rupesh mukhia3, narayan bhusal1, abhishek thapa1 1assistant professor, department of surgery, kist medical college and teaching hospital, kist hospital road, mahalaxmi municipality-1, lalitpur, nepal 2associate professor, department of paediatrics, kist medical college and teaching hospital, kist hospital road, mahalaxmi municipality-1, lalitpur, nepal 3professor, department of surgery, kist medical college and teaching hospital, kist hospital road, mahalaxmi municipality-1, lalitpur, nepal effectiveness of topical steroid in phimosis: a longitudinal observational study introduction: most non-retractile foreskins have been diagnosed with phimosis and referred for circumcision. however, many patients can be managed with corticosteroid cream. this study evaluates the effectiveness of the topical application of corticosteroid cream and manual prepucial stretching in the treatment of phimosis. methods: this was a longitudinal observational study carried out among children aged six months to 10 years with the diagnosis of phimosis between 1st september 2019 to 31st august 2020. the patients were advised to apply 1% hydrocortisone cream together with manual prepucial stretching twice daily for four weeks. patients were assessed at four weeks and six months at the outpatient clinic using kirkos grading for retractability. results: a total of 110 patients were diagnosed with phimosis during the study period. fourteen patients had pathological phimosis out of which four had balanitis xerotica obliterans and were excluded from the analysis. ninetysix patients with physiological phimosis were treated conservatively with 1% hydrocortisone and manual prepucial stretching. among them, 87 cases were successfully treated whereas five patients had a partial response with treatment failure in four cases. those five cases with partial response underwent adhesiolysis while circumcision was performed in the remaining four patients with treatment failure. prepucial retraction was possible in four weeks in most of the patients with physiological phimosis with successful results in 90.6% of cases. conclusions: all non-retractile prepuce are not pathological phimosis and doesn’t need circumcision. local application of a potent corticoid cream and foreskin stretching is a safe, simple, and effective long-term treatment for physiological unretractable foreskin in children. abstract *corresponding author sushil dhungel assistant professor, department of surgery, kist medical college and teaching hospital,kist hospital road, mahalaxmi municipality-1, lalitpur, nepal email: sushildhungel@hotmail.com article history received on : 22 apr, 2022 accepted on : 15 dec, 2022 funding sources: none conflicts of interest: none keywords: pathological phimosis, phimosis, physiological phimosis, prepucial adhesion online access doi: https://doi.org/10.3126/jnps.v42i2.44533 introduction phimosis is a condition with a failure to retract the foreskin, which may be due to either a narrowness of the opening of the prepuce, congenital adhesions between the glans and prepuce, or both.1 almost all boys (96%) are born with an unretractable foreskin. copyrights & licensing © 2022 by author(s). this is an open access article distributed under creative commons attribution license (cc by nc ) original article doi: 103126/jnps.v4113 j nepal paediatr soc | vol 42 | issue 02 |may-aug, 2022 27 original articlephimosis in children prepuce gradually becomes retractile by three years of age but can extend into older age groups (physiological phimosis).2 there is ongoing controversy regarding the use of neonatal circumcision for phimosis.3 the american academy of pediatrics has adopted a neutral or anti-circumcision stance on neonatal circumcision.4 physiological phimosis is more appropriately managed by conservative measures, such as “tincture of time”, or topical steroid therapy.5 topical steroids have now become an alternative to circumcision for the treatment of phimosis with high success rates1,6,7 since its introduction in 1993.8 pathological phimosis is defined as failure to retract the foreskin due to distal scarring of the prepuce that is seen as a contracted white, indurated, fibrous ring around the preputial orifice during physical examination. on the contrary, physiological phimosis lacks this scarring process and is a normal developmental phase of the prepuce and many physicians continue to have difficulty distinguishing one form from the other.3,4,9 the standard treatment for pathological phimosis is circumcision6,9 but dorsal slit and preputioplasty under general anesthesia and adhesiolysis under topical anesthetics are other modes of treatment. phimosis creates major concerns for parents and is responsible for significant numbers of consultations, referrals to paediatric surgeons, and circumcisions.10-12 recently alternative to circumcision and prepuce plasty, conservative treatments for phimosis with topical corticosteroids applied to the stenotic distal portion of the prepuce for four to eight weeks have been published with high rates of resolution. three large studies have recommended the initial treatment of phimosis with topical corticosteroids before any surgical intervention.6,13,14 the study aims to evaluate the effectiveness of the topical corticoid cream and manual prepuce stretching for non retractile prepuce. methods this longitudinal observational study was conducted at kist medical college and teaching hospital from 1st september 2019 to 31st august 2020 after ethical approval from institutional review committee (irc no 2076/77/15). children aged six months to 10 years, who were diagnosed with phimosis in the outpatient clinic of surgery and paediatrics were included in the study while children with current active balanoposthitis, recurrent urinary tract infections, balanitis xerotica obliterans, buried penis, and phimosis secondary to incomplete circumcision were excluded from the analysis. written informed consent was taken from the parents of the children after explaining the study. the treatment options for phimosis using topical steroids or surgery were discussed with the parents. grading of the degree of retractability of the foreskin was recorded at presentation and during follow-up visits at four weeks and six months after the corticosteroid treatment using kirkos grading.5 the parents and / or the patients were instructed to wash prepuce and apply 1% hydrocortisone ointment at the tip of the foreskin together with manual prepucial stretching twice daily for four weeks without stopping even if the foreskin became retractable without causing any pain. the patients were then followed up at four weeks to analyze the treatment effect. at four weeks of treatment, all patients were examined for phimosis using the same kirkos grading. the maximum duration of corticosteroid treatment was limited to four weeks. successful treatment at four weeks was defined as a retractile prepuce, patient / or parent satisfaction, and clinical examination suggesting circumcision was unnecessary. these patients were then followed again six months after treatment. those patients who did not come for follow-up were interviewed by telephone. failure of therapy at four weeks was defined as persistent phimosis with the inability to retract the outer foreskin and advised for circumcision. side effects such as striae, pigmentation, hypertrichosis, and telangiectasia as well as weight gain, and behavioral changes were recorded. during the treatment course, the patients or their parents were asked if the daily regime of retraction and cleansing of the retractable foreskin was diligently followed and whether there was an episode of balanitis during treatment. demographic data, age, and clinical data of patients were recorded in predesigned proforma and the same proforma was used to record the treatment outcomes during the follow-up. the data were entered and analyzed using the statistical package for social sciences (spss) version 27. frequencies and percentage values were calculated for the various variable using descriptive analysis. results a total of 110 patients ranging from six months to 10 years (mean = 4.28 years) were diagnosed as having phimosis during the study period. out of 110 patients, 14 (12.7%) patients had pathological phimosis of which four had balanitis xerotica obliterans. these 14 patients were excluded from the study and underwent circumcision. among 96 patients eligible for corticosteroid ointment therapy, 39.58% of patients had a grade 2 phimosis, 5.21% patients had grade 3 phimosis, 34.37% patients had grade 4 phimosis, and 20.83% patients had grade 5 phimosis at initial presentation as shown in table 1. during the study period, no patient had grade 0 and grade 1 phimosis. table 1. grades of phimosis of the study population grade number of patients percentage grade 0 0 0 grade 1 0 0 grade 2 38 39.58% grade 3 5 5.21% grade 4 33 34.37% grade 5 20 20.83% none of the patients were practicing daily retraction of their foreskin before entering the study. ninety-six patients were treated with 1% hydrocortisone cream and manual prepucial stretching according to protocol. four weeks after enrollment, 90.6% (87 patients) had a successful treatment while 5.21% (five patients) had a partial response, and 4.17% (four patients) were considered failures (table 2). the five patients with partial response to treatment were found to have symptomatic physiological preputial adhesion and were managed by simple adhesiolysis in the outdoor clinic under xylocaine spray. circumcision was performed for four treatment failure patients. at six months of follow-up, two patients were lost original article phimosis in children j nepal paediatr soc | vol 42 | issue 02 |may-aug, 202228 to follow-up while 88.5 % (85 / 96 patients) had a retractable prepuce without recurrence of phimosis. in all the cases, the treatment was well tolerated without evidence of adverse effects. in general, boys older than six years performed the retraction by themselves while parents performed the retraction procedure in younger boys. table 2. putcome of phimosis in kirko’s grades after steroid therapy grade number of patients percentage grade 0 87 90.62% grade 1 0 0 grade 2 4 4.17% grade 3 5 5.21% grade 4 0 0 grade 5 0 0 discussion this study found that local application of 1% hydrocortisone cream with manual prepucial stretching is an effective, safe treatment in patients with physiological phimosis. this finding was similar to several articles published recently on the treatment of phimosis using topical steroids with success rates from 70% to 90%.6,13,14 in a study, 233 patients received eight-week treatment with 0.02% clobetasol propionate cream, among which 181 (77.68%) showed full retraction of the foreskin, 28 (12.01%) experienced improvement (disappearance of the phimotic ring), and 24 (10.30%) failed to respond, with a total effectiveness rate of 89.70%.15 our result of 90.6 % success rate is similar to the above studies. moreover, this study followed patients and found that there was no recurrence in a six months follow-up. this study highlights that four weeks of conservative treatment could be tried before circumcision reducing the risk of anesthesia16 as well as complications associated with surgery.16,17 a tight foreskin may manifest with symptoms such as itching, smegma deposits, straining, ballooning, balanoposthitis, dysuria, or urinary infection termed as symptomatic or pathological phimosis. recently the general trend emerging all over the world is to perform circumcisions only in symptomatic cases and in possible cases to perform a prepuce conserving surgery like prepucioplasty, v-y plasty, etc.18 the dorsal slit has been known to cause scarring of the dorsal prepuce leaving an inadequate amount of ventral foreskin in 55% of the boys four years after the operation.19 surgical alternatives to circumcision, such as preputial plasty, also require anesthesia which has up to a 4% recurrence rate.18 the exact mechanism of action of topical steroids in phimosis is unknown. however proposed mechanisms are inhibition of prostaglandin release, downregulation of collagen synthesis, and moisturizing effect of steroids improving the elasticity of prepuce acid.1,5,7 steroids applied only to the foreskin (less than 0.1% of the total body surface area) have very minimal systemic side effects even in young boys less than four years of age5,7 and also topical steroid treatment for phimosis did not change morning cortisol levels.7 no local or systemic side effects with corticosteroid cream and foreskin stretching were noticed in our study. the risk of side effects is unlikely since the quantity of cream applied and the treated surface of the prepuce is very small. nevertheless, the parents and patients were informed accordingly and possible signs of toxicity such as headaches and vomiting were mentioned. the overall cost of topical steroid treatment is 25 to 35% of circumcision and preputial plasty, therefore costeffective.6,13 sometimes prepucial adhesion may be misdiagnosed as pathological phimosis and subjected to circumcision when it could have easily been managed by adhesiolysis as an outdoor procedure.10-12 gentle retraction of the prepuce, known as physiotherapy, was suggested as an important factor in the spontaneous resolution of physiologic phimosis.17 although forcible retraction of the prepuce should be avoided because of pain, bleeding, adhesion, and cicatrix formation, which might lead to secondary phimosis, careful and gentle retraction has been encouraged for the more rapid resolution of severe physiologic phimosis.6,17 a previous study found that gentle retraction of the foreskin with topical application, reported a 50% success rate even with placebo cream and physiotherapy and suggested that physiotherapy per se would be effective to resolve physiologic phimosis.6 in concurrence to these researches, we also found that steroid therapy would be an effective therapy for physiological phimosis. the limitation of this study is that it is a single-site study with a small number of children from the local region. hence the study should be further carried out in a large population from various centers to confirm the finding of this study to extrapolate to the general population. conclusions this study has shown that not all non-retractile prepuces are pathological phimosis and require circumcision. local application of 1% hydrocortisone cream along with foreskin stretching is a safe, simple, and effective long-term treatment for physiological unretractable foreskin in children. references 1. lund l, wai kh, mui lm, yeung ck. effect of topical steroid on non-retractile prepubertal foreskin by a prospective, randomized, double-blind study. scand j urol nephrol. 2000;34(4):267-9. doi: 10.1080/003655900750042013. pubmed pmid: 11095086. 2. gairdner d. the fate of the foreskin, a study of circumcision. br med j. 1949;2(4642):1433-7, illust. doi: 10.1136/bmj.2.4642.1433. pmid: 15408299; pmcid: pmcpmc2051968. 3. schoen ej, wiswell te, moses s. new policy on circumcision-cause for concern. pediatrics. 2000;105(3 pt 1):620-3. doi: 10.1542/peds.105.3.620. pmid: 10699119. 4. circumcision policy statement. american academy of pediatrics. task force on circumcision. pediatrics. j nepal paediatr soc | vol 42 | issue 02 |may-aug, 2022 29 original articlephimosis in children 1999;103(3):686-93. pmid: 10049981. 5. orsola a, caffaratti j, garat jm. conservative treatment of phimosis in children using a topical steroid. urology. 2000;56(2):307-10. doi: 10.1016/s0090-4295(00)00576-8. pmid: 10925099. 6. van howe rs. cost-effective treatment of phimosis. pediatrics. 1998;102(4):e43. doi: 10.1542/peds.102.4.e43. pmid: 9755280. 7. monsour ma, rabinovitch hh, dean ge. medical management of phimosis in children: our experience with topical steroids. j urol. 1999;162(3 pt 2):1162-4. doi: 10.1097/00005392-199909000-00074. pmid: 10458456. 8. jørgensen et, svensson a. the treatment of phimosis in boys, with a potent topical steroid (clobetasol propionate 0.05%) cream. acta derm venereol. 1993;73(1):55-6. doi: 10.2340/00015555735556. pmid: 8095754. 9. kikiros c, beasley s, woodward a. the response of phimosis to local steroid application. pediatr surg int. 1993;8(4):32932. doi: 10.1007/bf00173357 10. huntley js, bourne mc, munro fd, wilson-storey d. troubles with the foreskin: one hundred consecutive referrals to paediatric surgeons. j r soc med. 2003;96(9):449-51. doi: 10.1258/jrsm.96.9.449. pmid: 12949201; pmcid: 539600. 11. rickwood am, kenny se, donnell sc. towards evidence based circumcision of english boys: survey of trends in practice. bmj. 2000;321(7264):792-3. doi: 10.1136/bmj.321.7264.792. pmid: 11009516; pmcid: 27490. 12. spilsbury k, semmens jb, wisniewski zs, holman cd. circumcision for phimosis and other medical indications in western australian boys. med j aust. 2003;178(4):155-8. pmid: 12580740. 13. berdeu d, sauze l, ha-vinh p, blum-boisgard c. costeffectiveness analysis of treatments for phimosis: a comparison of surgical and medicinal approaches and their economic effect. bju int. 2001;87(3):239-44. doi: 10.1046/j.1464-410x.2001.02033.x. pmid: 11167650. 14. nobre yd, freitas rg, felizardo mj, ortiz v, macedo a, jr. to circ or not to circ: clinical and pharmacoeconomic outcomes of a prospective trial of topical steroid versus primary circumcision. int braz j urol. 2010;36(1):75-85. doi: 10.1590/s1677-55382010000100012. p m i d : 20202239. 15. wen y-l, wang a-g, zhang z-p, wu j, jiang t. topical application of clobetasol propionate cream in the treatment of phimosis in prepubertal children: a report of 237cases. zhonghua nan ke xue national journal of andrology. 2017;23(7):635-8. doi:10.13263/j.cnki.nja.2017.07.011 16. cooper gg, thomson gj, raine pa. therapeutic retraction of the foreskin in childhood. br med j (clin res ed). 1983;286(6360):186-7. doi: 10.1136/bmj.286.6360.186. pmid: 6401522. 17. matsuoka n. follow-up studies of children who underwent dorsal slit. jpn j clin urol. 1994;48:843-6. doi: 10.11477/mf.1413901308 18. cuckow pm, rix g, mouriquand pd. preputial plasty: a good alternative to circumcision. j pediatr surg. 1994;29(4):561-3. doi: 10.1016/0022-3468(94)90092-2. pmid: 8014816. 19. bloom da, wan j, key d. disorders of the male external genitalia and inguinal canal. clinical pediatric urology. 1992;2:1015-49. j nepal paediatr soc | vol 42 | issue 03 |sep-dec, 202256 original article doi: 103126/jnps.v4113 original article doi: 103126/jnps.v4113 original article doi: 103126/jnps.v4113 study of vitamin d level in paediatric asthma at a tertiary care center introduction: there are few studies that suggest low vitamin d levels are associated with asthma. present study was conducted to evaluate the association between vitamin d levels with asthma and its severity in children. methods: this cross-sectional study included 70 children (35 asthmatic and 35 controls) aged between three to 12 yrs. cbc, aec, ige, spirometry, chest x-ray, and serum vitamin d levels were measured. vitamin d levels were categorized according to guidelines of the american academy of paediatrics (2008) and compared between the two groups. the association between vitamin-d levels with asthma and its severity was studied. result: the mean age of study group cases was 9.23 ± 2.62 yr. the male:female ratio in the study group was 2.5: 1. cough and wheezing was the commonest manifestation (100%). most of the patients had mild asthma (45.71%). asthma was more common in the urban population (68.57%) and children belonging to lower socioeconomic status (51.43%). the markers of allergic disorders {eosinophils, aec and ige} were elevated (all p-values ≤ 0.0001) in study group. mean serum vitamin d levels in the study group and control group patients were 45.21 ± 28.52 nmol/l and 57.03 ± 40.01 nmol / l respectively. the mean levels of vitamin d in the study group were insufficient as per aap criteria. vitamin d levels were more deficient in severe asthma (26.19 ± 14.46 nmol/l). conclusions: in the present study vitamin d insufficiency was seen in patients with asthma. abstract 1 professor, department of paediatrics, s.n. medical college, agra, up, india. 2 junior resident, department of paediatrics, s.n. medical college, agra, up, india. pankaj kumar,1 rajesh kumar1, pratap bhanu singh2 *corresponding author pankaj kumar, professor, department of paediatrics, s.n. medical college, agra, up, india. email: drpankaj_peds@hotmail.com article history received on: 08 aug, 2022 accepted on: 26 apr, 2023 funding sources: none conflict of interest: none keywords: allergy; asthma; vitamin d deficiency online access doi: https://doi.org/10.3126/jnps.v42i3.47355 copyrights & licensing © 2022 by author(s). this is an open access article distributed under creative commons attribution license (cc by nc ) introduction asthma is a heterogeneous disease, usually characterized by chronic airway inflammation affecting one to 18% of the population in different countries.1 it is defined by the history of respiratory symptoms such as wheeze, shortness of breath, chest tightness, and cough that vary over time and in intensity, together with variable expiratory airflow limitation. symptoms are often triggered by factors such as exercise, allergen or irritant exposure, change in weather, or viral respiratory infections. recently, many studies have shown that the prevalence of asthma and allergic conditions have increased in various regions of the world.2 according to who, in 2005 around 300 million people suffered from asthma and 2,55,000 died of asthma.3 it is expected to increase to 400 million by 2025.4 numerous studies conducted in different countries have reported an increase in asthma prevalence of approximately 50% per decade.5 j nepal paediatr soc | vol 42 | issue 03 |sep-dec, 2022 57 original articlevitamin d level in paediatric asthma original article doi: 103126/jnps.v4113 asthma may have significant relation with serum vitamin d level. there has been extensive studies regarding this matter. however, indian studies are relatively lacking and inconclusive on this matter. for eg, somashekar et al in their study conducted in bangalore found that serum vitamin-d level was significantly lower in asthmatic children.6 on the other hand dogru m et al found that no significant difference was found between mean serum vitamin d levels in asthmatic children and control.7 keeping this background in mind, the present study was conducted to evaluate the vitamin d levels in asthmatic children and their association with the severity of asthma in north india. methods this cross-sectional study was conducted at a tertiary care center in agra, india between march 2015 and august 2016. children between three to 12 years of age attending paediatric opd and ipd, having clinical features suggestive of asthma, were enrolled as study subjects after taking informed consent from the parents or guardian. patients with bronchial asthma were diagnosed using gina guidelines (2015). only newly diagnosed cases of asthma were taken as study subjects. healthy children aged between three to 12 years, without a history or family history of asthma, coming to the paediatric outpatient department or ward, were enrolled as controls. children with other systemic illnesses or receiving vitamin d therapy or drugs that might affect vitamin d levels for the past one year were excluded from the study. data were collected on demographic variables (age, sex, height, weight, place of residence, socioeconomic status, and family history) and compared between the study and control group. the patients included in the study group underwent a detailed history, thorough clinical examination, and relevant investigations. complete blood count, absolute eosinophil count, serum ige levels, and vitamin d levels were done for both the study and the control groups. the serum ige levels were measured by chemiluminescence immunoassay (clia) by using a clia kit. vitamin d levels were determined by measuring 25-hydroxyvitamin d levels. estimation of serum vitamin d was done by electrochemiluminescence binding assay. an x-ray chest (plain radiograph) was done. spirometry was done in children above six years of age. for the bronchodilator reversibility test, salbutamol was given by mdi / nebulization. an increase in fev1 of > 12% predicted was considered positive for a diagnosis of asthma. study subjects were categorized based on their vitamin d levels as classified by the american academy of paediatrics (2008).8-10 severely deficient: ≤ 12.5 nmol / l (≤ 5 ng / ml) deficient: ≤ 37.5 nmol/l (≤ 15 ng / ml) insufficient: > 37.5 nmol/l to < 50 nmol / l (> 15 ng / ml to < 20 ng / ml) sufficient: ≥ 50 nmol / l to 250 nmol / l (≥ 20 ng / ml to 100 ng / ml) study was approved by ethical committee of the institute. the results of this study were analyzed and presented as numbers, percentages, or mean ± sd. statistical analysis was performed by applying the student “t” test, fisher’s exact test, and the analysis of variance (anova) technique. a p-value less than 0.05 was considered to be significant for the statistical hypothesis. results thirty-five children suffering from asthma and 35 healthy children were included in our study. the mean age of study and control group cases was 9.23 ± 2.62 yr and 7.94 ± 3.05 yr respectively. the male:female ratio was 2.5:1 and 1.7:1 in the study and control groups respectively. the socio-demographic profile of the study and control group is shown in table no.1. table 1: demographic and laboratory profile of study and control group demographic profile study (n=35) control (n=35) p-value age group 3-6 yr 6-9 yr 9-12 yr 3 (8.57%) 11 (31.43%) 21(60%) 6 (17.14%) 12 (34.29%) 17 (48.57%) 0.5018 gender male female 25 (71.43%) 10 (28.75%) 22 (62.86%) 13 (37.14%) 0.460 rural urban 11 (31.43%) 24 (68.57%) 12 (34.29%) 23 (65.71%) 1.000 ses lower middle upper 18 (51.43%) 15 (42.86%) 2 (5.71%) 17 (48.57%) 10 (28.57%) 8 (22.86%) 0.1192 laboratory parameters eosinophilscount (%) 6.97 ± 3.58 0.85 ± 2.40 < 0.0001 aec(cells/mm3) 690.23 ± 418.67 278.14 ± 96.07 < 0.0001 ige (iu/ml) 1035.69 ± 937.64 174.29 ± 217.31 < 0.0001 vit. d (nmol/l) 45.21 ± 28.52 57.03 ± 40.01 0.1597 cough and wheezing was the commonest presentation [35 / 35 (100%)] in the study group. dyspnea was present in 28 / 35 (80%) and night symptoms in 17 / 35 (48.57%) j nepal paediatr soc | vol 42 | issue 03 |sep-dec, 202258 original article vitamin d level in paediatric asthma of patients. twenty-one (60%) of asthmatic children had co-existing allergic rhinitis and four (11.4%) had atopic dermatitis. of the study group cases, 13 / 35 (37.14%) cases had a positive family history of allergy. of the total 35 children suffering from bronchial asthma, 16 (45.71%) had mild asthma, 15 (42.86%) had moderate asthma; only four (11.4%) children were suffering from severe asthma. mean fev1 values in mild, moderate, and severe asthma cases were 85.13 ± 3.72, 74.85 ± 7.50, and 51.33 ± 6.43 of the percentage predicted respectively. the value of fev1 / fvc in mild asthma was 94.67 ± 6.66, in moderate was 81.54 ± 4.91 and in severe asthma was 65.33 ± 6.43 of the percentage predicted value. comparisons of mean serum eosinophils count (%), aec, ige, and vitamin d levels in the study and control group had been described in table 1. mean eosinophils counts (6.96 ± 3.58%), aec (690.23 ± 418.67 cells / mm3) and ige (1035.69 ± 937.64 iu / ml) levels were higher in the study group than in the control group and the difference was statistically significant. mean vitamin d levels in the study group was 45.21 ± 28.52 nmol / l which was insufficient as per aap guidelines. mean serum vitamin d levels in mild, moderate and severe asthma cases were 52.56 ± 26.28, 42.44 ± 31.88 and 26.19 ± 14.46 nmol / l respectively. the level of vitamin d showed declining trend with increasing severity of asthma. patients with severe asthma have very low levels of vitamin d as compared to patients suffering from mild asthma (p = 0.0249). discussion the prevalence and severity of asthma and allergic diseases in children continue to increase worldwide. based on the results of various studies conducted in india as well as in other countries, the association of vitamin d deficiency with asthma is still controversial and confusing. several studies suggest a positive correlation between serum vitamin d levels and asthma but some studies are contradicting the same.6,7,11,12 the mean age group and male-female ratio in our study and control group were comparable to several other studies.6,13 in the study as well as in the control group most of the cases belonged to urban areas. this is comparable with several other studies where the prevalence of asthma was higher in children belonging to urban areas.14,15 increased number of cases from urban areas can be explained by the easy accessibility of health care facilities in urban areas. most of the cases in our study belonged to lower ses followed by middle and upper ses. asthma and allergic disorders are more related to tobacco smoking, air pollution (wood or coal-burning smoke, dust, etc.), poor housing conditions, and indoor allergens (animal dander, dust mites, cockroaches, molds), and diet which were related to lower ses.5 it may also be explained by the fact that most of the patients visiting government hospitals belonged to lower ses because of relatively cheaper healthcare facilities. in the study group, 37.14% of cases had a positive family history of asthma. other studies also showed similar trends of prevalence of family history.16,17 several studies show the co-existence of allergic disorders with asthma which is similar to our study.18,19 in our study subjects, most of the children were suffering from mild asthma followed by moderate and severe asthma which is consistent with a study done by lal et al.15 the mean value of eosinophil count (%), serum aec levels, and ige levels were significantly elevated in the study group than in control group (all p-value = < 0.0001). eosinophil count ≥ 4% in peripheral blood is a risk factor for early childhood asthma.5 the presence of > 500 eosinophils / ml in peripheral blood is the most common hematologic abnormality of allergic patients.20 average total ige levels are higher in the population of allergic patients than in comparable populations without allergic disease.20 ehlayel et al also observed the mean ige levels were significantly higher (pvalue = < 0.001) in asthmatic children.13 mean serum vitamin d levels in the study group and control group patients were 45.21 ± 28.52 nmol / l and 57.03 ± 40.01 nmol / l respectively. though the levels of vitamin d in the study group was insufficient as per aap criteria, the difference in the levels of vitamin d between the two group was not statistically significant (p-value = 0.1597).810 these findings were comparable with several studies done in various parts of the world.7,12,21 in the study subject vitamin d deficiency was present only in 48.57% of subjects which was consistent with a study done by yao tc et al in which vitamin d deficiency was present in 51.0% of study subjects.12 in our study cases from rural areas had sufficient vitamin d levels while cases belonging to urban areas had deficient levels of vitamin d and the difference was statistically significant (p-value = 0.0462). there was also an increased prevalence of asthma in the urban population. this is the only point that suggests some association between vitamin d levels and asthma. a similar result was seen in a study done in central ethiopia where vitamin d deficiency was significantly higher among students in an urban setting.22 the present study found that children from the upper and middle socioeconomic groups had insufficient vitamin d levels. while children belonging to low ses had sufficient vitamin d levels. prevalence of asthma was more in patients who belonged to lower ses as compared to upper ses. the maximum no. of patients in our study belonged to mild asthma followed by moderate and severe asthma. vitamin d levels in patients suffering from mild and moderate asthma were sufficient and insufficient respectively. only patients with severe asthma had vitamin d deficiency. increased severity of asthma was associated with decreased levels of j nepal paediatr soc | vol 42 | issue 03 |sep-dec, 2022 59 original articlevitamin d level in paediatric asthma vitamin d in several other studies.23 as the sample size of the present study is relatively small, more studies are needed to look for any consistent correlation between vitamin d deficiency and asthma. the small sample size in our study can be considered one of the limitations in our study. conclusions in the present study, vitamin d levels were insufficient (as per aap classification) in study group and deficiency was more pronounced in patients with severe asthma. references 1. gina guidelines 2015, retrieved april 26,2015from www. ginasthma.org. 2. von mutius e. the rising trends in asthma and allergic disease. clin exp allergy. 1998 nov;28 suppl 5:45-9; discussion 50-1. doi: 10.1046/j.1365-2222.1998.028s5045.x. pmid: 9988447. 3. schneider l, tilles s, lio p, boguniewicz m, beck l , lebovidge j, et al. atopic dermatitis: a practice parameter update 2012. j allergy clin immunol. 2013 feb;131(2):295-9.e127. doi: 10.1016/j.jaci.2012.12.672 4. pawankar r. allergic diseases and asthma: a global public health concern and a call to action. world allergy organ j 7, 1–3 (2014). doi: 10.1186/1939-4551-7-12 5. liu ah, covar ra, spahn jd, sicherer s.h. (2016) childhood asthma. in: kliegman rm, stanton bf, st geme iii, j.n. and behrman re, eds., nelson textbook of paediatrics, 20th edition, elsevier, philadelphia, 1095-1115. 6. somashekar ar, prithvi ab, gowda mn. vitamin d levels in children with bronchial asthma. j clin diagn res. 2014 oct;8(10):pc04-7. doi: 10.7860/jcdr/2014/10387.5055. pmid: 25478419; pmcid: pmc4253237. 7. dogru m, kirmizibekmez h, yesiltepe mutlu rg, aktas a, ozturkmen s. clinical effects of vitamin d in children with asthma. int arch allergy immunol. 2014;164(4):319-25. doi: 10.1159/000366279. epub 2014 sep 23. pmid: 25277142. 8. misra m, pacaud d, petryk a, collett-solberg pf, kappy m; drug and therapeutics committee of the lawson wilkins pediatric endocrine society. vitamin d deficiency in children and its management: review of current knowledge and recommendations. pediatrics. 2008 aug;122(2):398-417. doi: 10.1542/peds.2007-1894. pmid: 18676559. 9. holick mf. vitamin d deficiency. n engl j med. 2007 jul 19;357(3):266-81. doi: 10.1056/nejmra070553. pmid: 17634462. 10. holick mf. vitamin d status: measurement, interpretation, and clinical application. ann epidemiol. 2009;19(2):73–78. doi: 10.1016/j.annepidem.2007.12.001 11. uysalol m, mutlu lc, saracoglu gv, karasu e, guzel s, kayaoglu s, et al. childhood asthma and vitamin d deficiency in turkey: is there cause and effect relationship between them? ital j pediatr. 2013 dec 13;39:78. doi: 10.1186/1824-7288-39-78. pmid: 24330502; pmcid: pmc3892001. 12. yao tc, tu yl, chang sw, tsai hj, gu pw, ning hc, et al. suboptimal vitamin d status in a population-based study of asian children: prevalence and relation to allergic diseases and atopy. plos one. 2014 jun 3;9(6):e99105. doi: 10.1371/journal.pone.0099105. 13. ehlayel ms, bener a, sabbah a. is high prevalence of vitamin d deficiency evidence for asthma and allergy risks? eur ann allergy clin immunol. 2011 jun;43(3):81-8. pmid: 21789969. 14. pal r, dahal s, pal s. prevalence of bronchial asthma in indian children. indian j community med. 2009 oct;34(4):3106. doi: 10.4103/0970-0218.58389. pmid: 20165624; pmcid: pmc2822191. 15. lal a, kumar l, malhotra s. socio-economic burden of childhood asthma. indian pediatr. 1994 apr;31(4):425-32. pmid: 7875864. 16. jain a, vinod bhat h, acharya d. prevalence of bronchial asthma in rural indian children: a cross sectional study from south india. indian j pediatr. 2010 jan;77(1):31-5. doi: 10.1007/s12098-009-0308-6. pmid: 20091364. 17. dhduh maaa, sabri nam, fouda em. prevalence and severity of allergic diseases among egyptian pediatric in different egyptian areas. int j pharm sci res. 2015; 2: 107. doi:10.15344/2394-1502/2015/107 18. ober c, yao tc. the genetics of asthma and allergic disease: a 21st century perspective. immunol rev. 2011 jul;242(1):1030. doi: 10.1111/j.1600-065x.2011.01029.x. pmid: 21682736; pmcid: pmc3151648. 19. kumar g, roy g, subitha l, sahu sk. prevalence of bronchial asthma and its associated factors among school children in urban puducherry, india. journal of natural science, biology & medicine. 2014;5(1):59-62. doi:10.4103/0976-9668.127289 20. kim js, bunyavanich s, sicherer sh. diagnosis of allergic diseases. in: kliegman(ed). nelson textbook of pediartrics. first south asia edition. india: reed elsevier india pvt. ltd: 2016.1078-1082. 21. cairncross c, grant c, stonehouse w, conlon c, mcdonald b, houghton l, et al. the relationship between vitamin d status and allergic diseases in new zealand preschool children. nutrients. 2016jun1; 8(6).pii: e326. doi: 10.3390/nu8060326. 22. wakayo t, belachew t, vatanparast h, whiting sj. vitamin d deficiency and its predictors in a country with thirteen months of sunshine: the case of school children in central http://www.ginasthma.org http://www.ginasthma.org https://doi.org/10.1016/j.jaci.2012.12.672 https://doi.org/10.1016%2fj.annepidem.2007.12.001 file:///c:\users\asus\desktop\suboptimal%20vitamin%20d%20status%20in%20a%20population-based%20study%20of%20asian%20children%20%20prevalence%20and%20relation%20to%20allergic%20diseases%20and%20atopy.%20-%20pubmed%20-%20ncbi.htm https://doi.org/10.15344/2394-1502%2f2015%2f107 https://doi.org/10.4103/0976-9668.127289 file:///c:\users\asus\desktop\the%20relationship%20between%20vitamin%20d%20status%20and%20allergic%20diseases%20in%20new%20zealand%20preschool%20children.%20-%20pubmed%20-%20ncbi.htm j nepal paediatr soc | vol 42 | issue 03 |sep-dec, 202260 original article vitamin d level in paediatric asthma ethiopia. plosone.2015; 10(3): e0120963. doi:10.1371/journal.pone.0120963. 23. sharif a, haddad kashani h, sharif mr. association of 25-hydroxy vitamin d with asthma and its severity in children: a case-control study. clin mol allergy. 2020 may 4;18:7. doi: 10.1186/s12948-020-00122-9. pmid: 32390767; pmcid: pmc7197187. final nepas journal 30-1.indd -1-journal of nepal paediatric society january-june, 2010/vol 30/issue 1 original article january-june, 2010/vol 30/issue 1 is low hemoglobin level a risk factor for acute lower respiratory tract infections? malla t1, pathak ok2, malla kk3 1dr. tejesh malla, mbbs, md, assistant professor, 2 dr. om k pathak, mbbs, medical officer, 3dr. kalpana k malla, mbbs, md, associate professor. all from the department of paediatrics, manipal college of medical sciences, pokhara, nepal. address for correspondence: dr. tejesh malla, e-mail: tejeshmalla@hotmail.com abstract objective: this prospective study was conducted to evaluate whether a low hemoglobin level, was a risk factor for acute lower respiratory tract infections (alrti) in children. methods: 150 children of all age groups who came to the outpatient department and those admitted for alrti were included in the study. age and sex-matched 140 children, not having any respiratory illness, were taken as control. the study period was from march 2006 march 2007. detailed clinical and laboratory evaluation of the enlisted patients was done. all were subjected to detail investigations. results: radiological evidence of pneumonia was present in 70 (50 %) children.hyperinflated lungs were seen in 40 (29%) and was normal in 30 (21.4%) cases. blood culture was positive in 14 (10%) children of study group and none among control group. klebsiella was the commonest organism isolated 6 (4.2%) in blood culture positive cases. the mean hemoglobin (hb) level of study group was 9.88 gm% and it was 12 gm% in control group.96 (68.6%) of study group and 30 (21.42%) of control group had anemia. of the anemic children, 79 (82.3%) in study group had iron deficiency, and 17 (17.7%) had normocytic normochromic anemia. these values were 18 (33.3%) and 36 (66.6%) respectively for control group. low hemoglobin level was a risk factor (p<0.001) alrti. conclusion: anemic children were 3.2 times more susceptible to alrti compared to the control group and and iron deficiency anemia was predominating. supplemental iron therapy may reduce the incidence of alrti. prevention of anemia, due to whatever etiology is also essential. key words: alrti, anemia, hemoglobin. introduction anemia is a major nutritional global problem of immense public health signifi cance, affecting persons of all ages, sex and economic group. it is ranked as the commonest chronic malady of mankind affecting approximately 30% i.e. 1500 million people all over the world. iron defi ciency anemia in children occurs most frequently between the age of 6 months to 3 years1, the age when repeated infection occurs. on an average, children below 5 years of age suffer about 5-6 episodes of alrti per year2. with this view the present study was conducted to see if children with iron defi ciency anemia were at higher risk of alrti. methods this prospective study was carried out for a period of one year from march 2006 – march 2007 in pediatric department of manipal teaching hospital, pokhara. a total of 290 (150 cases and140 controls) children from 1 month to 5 years of age attending out patient department and those hospitalized were studied. controls were age and sex matched children not having respiratory problems. the inclusion criteria for cases were children with fever, cough, and fast respiratory rate, chest indrawing as per who criteria, and ronchi or crepitations on auscultation. the exclusion criteria was children suffering from other systemic illnesses like -2-january-june, 2010/vol 30/issue 1 journal of nepal paediatric society congenital heart disease, tuberculosis (any evidence plus montaux test positive cases) and protein energy malnutrition (pem > grade iii as per indian academy of pediatrics (iap) classifi cation). children who already received antibiotic from outside were also excluded from the study. the purpose of the study was explained to the parents or guardians. then consent was taken from parents or guardians before they were subjected to investigations. the investigations in both case and control included; complete blood count (cbc) with a peripheral smear, blood culture and sensitivity test, and x-ray chest, serum iron and serum iron binding capacity. serumferritin level was not done. syanmeth method by colorimeter was used to identify hb level. hemglobin level < 10 gm % will be considered low in this study. data were analysed using spss 10.0 by logistic multinomial regression analysis. results out of 290 children 150 were cases among which 10 were mantoux positive hence was excluded from the study. therefore the fi nal fi gure was 140 (99 males and 41 females) cases and 140 (94 males and 46 females) controls. among the cases 10 (8 males and 2 females) were less than 2 months, 69 (43 m and 26 f) were inbetween 2months – 1 year and 61 ( 48m and 13f ) were above 1 year. similarly for control group the distribution was 10 (6m/4f), 50(30m/20f), and 80 (58m/22f) were ≤2months, 2mo-1year and >1 year respectively. (table1). fever, cough and shortness of breath was main clinical features in the cases whereas fever, pain abdomen diarrhea, vomiting, seizures was main clinical features in the control group (table 2). radiological evidence of pneumonia was present in 70 (50 %) children, hyperinfl ated lungs in 40 (29%) cases and normal in 30 (21.4%) cases (fig 1). out of 40 children who had hyperinfl ated lung fi elds 20 (50%) had a history of recurrent wheeze ( fi g.2) and 10 ( 25%) gave a positive family history of asthma. blood culture was positive in 14 (10 %) children of study group (table 3) and none among control group. among the culture positives klebsiella was isolated in 6 (4.2%), and growth of staphylococcus, streptococcus pneumoniea, acintobacter and e.coli was noted in 2 (1.43%) cases each. the mean hb level of study group was 9.88 gm% and it was 12 gm% in control group.96 (68.6%) of study group and 30 (21.42%) of control group had anemia. of the anemic children, 79(82.3%) in study group had iron defi ciency, with mean mcv 64 (fl ),mean mch 17pg,mchc 25gm/dl,mean s. iron 35μg/dl,mean tibc 390μg/dl.17 (17.7%) had normocytic normochromic anemia. these values were 18 (33.3%) [with mean mcv 68.7 (fl ), mean mch 15pg, mean mchc 27gm/dl, mean s. iron 35μg/dl, mean tibc 350μg/dl] and 36 (66.6%) respectively for control group (table 4). there were 72 cases of bronchopneumonia among which 62 (86%) of them were anemic whereas rest 68 were wheeze associated alrti (bronchiolitis & recurrent wheeze ) of which 34 (50%) were anemic.(table 5). descriptive data regarding multivariate logistic regression analysis showing the risk factor of alrti. table 7 reveals the montaux positive cases which were excluded from the study. table 1: age and sex distribution of cases and control sex age total ≤2 months >2mo-1yr >1 -5 year case ; male=99 (70.7%) female=41(29.3%) 8 2 43 26 48 13 140 total 10 69 61 140 control: male= 94 (67%) female=46 (32.8%) 6 4 30 20 58 22 140 total 10 50 80 140 -3-journal of nepal paediatric society january-june, 2010/vol 30/issue 1 table 2: symptoms and signs at presentation symptoms case control n (n=140) percentage n(n=140 ) percentage fever 135 96.42% 114 81.42% cough 140 100% 8 5.71% shortness of breath 81 57.85% 4 2.85% convulsion 10 7.14% 30 21.42% vomiting 29 20.71% 77 55% diarrhea 10 7.14% 52 37.14% noisy breathing 50 35.71% 0 0% poor feeding 80 57.14% 60 42.85% chest pain 4 2.85% 0 0% irritable 20 14.28% 30 21.42% fast breathing 100 71.42% 0 0% delayed development 0 0% 6 4.28% headache 0 0% 2 1.42% pain abdomen 0 0% 35 25% sore throat 0 0 % 24 17% signs chest indrawings 120 85.71% 0 0% ronchi 70 50% 0 0% crepitations 60 42.85% 0 0% pyoderma 0 0% 4 2.85% dehydration 5 3.57% 35 25% table 3: blood culture of cases and control case (n=140) control (n=140) blood culture number percentage number percentage no growth growth klebsiella staphylococcus s. pneumniea acintobacter e.coli 126 14 6 2 2 2 2 90 % 10% 4.2% 1.43% 1.43% 1.43% 1.43% 140 0 100% 0% 0 10 20 30 40 50 ser 0% 0% 0% 0% 0% 0% pneum ies1 50 monia h 0% 50% yperinflation 29% 29% norma 21.40% 21.4 al % 40% series1 fig. 1: radiological fi ndings of cases -4-january-june, 2010/vol 30/issue 1 journal of nepal paediatric society table 4: hemogram of cases and control. case (n=140) control (n=140) hemoglobin number percentage number percentage mean hb 9.88gm % 12gm % hemoglobin ≤10gm%± >10gm% 96 44 68.6%) 31.4% 54 86 38.6% 61.4% anemia type mh* nn** 79 17 82.3% 17.7% 18 36 33.3% 66.6% mh anemia nn anemia mh anemia nn anemia mean mcv(fl )*** 64 80 68.7 79 mean mch(pg)**** 17 25 15 27 mean mchc(gm/dl)***** 25 32 27 34 s. iron 35μg/dl 95 35 μg/dl 100 mean tibc+ 390 μg/dl 268 350 μg/dl 270 note : anemic± = hb< 10gm/dl (normal hb in < 2mo 9 – 14 gm/dl and > 2months 11.5-15.5gm/dl)3 mh*= microcytic hypochromic, nn**=normocytic normochromic, mcv***=mean corpuscular volume ( 1month6 yrs = 7688(fl ),4mch****=mean corpuscular hemoglobin ( 1month6 yrs= 24-30 (pg ),4 mchc*****= mean corpuscular hemoglobin concentration ( 1month6 yrs=30-36gm/dl),4 s.iron (infants =100-400 μg/dl and above infancy =250-400 μg/dl)3,tibc+ -total iron binding capacity (all age =22-184 μg/dl )3 50% 50% h n h/o wheezing no wheezing fig. 2: histrory of wheezing 0 5 10 15 20 1 7 13 19 25 31 37 43 49 55 61 67 73 79 85 91 97 103 109 115 121 127 133 139 children in case and control h b le ve l i n gm % series1 series2 fig. 3: graph showing range of hb in case and control -5-journal of nepal paediatric society january-june, 2010/vol 30/issue 1 discussion acute lower respiratory tract infection (alrti) is a leading cause of mortality in children below 5 years of age in developing countries5. hence it is important to control the risk factors to prevent deaths from alrti. along with many risk factors like low birth weight, lack of breast feeding, severe malnutrition, smoke, cooking fuel6, low hemoglobin may also be a risk factor. present study was carried out to prove this fact.there were 140 cases (m=99 & f= 41) and 140 controls (m=94&f=46) among which in cases maximum children were between 2month – 1 year.this signifi es that alrti is most common in age group 2month to 1 year. this is the time when a child starts having low hemoglobin levels and also this is the period of adding supplemental feed which may be inadequate and inappropritate. the reasons for higher number of males may be gender biasness by the parents to bring them for hospital care. sign symptoms of patient had usual presentation of alrti. these patients came to us only after 4-5 days of illness or when the child became more ill as with less illness people of this region do not visit hospital. radiologically evidence of pneumonia was higher 70 (50 %) than hyperinfl ated lungs (bronchiolitis, recurrent wheeze) 40 (29%).this table 5: low hemoglobin with type of alrti bronchopneumonia (n= 72) wheeze associated lrti bronchiolitis / recurrent wheezing (n= – 68 ) number percentage number percentage hemoglobin ≤10gm% >10gm% 62 10 86.0 % 13.9 % 34 34 50 % 50 % total =140 72 100% 68 100% table 6: multivariate logistic regression analysis showing the risk factor of alrti parameters or 95% ci signifi cance hb≤10 5.6 2.7-11.7 df=1, p<0.001 s.iron 15.6 8.2-29.6 df=1, p<0.001 tibc 1.7 0.86-3.4 df=1, p= 0.119 c.i: confi dence interval, or: odds ratio,df:degree of freedom, lrtilower respiratory tract infection. table 7: history, clinical fi nding and cxr of mantoux positive cases. cases with mantoux positive 1 2 3 4 5 6 7 8 9 10 recurrent ari + + + + + + + + esr > 50mm/1st hr + + + + + + + + + + contact with tb + + + _ _ + weight loss + + + + + + + + cxr – compatible with tuberculosis + + + + gastric lavage for afb _ _ _ _ _ _ _ _ _ _ crofton, horne and miller scoring > 7 9 9 8 10 8 7 8 9 9 8 may indicate that low hemoglobin has higher risk for pneumonia than bronchiolitis. table 5 reveals that among pneumomia cases 86% were anemic whereas only 50% cases were anemic among bronchiolitis cases.there was no specifi c golden criterion to differentiate bacterial (pneumonia) or viral (bronchiolitis) alrti but ill looking child, crp positive,neutrophilic leukocytosis,blood culture positive was considred bacterial infection and viral infection were assessed clinically and leucopenia was considered. literature related to this fi nding was not available. it was observed that 20(50%) children with hyperinfl ated lung fi elds had a history of wheeze and 10 ( 25%) gave a positive family history of asthma. this maybe due to the fact that bronchiolitis is more prone to occur if there is a genetic predisposition or has a history of atopy rather than low hemoglobin. these population was not excluded from the study as they had features of secondary infection clinically with high fever, ill looking, not feeding well and biochemically with either neutrophilic leukocytosis or crp positive. blood culture showed growth in only 10 % of cases and klebsiella was most common (4.2%) organism isolated. in this study hb ≤ 10gm/dl was considered anemia (normal hb = -6-january-june, 2010/vol 30/issue 1 journal of nepal paediatric society <2mo 9-14gm/dl and > 2months -11.5 – 15.5gm/dl)3. mean hb level in this study was 9.58 gm% for cases &12gm% for control group. ramakrishnan k, harish ps in their study found that anemic children were 5.75 times more susceptible to lrti7 which was 3.2 times in this study. several risk factors for developing alrti had been reported in different studies. baskaran et al8 in their study in children between 3-5 years had found 83% with pneumonia had hemoglobin less than 11 g/dl. in this study microcytic and hypochromic picture was seen in maximum children (82.3%). in another study of iron defi ciency anemia and respiratory infection by de-silva a et al9, an over all prevalence of anemia was found in 52.6%. the role of low hemoglobin level per se, as a risk factor for developing alrti are reported only in few literatures7. they had found that reduced hemoglobin level due to whatever etiology was a signifi cant risk factor for developing alrti. unlike those studies here it was found that low hemoglobin due to iron defi ciency anemia was the main cause for alrti. iron defi ciency anemia was detected based on low mcv(normal=76-88 fl ), low mch(normal=24-30 pg ), low mchc(normal=3036gm/dl), low s.iron [normal=infants 100-400 μg/dl and above infancy 250-400 μg/dl] and increased tibc[ normal =all age=22-184 μg/dl]. serum ferritin level was not done due to unavailability of this test and ferritin level is not reliable in cases with infection as it increases probably as acute phase protein10. if you look at the normal function of hemoglobin it facilitates oxygen (o2) and carbon dioxide (co2) transport. it caries and inactivates nitric oxide (no) and also play the role of a buffer11. hemoglobin in the blood is mainly responsible for stabilizing the oxygen pressure in the tissues 12. therefore quantitative and/or qualitative reduction in hb, may adversely affect the normal functions. iron is principally required for haemoglobin synthesis.13 intestinal iron absorption is related to erythropoietic requirements, although the regulatory mechanism(s) remain unknown. the usual source of iron in the lung is serum iron which is derived from catabolised erythrocytes and absorbed iron13. probably it may be the reason for low hemoglobin level found to be as a serious risk factor for developing alrti. further studies including other risk factors like low birth weight, lack of breast feeding, severe malnutrition, smoke, cooking fuel etc along with low hemoglobin should be considred as future perspective. conclusion to conclude hb was a risk factor for lrti (p<0.001). iron defi ciency anemia was the main cause detected. anemia was responsible for pneumonia more than bronchilitis.iron supplementation in age group 1month to 5 years may reduce the incidence of lrti and prevention of anemia, due to whatever etiology is also essential. the limitation of this study is that other variables were not considered in this study. it is diffi cult to correlate the one point prevalence of pneumonia with anaemia as the control group could present with pneumonia within another few months. only way to see is to follow a group of children with normal and low haemoglobin over a period of time for an episode of pneumonia. acknowledgements: we thank all the children who had participated in this study. a special thanks to dr. praveen shrestha who had helped with data collecton. funding: none confl ict of interest: none references 1. demaeyer eh, adiels tegman m. the prevalence of anemia in the world. world health statistics 1985, 38, 302-316. 2. wald er. recurrent and non resolving pneumonia in 20 children. sem resp infect 1993; 8: 46-58. 3. michael a.pesce. reference ranges for laboratory tests and procedures. in richard e. behrman, robert kleigman, hal b jenson, ed. nelson text book of pediatrics. 18th ed. philadelphia; saunders, 2008: 2944. 4. forfar & arneil. text book of pediatrrics 5th edition 1998; campbell a.g.m. & mcintosh n, bpc wheatons ltd; exeter; 1939. 5. rasmussen z,pio a, enarson p. case management of childhood pneumonia in developing countries: relevant research and current initiatives. int j tuber lung dis 2000:4;807-827 6. behrman s. epidemiology of acute respiratory infection in children of developing countries. rev infect dis 1991: (suppl): s454-s462. 7. ramakrishnan k, harish ps. hemoglobin level as a risk factor for lower respiratory tract infections. indian j pediatr. 2006:73:10:881-883. 8. bhaskaran p, madhavan nair k, balakrishnan n. serum transferrin receptors in children with respiratory infections. eur j nutr. 2003; 57: 75-80. 9. de-silva a, atukorola s, weerasinghel. iron supplementation improves iron status and reduces morbidity in childrenwith or with out urti. am j clin nutr 2003; 77: 234-241. -7-journal of nepal paediatric society january-june, 2010/vol 30/issue 1 10. ryan tp, krzesicki rf, blakeman dp, et al. pulmonary ferritin: differential effects of hyperoxic lung injury on subunit mrna levels. free radic biol med 1997;22:901-908 11. william f ganong. gas transport between the lungs and the tissues. review of medical physiology. 22nd ed. new york; mc graw-hill, 2005: 666-669 12. guyton & hall. effect of hemoglobin to ‘buffer’ the tissue po2. text book of medical physiology. 11th ed. philadelphia; saunders, 2006: 507-508. 13. fernando mateos, jeremy h brock, josé luis pérez-arellanoa. iron metabolism in the lower respiratory tract. thorax. 1998; 53:594-600. final nepas journal 30-1.indd -37-journal of nepal paediatric society january-june, 2010/vol 30/issue 1 original article january-june, 2010/vol 30/issue 1 single stage anterior sagittal anorectoplasty (asarp) for anorectal malformations with vestibular fistula and perineal ectopic anus in females: a new approach chaudhary rp1, thapa b2, thana s3, singh pb4 1dr. ramananda prasad chaudhary, mbbs.ms. consultant pediatric surgeon, 2dr. bijay thapa, 3dr. santosh thana, 4dr. pradeep b. singh. medical officers, kanti children’s hospital, kathmandu, nepal. address for correspondence: dr. rp chaudhary, e-mail: drchaudharyrp@hotmail.com abstract introduction: despite a better understanding of the embryology, anatomy of anorectal malformations and of the physiology of continence, the management of children born with imperforate anus continues to be a surgical challenge and is still fraught with numerous complications and often leads to less than perfect qualitative results. pediatric patients with recto-vestibular fistula have good prognoses in terms of bowel function when properly treated. aim & objective: the study was designed to assess the surgical morbidity of single stage anterior sagittal anorectoplasty (asarp). methodology: this prospective study was carried for a period of 26 months. it included a total of 48 female patients (aged 0 – 14 years) with diagnosis of anorectal malformations (arm) with vestibular fistula or perineal ectopic anus. in asarp, patient in lithotomy position, the anterior portion of sphincter muscles were cut through a midline perineal skin incision, rectum was separated from the vagina & then rectum was pulled through the center of these muscles. the perineal body was reconstructed and the normal appearance of perineum was achieved. results: short-term surgical outcome was satisfactory in all cases. no one needed colostomy. conclusion: single-stage asarp is a good approach in experience hands for arm with vestibular fistula and perineal ectopic anus in females and thereby complications and time involved in staged procedures including colostomy can be avoided. key words: anterior sagittal anorectoplasty, anorectal malformations, perineal ectopic anus, vestibular fistula. introduction vestibular fi stula and perineal ectopic anus are the most common anorectal malformations in female children1. imperforate anus without a fi stula is a rather unusual defect2. despite a better understanding of the embryology, anatomy of anorectal malformations and of the physiology of continence, the management of children born with imperforate anus continues to be a surgical challenge and is still fraught with numerous complications and often leads to less than perfect qualitative results3. pediatric patients with rectovestibular fi stula have good prognoses in terms of bowel function when properly treated. the bowel opens immediately behind the hymen in the vestibule of the female genitalia. immediately above the fi stula, rectum and vagina are separated by a thin common wall. these patients usually have well-developed muscles and a normal sacrum and nerves. a meticulous inspection of the newborn genitalia is needed for the diagnosis2. the conventional surgical treatment has not always been satisfactory. the previously used surgical techniques include cutback, perineal anal transplant, yv plasty, sacroperineal repair, and colostomy followed by minimal posterior sagittal anorectoplasty. these procedures have been limited by incomplete anatomic exposure, blind tunneling of the rectum, lack of -38-january-june, 2010/vol 30/issue 1 journal of nepal paediatric society reconstruction of the perineal body, need for a colostomy and a displeasing appearance of the perineum, with anterior migration of the anus in the long term. these limitations have been offset by asarp1, 3. the optimal surgical repair should allow easy access to the fi stula, minimize the dissection to spare pelvic nerves, guide the rectal pouch through all the muscles of continence, restore a physiological anorectal angle, create good anal opening and take advantage of all existing structures. most contemporary techniques attempt to preserve the major elements of continence, often at the cost of “lesser” factors, increased dissection or poorer exposure4. de vries and pena in 1982 reported posterior sagittal anorectoplasty as an operative procedure for high or intermediate imperforate anus. by the reference to the idea of this operative procedure, okada et al devised a new approach, anterior sagittal anorectoplasty (asarp) for repair of a vestibular fi stula, in which, in the lithotomy position, sphincter muscles are cut from the anterior aspect longitudinally through a median perineal skin incision and then the rectum is passed through the central portion of the sphincter muscle5. a number of pediatric surgeons repair this defect, primarily without a protective colostomy 6. methodology in this prospective study, primary asarp was performed from september 2004 to march 2009 (55 months) on 48 patients of anoretal malformations with vestibular fi stula or perineal ectopic anus in kanti children’s hospital and ishan children’s nursing and maternity home (table 1) their age range was from neonates (3 days) to fi ve years old children (table 2). table 1: showing the diagnosis of patients. diagnosis no. of patients vestibular fistula 44 perineal ectopic anus 4 all patients who underwent asarp were included in this study. patients with anorectal malformations with colostomy, with prematurity, with septicemia, with birth asphyxia and patients with major defects making them unfi t for ga were excluded from the study. table 2: showing the age distribution of patients. age no. of patients neonates 15 1 -12 months 25 >1 year 8 surgical anatomy: in normal individual, the voluntary striated muscle structures responsible for fecal control are represented by funnel-like muscle structure. anatomically, the levator ani (also called the pelvic diaphragm) is arbitrary divided into three striated muscle groups: ileococcygeus, pubococcygeus and puborectalis. functionally & anatomically, the puborectalis is therefore more likely linked to the external anal sphincter as a part of the striated muscle complex7. often acting as a single unit,they are sometimes referred to as the striated muscle complex8. apart from this, the superfi cial and deep transverse perinei muscle muscle fuse in the midline to form perineal body along with the anterior most fi bers of the external sphincter. in patients with a vestibular fi stula, the development of the sphincteric muscle is normal and the ‘anus’ or ‘fi stula’ opens into the vestibule between the hymen and the fourchette, surrounded by moist mucous membrane rather dry skin9. in cases of perineal ectopic anus, only the postetior aspect of the anorectum is enclosed by the vertical muscle complex. they attributed the constipation seen with this defect to a ‘shelf’ effectof the ‘levator ani, or pelvic fl oor behind the abnormal anterior opening10. operative technique: patients were investigated to fi nd out any associated anomalies. pre-operative preparations; they were treated for constipation with laxatives (lactulose and fi stula irrigation with normal saline). they were kept nothing per oral for 48 hours in infants and older children and 24 hours in neonates. oral antibiotics (erythromycin 50 mg /kg and metronidazole 20 mg /kg in divided doses) were started 48 hours before surgery. the operation was performed under general anaesthesia along with caudal block. proposed anal site was determined by the anal mark and confi rmed by the use of an electrostimulator. with patient in lithotomy position, a foley catheter was passed through urethra into the bladder before surgery. pre-operative calculated doses of i.v. antibiotics (cephalosposin, gentamicin & metronidazole) were given with the induction of anaesthesia. a circular incision was made in the mucocutaneous junction at the opening of the fi stula and extended posteriorly along the median line to reach the anal dimple. several fi ne silk traction sutures were inserted around the fi stula orifi ce. through the incision, the fi stula (anorectum) was fi rst dissected free bluntly little by little from surrounding tissues with meticulous care being taken not to cause damage to the musculature enclosing the rectum. the rectum was separated from the posterior wall of the vagina by sharp dissection; side by side retrorectal dissection is also done. the length of -39-journal of nepal paediatric society january-june, 2010/vol 30/issue 1 the rectal tube to be dissected was 4 to 5cm. bleeding vessels were controlled by electrocoagulation. the fi rst muscle to come in sight was the superfi cial external sphincter muscle; next to come was the deep external sphincter muscle. the latter muscle soon joined the longitudinal muscles that spread out in a fan-shaped pattern. behind these thin muscles lied puborectalis muscle, enclosing the rectum and was defi nitely palpable. posterior part of those fan-shaped longitudinal muscle fi bers and puborectalis muscle was carefully preserved. next, the dissected rectal tube was mobilized backward to be placed at the center of the fan-shaped muscles. anterior ends of the fan shaped muscle fi bers were apposed and sutured by interrupted suture with several stitches of 4-0 vicryl suture beginning from the lower depths upward and outward. the rectum was fi xed to the deep external sphincter muscles over its entire circumference by 4-0 interrupted stitches. sutures were placed between the rectal stump and surrounding skin by about 12 -16 stitches of 4-0 vicryl (anoplasty). the neoanus should allow for the initial passage of at least number 10, hegar dilator in neonates. then, subcutaneous adipose and connective tissues extending up to the vaginal wall were approximated and sutured with interrupted 4-0 vicryl. the operation ended with closure of the surgical wound by suturing the perineal skin intradermally, with resultant creation of the perineal body. post-operatively, patients were managed by – analgesic for infants was paracetamol drop (15 mg / kg / dose) and that for others was intramuscular pethidine (11.5 kg/dose) 6 hourly. foley catheter was kept in situ up to 5th postoperative days for better perineal care. aqeuous povidone iodine solution was applied over operated wound and neoanus several times a day. patient was kept nothing per oral for 4 days. oral feeding was started on 5th pod. catheter was removed on 5th post operative day. intravenous antibiotics were continued up to 5th postoperative days, followed by oral cephalosporin for 2 more days. majority of the patients were discharged within 6 to 8 days of surgery unless indicated for some reasons. parents were informed about the planned schedule of follow up and anal dilatation was started on 14th post operative day continued up to approximately 8 months as per pena’s schedule12. the parents were demonstrated how to perform dilatation at home. fig 1: showing arm with vestibular fistula. fig 2: showing arm with perineal ectopic anus fig. 3: detailed normal anorectal anatomy, coronal view (pena 1990). fig. 4: detailed normal anorectal anatomy, sagittal view (pena 1990). -40-january-june, 2010/vol 30/issue 1 journal of nepal paediatric society fig. 8: clinical presentations of the patients results anterior sagittal anorectoplasty was performed on 32 female patients for anorectal malformations with vestibular fi stula or perineal ectopic anus from november, 2002 through june, 2004. during perineal examinations following fi ndings were noted: type of fi stula, look of perineum, anal dimple and mark, anal pigmentation, midline groove, sacrum and perineal muscle contraction. 16 (33.33 %) patients had associated anomalies. in all cases, operation was performed following the forementioned procedures after confi rming the site of fig. 5: vestibular fistula (pena 1995). fig. 6: female perineal anus (pena 1995). fig. 7: operative technique: anterior sagittal anorectoplasty (okada et al 1992). anus by nerve stimulator. one patient who developed post operative complication of perianal excoriation, recovered well after 2 weeks of surgery. on assessment of results (table 4), the site, size and appearance of the neoanus was normal in all cases. the frequency of bowel movement was 1-2 times in 40 patients and 2-3 times in 8 patients. the consistency of fecal matter was normal (formed or semisolid) in all 48 cases. none of the patients in this study, suffered from constipation and soiling. all the parents of children were satisfi ed with the outcome. follow up period was minimum of 6 months to 55 months. accordingly, the patient visited the hospital as follows: 1st follow up at 14 days of surgery, 2nd follow up at 1 month of surgery and 3rd follow up at 3 months of surgery. during each visit, following points were noted: i) neoanual size, site and appearance. ii) perineal wound. iii) voluntary bowel movements. iv) fecal incontinence. v) constipation. vi) any complications. 0 10 20 30 40 50 abscess of an anal opening at normal site 48 constipation 8 passage of meconium/ stool through an ectopic opening 48 -41-journal of nepal paediatric society january-june, 2010/vol 30/issue 1 table 3: associated anomalies associated anomalies number of patients urogenital defects hydronephrosis (3 cases), ), indirect inguinal hernia (2 cases) dysplastic kidney(1 case) and renal agenesis (1 case) vur (1 case) 8 (16.66%) cardiac defects vsd (3 cases), tof with complex anomaly (1 case) 4 (8.33%) skeletal defects polydactyly (2 cases), rudimentary index fi nger (1 case) 3 (6.25%) others talipes equino-varus (1 case) 1(3.13%) table 4: showing the results of asarp neoanus appearance round 48 (100%) oval site correct 48 (100%) incorrect size adequate 48 (100%) stenosis frequency of defaecation (per day) 1-2 times 40 (83.33%) 2-3 times 8 (16.66%) consistency normal 48 (100%) loose constipation absent 348(100%) present soiling absent 48 (100%) present neoanal retraction absent 48 (100%) present parental reaction satisfi ed 48 (100%) unsatisfi ed discussion the technique described herein has several advantages over the posterior sagittal approach and other anterior perineal techniques. conceptually, continence is dependent on the integrated function of the puborectalis, the internal and external sphincters, normal sensation of rectal fullness, and normal discrimination by the anoderm13. this technique has the advantages of the anterior perineal approach popularizes by mollard14,15. previous operative procedures like cut back operation or its modifi cation ‘ v-y plasty’ for the repair of anovestibular fi stula, however have the disadvantage of the contamination of the vagina and urethra with consequent vaginitis and urethritis often results and soiling or staining due to mucosal involvement may occur at times16,17. the limitations like incomplete anatomic exposure, blind tunneling of the rectum, lack of reconstruction of the perineal body, need for a colostomy and a displeasing appearance of the perineum, with anterior migration of the anus in the long term have been offset by asarp; colostomy is obviated, can be performed even in neonates, mobilizqtion of the rectum is visualized, only the anterior aspect of the sphincteric muscle complex is divided, continence mechanism is preserved (puborectalis muscle is preserved), there is minimal dissection, allows better anatomic exposure for separation of rectum from vagina, sphincter muscles and the perineal body are accurately reconstituted, easytoperform approach of salient repairing effi cacy. the extensive preoperative and postoperative measures advocated by okada et al 18 weren’t needed in this study. chatterjee advocated use of a colostomy if the patient is over 5 years of age or in cases of mega rectum or a small fi stula that prevents bowel preparation19. but no colostomy was performed in these cases too and there weren’t any serious complications. none of the 2 (6.25%) fig. 9: showing the perinium after asarp -42-january-june, 2010/vol 30/issue 1 journal of nepal paediatric society patients who suffered peroperative complications of vaginal wall tear developed further complications. one patient who suffered post-operative complication (of perianal skin excoriation improved with local application of zinc oxide paste. in this series complications were less than wakhlu et al (1996) series (11.53%) but higher than okada et al (1992) & okada et al (1993) series where, in all cases, the postoperative course was uneventful without any wound infection or laceration and their bowel habits & anorectal discharge control were nearly satisfactory in all the infants. short-term surgical outcome was acceptable in all 48 cases and all patients had normal size, round shaped neoanus at normal site. there is evidence that the cortical intergration of somatosensory input from the anal skin may be lost after the third or fourth month of life20 if unused. this strongly supports early repair of high imperforate anus, enabling the development of normal stooling patterns at the appropriate time. when an anterior sagittal incision is used in a neonate, the preoperative differentiation between low, intermediate and high types becomes less necessary21. colostomy or not, the important thing is to establish normal stooling as soon as possible. the modern trend is to operate on newborns with anorectal malformations, primarily without a protective colostomy. even more ambitious and bold is the tendency to repair these babies primarily without a protective colostomy and laparoscopically11. i believe that this technique makes maximal use of native tissues, with minimal dissection and optimal reconstruction of this deformity. this study demonstrates that single stage asarp can be performed with acceptable surgical outcome in cases of arm with vestibular fi stula or perineal ectopic anus in female. i would like to follow up these patients to see the longterm outcome of the approach. conclusion outcome of single-stage anterior sagittal anorectoplasty is good and is an acceptable approach for arm with vestibular fi stula or perineal ectopic anus in female and thereby complications and time involved in staged procedure including colostomy can be avoided. acknowledgement: none funding: nil confl ict of interest: none references 1. wakhlu a, pandey a, prasad a, kureel sn, tandon rk, wakhlu ak. anterior sagittal anorectoplasty for aorectal malformatios and perineal trauma in the female child. j pediatr surg.1996; 31:12361240. 2. pena a. imperforate anus and cloacal malformations in pediatric surgery, 3rd. edn, ed. k.w.ashcraft, w.b.saunders company, philadelphia, 2000: 473492. 3. smith ed. the bath water needs changing, but don’t throw out the baby: an overview of anorectal anomalies. j. pediatr surg. 1987; 22: 335-348. 4. yazbeck s, luks fi, st-vil d. anterior perineal approach and three-flap anorectoplasty for imperforate anus: optimal reconstruction with minimal destruction. j. pediatr surg. 1992; 27: 190-195. 5. okada a, shinkichi k, imura k, fukuzawa m, kubota a, yagi m, azuma t, tsuji h. anterior sagittal anorectoplasty for rectovestibular and anovestibular fistula. j.pediatr surg. 1992; 27: 85-88. 6. moore tc. advantages of performing the sagittal anoplasty operation for imperforate anus at birth. j. pediatr surg. 1990; 25: 276-277. 7. stafford, p.e.w. other disorders of the anus and rectum, anorectal function in pediatric surgery, 5th. edn, vol-2, eds. j.a. o’neill,jr., m.i. rowe, j.l.grosfeld, e.w.fonkalsrud, & a.g.coran, mosby-year book, st.louis, 1998; pp. 1449-1460. 8. weinberg g, boley sc. anorectal continence and management of constipation in pediatric surgery, 3rd. edn, ed. k.w. ashcraft, w.b.saunders company, philadelphia, 2000; pp.502-510. 9. freeman nv. anorectal malformations’, in surgery of the newborn, eds. n.v.freeman, d.m. burge, m.griffi ths and p.s.j.maline, churchill livingstone, edinburgh, 1994; pp. 171-199. 10. de vries pa. complications of surgery for congenital anomalies of the anorectum in complications of pediatric surgery, eds. p.a.devries, s.r.shapiro, john wiley & sons, new york, 1982; pp. 233-262. 11. pena a., ‘an atlas of surgical management of anorectal malformations’, spring-verlag, newyork, 1990 pp. 195. -43-journal of nepal paediatric society january-june, 2010/vol 30/issue 1 17. nixon hh. review of anorectal anomalies. j r soc med, 1984(suppl); 77:27-29. 18. okada a, tamada h, tsuji h, azuma t, yagi m, kubota a, kamata s. anterior sagittal anorectoplasty as a redo operation for imperforate anus. j.pediatr surg. 1993; 28: 933-938. 19. chaterjee sk. lesions in the wingspread list management in the neonatal period, in chaterjee sk ed: anorectal malformations – a surgeons experience, oxford university, new delhi, india 1991 pp 48-64. 20. freeman nv, burge dm, soar js et al. anal evoked potentials. z kinderchir, 1980; 31:22-30. 21. sigalet dl, laberge jm, adolph vr, guttman fr. the anterior sagittal approach for high imperforate anus: a simplifi cation of the mollard approach. j. pediatr surg. 1996; 31:625-629. 12. pena a. ‘anorectal anomalies’, in newborn surgery, 2nd. edn, ed. p. puri, arnold, london, 2003; pp. 535-552. 13. holschneider am, freeman nv. anatomy and function of the normal rectum and anus, in stephen fd, smith ed, paul nw eds. anorectal malformations in children: update 1988, ny liss, newyork,1988 pp. 125-154. 14. mollard p, marechal jm, de beaujeu mj. surgical treatment of high imperforate anus with defi nition of the puborectalis sling by an anterior perineal approach. j. pediatr surg. 1978; 13: 499-504. 15. mollard p, soucy p, louis d et al. preservation of infralevator structures in imperforate anus repair. j pediatr surg, 1989; 27: 185-189. 16. ishihara m, morita k. techniques and indication of the cut back procedure and potts method for low deformities. jpn j pediatr surg. 1981; 13: 11991204. j nepal paediatr soc | vol 42 | issue 02 |may-aug, 2022 61 original articlebreastfeeding motivation and exclusive breastfeedingoriginal article doi: 103126/jnps.v4113 ashish jain1, sudharshan raj chitgupikar2, madhavi bhardwaraj1, preethi subramanian2 1department of of neonatology, maulana azad medical college and associated lok nayak hospital, bahadur shah zafar marg, new delhi, india 2department of of paediatrics, mediciti institute of medical sciences, medchal mandal, ghanpur, hyderabad, india nasal anthropometry among term and preterm indian neonatesdoes size matter? introduction: the binasal prongs are used in neonatal intensive care unit (nicu) to provide nasal continuous positive airway pressure (n-cpap) in preterm neonates. a variable degree of nasal trauma is the most common complication of n-cpap therapy. this can be reduced by use of appropriately sized binasal prongs that are customized to the anatomical measurements of noses. the aim was to estimate the nasal two-dimensional anthropometrical parameters among indian neonates across different gestations; to compare between both sexes and between neonates with different intra uterine growth status and gestational ages. methods: hundred and one neonates across different gestational ages (24 – 42 weeks) and intrauterine growth status who were admitted to the nicu of a tertiary teaching hospital were enrolled and grouped into five categories based on their gestational ages viz. 37 – 42 weeks, 34 – 36 weeks, 31 – 33 weeks, 28 – 30 weeks, and 24 – 27 weeks. seven nasal and para-nasal measurements were taken (nostril width, nostril length, columella width, columella length, nose width, nose length and philtrum length) for each neonate using a vernier caliper. results: all anthropometrical measurements differed significantly across gestations especially between 37 42 and 24 27 wks (p = 0.00). males differed from females only with respect to nostril width (p = 0.032). the measurements varied significantly when compared among small for gestational age, appropriate for gestational age and large for gestational age neonates. conclusions: the nasal parameters differed significantly among various ga and intra-uterine growth statuses, which should help design appropriate sizes of bi-nasal prongs. abstract *corresponding author ashish jain associate professor, department of of neonatology, maulana azad medical college and associated lok nayak hospital, bahadur shah zafar marg, new delhi, india email: neoashish2008@gmail.com article history received on : 05 apr, 2022 accepted on : 11 dec, 2022 funding sources: none conflict of interest: none keywords: inappropriately sized nasal prongs; nasal parameters; nasal trauma online access doi: https://doi.org/10.3126/jnps.v42i2.44302 introduction it is a well-known fact that respiratory distress syndrome (rds) affects a significant proportion of preterm neonates1 and is one of the major causes of neonatal mortality. hence, the recent neonatal resuscitation program (nrp) guidelines recommend a delivery room respiratory support in the form of nasal continuous positive airway pressure (n-cpap) which can provide a positive end-expiratory pressure of around 5 cm h2o, for these vulnerable preterm babies. 2,3 the use of n-cpap in these babies has been reported to lead to earlier transition in the delivery room area, lower mortality, lower rates of intraventricular hemorrhage and bronchopulmonary dysplasia as copyrights & licensing © 2022 by author(s). this is an open access article distributed under creative commons attribution license (cc by nc ) j nepal paediatr soc | vol 42 | issue 02 |may-aug, 202262 original article nasal anthropometry of indian neonates compared to use of mechanical ventilation directly.4 various cpap generators as well as nasal interfaces, have been innovated and improvised in the past. diverse interface devices like nasopharyngeal tube, single nasal prongs, bi-nasal prongs, nasal cannula have been used to deliver n-cpap in neonates however, each of these have their own merits and limitations. short bi-nasal prongs are commonly used in recent times and have shown to have better respiratory outcomes in neonates with rds as compared to use of nasopharyngeal or single nasal prongs.5 the major and the most common complication of n-cpap therapy in preterm neonates is the variable degree of nasal trauma.6,7 even though there is no recognized classification specifically addressing to the nasal trauma secondary to n-cpap in neonates, the us national pressure ulcer advisory panel8,9 guidelines for staging have been often used in most of the neonatal studies. this classification has attempted to grade nasal injury from early stage of non-blanchable erythema with intact skin to an advanced stage of necrosis, with full thickness skin loss. in a study using the above classification of nasal trauma in neonates on n-cpap, 42.5% of neonates developed nasal trauma. 88.3% had stage i nasal trauma, 11% stage ii trauma and 0.7% had stage iii trauma.9 a similar incidence (20 60%) with nasal septum being the commonest site for injury was reported by another study.10 fischer et al, in a large cohort of preterm with different gestational ages and weights, reported an increase in the relative risk of nasal injury when the gestational age was < 32 weeks, birth weight < 1500 grams, n-cpap duration < 5 days and neonatal intensive care unit (nicu) stay was < 14 days.9 the higher incidence of nasal trauma in smaller babies is most of the times attributed to the soft, fragile skin, ischemia prone underlying tissue and more importantly the ill-fitting, inappropriately sized binasal prongs.6 the number of the injuries and their severity can be reduced if an appropriate size binasal prongs are used, that are customized to the anatomical measurements of the noses. even though it is undoubtedly an important prerequisite to have the nasal sizes for development of the safe nasal interface sizes, there are few studies that look at the nasal anatomical parameters in indian neonates of different gestations. most of the prongs that are used in the indian setup are based on the studies and sizes of the western babies. our study aims to assess nasal parameters in neonates of different gestations for development of appropriate size binasal prongs. methods this observational study was performed in the neonatology department of a tertiary care and teaching hospital of north india between january 2016 and april 2016. the study was approved by the ethical committee of the institution. all the procedures followed were in accordance with the ethical standards of the responsible committee on human experimentation (institutional or regional) and with the helsinki declaration of 1964, as revised in 2000. all neonates born (live or stillbirth) were enrolled in the study within 72 hours of birth. the neonates who had any congenital malformation or facial dysmorphism of the nasal or para-nasal area were excluded from the study. it was pre-decided to measure at least five babies (male or female) in each gestational category for appropriate representation in each group. the gestational categories in which the measurements were taken were 24+1 27 completed weeks; 28+1 30 completed weeks; 31+1 33 completed weeks; 34+1 36 completed weeks and 37+1 42 completed weeks. a gestational assessment was done within 72 hours of birth using the new expanded ballard score.11 based on ghosh et al,12 who reported the mean width of columella of term neonates as 2.5 mm with sd of 0.1, we calculated the sample size with a precision of 0.2 mm and confidence interval of 95% which came to 100 neonates. candidates were enrolled after taking due informed consent from the parents or the primary caregiver. baseline information including age in completed days, sex, and birth weight, appropriateness of growth for the estimated gestational age and date of birth were recorded. a vernier caliper with a least count of 0.1 mm was used for measuring the various parameters. for this study, all the measurements were made by a vernier caliper. the measurements were done using aseptic technique by an experienced paediatrician (figures 1 & 2). special care was taken to perform the same with all asepsis when the baby was quiet and sleeping. the mother was always with the baby when the measurements were taken. each measurement was done at least thrice successively, and the mean of the measurements was finally recorded. seven nasal and paranasal parameters were measured (in mm), which were: (1) nasal length ; measurement from the base of the nose to the tip of the nose (2) nasal width (outer ala to ala distance) (3) nostril length (base of the nostril to the highest point) (4) nostril width (the horizontal measurement from one side to other) (5) columella length (the length from the base to the highest point on columella in the center) (6) columella width (this was taken at the base of the columella) (7) philtrum length (the length from the point of attachment of columella to the upper margin of the upper lip). figure 1. measuring nasal width using vernier caliper j nepal paediatr soc | vol 42 | issue 02 |may-aug, 2022 63 original articlenasal anthropometry of indian neonates figure 2. measuring columellar width using vernier caliper measurements were statistically analyzed and tabulated. arithmetic mean for each parameter in each group with standard deviations were calculated. one way analysis of variance (anova) was used for comparing mean values of different groups; post hoc tukey’s hsd (honestly significant difference) test was used to identify the significance of differences of mean among these groups and student t-test was used for comparing two groups with continuous variables. all the calculations / tests were performed with the help of spss 2.0. a p-value of < 0.05 was considered statistically significant. results 101 neonates were enrolled in the study from ga of 24 weeks till 42 weeks. among the study group, 50.5% (51 / 101) were males. 23.76% (24 / 101) neonates were extremely low birth weight (elbw), 24.75% (25 / 101) were very low birth weight (vlbw) and 28.71% (29 / 101) were low birth weight (lbw). among all, 72.28% (73/101) were preterms. the comparison of nasal measurements among different gestational age groups using one way anova is shown in table 1. on comparison of the five gestational groups, the estimated p values were highly significant for all the parameters. post hoc analysis for nostril width revealed significant difference between 37 42 weeks group and 28 30 weeks group (p = 0.0052) and between 37 42 weeks and 24 27 weeks group (p = 0.008). for nostril length post hoc analysis revealed significance only for difference in measurements between 37 42 weeks and 24 27 weeks (p = 0.0273). with respect to columella width, the difference was significant when compared among the groups: 37 42 weeks vs 28 30 weeks (p = 0.0000); 37 42 weeks vs 24 27 weeks (p = 0.0000); 34 36 weeks vs 28 30 weeks (p = 0.0272); 34 36 weeks vs 24 27 weeks (p = 0.0001) and 31 33 weeks vs 24 27 weeks (p = 0.0101). for columella length post hoc analysis revealed significant difference for 37 42 weeks vs 34 36 weeks (p = 0.0057); 37 42 weeks vs 31 33 weeks (p = 0.0062); 37 42 weeks vs 28 30 weeks (p = 0.0005); 37 42 weeks vs 24 27 weeks (p = 0.0000) and 31 33 weeks vs 24 27 weeks (p = 0.0442). for nose width the difference among groups were significant except for 34 36 weeks vs 31 33 weeks and 28 30 weeks vs 24 27 weeks. for nose length, post hoc analysis revealed significant difference among all groups except 34 36 weeks vs 31 33 weeks and 28 – 30 weeks vs 24 27 weeks. philtrum length showed significant difference only between 37 42 weeks vs 28 30 weeks (p = 0.0343) and 37 42 weeks vs 24 – 27 weeks (p = 0.0239). table 1. comparison of parameters among different gestational groups [mean (mm) ± s.d.] gestational age (wks) n (%) nostril width nostril length columella width columella length nose width nose length philtrum length 42 – 37 n = 28 (27.72) 1.77 ± 5.68 0 ± 3.77 0.35 ± 4.11 1.77 ± 8.02 1.06 ± 20.71 1.77 ± 17.36 2.12 ± 7.89 36 – 34 n = 21 (20.79) 0 ± 4.74 1.06 ± 3.29 0.707 ± 3.67 0.707 ± 7 2.12 ± 18.14 1.41 ± 15.45 0 ± 7.52 31 – 33 n = 22 (21.78) 2.47 ± 4.77 1.06 ± 3.68 0.70 ± 3.36 0 ± 7.02 1.41 ± 16.84 1.06 ± 14.45 1.06 ± 7.52 30 – 28 n = 23 (22.78) 0.35 ± 4.17 0.71 ± 3.6 0 ± 3.22 0 ± 6.83 2.12 ± 15.07 0.71 ± 13.22 0.35 ± 6.87 27 – 24 n = 7 (6.93) 0.71 ± 3.5 0 ± 2.79 0 ± 2.64 0 ± 5.79 0 ± 14.28 0.35 ± 12.07 0 ± 6.29 p-value (anova) 0.000 0.005 0.000 0.000 0.000 0.000 0.001 s.d. – standard deviation; anova: analysis of variance. the comparison of the parameters between both sexes using independent student t-test (table 2), did not show any significant difference except nostril width (p = 0.032). j nepal paediatr soc | vol 42 | issue 02 |may-aug, 202264 original article nasal anthropometry of indian neonates table 2. comparison of parameters between both sexes [mean (mm) ± sds sex nostril width nostril length columella width columella length nose width nose length philtrum length male (n = 51) 0.941 ± 4.99 0.636 ± 3.66 0.668 ± 3.56 0.997 ± 7.23 2.690 ± 17.62 1.992 ± 15.13 1.202 ± 7.53 female (n = 50) 0.913 ± 4.59 0.776 ± 3.43 0.523 ± 3.54 0.821 ± 7.10 2.637 ± 17.59 2.274 ± 14.91 1.051 ± 7.25 p-value (independent t test) 0.032 0.111 0.875 0.492 0.959 0.610 0.217 table 3 depicts the comparison of nasal parameters among neonates with different growth status using one way anova. on comparison of the three groups, the p values for nose width (0.000), columella width (0.01) and philtrum length (0.004) were found to be significant. post hoc analysis revealed significant difference between aga and sga (p=0.0193) for columella width; between aga vs lga (p = 0.0004) and sga vs lga (p = 0.0000) for nose width and between aga vs lga (p = 0.0030) and sga vs lga (p = 0.0051) for philtrum length. table 3. comparison of parameters among neonates with different growth status [mean (mm) ± s.d.] growth status n(%) nostril width nostril length columella width columella length nose width nose length philtrum length (aga) n = 71 (70.29) 0.996 ± 4.76 0.751 ± 3.58 0.609 ± 3.63 0.981 ± 7.22 2.617 ± 17.66 2.235 ± 15.06 0.904 ± 7.31 (sga) n = 25 (24.75) 0.777 ± 4.70 0.612 ± 3.40 0.523 ± 3.26 0.645 ± 6.90 1.947 ± 16.54 1.719 ± 14.54 1.061 ± 7.30 (lga) n = 5 (4.96) 0.447 ± 5.70 0.671 ± 3.70 0.274 ± 3.80 0.758 ± 7.70 0.822 ± 22.10 1.475 ± 16.90 2.761 ± 9.00 p-value (anova) 0.083 0.481 0.01 0.080 0.000 0.073 0.004 s.d. – standard deviation; anova: analysis of variance. discussion the present study is an attempt to determine the basal values for various nasal parameters in different gestational age groups of the local population of neonates belonging to the northwestern part of india. the study suggests that, as the gestation progresses, all the seven parameters assessed increase and values differ significantly between the gestation groups more so in the first and the last categories (37 42 weeks and below 28 weeks). js et al13published a cross sectional study in 2019 on discrepancy in physical dimensions of nose and cpap interface in 32 preterm neonates from 26+0 34+6 weeks gestation. the various anthropometric measurements were done using 3d photogrammetry. the measurements were observed to increase as weight of neonates’ weight increased. on comparison of male and female participants of the study, it was found that nostril width differs significantly between the two groups, being larger in males. this finding was comparable to the study done by khandekar et al,14 who reported mean nasal widths as 15.5 mm and 14.5 mm in male and female neonates respectively. they also found that mean columellar height in term neonates was 4.2 mm in male babies and 4.1 mm in females. mean columellar width was 4.5 mm in male neonates and 4.4 mm in females. no comparison of means was done to assess whether the two groups differ with respect to the parameters assessed. also, preterm babies were not a part of this study. our study found lesser mean columellar width but larger columellar length and nasal width values compared to their study. our study also noted no significant difference in measured parameters (except nostril width) between male and female babies. ghosh a et al12 studied craniofacial anthropometric measurements of 1860 term neonates (1060 normal birth weight and 800 low birth weight) of northeastern india and reported that there was significant difference in measurements of nose length, columella width, and length and width of philtrum between males and females with male neonates having higher measurements. but preterm neonates were not included in this study. our study did not find such statistically significant difference between the two sexes except for nostril width. this could probably be attributed to lesser sample size. in another study done by agnihotri et al15 for assessment of craniofacial anthropometry in infants and newborns, it was found that philtrum length differs in male and female neonates with p-value less than 0.05 while no such difference was found in the present study. preterm neonates were again not a part of this study. wong et al16 assessed comparability of 3d digital photogrammetric images and direct methods and found them to be comparable on validity and reliability for craniofacial assessment. a comparison among aga, sga and lga groups, showed that columella width, nose width and philtrum length differ significantly between the three groups being the largest in lga babies and the smallest in sga babies. these would be an important data looking at the proportion of the sga babies in the indian population, as the current interfaces available are mainly derived from aga preterm population. when we analyzed our data and applied post hoc (tukey’s hsd) after anova, we found significant difference in nostril width and length; columellar width and length in the categories of ga < 28 weeks; 31 33 and ≥ 37 weeks. the nomograms of the nasal and the paranasal parameters of the indian babies of different gestation would be vital data to develop the indigenous (indian), novel and customized nasal prongs for the indian babies, translating into lesser nasal injury and better j nepal paediatr soc | vol 42 | issue 02 |may-aug, 2022 65 original articlenasal anthropometry of indian neonates outcomes. the variation in the measurements between different gestation categories would be important to develop the minimum range of the nasal prongs that can fit most of the indian neonates, this minimum range would add to reduction in the running cost in the widely existent constraint settings. although our study is an important study for the nasal anthropometry among indian neonates, one major limitation is lesser number (n = 7) of extremely preterm infants available for enrollment. conclusions post-hoc hsd suggests the use of at-least three different sizes of nasal prongs in indian neonates (< 28 weeks; 31 33 and ≥ 37 weeks). larger and multicentric studies are required before this can be put into use for manufacturing customized nasal prongs for indian neonates. references 1. mishra kn, kumar p, gaurav p. aetiology and prevalence of respiratory distress in newborns delivered at dmch, darbhanga, bihar, india. j evolution med dent sci. 2020;9:3655-3659. doi: 10.14260/jemds/2020/802. 2. aziz k, lee hc, escobedo mb, hoover av, kamath-rayne bd, kapadia vs, et al. part 5: neonatal resuscitation: 2020 american heart association guidelines for cardiopulmonary resuscitation and emergency cardiovascular care. circulation 2020;142(16_suppl_2): s524-s550. doi: 10.1161/cir.0000000000000902. 3. neonatal resuscitation: india. 3rd ed. national neonatology forum of india; 2019. 4. morley cj, davis pg, doyle lw, brion lp, hascoet jm, carlin jb; coin trial investigators. nasal cpap or intubation at birth for very preterm infants. n engl j med. 2008;358: 700-8. doi: 10.1056/nejmoa072788. 5. de paoli ag, davis pg, faber b, morley cj. devices and pressure sources for administration of nasal continuous positive airway pressure (ncpap) in preterm neonates. cochrane database syst rev. 2002;(4):cd002977. d o i : 1 0 . 1 0 0 2 / 1 4 6 5 1 8 5 8 . c d 0 0 2 9 7 7 . update in: cochrane database syst rev. 2008;(1):cd002977. pmid: 12519580. 6. ribeiro dfc, barros fs, fernandes bl, nakato am, nohama p. nasal prongs: risks, injuries incidence and preventive approaches associated with their use in newborns. j multidiscip healthc. 2020;13:527-537. doi: 10.2147/jmdh.s252017 7. goel s, mondkar j, panchal h, hegde d, utture a, manerkar s. nasal mask versus nasal prongs for delivering nasal continuous positive airway pressure in preterm infants with respiratory distress: a randomized controlled trial. indian pediatr. 2015;52:1035-40. doi: 10.1007/s13312-015-0769-9. 8. buettiker v, hug mi, baenziger o, meyer c, frey b. advantages and disadvantages of different nasal cpap systems in newborns. intensive care med. 2004 may;30(5):926-30. doi: 10.1007/s00134-004-2267-8. pmid: 15042289. 9. fischer c, bertelle v, hohlfeld j, forcada-guex m, stadelmann-diaw c, tolsa jf. nasal trauma due to continuous positive airway pressure in neonates. arch dis child fetal neonatal ed. 2010 nov;95(6):f447-51. doi: 10.1136/adc.2009.179416. 10. yong sc, chen sj, boo ny. incidence of nasal trauma associated with nasal prong versus nasal mask during continuous positive airway pressure treatment in very low birthweight infants: a randomised control study. arch dis child fetal neonatal ed. 2005 nov;90(6):f480-3. doi: 10.1136/adc.2004.069351. 11. ballard jl, khoury jc, wedig k, wang l, eilers-walsman bl, lipp r. new ballard score, expanded to include extremely premature infants. j pediatr. 1991;119:417-23. doi: 10.1016/s0022-3476(05)82056-6. 12. ghosh a, manjari c, mahapatra s. the craniofacial anthropometric measurement in a population of normal newborns of kolkata. nepal j med sci. 2013; 2:125–129. doi:10.3126/njms.v2i2.8955. 13. dalal js, ajmera sk, prajapat k, sahoo t, yadav cp, rao mpv, et al. discrepancy in the physical dimensions of nose and continuous positive airway pressure (cpap) interface: a possible reason for high rates of nasal injury in indian neonates. bmj innov. 2020; 0:1-7. doi: 10.1136/bmjinnov-2019-000372. 14. khandekar b, srinivasan s, mokal n, thatte mr. anthropometric analysis of lip-nose complex in indian population. indian j plast surg. 2005;38:128-31. doi: https://doi.org/10.1055/s-0039-1699120 15. agnihotri g, singh d. craniofacial anthropometry in newborns and infants. iran j pediatr. 2007;17:332–8. 16. wong jy, oh ak, ohta e, hunt at, rogers gf, mulliken jb, et al. validity and reliability of craniofacial anthropometric measurement of 3d digital photogrammetric images. cleft palate craniofac j. 2008; 45:232-9. doi: 10.1597/06-175. https://doi.org/10.1055/s-0039-1699120 j nepal paediatr soc | vol 42 | issue 02 |may-aug, 202266 original article platelet volume as a marker of sepsisoriginal article doi: 103126/jnps.v4113 arvind kumar1, harmeet singh arora2, vikas marwah1, satish chandra mishra3, akhil k r1, dinesh kumar kalra4, subhash chandra shaw2 1department of respiratory and sleep medicine, army institute of cardio-thoracic science, pune-411040, maharashtra, india 2department of paediatrics, army hospital (research and referral), new delhi-110010, india 3department of cardiology, army institute of cardio-thoracic science, pune-411040, maharashtra, india 4department of pathology, command hospital, pune-411040, maharashtra, india pediatric severe acute respiratory syndrome coronavirus 2 (sars-cov-2): clinical characteristics and cycle threshold value (ct value) of reverse transcriptase polymerase chain reaction (rt-pcr) of nasopharyngeal samples introduction: we aimed to characterize epidemiological and clinical characteristics of children and adolescents with severe acute respiratory syndrome coronavirus 2 (sars-cov-2) infection, and to evaluate relationship of cycle threshold value (ct value) of reverse transcriptase polymerase chain reaction (rt-pcr) test (as surrogate marker of viral load) with patient age and severity of infection. methods: we retrospectively collected data of children and adolescents admitted in our center from april 2020 to july 2020 with positive rt-pcr test for sars-cov-2. results: total 62 children, with median (iqr) of age 96 (54 122) months and 39 adolescents with median (iqr) of age 19.5 (18.2 20) years were included. 56 (90%) children and 34 (87%) adolescents had history of sarscov-2 positive cases in their family. only nine (14%) children had associated risk factor for severe sars-cov-2 infection. fever was the commonest symptom which was present in 24 (39%) children and 16 (41%) adolescents. cough was present in 17 (27%) children and 10 (26%) adolescents. diarrhea was found in 14 (23%) children and three (8%) adolescents. ct values of rt-pcr test were similar in children and adolescence (p = 0.48). however, asymptomatic children had higher ct values than symptomatic children (p = 0.01). conclusions: majority of children have asymptomatic or mild sars-cov-2 infection with similar ct values in children and adolescents. abstract *corresponding author subhash chandra shaw professor (paediatrics) and neonatologist, department of paediatrics, army hospital (research and referral), new delhi-110010, india. email: drscshaw77@gmail.com article history received on : 20 dec, 2021 accepted on : 09 dec, 2022 funding sources: none conflict of interest: none keywords: covid-19; children; cycle threshold value; rt-pcr test; vaccination online access doi: https://doi.org/10.3126/jnps.v42i2.41521 introduction it has been more than two years since outbreak of corona virus disease 2019 (covid-19) caused by severe acute respiratory syndrome corona virus 2 (sarscov-2).1 globally studies have shown that primary and secondary attacks rate of sars-cov-2 in children are same as in adults population but children were rarely index cases in household, and adult age group patients are often the index cases in household who had travelled or moved out in public places.2 copyrights & licensing © 2022 by author(s). this is an open access article distributed under creative commons attribution license (cc by nc ) j nepal paediatr soc | vol 42 | issue 02 |may-aug, 2022 67 original articleclinical characteristics and ct value of rt-pcr in paediatric sars-cov-2 there are limited studies, as such from india, about epidemiology, clinical manifestations and risk of infectivity of sars-cov-2 in different age groups of children. moreover, there has also been association between viral load and risk of transmission, as seen in other similar respiratory diseases.3-5 there is uncertainty about the extent to which children can be source of infection as indian adult citizens are being immunized, with the largest vaccination program of the world.6 hence, we aimed to study the epidemiology, clinical characteristics and viral loads in nasopharyngeal swabs as a surrogate marker of infectivity in different age groups of children. we assessed viral loads by measuring cycle threshold value (ct value) of reverse transcriptase polymerase chain reaction (rt-pcr) test for sarscov-2 in nasopharyngeal sample of covid-19 patients. the ct value is the number of replications cycle required for rt-pct test to become positive. methods we did retrospective review of 101 patients’ (children between one month to 15 years and adolescents between 15 years to 20 years) admitted from 01 april 2020 to 31 july 2020 in a dedicated tertiary care covid-19 center in maharashtra, india. the study was approved by the institute ethics committee. covid-19 case was identified by detection of nucleic acid of sars-cov-2 in nasopharyngeal swab using rt-pcr test. details of each admitted cases including demographic data, contact and travel history, vaccination history, living condition, clinical manifestation, co-morbid condition, treatment received and outcome were entered in pre-designed proforma. children were classified as asymptomatic infection who had positive rt-pcr test without any clinical features, upper respiratory tract infection when there was fever, cough, nasal congestion, etc without signs of pneumonia in chest radiology, mild covid-19 pneumonia with respiratory symptoms and radiological evidence whereas severe covid-19 pneumonia was defined in presence of fast breathing, hypoxemia (spo2 < 90%), altered sensorium, feeding difficulty. 7 critical infection had any one of following: respiratory failure, shock or multiple organ failure.8 sample for rt-pcr were taken from nasopharyngeal swab with allplextm 2019-ncov assay by seegene inc. ct value represents the number of replication cycles required for detection of virus. it is inversely correlated to amount of viral nucleic acid in sample. sample was considered positive when ct value of all genes were less than 40 cycles. in case of repeat rt-pcr testing, we included only initial rt-pcr test ct value for this study. all data were analyzed by using stata / ic statistical software version 16.0 (statacorp). the quantitative variables of different groups were measured in median with interquartile ranges and compared using krushal-wallis test, whereas all categorical data were analyzed by chi 2 test. we used spearman correlation test to evaluate relationship between two variables. a p value of < 0.05 was considered statistically significant. results a total of 101 patients were enrolled in this study. the demographic, clinical characteristics and ct value and clinical severity have been depicted respectively in tables 1, 2 and 3 and figure 1. table 1. clinical characteristics of the study population variable children less than 15 years of age (n = 62 ) adolescents age (< 15 years to 20 years ), n = 39 p value presence of risk factor, n (%) 9 (7+2) (14%) 4 (10%) p = 0.54 fever, n (%) 24 (39%) 16 (41%) p = 0.81 cough, n (%) 17 (27%) 10 (26%) p = 0.84 running nose, n (%) 10 (16%) 6 (15%) p = 0.92 sore throat, n (%) 11 (18%) 9 (23%) p = 0.51 body ache, n (%) 4 (6%) 6 (15%) p = 0.15 loss of appetite, n (%) 3 (5%) 5 (13%) p = 0.25 diarrhea, n (%) 14 (23 %) 3 (8%) p = 0.04 loss of smell, n (%) 8 (13%) 6 (15%) p = 0.62 loss of taste, n (%) 4 (6%) 5 (13%) p = 0.26 symptoms category asymptomatic, n (%) 34 (55%) 28 (45%) 0 22 (57%) 15 (38%) 2 (5%) p = 0.17mild, n (%) moderate, n (%) j nepal paediatr soc | vol 42 | issue 02 |may-aug, 202268 original article clinical characteristics and ct value of rt-pcr in paediatric sars-cov-2 table 2. demographic characteristics of the study population children less than 15 years of age (n = 62 ) adolescents age (< 15 years to 20 years ), (n = 39) p value bmi (kg / m2), median (iqr) 15.6 (14.0, 17.7) 20.2 (19.1, 21.2) p = 0.001 ct value, median (iqr) 25.5 (22, 30) 24 (22, 28) p = 0.59 male, n (% ) 34 (55%) 20 (51%) p = 0.75 residence rural, n (%) 18 (29%) 3 (8%) p = 0.01 urban, n (%) 44 (71%) 36 (92%) father occupation govt. sec., n (%) 47 (76%) 22 (56%) p = 0.002 agriculture, n (%) 10 (16%) 3 (8%) private sec., n (%) 5 (8%) 14 (36%) (size of family (numbers three , n (%) 12 (19%) 36 (58%) 8 (13%) 2 (3%) 12 (31%) 16 (41%) 9 (23%) 2 (5%) p = 0.21 four, n (%) five, n (%) six, n (%) vaccination received bcg + mmr, n (%) 40 (65%) 11 (18%) 4 (6%) 7 (11%) 34 (88%) 1 (2%) 1 (2%) 3 (8%) p = 0.06 bcg+mmr+ pneumococcal, n (%) bcg+mmr+ pneumococcal+ influenza, n (%) only bcg , n (% ) travel history present, n (%) 4 (6%) 4 (10%) p = 0.40 family member infected, n (%) 56 (90%) 34 (87%) p = 0.30 infected member in family father alone, n (%) 22 (35%) 4 (6%) 12(19%) 16 (26%) 7 (11%) 21 (54%) 4 (10%) 7 (18%) 7 (18%) 0 p = 0.15 mother alone, n (%) sibling alone, n (%) both parent, n (%) all family members, n (%) bmi-body mass index, ct value – cycle threshold value, bcgbacille calmette-guérin, mmr-measles, mumps, rubella. table 3. severity of symptoms and cycle threshold (ct) values severity of symptoms ct values ( median , iqr) p value asymptomatic (n = 56) 26 (24, 28) 0.01 mild (n = 43) 22 (22, 30) moderate (n = 2) 17.5 (14, 21) severe (n = 0) figure 1. severity of symptoms and cycle threshold (ct) values we did not notice any statistically significant correlation between severity of infection and vaccination received or presence of risk factors of covid-19 infection either. we noted that ct value had weak negative but statistically significant correlation with severity of covid infection (table 3, fig 1). discussion since covid-19 is a novel infection, there is limited knowledge about it, especially from paediatric age group. to date, majority of covid-19 related paediatric studies have originated from either china or western countries, data from indian subcontinent are scarce.9,10 as many country specific factors (climate, food habit, socio-economic status and health care facilities etc) may affect natural course of any pandemic, we reported an observation study of epidemiology, clinical characteristics and ct value of rtpcr (as surrogate marker of viral load) in nasopharyngeal swab of sars-cov-2 infected children from a large city in southern part of india. it was one of the epicenters of covid-19 cases in india.11 there was male predominance (55%) in admitted children in our study which is almost similar to another large study from china.8 as seen in previous studies, main source of infection in majority of children was house hold contact.12,13 this was prominently seen in our study too. clinically, almost all children in this study had j nepal paediatr soc | vol 42 | issue 02 |may-aug, 2022 69 original articleclinical characteristics and ct value of rt-pcr in paediatric sars-cov-2 asymptomatic or mild sars-cov-2 infection. previous multicenter studies and recent meta-analysis too, revealed that majority of sars-cov-2 infected children had mild or no symptoms of infection worldwide.12,14 fever (39%), cough (27%) and diarrhea (23%) were common symptoms in children in our study. similar clinical characteristics were reported in cohorts of previous large studies from china too.8,9 we noticed that higher proportion of children had symptomatic infection with increasing age. complaints of loss of taste, loss of appetite and body ache were more prevalent in < 15 years of adolescence than children, whereas diarrhea was more common in children (23% vs 8%) in our study. similar results were reported in another multicenter study including tertiary centers of china, france and germany.15 however, we found that higher proportion of children in our study had diarrheal episodes compared to previous studies from us and china.16,17 the reason for this differences may be due to sanitation, food habits, vaccination coverage, and poor nutritional status. we did not have any child with severe or critical infection, which is also supported by finding of the covid-19 data summary-nyc heath, in which less than 1% children had critical infection. 18 besides diagnosing, it is also important to know whether infected person is infectious or not, for containing pandemic. though, viral cell culture is gold standard to determine infectivity but in the absence of it, one may use viral load / ct value of rtpcr as a surrogate marker of likelihood of infectivity. the correlation between ct values of rt-pcr test and viral cell culture was found significant in a study in france.19 similarly, another study from canada showed there was significant correlation between ct values and infectivity in cell culture; and concluded that infectivity of patients may be low who has ct value <24.20 however, a study from us showed positive culture growth from samples with ct values of 34.21 later, systematic review from stanford university analyzed this study and revealed that study population (elderly patients in elder care facility) of this study might not represent the general population.22 recently, authors of another systematic review including 17 studies on viral load (ct values) and symptom severity, concluded that infectivity decreases after a week of viral shedding and cycle threshold value of 24.23 in our study, we did not find any significant difference in ct values of paediatric and adolescence age groups. since the early phase of this pandemic, many studies worldwide assessed relationship between severity of infection, age and infectivity. earlier this year, a study from chicago, u.s. including 97 children and 48 adults revealed similar ct values in older children (< 5 years to 17 years of age) and adults. however, younger children (< 5 years of age ) had significant lower ct values than adults.24 in another study from switzerland, authors compared viral load values of 53 children with values of 352 adults and found no significant difference.25 similarly, a study of 201 children from greece reported no difference in viral load of paediatric and adolescence age groups, neither any difference of viral loads between symptomatic and asymptomatic children.26 however, another study from us comprising of 339 asymptomatic and 478 symptomatic children found significantly higher ct values of asymptomatic children than symptomatic children of all age groups.27 we too found significant higher median (iqr) ct values of asymptomatic children than mild symptomatic children, 26 (24, 28) vs 22 (22, 30), p = 0.01. our study has some limitations. asymptomatic children in our study may not be representing recent infection due to possibility of picking up remote infection in the screening tests. however, the strength of our study is that the nasopharyngeal swabs were taken by single team of trained health care staff and analyzed by same commercial kit in microbiological laboratory of our center. conclusions to conclude, majority of children have asymptomatic or mild sarscov-2 infection with similar ct values in children and adolescents. further studies are needed to evaluate the role of children in the ongoing pandemic with upcoming new mutations. references 1. wang c, horby pw, hayden fg, gao gf. a novel coronavirus outbreak of global health concern. lancet. 2020 feb 15;395(10223):470-473. doi: 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10.3201/eid1608.091918 5. lee n, chan pk, hui ds, rainer th, wong e, choi kw, et al. viral loads and duration of viral shedding in adult patients hospitalized with influenza. j infect dis. 2009 aug 15;200(4):492-500. doi: 10.1086/600383. pmid: 19591575; pmcid: pmc7110250. 6. kumar vm, pandi-perumal sr, trakht i, thyagarajan sp. strategy for covid-19 vaccination in india: the country with the second highest population and number of cases. npj vaccines 6, 60 (2021). doi: https://doi.org/10.1038/s41541-021-00327-2 7. sahi pk, jhamb u, dabas a. pediatric coronavirus disease 2019: clinical features and management. indian pediatr. 2021 may 15;58(5):453-460. doi: 10.1007/s13312-021-2216-4. pmid: 33612488; pmcid: pmc8139223. 8. dong y, mo x, hu y, qi x, jiang f, jiang z, et al. epidemiology of covid-19 among children in china. pediatrics. 2020 jun;145(6): e20200702. doi: 10.1542/peds.2020-0702. pmid: 32179660. 9. bai k, liu w, liu c, fu y, hu j, qin y, et al. clinical analysis https://doi.org/10.3201%2feid1608.091918 https://doi.org/10.1038/s41541-021-00327-2 j nepal paediatr soc | vol 42 | issue 02 |may-aug, 202270 original article clinical characteristics and ct value of rt-pcr in paediatric sars-cov-2 of 25 covid-19 infections in children. pediatr infect dis. 2020 jul 1;39(7): e100-3. doi: 10.1097/inf.0000000000002740. pmid: 32520888. 10. shekerdemian ls, mahmood nr, wolfe kk, riggs bj, ross ce, mckiernan ca, et al. characteristics and outcomes of children with coronavirus disease 2019 (covid-19) infection admitted to us and canadian pediatric intensive care units. jama pediatr. 2020 sep 1;174(9):868-873. doi: 10.1001/jamapediatrics.2020.1948. pmid: 32392288;pmcid: pmc7489842. 11. bogam p, joshi a, nagarkar s, jain d, gupte n, shashidhara ls, et al. burden of covid-19 and case fatality rate in pune, india: an analysis of the first and second wave of the pandemic. ijid regions. 2022 mar 1; 2: 74-81. doi: http//doi.org/10.1016/j.ijregi.2021.12.006. 12. zimmermann p, curtis n. coronavirus infections in children including covid-19: an overview of the epidemiology, clinical features, diagnosis, treatment and prevention options in children. pediatr infect dis j. 2020; 39(5): 355368. doi: 10.1097/inf.0000000000002660. pmid: 32310621; pmcid: pmc7158880. 13. choi sh, kim hw, kang jm, kim dh, cho ey. epidemiology and clinical features of coronavirus disease 2019 in children. clin exp pediatr. 2020 apr; 63 (4): 125-32. doi: 10.3345/cep.2020.00535. pmid: 32252139; pmcid: pmc7170785. 14. meena j, yadav j, saini l, yadav a, kumar j. clinical features and outcome of sars-cov-2 infection in children: a systematic review and meta-analysis. indian pediatr. 2020 sep;57(9):820-826. doi: 10.1007/s13312-020-1961-0. pmid: 32583808; pmcid: pmc7498550. 15. qiu c, cui c, hautefort c, haehner a, zhao j, yao q, et al. olfactory and gustatory dysfunction as an early identifier of covid-19 in adults and children: an international multicenter study. otolaryngol head neck surg. 2020; 163: 714–21. doi: 10.1177/0194599820934376. epub 2020 jun 16. pmid: 32539586; pmcid: pmc7298561. 16. cdc covid-19 response team. coronavirus disease 2019 in children united states, february 12-april 2, 2020. mmwr morb mortal wkly rep. 2020 apr 10;69(14):422-426. doi: 10.15585/mmwr.mm6914e4. pmid: 32271728; pmcid: pmc7147903. 17. li q, guan x, wu p, wang x, zhou l, tong y, et al. early transmission dynamics in wuhan, china, of novel coronavirus-infected pneumonia. n engl j med. 2020 mar 26;382(13):1199-1207. doi: 10.1056/nejmoa2001316. pmid: 31995857; pmcid: pmc7121484. 18. covid-19: data summary—nyc health. https:// www1. nyc.gov/site/doh/covid/covid-19-data.page (17 august 2020, date last accessed). 19. la scola b, le bideau m, andreani j, hoang vt, grimaldier c, colson p, et al. viral rna load as determined by cell culture as a management tool for discharge of sars-cov-2 patients from infectious disease wards. eur j of clin microbiol & infect dis. 2020;39(6):1059-1061. doi: 10.1007/s10096-020-03913-9. pmid: 32342252; pmcid: pmc7185831. 20. bullard j, dust k, funk d, strong je, alexander d, garnett l, et al. predicting infectious severe acute respiratory syndrome coronavirus 2 from diagnostic samples. clin infect dis. 2020 dec 17;71(10):2663-2666. doi: 10.1093/cid/ciaa638. pmid: 32442256; pmcid: pmc7314198. 21. arons mm, hatfield km, reddy sc, kimball a, james a, jacobs jr, et al. presymptomatic sars-cov-2 infections and transmission in a skilled nursing facility. n engl j med. 2020 apr 24; 382:2081-2090. doi: 10.1056/nejmoa2008457. pmid: 32329971; pmcid: pmc7200056. 22. savvides c, siegel r. asymptomatic and presymptomatic transmission of sars-cov-2: a systematic review. medrxiv [preprint]. 2020 jun 17:2020.06.11.20129072. doi: 10.1101/2020.06.11.20129072. pmid: 32587980; pmcid: pmc7310638. 23. jefferson t, spencer ea, brassey j, heneghan c. viral cultures for coronavirus disease 2019 infectivity assessment: a systematic review. clin infect dis. 2021 dec 6;73(11): e3884-e3899. doi: 10.1093/cid/ciaa1764. pmid: 33270107; pmcid: pmc7799320. 24. heald-sargent t, muller wj, zheng x, rippe j, patel ab, kociolek lk. age-related differences in nasopharyngeal severe acute respiratory syndrome coronavirus 2 (sarscov-2) levels in patients with mild to moderate coronavirus disease 2019 (covid-19). jama pediatr. 2020 sep 1;174(9):902-903. doi: 10.1001/jamapediatrics.2020.3651. pmid: 32745201; pmcid: pmc7393583. 25. baggio s, l’huillier ag, yerly s, bellon m, wagner n, rohr m, et al. severe acute respiratory syndrome coronavirus 2 (sars-cov-2) viral load in the upper respiratory tract of children and adults with early acute coronavirus disease 2019 (covid-19). clin infect dis. 2021 jul 1;73(1):148150. doi: 10.1093/cid/ciaa1157. pmid: 32761228; pmcid: pmc7454380. 26. maltezou hc, magaziotou i, dedoukou x, eleftheriou e, raftopoulos v, michos a, et al. for greek study group on sars-cov-2 infections in children. children and adolescents with sars-cov-2 infection: epidemiology, clinical course and viral loads. pediatr infect dis j. 2020 dec;39(12): e388-e392. doi: 10.1097/inf.0000000000002899. pmid:33031141. 27. kociolek lk, muller wj, yee r, dien bard j, brown ca, revell pa, et al. comparison of upper respiratory viral load distributions in asymptomatic and symptomatic children diagnosed with sars-cov-2 infection in pediatric hospital testing programs. j clin microbiol. 2020 dec 17;59(1): e02593-20. doi: 10.1128/jcm.02593-20. pmid: 33093026; pmcid: pmc7771452. original article peripapillary and macular nerve fiber layer thickness in glaucoma j nepal paediatr soc | vol 42 | issue 02 |may-aug, 202230 *pragati adhikari gautam1, hira nath dahal2, jyoti baba shrestha3 1lecturer, department of ophthalmology, b p koirala lions center for ophthalmic studies, maharajgunj medical campus, tribhuvan university, kathmandu, nepal 2optomtery instructor, department of ophthalmology, b p koirala lions center for ophthalmic studies, maharajgunj medical campus, tribhuvan university, kathmandu, nepal 3associate professor, department of ophthalmology, b p koirala lions center for ophthalmic studies, maharajgunj medical campus, tribhuvan university, kathmandu, nepal evaluation of peripapillary and macular nerve fiber layer thickness in paediatric glaucoma suspects introduction: glaucoma patients are known to have optic disc cupping, leading to loss of retinal ganglion cell axons. this peripapillary nerve thinness is known to be associated with glaucoma in adult patients. hence, we intended to evaluate the peripapillary and macular nerve fiber thickness in eyes of pediatric glaucoma suspects using spectral domain optical coherence tomography (oct). methods: this cross-sectional analytical study included 44 eyes of 22 paediatric glaucoma suspects, who were compared with 40 eyes of 20 normal paediatric eyes. pearson correlation coefficients were calculated to assess the relations between the peripapillary retinal nerve fiber layer (rnfl) thickness and ganglion cell complex (gcc). receiver operating characteristics (roc) curve was plotted for oct parameters in both the groups along with area under the curve (auc) calculation. p values < 0.05 were considered as statistically significant. results: we found the rnfl to be thickest in the superior, inferior, nasal and temporal quadrants in both glaucoma suspects and normal group. when compared among quadrants in rnfl, all the values in different quadrants were statistically significant from each other and in between groups with p < 0.01. the rnfl thickness was statistically different in glaucoma when compared with normal in superior, temporal, and nasal quadrants however, no statistically significant difference was found in inferior quadrant rnfl. the largest auc for discrimination of glaucoma suspect eyes from normal in peripapillary rnfl in was nasal quadrant in right eye, followed by temporal quadrant whereas it was largest in superior followed by nasal quadrants then average in left eye. however, auc for discrimination of glaucoma suspects from normal in gcc was poor. conclusions: glaucoma suspect paediatric eyes showed significant thinning in peripapillary nerve fiber layer thickness compared to normal subjects. abstract *corresponding author pragati gautam adhikari lecturer, department of ophthalmology, b p koirala lions center for ophthalmic studies, maharajgunj medical campus, maharajgunj, kathmandu, nepal. e-mail: pragatigautam@hotmail.com article history received on : 18 jan, 2022 accepted on : 09 dec, 2022 funding sources: none conflicts of interest: none keywords: ganglion cell complex; pediatric glaucoma; retinal nerve fiber layer; spectral domain oct online access doi: https://doi.org/10.3126/jnps.v42i2.42495 introduction glaucomatous optic disc cupping is associated with the loss of retinal ganglion cell axons.1 normal optic disc usually has the inferior neuroretinal rim as the thickest portion of the rim, followed by the superior rim, and then the nasal rim, with the temporal rim being the thinnest portion.2 violation of this isnt rule has shown to have predictive value in diagnosing glaucoma in adults.3,4 original article doi: 103126/jnps.v4113 copyrights & licensing © 2022 by author(s). this is an open access article distributed under creative commons attribution license (cc by nc ) j nepal paediatr soc | vol 42 | issue 02 |may-aug, 2022 31 original articleperipapillary and macular nerve fiber layer thickness in glaucoma the faster image acquisition with spectral-domain oct (sd oct) like rtvue has made oct even more feasible in children.5-9 oct has been used in eyes of normal children but published reports of the use of oct in paediatric glaucoma suspects are sparse. this study tries to explore and correlate the measurements of peripapillary rnfl thickness and macular thickness in paediatric glaucoma suspects with that of normal using sd oct. to the best of our knowledge, this is one of the first studies using the oct machine to investigate the correlation between the macular thickness and the peripapillary rnfl thickness in pediatric glaucoma and suspects. methods this was a hospital-based cross-sectional comparative study. written informed consent was obtained from all parents or guardians. approval from the institutional review committee of institute of medicine was taken. the study duration was from 15th june 2021 to 15th december 2021. from a prevalence of childhood glaucoma from previous study done in nepal,9 a sample size of 22 was calculated for glaucoma suspects and 20 cases were taken in for control. the non-probability purposive sampling method was applied. children aged between five to 17 years with glaucoma suspect were included in study. children less than five years, those with hypermetropia more than + 3d, myopia more than – 5d, or astigmatism more than 2d, pseudophakia and prematurity at birth, corneal lesions, chronic uveitis, secondary glaucoma, optic neuropathy other than glaucoma, retinal pathology, maculopathy and previous ocular trauma history were excluded from this study. the peripapillary retinal nerve fiber layer (rnfl) and macular thickness mainly ganglion cell complex (gcc) was compared amongst two groups: normal eyes, eyes with glaucoma suspects. glaucoma suspects were defined as eyes with (cup disc ratio < 0.4 and < 0.7, iop < 21), with or without family history of glaucoma, vertical cup-to-disc ratio asymmetry < 0.2 between the two eyes without focal notching or generalized loss of the neuroretinal rim, excavation of the optic disc was taken as glaucoma suspects. the normal subjects were from patients coming for routine ophthalmic examination, with no clinical suspicion of glaucoma, who also had optic nerves and retinas that were considered clinically normal. family history in these individuals was negative for glaucoma. all subjects underwent a complete ophthalmological examination. the intraocular pressure (iop) was measured with keeler, uk pulse air desktop non-contact applanation tonometry and any high reading < = 21 were be reconfirmed with goldman applanation tonometry. for peripapillary rnfl and ganglion cell complex in macular thickness measurements the rtvue -100 software was used. peripapillary rnfl thickness measurements were performed by the same investigator using rtvue oct (optovue, fremont, ca). the rnfl thickness was measured by averaging the results of three sequential circular scans. images with signal strength index ≥ 50 were included in the study. both eyes from each individual were included in the analyses. the statistical package for social sciences (spss) version 22.0 was used in the analysis. pearson correlation coefficients were calculated to assess the relations between the peripapillary rnfl thickness and gcc. interocular oct parameters difference among gender was calculate with paired t test. interocular oct parameters difference was calculated with paired t test among glaucoma suspects and control. the oct parameters were compared among the two groups by one-way anova test. receiver operating characteristics (roc) curve was plotted for oct parameters in both control and glaucoma suspect along with area under the curve (auc) calculation. the diagnostic specificity and sensitivity of the main parameters (auc < 0.7) for distinguishing fellow eyes from normal eyes were evaluated, and cut-off points were presented. a probability value (p value) less than 0.05 was considered as significant at 95% confidence level. results the mean age of the subjects in the study was 14.59 ± 1.89 years (11 years 17 years). of the total 22 subjects in the study, 54.5% (n = 12) were males and 45.5% (n = 10) were females. the mean cup disc ratio was 0.56 ± 0.078 in right eyes and 0.55 ± 0.071 in left eyes. twenty age and gender matched normal children were also included in the study. the mean age of normal group was 13.80 ± 2.14 years (11 17 years). male to female ratio was 1:1 in control group. all subjects had best corrected visual acuities of 0.0 logmar or better. none of subjects had glaucoma, ocular hypertension. there was no effect of age on rnfl thickness in glaucoma suspects and normal as presented in table 1. average oct parameters were not statistically significant in relation to gender (re, p = 0.630, le p = 0.178, independent t test) in glaucoma suspect group. original article peripapillary and macular nerve fiber layer thickness in glaucoma j nepal paediatr soc | vol 42 | issue 02 |may-aug, 202232 table 1. oct parameters in relation to gender oct parameters (µm) male female p value (independent t-test) right eye superior 22.77 ± 130 16.15 ± 129.60 0.876 inferior 24.05 ± 127.83 14.54 ± 121.00 0.422 temporal 12.39 ± 75.75 7.43 ± 72.70 0.485 nasal 12.50 ± 74.66 12.77 ± 82.10 0.186 average 17.48 ± 104.37 11.26 ± 101.35 0.630 left eye superior 13.36 ± 133.33 12.73 ± 124.10 0.114 inferior 18.77 ± 128.91 15.74 ± 120.00 0.240 temporal 8.14 ± 77.50 8.52 ± 71.30 0.099 nasal 8.80 ± 76.66 11.00 ± 79.20 0.564 average 9.24 ± 104.10 9.02 ± 98.65 0.178 in this study, we found rnfl to be thickest in the superior, inferior, nasal and temporal quadrants, in that order in both glaucoma suspects and control group. when compared among quadrants in rnfl, all the values in different quadrant were statistically significant from each other and in between groups with p < 0.01 when tested with anova test. this means, there was statistically significant difference in rnfl thickness quadrant wise and in between groups. rnfl thickness was statistically different in glaucoma suspects when compared with control in superior, temporal, and nasal quadrants as presented in table 2. table 2. intraocular differences in peri papillary nerve fiber thickness along different quadrant oct characteristics (µm) right eye p value (anova) left eye p value (anova)glaucoma suspects normal glaucoma suspects normal superior 19.59 ± 130.31 142.31±7.10 0.005 13.60 ± 129.13 6.22 ± 144.60 0.000 inferior 20.14 ± 124.72 9.73 ± 124.72 0.680 17.65 ± 124.86 8.60 ± 126.10 0.744 temporal 22.79 ± 78.90 4.75 ± 82.80 0.002 8.70 ± 74.68 5.77 ± 82.40 0.005 nasal 12.89 ± 78.04 9.73 ± 93.20 0.000 9.70 ± 77.81 10.22 ± 90.30 0.000 average 14.72 ± 103.00 6.14 ± 110.85 0.019 9.34 ± 101.62 6.44 ± 110.85 0.001 the macular ganglion cell complex thickness was similar in both groups with no statically significant difference quadrant wise in both right eye and left eye respectively. (table 3) table 3. distribution of macular ganglion cell thickness ganglion cell thickness (µm) right eye p value (anova) left eye p value (anova) glaucoma suspects normal glaucoma suspects normal superior quadrant 12.20 ± 94.50 2.68 ± 95.10 0.648 7.08 ± 94.59 3.78 ± 93.10 0.458 inferior quadrant 10.45 ± 93.68 3.22 ± 95.20 0.296 6.83 ± 94.90 3.52 ± 92.70 0.291 average 10.98 ± 94.09 2.92 ± 95.15 0.460 6.69 ± 94.75 3.58 ± 92.9 0.349 however, there was no statistically significant interocular difference quadrant wise in right eye and left eye respectively. (table 4) j nepal paediatr soc | vol 42 | issue 02 |may-aug, 2022 33 original articleperipapillary and macular nerve fiber layer thickness in glaucoma table 4. comparison between mean peri-papillary nerve fiber thickness between right eye and left eye in glaucoma suspect group quadrant right eye left eye p value (t-test) superior 19.59 ± 130.31 13.60 ± 129.13 0.765 inferior 20.14 ± 124.72 17.65 ± 124.86 0.972 temporal 22.79 ± 78.90 8.70 ± 74.68 0.887 nasal 12.89 ± 78.04 9.70 ± 77.81 0.926 average 14.72 ± 103.00 9.34 ± 101.62 0.584 the peri-papillary rnfl in all quadrants showed a positive correlation with gcc in right eyes whereas no such correlation was found in left eyes and in both eyes of control group. pearson correlation coefficients among the different quadrants to that of gcc displayed significant correlation with that of all quadrants and average parameters in right eye and only superior quadrant in left eye in glaucoma suspect group. (table 5) table 5. correlation between peri papillary nerve fiber thickness and average macular ganglion cell thickness (pearson correlation) glaucoma suspect control quadrant (gcc (re (gcc (le (gcc (re (gcc (le superior (r = 0.755, p = 0.000) (r = 0.410, p = 0.058) (r = 0.343, p = 0.331) (r = 0.371, p = 0.291) inferior (r = 0.587, p = 0.004) (r = 0.077, p = 0.734) (r = -0.177, p=0.625) (r = -0.066, p = 0.856) temporal (r = 0.785, p = 0.000) (r = 0.252, p = 0.257) (r = 0.314, p = 0.377) (r = -0.100, p = 0.784) nasal (r = 551, p = 0.008) (r = 0.392, p = 0.071) (r = -0.256, p = 0.475) (r = -0.228, p = 0.527) average (r = 0.658, p = 0.001) (r = 0.346, p = 0.115) (r = -0.007, p = 0.984) (r = -0.045, p = 0.901) roc curves were plotted and auc calculated for right eye and left eye for rnfl thickness and gcc thickness respectively. the largest auc for discrimination of glaucoma suspect eyes from normal in peripapillary rnfl in was nasal quadrant (0.84) in right eye, followed by temporal quadrant (0.78) whereas it was largest in superior (0.85) followed by nasal quadrants (0.83) then average (0.80) in left eye. this was statistically significant to diagnose glaucoma suspects from normal eye. however, auc for discrimination of glaucoma suspects from normal in gcc was poor in right eye (0.54) and had no discrimination in left eye (0.38). discussion the present study showed that average peripapillary rnfl and peripapillary rnfl of all sectors except for inferior was significantly thinner in glaucoma suspects compared to normal children. since glaucomatous optic neuropathy has been documented to develop locally rather than globally3 the normal group of participants had thicker rnfl using sd-oct as compared to earlier studies,3,4 with maximum thickness in the superior quadrant, contradicting the isnt rule. some researchers have reported the thinning of average rnfl thickness in all sectors between paediatric glaucoma and controls. however, other researchers have found that children with glaucoma and normal participants have comparable rnfl thickness in some regions such as the temporal, nasal, and inferior nasal quadrants. the average peripaillary thickness was 110.85 ± 6.14 in right eyes and 110.85 ± 6.44 in left eyes in normal group which is slightly higher than reported in various studies11-14 but almost similar to reported in others.15-17 this may be attributed to ethnic differences and differences in the oct version used in earlier studies. studies of the sensitivity and specificity of oct for the diagnosis of glaucoma have primarily focused on glaucoma patients who already have visual field defects.18,19 however, in our study, the largest auc for discrimination of glaucoma suspect eyes from normal in peripapillary rnfl in was nasal quadrant in right eye, followed by temporal quadrant whereas it was largest in superior followed by nasal quadrants then average in left eye. budenz and associates found higher auc values for mean rnfl thickness, superior quadrant and inferior quadrant.8 similarly, in a study comparing sd-oct to stratus oct, the rnfl parameters with the highest aucs were in similar locations as those in the stratus oct.20 the ability to discriminate normal from glaucomatous results is directly proportional to the magnitude of disease severity as suggested in previous studies.19,21,22 the changes of prnfl thickness are meaningful in these children with less severe glaucomatous optic nerve damage, where the damage of visual function may be difficult to measure. age-related decrease in rnfl has been reported in adults11,23 using oct however it was not found in our study of paediatric age group. similar to our finding, rao et al also showed that peripapillary rnfl thickness was independent of age in children.24 alamouti and funk have suggested that the rate of decrease in rnfl thickness is slower in the younger age.23 however, it is still unclear as to the exact age at which the thinning starts. more so, it is possible that such an age-related process starts in adult life, which may explain the lack original article peripapillary and macular nerve fiber layer thickness in glaucoma j nepal paediatr soc | vol 42 | issue 02 |may-aug, 202234 of correlation of age with rnfl thickness in the paediatric group.24 gender also did not contribute to the variability in rnfl thickness in our study. similarly, such gender related difference in the pediatric age group was not found in other studies too.25,26 in our study, we did not find significant differences between the two eyes with symmetric rnfl and macular parameters. similarly, no significant difference in mean rnfl thickness of the four quadrants was seen between the two eye in other studies.7,24 however, eye side had significant influence on rnfl thickness in some studies.8,10,13 the mechanism underlying interocular differences is unclear in normal children, it may be physiological however in children with glaucoma suspects or glaucoma it could be attributed to the asymmetrical nature of the disease itself. in addition to peripapillary rnfl thickness, macular thickness especially gcc has been shown as an important parameter for the early detection of glaucoma in adults, with significant differences reported by some between glaucomatous and normal eyes. in the study by hess et al, significant differences were found in the oct scans obtained from glaucoma patients compared with those of normal children, using both the rnfl map protocol and the fast macular map.27 however, another study has showed that macular thickness had lower diagnostic capability compared with peripapillary rnfl thickness in early glaucoma.19 similarly, no much difference was found in our study too. since diagnostic accuracy of gcc is significantly influenced by disease severity. moreso, this difference may not be pronounced in early disease state. our group of glaucoma suspect eyes based on optic nerve cupping. even though we did not correlate rnfl and cupping, a study had correlated rnfl and cupping and found that the degree of optic nerve cupping does not correlate with the rnfl thickness.10 the glaucoma suspects patients may represent a mild or early stage of glaucomatous damage. this provides us with a standard comparison for glaucoma screening since any deviation from the normative database would be definite along with other factors. inclusion of eyes with glaucoma in the group would probably have made the differences between the glaucoma and normal groups even larger. the axial length was not taken into account in our study and our measurements were obtained in children without ocular disease and significant refractive error as previous studies showed that they did not affect the rnfl and gcc parameters significantly.13,15 the strength of the study is that it included a heterogeneous mixture of nepalese ethnic group visiting a tertiary eye care center in kathmandu, nepal. the major limitation of the present study is related to the characteristics of the subjects enrolled ranging mainly in age group 11 17 years, so results may not be applicable to younger age groups. more so, a larger sample size will help to differentiate better predictive value of each parameter. longitudinal follow-up of eyes with paediatric glaucoma suspects, and serial oct measurements in these same eyes, will assess the use of this technology in monitoring glaucoma progression and evaluate outcomes in more detail along with identification of additional risk factors in the development of glaucoma in paediatric glaucoma suspects. conclusions this study demonstrated that spectral domain oct of rnfl can quantitatively measure structural changes that are known to occur with glaucoma, in glaucoma suspects. in our study, paediatric glaucoma suspects had significantly reduced average quadrants rnfl thickness compared with healthy controls along with largest auc for discrimination of glaucoma suspect eyes from normal in nasal quadrant and temporal quadrant in right eye, and superior, nasal quadrants and average rnfl in left eye. we would like to highlight sd-oct has potential clinical value in early diagnosis in pediatric glaucoma suspects. references 1. zhang yx, huang hb, wei sh. clinical characteristics of nonglaucomatous optic disc cupping. exp ther med. 2014;7(4):995-9. doi: https://doi.org/10.3892/etm.2014.1508 2. jonas jb, gusek gc, naumann go. optic disc, cup and neuroretinal rim size, configuration and correlations in normal eyes. invest ophthalmol vis sci. 1988;29(7):1151-8. 3. jonas jb, fernández mc, stürmer j. pattern of glaucomatous neuroretinal rim loss. ophthalmology. 1993;100(1):63-8. doi: https://doi.org/10.1016/s0161-6420(13)31694-7 4. harizman n, oliveira c, chiang a, tello c, marmor m, ritch r, et al. the isnt rule and differentiation of normal from glaucomatous eyes. arch ophthalmol. 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[role of sd-oct in pediatric glaucoma suspects]. j fr ophtalmol. 2015;38(10):e235-6. doi:https://doi.org/10.1016/j.jfo.2014.09.030 6. pazos m, biarnés m, blasco-alberto a, dyrda a, luquefernández m, gómez a, et al. sd-oct peripapillary nerve fibre layer and ganglion cell complex parameters in glaucoma: principal component analysis. br j ophthalmol. 2021;105(4):496-501. doi: https://doi.org/10.1136/bjophthalmol-2020-316296 7. ahn hc, son hw, kim js, lee jh. quantitative analysis of retinal nerve fiber layer thickness of normal children and adolescents. korean j ophthalmol. 2005;19(3):195-200. doi:https://doi.org/10.3341/kjo.2005.19.3.195 8. budenz dl, anderson dr, varma r, schuman j, cantor l, savell j, et al. determinants of normal retinal nerve fiber layer thickness measured by stratus oct. ophthalmology. 2007;114(6):1046-52. doi: https://doi.org/10.1016/j.ophtha.2006.08.046 9. shakya s ks, pradhan e. pattern of childhood glaucoma. j nepal med assoc. 2013;39(144):4. doi: https://doi.org/ https://doi.org/10.31729/jnma.679 j nepal paediatr soc | vol 42 | issue 02 |may-aug, 2022 35 original articleperipapillary and macular nerve fiber layer thickness in glaucoma 10. gherghel d, orgül s, prünte c, gugleta k, lübeck p, gekkieva m, et al. interocular differences in optic disc topographic parameters in normal subjects. curr eye res. 2000;20(4):276-82 11. parikh rs, parikh sr, sekhar gc, prabakaran s, babu jg, thomas r. normal age-related decay of retinal nerve fiber layer thickness. ophthalmology. 2007;114(5):921-6. doi: https://doi.org/10.1016/j.ophtha.2007.01.023 12. gupta g, donahue jp, you t. profile of the retina by optical coherence tomography in the pediatric age group. am j ophthalmol. 2007;144(2):309-10. doi: https://doi.org/10.1016/j.ajo.2007.03.029 13. huynh sc, wang xy, rochtchina e, crowston jg, mitchell p. distribution of optic disc parameters measured by oct: findings from a population-based study of 6-year-old australian children. invest ophthalmol vis sci. 2006;47(8):3276-85. doi: https://doi.org/10.1167/iovs.06-0072 14. larsson e, molnar a, holmström g. repeatability, reproducibility and interocular difference in the assessments of optic nerve oct in childrena swedish population-based study. bmc ophthalmol. 2018;18(1):270. doi: https://doi.org/10.1186/s12886-018-0940-x 15. el-dairi ma, asrani sg, enyedi lb, freedman sf. optical coherence tomography in the eyes of normal children. arch ophthalmol (chicago, ill : 1960). 2009;127(1):50-8. doi: https://doi.org/10.1001/archophthalmol.2008.553 16. tsai dc, huang n, hwu jj, jueng rn, chou p. estimating retinal nerve fiber layer thickness in normal schoolchildren with spectral-domain optical coherence tomography. japanese journal of ophthalmology. 2012;56(4):362-70. doi: https://doi.org/10.1007/s10384-012-0142-7 17. repka mx, goldenberg-cohen n, edwards ar. retinal nerve fiber layer thickness in amblyopic eyes. am j ophthalmol. 2006;142(2):247-51. doi: https://doi.org/10.1016/j.ajo.2006.02.030 18. shah nn, bowd c, medeiros fa, weinreb rn, sample pa, hoffmann em, et al. combining structural and functional testing for detection of glaucoma. ophthalmology. 2006;113(9):1593-602. doi: https://doi.org/10.1016/j.ophtha.2006.06.004 19. parikh rs, parikh s, sekhar gc, kumar rs, prabakaran s, babu jg, et al. diagnostic capability of optical coherence tomography (stratus oct 3) in early glaucoma. ophthalmology. 2007;114(12):2238-43. doi: https://doi.org/10.1016/j.ophtha.2007.03.005 20. ghasia ff, freedman sf, rajani a, holgado s, asrani s, el-dairi m. comparison of stratus and spectralis optical coherence tomography (oct) in pediatric glaucoma evaluation. journal of american association for pediatric ophthalmology and strabismus {jaapos}. 2012;16(1):e17. doi: https://doi.org/10.1016/j.jaapos.2011.12.065 21. sehi m, grewal ds, sheets cw, greenfield ds. diagnostic ability of fourier-domain vs time-domain optical coherence tomography for glaucoma detection. am j ophthalmol. 2009;148(4):597-605. doi: https://doi.org/10.1016/j.ajo.2009.05.030 22. wu h, de boer jf, chen tc. diagnostic capability of spectraldomain optical coherence tomography for glaucoma. am j ophthalmol. 2012;153(5):815-26.e2. doi: https://doi.org/10.1016/j.ajo.2011.09.032 23. alamouti b, funk j. retinal thickness decreases with age: an oct study. br j ophthalmol. 2003;87(7):899-901. doi: https://doi.org/10.1136/bjo.87.7.899 24. rao a, sahoo b, kumar m, varshney g, kumar r. retinal nerve fiber layer thickness in children <18 years by spectraldomain optical coherence tomography. semin ophthalmol. 2013;28(2):97-102. doi: https://doi.org/10.3109/08820538.2012.760626 25. salchow dj, oleynikov ys, chiang mf, kennedy-salchow se, langton k, tsai jc, et al. retinal nerve fiber layer thickness in normal children measured with optical coherence tomography. ophthalmology. 2006;113(5):786-91. doi: https://doi.org/10.1016/j.ophtha.2006.01.036 26. pawar n, maheshwari d, ravindran m, ramakrishnan r. interocular symmetry of retinal nerve fiber layer and optic nerve head parameters measured by cirrus high-definition optical coherence tomography in a normal pediatric population. indian j ophthalmol. 2017;65(10):955-62. doi: https://doi.org/10.4103/ijo.ijo_71_17 27. hess db, asrani sg, bhide mg, enyedi lb, stinnett ss, freedman sf. macular and retinal nerve fiber layer analysis of normal and glaucomatous eyes in children using optical coherence tomography. am j ophthalmol. 2005;139(3):509-17. doi: https://doi.org/10.1016/j.ajo.2004.10.047 j nepal paediatr soc | vol 42 | issue 02 |may-aug, 202246 original article outcome of respiratory distress syndrome in nepaloriginal article doi: 103126/jnps.v4113 pitaloka ada1, nurhayati e2, hadi h1, lee k3, *paramashanti ba1 1department of nutrition, faculty of health sciences, universitas alma ata, yogyakarta 55183, indonesia 2department of midwifery, faculty of health sciences, universitas alma ata, yogyakarta 55183, indonesia 3department of international health, bloomberg school of public health, the johns hopkins university, maryland 21205-2179, usa. is maternal breastfeeding motivation and exclusive breastfeeding influenced by early marriage age? introduction: exclusive breastfeeding is beneficial for mother and child health. however, exclusive breastfeeding rate is low among young mothers. thus, our study aimed to examine the relationship between maternal breastfeeding motivation and exclusive breastfeeding and determine whether the early marriage age influences both variables in gunung kidul district, yogyakarta special region, indonesia. methods: a cross-sectional design was conducted among young adolescent mothers. a total of 144 married women who had young children aged six to 12 months were selected using simple random sampling. exclusive breastfeeding was the dependent variable, while maternal breastfeeding motivation was the independent variable. descriptive statistics, chi-square test, multiple logistic regression, and mantel haenzel tests were performed to analyse the relationship between variables using stata 14.2. results: maternal age at marriage < 20 years (or = 2.98; 95%ci: 1.15-7.74) and good maternal breastfeeding motivation (or = 22.02; 95%ci: 7.55-64.2) were associated with exclusive breastfeeding practice. in the stratification analysis, a larger association was found between maternal breastfeeding motivation and exclusive breastfeeding among younger mothers (or=3.96; 95%ci: 2.25-6.97) compared to older mothers (or = 1.79; 95%ci: 1.29-2.48). conclusions: maternal breastfeeding motivation positively influences exclusive breastfeeding practice. notably, young mothers are more likely to be motivated to breastfeed their children exclusively than older mothers. the results suggest a need to improve maternal breastfeeding motivation through quality prenatal and postnatal care services that involve other family members. abstract *corresponding author bunga astria paramashanti universitas alma ata jalan brawijaya no. 99, kasihan, bantul, daerah istimewa yogyakarta55183, indonesia. email: bunga@almaata.ac.id. introduction exclusive breastfeeding for the first six months of life, with the introduction of complementary food and continued breastfeeding thereafter, has been recommended since 2001 by the world health organization (who).1 copyrights & licensing © 2022 by author(s). this is an open access article distributed under creative commons attribution license (cc by nc ) online access doi: https://doi.org/10.3126/jnps.v42i1.38053 article history received on : 25 june, 2021 accepted on : 05 apr, 2022 funding sources: none conflicts of interest: none keywords: adolescent mothers; exclusive breastfeeding; indonesia; motivation; young mothers j nepal paediatr soc | vol 42 | issue 02 |may-aug, 2022 47 original articlebreastfeeding motivation and exclusive breastfeeding also, breastfeeding is linked with sustainable development goals by improving nutrition, health, economics, education, equity, and environmental aspects.2 non-exclusive breastfeeding is responsible for high mortality and disease burden, especially among children under the age of five.3 in addition, breastfeeding promotion has the potential to decrease the disease burden of mothers.4 it is estimated that scaling up breastfeeding could save up to 823,000 child deaths and 20,000 deaths due to breast cancer annually.5 globally, the improvement of exclusive breastfeeding is still very modest in the past few decades.6 in low and middle income countries, exclusive breastfeeding coverage is only 37% among children less than six months of age.5 based on the nationally representative survey in indonesia, the proportion of exclusive breastfeeding decreased slightly from 38.0% in 2013 to 37.3% in 2018.7 further analysis showed that exclusive breastfeeding was even lower among employed mothers, mothers who lived in the eastern part of indonesia, and those in urban areas.8 maternal breastfeeding behaviour is complex and is affected by socio-cultural and physiological factors.9 a systematic review in brazil showed that exclusive breastfeeding was associated with living residence, maternal age, maternal education, maternal employment, child’s age, the use of a pacifier, and financing primary health care.10 similarly, a review conducted in the middle-east suggested that exclusive breastfeeding was linked to delivery factors, maternal employment, maternal age, and maternal education.11 few earlier studies examined the relationship between mothers’ motivation and exclusive breastfeeding practices in indonesia.12,13 in this study, we aimed to analyse the relationship between maternal breastfeeding motivation and exclusive breastfeeding in gunung kidul district, yogyakarta, indonesia. gunung kidul district was selected since it is a district with lower exclusive breastfeeding coverage and a high prevalence of young maternal age.14 the district consists of coastal, noncoastal, slope, and valley areas. the condition could be one of the reasons, in addition to the inequality in distribution of health professionals and limited health budget, that may lead to poor health care service in the district.15 gunung kidul, as in other districts / municipalities in yogyakarta, has strong cultural factors that may contribute to the population feeding practices.16 methods a cross sectional observational design was used for this study. the population included all married women in tepus subdistrict, gunung kidul district, yogyakarta special region, indonesia. from the list of mothers registered at the tepus ii phc, 72 married women were selected using a simple random sampling with a lottery method. we then added another 72 women from the list by matching their age at marriage with a ratio of 1:1. thus, for every woman who married at an age < 20 years, one woman whose marriage age was < 20 years, was added resulting in a total of 144 participants. the inclusion criteria were mothers of children aged six to 12 months and those who were agreed to participate in this study, evidenced by signing an informed consent form. if mothers had two children under-fives, then we only took data from the youngest child. we collected data on women’s characteristics, history of exclusive breastfeeding, and breastfeeding motivation by a home-visit interview for all women. we also derived data on marriage information (e.g., identity, marriage age) from the tepus office of religion affairs report in 2016. data on mothers who had young children aged six to 12 months was obtained from tepus ii phc. we then created a list of subjects based on data from the tepus office of religious affairs and tepus ii phc and randomised it to select participants. all participants were asked for their willingness to join the study by signing an informed consent form. the dependent variable of this study was a history of exclusive breastfeeding as recommended by the who.1 the independent variable was the maternal motivation for breastfeeding. maternal motivation for breastfeeding was categorised into favourable or unfavourable factoring in the maternal decision process related to breastfeeding. it covered intrinsic motivation related to maternal and child health and extrinsic motivation covering self-control, support, and social aspects.17 we categorised the motivation variable into two categories: good (50 100%) and poor (< 50%). other variables that were analysed in this study included child’s sex, child’s birth rank, parity, mother’s age at marriage, mother’s level of education, mother’s employment status, spouse’s age, and spouse’s educational level. we used a structured questionnaire on maternal characteristics, breastfeeding practices, and a motivation checklist. this checklist has been translated to bahasa indonesia and validated in a previous study.18 characteristics of mothers, children, their household, and the distribution of main variables were described with descriptive statistics. a chi-square test was used for bivariate analysis. variables that showed an association with the history of exclusive breastfeeding with a p-value of < 0.25 were entered into multiple logistic regression. in the multivariate analysis, the p-value < 0.05 was considered to be significant and the confidence interval for odds ratios was set at 95%. to examine confounding factors and the effect of the modification, we used the mantel haenzel test for stratification analysis. all of the analyses were performed using stata 14.2 (stata corporation, college station, tx). results almost half of women were married at a young age, less than 20 years, whereas most spouses married < 20 years (90.3%). our study participants had children who were primarily male (53.47%) and the first child (77.78%), with an average age of 8.7 months. most of our study participants and their spouses completed junior high school as their highest educational attainment, 57.6% and 47.9%, respectively. most women were unemployed (81.25%). sixty-one per cent of the sample reported exclusive breastfeeding at six months. 45.1% of mothers’ motivation for breastfeeding was scored as good. table 1 shows the characteristics and the distribution of the main variables in this study. j nepal paediatr soc | vol 42 | issue 02 |may-aug, 202248 original article breastfeeding motivation and exclusive breastfeeding table 1. characteristics of children and parents and the distribution of main variables (n = 144 households) characteristics % / mean + sd child characteristics child’s age (months) 8.7 + 2.1 child’s sex male 53.5 female 46.5 birth rank 1st 77.8 2nd 22.2 parity 1 81.9 <2 18.1 parental characteristics mother’s age at marriage < 20 years 48.6 < 20 years 51.4 mother’s education not completed primary school 1.4 completed elementary school 18.1 completed junior high school 57.6 completed senior high school 20.1 completed tertiary education 2.8 maternal working status not working 81.3 working 18.7 spouse’s age at marriage < 20 years 9.7 < 20 years 90.3 spouse’s education not completed primary school 3.5 completed elementary school 28.5 completed junior high school 47.9 completed senior high school 18.0 completed tertiary education 2.1 main variables exclusive breastfeeding no 38.9 yes 61.1 maternal breastfeeding motivation poor 54.9 good 45.1 being a later-born child (or = 2.78; 95%ci: 1.13-6.79), parity more than one (or = 3.20; 95%ci: 1.16-8.73), older maternal age at marriage (or = 3.69; 95% ci: 1.83 7.47), and good maternal motivation for breastfeeding (or = 21.86; 95% ci: 8.06 58.79) were significantly associated with exclusive breastfeeding at six months. variables such as the child’s sex, mother’s educational level, mother’s employment status, and spouse’s age at marriage were not related to exclusive breastfeeding. in the multivariate results, factors associated with breastfeeding exclusively included maternal age at marriage <20 years (or = 2.98; 95% ci: 1.15 7.74). having good motivation for breastfeeding was associated with a 22 times greater likelihood for exclusive breastfeeding for at least six months. table 3. stratification analysis between maternal breastfeeding motivation and exclusive breastfeeding by maternal age at marriage strata maternal breastfeeding motivation or (95%ci) mother’s age at marriage < 20 years poor (ref) good 3.96* (2.25 6.97) < 20 years poor (ref) good 1.79*(1.29 2.48) cor 2.60* (1.91 3.54) m-h or 2.38* (1.79 3.16) *level of significance at < 0.05 cor = crude odds ratio; m-h or = mantel-haenzel odds ratio j nepal paediatr soc | vol 42 | issue 02 |may-aug, 2022 49 original articlebreastfeeding motivation and exclusive breastfeeding table 2. bivariate and multivariate analysis of exclusive breastfeeding determinants characteristics exclusive breastfeeding cor (95% ci) p aor (95% ci) p child’s sex male (ref) female 0.89 (0.46 1.75) 0.75 birth rank 1st (ref) 2nd 2.78 (1.13 6.79) *0.03 1.87 (0.21 16.31) 0.57 parity 1 (ref) < 2 3.20 (1.16 8.73) *0.02 1.18 (0.09 14.14) 0.89 mother’s age at marriage < 20 years (ref) < 20 years 3.69 (1.83 7.47) *0.00 2.98 (1.15 7.74) *0.02 mother’s education primary school or below (ref) secondary school or above 0.85 (0.36 1.97) 0.70 maternal breastfeeding motivation poor (ref) good 21.86 (8.06 58.79) *0.00 22.02 (7.55 64.2) *0.00 maternal working status not working (ref) working 1.10 (0.47 2.57) 0.83 spouse’s age at marriage < 20 years (ref) < 20 years 0.86 (0.28 2.60) 0.79 spouse’s education primary school or below (ref) secondary school or above 1.97 (0.97 4.00) *0.06 2.00 (0.78 5.14) 0.15 *level of significance at < 0.05 cor = crude odds ratio; aor = adjusted odds ratio we analysed the relationship between maternal motivation and exclusive breastfeeding by maternal age at marriage (table 3). the association between maternal motivation and exclusive breastfeeding was not confounded by maternal age at marriage because there was only a small difference between crude odds ratio (or = 2.60; 95% ci: 1.91 3.54) and adjusted odds ratio (or = 2.38; 95% ci: 1.79 3.16). there was a larger effect of maternal motivation on exclusive breastfeeding among younger mothers (or = 3.96; 95% ci: 2.25 6.97) compared to older mothers (or = 1.79; 95% ci: 1.29 2.48). discussion our study demonstrated that the proportion of infants who were breastfed exclusively for six months was higher than the national proportion in indonesia.7 however, it remained below national and global targets for 80% and 90% coverage respectively.19,20 thus, to design interventions aiming at improving the rate of exclusive breastfeeding, it is essential to identify its associated factors. in this study, the mother’s age at marriage and maternal motivation for breastfeeding were significantly associated with exclusive breastfeeding. women who married at an age < 20 j nepal paediatr soc | vol 42 | issue 02 |may-aug, 202250 original article breastfeeding motivation and exclusive breastfeeding years were more likely to breastfeed their infants exclusively. our study in line with a previous study reporting that mother’s age at 20 years was linked with the higher exclusive breastfeeding proportion than mothers of younger age.21 maternal experiences in infant management may increase as maternal age increases.22 conversely, younger mothers may think about the change of their physical appearance (e.g., breast size) if they breastfeed their infant for a longer time and thus stop providing breastmilk.23 in yogyakarta, strong cultural factors such as giving sugar water or tajin water from boiled rice may also contribute to the early introduction of complementary food.16 additionally, mother’s decision making on breastfeeding could be explained by cultural and family factors.24 our finding indicates that young mothers are vulnerable to nonexclusive breastfeeding practices, especially in settings where culture and social norms are highly influential. mothers who had good motivation for breastfeeding were more likely to breastfeed their infants exclusively. when we stratified maternal motivation by maternal age at marriage, the result showed that mothers with good motivation tend to provide exclusive breastfeeding, especially among younger mothers. a previous study conducted among low-income women divided maternal motivation on breastfeeding into three categories: 1) extrinsically motivated, if women hadn’t succeeded breastfeeding previously but chose to breastfeed based on others’ suggestions, 2) intrinsically motivated, if women hadn’t succeeded breastfeeding previously but chose to breastfeed based on their or their infant’s health reasons, and 3) successfully experienced, if women had successfully breastfed previously and had a family with breastfeeding history, then chose to breastfeed for their and their infant’s health.25 in our study, mothers who married at a younger age might have no breastfeeding experience success as they just had one child. thus, intrinsic motivation arising from maternal selfdecision making and extrinsic motivation influenced by family and social factors may play essential roles in the success of exclusive breastfeeding. a qualitative study among adolescent mothers demonstrated that the decision to breastfeed was made prenatally. therefore, during prenatal and early postpartum health service, counselling strategy may benefit adolescents with low levels of motivation to breastfeed their infant.26 on the contrary to the earlier study, we found that maternal education was not related to exclusive breastfeeding.21 based on susiloretni et al,27 the only factor associated with a longer duration of exclusive breastfeeding was maternal breastfeeding knowledge in central java, indonesia. maternal knowledge on breastfeeding can have a significant impact on the success of exclusive breastfeeding.28 on the other hand, high educational attainment does not always mirror the favourable knowledge about breastfeeding.8 in this case, it is the responsibility of health care providers to deliver messages on breastfeeding and early child feeding practices to mothers since preconception and their husbands / spouses and other family members. besides, supports from families and the workplace may benefit the practice of breastfeeding.29,30 to our knowledge, this is the first study to analyse the association between maternal motivation for breastfeeding and exclusive breastfeeding that considers maternal marriage age in indonesia. findings for this study can be generalised to other populations with a high prevalence of adolescent marriage, as in the gunung kidul district. however, the use of a cross-sectional design prevents us from concluding any cause-effect relationship between maternal motivation for breastfeeding and exclusive breastfeeding. further research with larger sample sizes and advanced study designs (e.g., cohort and community trial) may be needed to examine the association between both variables. conclusions maternal breastfeeding motivation positively influences exclusive breastfeeding practice. notably, young mothers are more likely to be motivated to breastfeed their children exclusively than older mothers. the results suggest a need to improve maternal breastfeeding motivation through quality prenatal and postnatal care services, which involve mothers and family members. such programs should engage mothers of all ages in the community. references 1. who. report of the expert consultation on the optimal duration of exclusive breastfeeding. 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hadi h, prabandari ys, soenarto ys, wilopo sa. what works to improve duration of exclusive breastfeeding: lessons from the exclusive breastfeeding promotion program in rural indonesia. matern child health j. 2015; 19(7): 1515-25. doi:10.1007/s10995-014-1656-z. 28. kornides m, kitsantas p. evaluation of breastfeeding promotion, support, and knowledge of benefits on breastfeeding outcomes. j child health care. 2013; 17(3): 264-73. doi:10.1177/1367493512461460. 29. ratnasari d, paramashanti ba, hadi h, anafrin y, astiti d, nurhayati e. family support and exclusive breastfeeding among yogyakarta mothers in employment. asia pac j clin nutr. 2017; 26: s31-s5. doi:10.6133/apjcn.062017.s8. 30. indrawanto y, paramashanti ba, hadi h, rahmawati ni, amna fk. breastfeeding support and facilities for mothers in the workplace. j ners kebidanan indonesia. 2018; 5(3): 200-8. doi: 10.21927/jnki.2017.5(3).200-208. original article childhood asthma and its associated factors among children j nepal paediatr soc | vol 42 | issue 02 |may-aug, 202240 original article doi: 103126/jnps.v4113 1associate professor, department of paediatrics, institute of medicine, maharajgunj medical campus, kathmandu, nepal 2assistant professor, department of paediatrics, institute of medicine, maharajgunj medical campus, kathmandu, nepal 3professor, department of paediatrics, institute of medicine, maharajgunj medical campus, kathmandu, nepal incidence, outcome and predictors of mortality in respiratory distress syndrome (rds): a prospective cohort study at tertiary care hospital in nepal introduction: with advances in therapies during antenatal and perinatal period, there has been apparent decrease in incidence and mortality due to respiratory distress syndrome (rds). however, there is paucity of data on exact incidence and outcome of rds in resource limited setting. this study was conducted with the primary aim to describe the outcome of rds and analyze the predictors for mortality. methods: a prospective observational study was conducted in the neonatal intensive care unit (nicu) and neonatal unit of tribhuvan university teaching hospital (tuth), kathmandu, nepal from october 2019 to april 2021. results: a total of 94 preterm newborns developed rds giving prevalence of 20.5 per 1000 live birth cohort at tuth. incidence of rds among preterm babies was 14.6%. the median duration of continuous positive airway pressure (cpap) was 48 hours (range 8 192 hours). inhospital mortality rate was 15 (15.96%). lower gestational age and premature rupture of membrane (prom) were significantly associated with higher mortality whereas normal vaginal delivery (nvd) was associated with lower mortality. logistic regression analysis for risk of dying for the cohort predicted that lower birth weight (aor = 0.99; 95% ci = 0.99 0.99; p = 0:01), sepsis (aor = 145.14; 95% ci = 5.04 4175.15; p = 0:004) are independently associated with increased risk of dying whereas increase duration of nicu stay decreased the risk (aor = 0.71; 95% ci = 0.54 0.91; p = 0:01). conclusions: the mortality rate decreases with increasing gestational age and birth weight. a number of neonatal factors, either in isolation or in combination, were significantly associated with in-hospital mortality abstract *corresponding author srijana basnet associate professor, department of paediatrics, institute of medicine, maharajgunj medical campus, kathmandu, nepal. emaildrsrijanabasnet@yahoo.com article history received on : 06 apr, 2022 accepted on : 15 dec, 2022 funding sources: none conflict of interest: none keywords: cpap; mortality; rds; surfactant online access doi: https://doi.org/10.3126/jnps.v42i1.41183 introduction it is estimated that 15 million babies are born preterm, with 60% occurring in south asia and sub-saharan africa and this number is rising.1,2 respiratory distress syndrome (rds) occurs in 60 80% of infants < 28 weeks, 50% of born between 28 – 32 weeks, 15 30% of infant 32 36 weeks and rarely in those < 37 weeks gestational age.3,4 the incidence of rds is reported to be 6.8 14.1% of preterm live births and contributed 13.5% of total neonatal deaths in india.5,6 however, incidence of rds and its mortality rate has substantially reduced by improved antenatal and intrapartum care of preterm pregnancies. similarly, optimal respiratory support in the form of continuous positive airway pressure (cpap) or mechanical ventilation and surfactant replacement therapy copyrights & licensing © 2022 by author(s). this is an open access article distributed under creative commons attribution license (cc by nc ) srijana basnet1, surabhi aryal2, laxman shrestha3 j nepal paediatr soc | vol 42 | issue 02 |may-aug, 2022 41 original articleoutcome of respiratory distress syndrome in nepal (srt) has been identified as important interventions that could save many premature babies suffering from rds.7,8 but the important fact is that these recommendations are based on published trials done in high-income countries where care for premature babies in neonatal intensive care unit (nicu) is undertaken by trained doctors and nursing staff, facilities for round the clock monitoring, laboratory and radiological services and optimal cpap devices are available. without optimal equipment and skilled manpower, it is likely that these therapies may not be as effective and possibly less safe. it has been more than a decade that cpap and srt are in use for the management of rds at level iii nicu in nepal. however, these technologies for rds treatment are used inconsistently in different health care settings and there has been wide variation in the optimal care that the newborn receive in nicu. thus, it is crucial for us to analyze the impact of providing special care and respiratory support to preterm infants on rds related survival and to identify important factors contributing to mortality. therefore, this study was designed with the aim to determine the incidence, describe the outcome and evaluate the predictors of mortality among preterm infant delivered at tertiary care center of nepal. methods this is a prospective cohort study was conducted over one and half year period between october 2019 to april 2021, at tribhuvan university teaching hospital (tuth), kathmandu, nepal after approval from institutional research committee. all neonates born before 37 weeks of gestation delivered at tuth and clinically diagnosed as rds based on the need of respiratory support within six hours for at least for 24 hours were enrolled in the study. respiratory support was given if at least two out of five clinical signs (respiratory rate < 60 / min, expiratory grunting, suprasternal / intercostal / subcostal retraction, cyanosis at room air, flaring of the alae nasi) were present. the neonates were excluded if they were < 22 weeks of age or < 500 gm, and had any one of the following; perinatal asphyxia requiring endotracheal intubation at birth, major or life threatening congenital abnormality, suspected heart disease in antenatal echocardiography, delivered through meconium stained liquor, confirmed diagnosis of congenital pneumonia or early onset (within 72 hours) sepsis. all eligible premature babies fulfilling inclusion criteria and admitted in nicu were enrolled in the study after obtaining written consent from their parents. all these babies were monitored for the signs of respiratory distress using silverman’s anderson scale and were followed up every day until discharge. a detailed history, clinical examination and hospital course were recorded in pre designed study performa. respiratory support for rds was provided as per the nicu protocol of the department which are as follows • for newborn < 30 weeks’ gestation and less than 1500 gm who do not need intubation for stabilization, was started on prophylactic cpap immediately after birth even in absence of signs of respiratory distress. • for newborn < 30 week gestation and more than 1500 gm, they were observed for signs of respiratory distress using silverman’s scoring. if they developed any signs of respiratory distress, they were put on oxygen by head box. if rds score was ≥ 3 cpap was started. all new born started on cpap were re-assessed every 15 minutes. cpap was increased up to 7 cm h2o unless rds score is ≤ 3. even after ncpap of at least 7 cm for at least 15 minutes, if the infant requires fio2 < 0.30 to maintain oxygen saturation between 88 92% or rds score persistently remain < 3, early rescue surfactant was given. the srt preferably be given within two hours after birth. primary outcomes were death or discharge from hospital and the secondary outcome were bpd defined as oxygen dependency at twenty eight days of life. other outcome includes neonatal sepsis, pneumothorax and retinopathy of prematurity. data collected were entered into spss version 26 for analysis. descriptive statistics were used for continuous variables whereas frequency listings and percentages were used to describe categorical variables. the log-rank test was used to test for associations among various ga and birth weight categories presented in the kaplan-meier survival curves. the fischer’s exact and pearson’s chi square tests were used to test for associations among socio-demographic and treatment variables with the outcome death. a p-value < 0.05 was considered statistically significant at 95% confidence interval. multiple logistic regression (odds ratio) was used to determine independent variables for the outcome death. results during the study period, there was 4578 live delivery and among them 644 (10%) were preterm. 94 developed rds giving prevalence of 20.5 per 1000 live birth cohort. incidence of rds among preterm babies was 14.6%. a total of 739 inborn newborns were admitted to nicu and neonatal unit and rds accounted for 12.7% of all neonatal admissions for the study period. as demonstrated in table 1, among 94 premature neonates with rds, 49 (52.1 %) were males. the mean gestation was 31.96 ± 2.04 weeks and mean birth weight was 1581.61 ± 430.69 grams. majority of them were between 28 to < 34 weeks of gestation. incidence of rds was highest among preterm babies between 28 to < 32 weeks (80.49%). j nepal paediatr soc | vol 42 | issue 02 |may-aug, 202242 original article outcome of respiratory distress syndrome in nepal table 1. neonatal demographic characteristics and maternal risk factors for premature delivery characteristics study population (n = 119) gender male, n (%) female, n (%) 49 (52.1) 45 (47.9) mode of delivery nvd, n (%) lscs, n (%) 25 (26.6) 69 (73.4) birth weight at admission (grams) <1000, n (%) 1000 1499 grams, n (%) 1500 2500 grams, n (%) 6 (6.4) 39 (41.5) 49 (52.1) mean gestational age (± sd) 31.96 (2.04) gestational age (weeks) < 28, n (%) 28 to < 32, n (%) 32 to < 34, n (%) 34 to < 37, n (%) 3 (3.2) 33 (35.2) 33 (35.2) 25 (26.6) gestational age (weeks) 28 < to < 32 28 to < 34 32 to < 37 34 mean birth weight in grams (± sd) 1181.67 (189.24) 1332 (338.73) 1670.91 (376.13) 1841.21 (432.86) weight for gestational age according to who fetal growth chart appropriate for gestational age (aga), n (%) small for gestational age (sga), n (% large for gestational age (lga), n (%) 37 (39.4) 53 (56.4) 4 (4.3) (%) resuscitation required, n 17 (18.1) mean maternal age (± sd) 28.98 (4.64) parity primgravida, n (%) multigravida, n (%) 48 (51.1) 46 (48.9) antenatal steroids complete, n (%) incomplete, n (%) none, n (%) 54 (57.4) 26 (27.7) 14 (14.9) prom for more than 18 hours, n (%) 24 (25.5) pregnancy induced hypertension (pih), n (%) 29 (30.9) gestational diabetes mellitus (gdm), n (%) 6 (6.4) table 2 shows incidence of rds and comparison of respiratory support given to neonates according to the gestational age. incidence of rds was highest in gestational age between 28 to < 32 weeks (80.49%). majority of the enrolled preterm babies (86.18%) were managed on cpap. insure was done in 50 (52.6%) babies and one them required repeat dose. there were 15 (15.96%) deaths during the study period with six (40%) occurring within the first week of life. j nepal paediatr soc | vol 42 | issue 02 |may-aug, 2022 43 original articleoutcome of respiratory distress syndrome in nepal table 2. incidence of rds and comparison of treatment in different gestational age gestational age (weeks) < 28 (n = 3) 28 to < 32 (n = 33) 32 to < 34 (n = 33) 34 to < 37 (n = 25) total (n = 94) incidence of rds among preterm babies, (%) 21.43 80.49 40.74 4.93 14.6 primary respiratory support o2, only, n (%) cpap, n (%) mechanical ventilation (mv), n (%) 0 2 (66.7) 1(33.3) 1 (3.03) 30 (90.91) 2 (6.61) 4 (12.13) 28 (84.85) 1 (3.03) 3 (12) 21 (84) 1 (4) 8 (8.4) 81 (86.18) 5 (5.2) srt, n (%) 3 (100) 23 (69.7) 11 (30) 13 (52) (52.6) 50 mv required, n (%) primary respiratory support, n (%) cpap failure, n (%) after 96 hours of life, n (%) 2 (66.7) 1 1 0 14 (42.43) 2 6 6 2 (6.07) 1 1 0 8 (32) 1 5 2 26 (28.43) 5 13 8 death, n (%) early late 2 (66.7) 2 10 (30.31) 4 6 1 (3.04) 1 2 (8) 2 15 (15.96) 6 9 the median duration of cpap was 48 hours (range 8 192 hours). the median duration of hospital stay was 14 days (range 4 54 days). the most common complication was sepsis followed by apnea. (table 3). table 3. duration of respiratory support and morbidity profile of survived newborns duration of respiratory support: median duration of cpap (range) median duration of mv (range) median duration of respiratory support (range) median duration of hospital stay 48 (8 192) hours 96 (32 264) hours 96 (22 632) hours 14 (4 54)days complications: retinopathy of prematurity (%) bronchopulmonary dysplasia (%) pneumothorax (%) sepsis (%) apnea (%) 3 (3.2) 1 (1.1) 3 (3.2) 27 (28.7) 10 (12.7) survival by gestational age is illustrated in figure 1 by kaplan meier graph. for those with ga of 32 weeks and above, survival was 97%, 95% and 95% on day 10, 20 and 30 respectively. however, for those with ga below 32 weeks, survival was 80%, 66% and 61% on day 10, 20 and 30 respectively. from the log rank test, survival distribution of different ga categories comparing those born with a ga of < 32 weeks and ≥ 32 weeks was statistically significant (p = 0.015) indicating that having a ga above 32 weeks is significantly associated with better survival. figure 1. chance of survival by gestational age table 4 describes the maternal characteristics and risk factors for survivors versus non-survivors. lower gestational age (p = < 0.001) and prom (or 3.19; 95%ci 1.02 ± 10.05; p = 0.04) were significantly associated with higher mortality whereas nvd (or 0.240, 95% ci 0.08 ± 0.76; p = 0.01) is associated with lower mortality. j nepal paediatr soc | vol 42 | issue 02 |may-aug, 202244 original article outcome of respiratory distress syndrome in nepal table 4. comparison of maternal characteristics between survivors and non-survivors survivor n (%) non survivor n (%) relative risk p value total 79 (84.05) 15 (15.96) maternal age 29.02 ± 4.81 28.81 ± 3.77 0.88 parity 1.7722 ± .98644 1.8667 ± .91548 0.74 delivery mode nvd 17 (21.5) 8 (53.34) 0.240 (0.08 ± 0.76) 0.01 mean gestational age ± sd (weeks) < 28 (n= 3) 28 to < 32 (n = 33) 32 to < 34 (n = 33) 34 to < 37 (n = 25) 32.33 ± 1.81 1 (33.3) 23 (69.7) 32 (97) 13(92) 30.0 ± 2.14 2 (66.7) 10 (30.3) 1(3) 2(8) <0.001 maternal pih 24 (30.4) (33.33) 5 1.15 (.354 ± 3.72) 0.82 maternal heart disease 4 (5.06) 1 (6.67) 1.34 (0.14 ± 12.90) 0.80 maternal gdm 5 (6.33) 1 (6.67) (9.76 ± 0.12) 1.06 0.97 prom 17 (21.52) 7 (46.67) (10.05 ± 1.02) 3.19 0.04 twin pregnancy 8 (10.12) 1 (6.67) (5.48 ± 0.08) 0.64 0.67 aph 11 (13.93) 1 (6.67) (3.71 ± 0.05) 0.45 0.45 table 5 depicts the finding of logistic regression analysis for risk of dying for the cohort. lower birth weight (aor = 0.99; 95% ci = 0.99-0.99; p=0:01), sepsis (aor = 145.14; 95%ci = 5.04 4175.15; p = 0:004) were independently associated with increased risk of dying whereas increase duration of nicu stay decreased the risk (aor = 0.71; 95%ci = 0.54 0.91; p = 0:01). table 5. logistic regression model for mortality in rds adjusted or 95% ci p value birth weight 0.99 0.99 0.99 0.01 male sex 0.41 0.04 4.06 0.45 need of resuscitation at birth 0.23 .01 5.75 0.36 pneumothorax 36 .01 36 0.38 sepsis 145.14 5.04 – 417.51 0.004 srt 0.34 .026 4.320 0.41 mv needed 0.01 0.010.01 0.99 length of nicu stay 0.71 0.54 0.91 0.01 duration of respiratory support 1.00 0.99 1.01 0.47 apnea 0.97 .02 76.76 0.99 discussion the incidence of rds among preterm in our study was 14.6% which was similar to the incidence reported from india.5,6 lower the gestational age higher would be rds incidence. however, we observed higher incidence of rds among infant born between 28 32 weeks as compared to those < 28 week ((80.49 vs 21.43%). this contrast finding of the study may be due to the fact that only newborn admitted in nicu or neonatal unit were enrolled in the study and considering poor outcome of extremely premature babies in our setup, most of the family opted not to treat their babies. in the present study, the neonatal mortality rate due to rds was 15.94%. various studies done in nicu of medical colleges of india over last 10 years have reported the mortality rate as 44.7% to 15%.9-11 in the retrospective study done over three months period at nicu of tertiary center in western nepal among preterm newborn, the rds prevalence was found to be 14% with mortality rate of 46.15%.12 in a similar study done in kenya in 2015, the mortality rate due to rds was found to be 72.3%. in our study cpap was provided in 86.18% and 5.2% of the preterm babies were mechanically ventilated as a primary respiratory support. in the study done by sambhaji s w et al,11 87% of newborn received cpap and 13% received ventilation as compared to study done by phirke ds et al13where 78% received cpap and 22% were ventilated. in our nicu, we follow protocol of providing prophylactic cpap for newborn born less than 30 weeks and early cpap for those born above 30 weeks. this might be the plausible explanation for less frequent mechanical ventilation. incidence of bpd and rop observed in this study is low compared to the findings from western world.14 this could be due to low survival rate among babies less than 28 weeks in our center. nevertheless, resuscitation in room air and meticulous fio2 monitoring while providing respiratory support could decrease the oxidative stress and related pulmonary and eye injury. gestational age is a key factor in the survival of preterm babies world-wide. this is demonstrated in the present study as well where we found significant association between lower ga and birth with higher mortality in bivariate analysis. two thirds of the babies less than 28 weeks died hence our biggest challenge is to save lives of babies less than 28 weeks. in the present study, sepsis was observed in 28.7% of neonates and was strongly associated with neonatal mortality. studies in india have shown the incidence of sepsis ranging 31.8% to 43.5%.11,15 sepsis was the most common comorbidity among newborn with rds receiving srt. mortality due to sepsis among j nepal paediatr soc | vol 42 | issue 02 |may-aug, 2022 45 original articleoutcome of respiratory distress syndrome in nepal babies with rds ranges from 25 to 49% in the other studies done in india.9,16 sepsis rate in our study is higher than that observed in korean study17and studies from greece18 indicating importance of infection prevention practices for improving preterm survival. this is one of the few prospective studies on the outcome of rds at tertiary care center of nepal. however, there are few limitations of the study. chest x-ray was not evaluated in present study as most of the time chest x-ray was taken after surfactant administration and radiologist report was not available for many of them. since, our nicu protocol for starting respiratory support is based on the clinical monitoring, we did not analyze admission abg which was not available in many newborns. similarly, due to limited oxygen blender we could not also keep record of highest fio2 requirement in all newborns. hence, findings of chest x-ray, fio2 requirement and abg parameters could not be done analyzed as predictors of mortality. conclusions our study provides gestational age specific incidence of rds at tertiary care center of nepal. lower gestational age, birth weight and prom were significantly associated with higher mortality whereas nvd is associated with lower mortality. considerable attention should be directed toward prevention of infection for better survival of preterm babies with rds. references 1. preterm birth. world health organisation [internet]. [updated2022 mar 12]. available from: https://www.who. int/news-room/fact-sheets/detail/preterm-birth. 2. blencowe h, cousens s, oestergaard mz, chou d, moller a-b, narwal r, et al. national, regional, and worldwide estimates of preterm birth rates in the year 2010 with time trends since 1990 for selected countries: a systematic analysis and implications. the lancet. 2012 jun;379(9832):2162–72. doi: 10.1016/s0140-6736(12)60820-4. 3. kliegman rm. nelson textbook of pediatrics. 21st ed. canada: elsevier; 2020. 15739p. 4. national neonatal perinatal database [internet]. report for the year 2002-03. available from: http://www.newbornwhocc. org/pdf/nnpd_report_2002-03. pdf. 5. soll r, blanco f. natural surfactant extract versus synthetic surfactant for neonatal respiratory distress syndrome. in: the cochrane collaboration, editor. cochrane database of systematic reviews [internet]. chichester, uk: john wiley & sons, ltd; 2001. doi: 10.1002/14651858.cd000144. 6. rubaltelli f, dani c, reali m, bertini g, wiechmann l, tangucci m, et al. acute neonatal respiratory distress in italy: a one-year prospective study. acta paediatr. 2007 jan 2;87(12):1261–8. doi: 10.1080/080352598750030951. 7. world health organization. who recommendation on continuous positive airway pressure therapy for the treatment of preterm newborns with respiratory distress syndrome. extranet. available from: who.int/rhl/topics/newbornhealth/care-newborninfant/who-recommendation-continuouspositive-airwaypressure-therapy-treatment-preterm-newborns. 8. papile l-a, baley je, benitz w, cummings j, eichenwald e, et al. committee on fetus and newborn. respiratory support in preterm infants at birth. pediatrics. 2014 jan 1;133(1):171–4. doi: 10.1542/peds.2013-3442. 9. pattar m, das l. a study of morbidity and mortality profile among preterms suffering from hyaline membrane disease in a tertiary care hospital in cuttack, india. int j contemp pediatr. 2018 jun 22;5(4):1330. doi: http://dx.doi.org/10.18. 10. wagh ss, phirke ds, bantewad s. a study of clinical profile of respiratory distress syndrome (rds) in preterm babies. int. j. recent trends sci. technol. 2016;21(1):34-7. 11. femitha p, rojo j, adhisivam b, prasad k, bahubali dg, bhat vb, et al. surfactant replacement therapy (srt) in respiratory distress syndrome (rds). curr pediatr res. 2012;16(2):13436. issn 0971-9032 12. paudel l, kalakheti b, sharma k. prevalence and outcome of pre-term neonates admitted to neonatal unit of a tertiary care center in western nepal. j lumbini med coll. 2018;6(2):6 pages. doi: 10.22502/jlmc.v6i2.218. epub: 2018 dec 8. 13. phirke ds, bantewad s, khot s. study of different treatment modalities and outcome in preterm babies with respiratory distress syndrome. j evolution med dent. sci. 2017;6(72):5113-5116, doi: 10.14260/jemds/2017/1111. 14. schmolzer gm, kumar m, pichler g, aziz k, o’reilly m, cheung py. non-invasive versus invasive respiratory support in preterm infants at birth: systematic review and metaanalysis. bmj (clinical research ed). 2013;347:f5980. doi: https://doi.org/10.1136/bmj.f5980. 15. gahlawat v, chellani h, saini i, gupta s. predictors of mortality in premature babies with respiratory distress syndrome treated by early rescue surfactant therapy. j neonatal-perinat med. 2021 nov 12;14(4):547–52. doi: 10.3233/npm-190244. 16. narang a, kumar p, dutta s, kumar r. surfactant therapy for hyaline membrane disease: the chandigarh experience. indian pediatr. 2001 jun;38(6):640–6. pmid: 11418729 17. bae c-w, hahn w-h. surfactant therapy for neonatal respiratory distress syndrome: a review of korean experiences over 17 years. j korean med sci. 2009;24(6):1110. pmid: 19949668. 18. verder h, albertsen p, ebbesen f, greisen g, robertson b, bertelsen a, et al. nasal continuous positive airway pressure and early surfactant therapy for respiratory distress syndrome in newborns of less than 30 weeks’ gestation. pediatrics. 1999 feb 1;103(2):e24–e24. doi: 10.1542/peds.103.2.e24. https://doi.org/10.1016/s0140-6736(12)60820-4 https://doi.org/10.1002/14651858.cd000144 https://doi.org/10.1080/080352598750030951 https://doi.org/10.1542/peds.2013-3442 https://doi.org/10.1136/bmj.f5980 https://doi.org/10.3233/npm-190244 https://www.ncbi.nlm.nih.gov/pubmed/19949668 https://doi.org/10.1542/peds.103.2.e24 nepas journal 32-1 cs5 final.indd original article <1>j. nepal paediatr. soc. antropometric measurements in different ethnic groups of nepalese new borns malla k1, mall t2, rao s3, gauchan e4, basnet s5, koirala dp6 1dr. kalpana k malla, mbbs, md, associate professor, 2dr. tejesh malla, mbbs, md, associate professor, 3sheshagiri rao, mbbs, md, professor, 4dr. eva gauchan, mbbs, md, lecturer, 5dr. sahisnuta basnet, mbbs, md, lecturer, 6dr. deepak prasad koirala, mbbs, md, lecturer. all from the department of paediatrics, manipal college of medical science, pokhara, nepal. address for correspondence: dr. kalpana malla, e-mail: kalpana_malla@hotmail.com abstract introduction: there is a wide variation in normal birth weight, length and head circumference of newborns. the standards formulated by western workers may not be very reliable to this part of world because of wide variations in normal range of length, weight and head circumference in different ethnic groups. this study aims to determine the anthropometric values – birth weight, length and head circumference in term and preterm newborns of different ethnic groups in this region of the country and to see if this can be used as a standard for taking the anthropometric measurements. materials and methods: a prospective study of 600 newborns born in manipal teaching hospital, pokhara from july 2009–june 2010. a detailed anthropometric measurement (weight, length, and head circumference) of all newborns was taken on 3rd day of life. results: six major ethnic groups were noted brahmin, gurung, dalit, chettri, magar and newar. there were 54 % males and 46% females among which 18.16% were preterms, 20.66% small for gestation age, 81.50% term and 0.33% posterm. the mean weight, length and head circumference of term babies were 2.817±0.61 gms, 47.68±2.48 cm, 33.56±2.02 cm and for preterm babies it was 2.215± 0.41 gms, 46.36±2.39 cm, 32.23±2.03 cm respectively. there were 25% low birth weight (n=151, highest number in brahmins-27%), 74.16% normal weight (n=445) and 0.66% over weight (n=4, all were gurungs) babies. in term newborns weight, length and head circumference was noted to be highest in gurungs (3.3004gms, 49.35cm, 34.72cm) and was statistically significant (p<0.000). weight and length of brahmins was lowest (2.578 gms, 45.49cm) and head circumference was lowest in dalits (30.88cm, statistically significant<0.000). in case of preterms highest weight and length was seen in magars (2.387gms, 47.90cm) but head circumference was highest in gurungs (34.18cm) whereas weight was lowest in chettri (2.1609gms), length in brahmin (44.61cm) and ofc in dalits (29.92cm). these parameters were directly proportion with gestation age and was statistically significant (p<0.000). conclusion: the present study highlights the mean weight, length and head circumference of term and preterm newborns in different ethnic groups and gestation age. these parameters were directly proportion to gestation age but were variable in different ethnic groups. therefore a study in larger population could give us a different standard for anthropometric measurements in nepalese newborns. key words: anthropometry measurements, n.ewborn, nepal introduction there is a wide variation in normal birth weight, length and head circumference of newborns. diff erent standards of norms are used in diff erent parts of the world. therefore the standards formulated by western workers may not be very reliable to this part of world because of wide variations in normal range of length, weight and head circumference in diff erent ethnic groups. this study aims to determine the anthropometric values – birth weight, length and head circumference in term and preterm newborns of diff erent ethnic groups in this region of the country and to see if this can be used as a standard for taking the anthropometric measurements. materials and methods this is prospective observational study which was undertaken over a period of 1 year from july 2009 to june 2010. cases were enrolled from postnatal ward, these included babies delivered in manipal teaching hospital, pokhara (a tertiary care hospital of western region of the january-april, 2012/vol 32/issue 1 doi: http://dx.doi.org/10.3126/jnps.v32i1.4880 <2> j. nepal paediatr. soc. country). consent was taken from mothers after they were explained about the measurements. a total of 2000 healthy term and preterm neonates were subjected to clinical examination. gestational age was assessed according to the fi rst day of last menstrual period and was cross checked by ballard scoring1. anthropometric parameters that included; weight, length and occipito frontal circumference (ofc) were recorded on day 3 of life so that the error due to skull molding and caput succedaneum for the head circumference and excess body fl uid loss in the initial days for weight loss could be eliminated. common ethnic groups noted were brahmins, gurungs, chettris, dalits, magars and newars. out of these 2000 babies 100 consequitive babies from each of the above mentioned ethnic groups (n= 600) were selected for the study. the remaining 1400 babies were excluded from the study. normal and caeserian delivered babies of any sex, gestation age >32weeks and those from a high socioeconomic background (family income >5000 rupees per month) were also included in the study. exclusion criteria consisted of babies with congenital anomalies, chromosomal anomalies, babies measured 3 days before or after birth, gestational age ≤ 32 weeks, birth weight ≤ 1500gms, preterm babies with complications, ill babies and maternal illness during antenatal period. detail anthropometric measurements were taken by the same persons to minimize subjective error. same weighing scale and same measuring tape were used to take the weight and head circumference respectively. infantometer was used to take the length. data collected was tabulated and analyzed using statistical package spss 19.0 version. microsoft excel (2003) and spss were used for plotting fi gures. two tailed t-test was used to compare the anthropometric parameters (birth weight, length, head circumference) of diff erent ethnic groups. results there were 326/600 (54%) males and 274/600 (46%) females (figure 1). classifi cation of newborns in relation to gestation age showed that small for gestation age (sga) babies (20.66%) were more than the preterm babies (18.16%, table 1). there were 77.16% appropriate for age (aga) and 2.16% large for gestation age (lga) babies. highest number of both sga (27.41%) and preterms (33.02%) were observed in the brahmin population (figure 2). preterms were higher in brahmin, newar and magar and sga was higher in dalit, gurung and chettri populations (figure 2). 9/13 lga was noted in gurungs (figure 2 and table 1). the mean±sd for weight, length and ofc for term babies were 2.8177±0.61gms, 47.68±2.48cm, 33.56±2.02cm respectively and for preterm babies it was 2.2153±0.41gms, 46.36±2.39cm, 32.23± 2.03 cm respectively (table 2). table 2 also shows these parameters in diff erent ethnic groups where the mean±sd for weight, length, ofc for brahmin was 2.5788±0.59gms (term), 2.2016±0.49gms (preterm); 45.49±3.48cm (term) 44.61±3.22cm (preterm); 33.41±2.29cm (term) 33.02 ±2.16cm (preterm) respectively. similarly for gurung this was 3.3004±0.58gms (term) 2.1166±0.48gms(preterm); 49.35±1.41cm (term), 47.83±1.38cm(preterm); 34.72±0.81cm (term), 34.18±0.78cm(preterm); dalit 2.6079± 0.43gm (term), 2.2153±0.41gms (preterm); 46.49±2.22cm (term), 45.07±2.16cm (preterm); 30.88 ±1.96cm (term), 29.92±1.87cm (preterm); chettri 2.7305±0.52gms (term) 2.1609±0.38gms (preterm; 47.93±1.84cm (term), 47.45cm±1.81 (preterm; 34.08±1.10cm (term), 33.81±0.98cm (preterm); magar 2.8870±0.6gms (term), 2.3875±0.87gms (preterm), 48.49±1.61cm (term), 47.90±1.56cm (preterm); 33.79±1.52cm (term), 33.55±1.46cm (preterm); newar 2.8000±0.56gms (term), 2.3260±0.48gms (preterm); 48.33±1.37cm (term) 47.69±1.28cm (preterm); 34.51±1. (term) 34.09±1.27cm (preterm) respectively. figure 3 shows weight and figure 4 shows ofc in diff erent ethnic groups where it is observed that highest number of low birth babies (lbw) was found in brahmins (27.15%) followed by magar (19.86%), newar (17.21%), dalit (14.56 %), chettri (13.90%). only 7.28% lbw was seen in gurung population (figure 3). ofc was < 33cm in maximum babies (80%) in dalits, which was statistically signifi cant (table 3). correlation of weight, length and ofc with gestation age was also seen (table 4) and these parameters were directly proportion to gestation age and was statistically signifi cant (p<0.000). female 46% male 54% fig 1: distribution of sex of babies antropometric measurements in different ethnic groups of nepalese new borns <3>j. nepal paediatr. soc. table 1: mean birth weight for gestational age in six ethnic groups. gestation age sga aga lga total brahmin : preterm term posterm 19 15 0 17 49 0 0 0 0 36 64 0 gurung: preterm term posterm 3 4 0 3 81 0 0 9 0 6 94 0 dalit : preterm term posterm 6 12 0 7 75 0 0 0 0 13 87 0 chettri: preterm term posterm 9 10 0 2 75 2 0 2 0 11 87 2 magar: preterm term posterm 10 14 0 10 64 0 0 2 0 20 80 0 newar: preterm term posterm 13 9 0 10 68 0 0 0 0 23 77 0 total 124 (20.66%) 463 (77.16%) 13 (2.16%) 600 *preterm (<37 weeks) n=109 (18.16%), **term (37-42 weeks) n= 489 (81.50%), ***posterm (>42 weeks) n=2 (0.33%), ***small for gestation age n=124 (20.66%) table 2: mean± sd of weight, length and ofc of babies in diff erent ethnic groups ethnic group of babies mean weight ± sd mean length± sd mean ofc± sd brahmin: term preterm 2.5788±0.59632 2.2016 ± 0.49012 45.4900±3.48299 44.6109±3.22456 33.4100±2.29666 33.027±2.16751 gurung: term preterm 3.3004±0.58373 2.2166± 0.48465 49.3500±1.41689 47.8331±1.38796 34.7200±0.81749 34.1830±0.78910 dalit : term preterm 2.6097±0.43009 2.2153±0.41981 46.4900±2.22699 45.0760±2.16589 30.8800±1.96062 29.9230±1.87675 chettri: term preterm 2.7305±0.52709 2.1609±0.38976 47.9300±1.84913 47.4541±1.81987 34.0800±1.10718 33.8180±0.98478 35.00% 30.00% 25.00% 20.00% 15.00% 10.00% 5.00% 0.00% brahmin chettri magar newar preterm sga gurung dalit fig 2: distribution of preterm and sga babies in diff erent ethnic groups malla k et al <4> j. nepal paediatr. soc. magar: term preterm 2.8870±0.65738 2.3875±0.8798 48.4900±1.61117 47.9000±1.56689 33.7900±1.52617 33.5500±1.46876 newar: term preterm 2.8000±.56707 2.3260±0.48065 48.3300±1.37109 47.6956±1.2887 34.5100±1.30651 34.0998±1.27651 total: term preterm 2.8177±0.61149 2.2153±0.41075 47.6800±2.48083 46.3600±2.39100 33.5650±2.02961 32.2310± 0.03891 table 3: comparison of anthropometric measurements in diff erent ethnic groups t df p value t df p value brahmin vs gurung weight length ofc -8.647 -10.266 -5.374 198 198 198 0.000 0.000 0.000 gurung vs newar weight length ofc 6.149 5.173 1.363 198 198 198 0.000 0.000 0.000 brahmin vs dalit weight length ofc -.420 -2.419 8.378 198 198 198 0.675 0.016 0.000 dalit vs chettri weight length ofc .171 -1.279 -13.519 198 198 198 0.864 0.203 0.000 brahmin vs chettri weight length ofc -1.906 -6.188 -2.628 198 198 198 0.058 0.000 0.009 dalit vs magar weight length ofc -1.289 -1.279 -9.522 198 198 198 0.199 0.203 0.000 brahmin vs magar weight length ofc -3.472 -7.817 -1.378 198 198 198 0.001 0.000 0.170 dalit vs newar weight length ofc .186 -1.396 -10.446 198 198 198 0.754 0.138 0.000 brahamin vs newar weight length ofc -2.688 -7.587 -4.163 198 198 198 0.008 0.000 0.000 chettri vs magar weight length ofc -1.461 .000 2.443 198 198 198 0.146 1.000 0.015 gurung vs dalit weight length ofc -.469 3.590 17.910 198 198 198 0.640 0.000 0.000 chettri vs, newar weight length ofc -.898 -1.738 -2.511 198 198 198 0.370 0.084 0.013 gurung vs chettri weight length ofc -.315 2.809 4.820 198 198 198 0.753 0.005 0.000 magar vs newar weight length ofc 1.002 .756 -3.584 198 198 198 0.318 0.450 0.000 gurung vs magar weight length ofc -1.643 2.809 6.482 198 198 198 0.102 0.005 0.000 antropometric measurements in different ethnic groups of nepalese new borns <5>j. nepal paediatr. soc. 100.00% 90.00% 80.00% 70.00% 60.00% 50.00% 40.00% 30.00% 20.00% 10.00% 0.00% brahmin newargurung lbw normal weight overweight dalit chettri magar fig 3: distribution of weight in diff erent ethnic groups ***lbw (<2500gms, n=151) 25%, **normal weight (2500gms-3.999gms, n=445) 74.16%, * overweight (>4000gm, n=4) 0.66% table 4: correlation of weight, length and ofc with gestation age gestation age of children weight spearman correlation value approx. sig p value normal lbw lga n0. % no. % no. % <37 weeks (n=109) 25 22.9 84 77 0 0 0.544 0.000 37-42 weeks (n=489) 418 85 67 13.7 4 0.81 >42 weeks (n=2) 2 100 0 0 0 0 total (n=600) 445 100 151 0 4 0 gestation age of children length spearman correlation value approx. sig <45cm 45-50cm >50cm no. % no. % no. % <37 weeks (n=109) 30 27.5 78 71.55 1 0.91 0.262 0.00037-42 weeks (n=489) 34 6.92 405 82.8 50 10.22 >42 weeks (n=2) 0 0 1 50 1 50 total (n=600) 64 485 51 gestation age of children ofc spearman correlation value approx. sig <33cm 33-35cm >35cm no. % no. % no. % <37 weeks (n=109) 38 34.86 71 65.13 0 0 0.146 0.006 37-42 weeks (n=489) 107 21.88 351 71.77 31 6.33 >42 weeks (n=2) 0 0 1 50 1 50 total (n=600) 145 424 31 malla k et al <6> j. nepal paediatr. soc. discussion the term anthropometric refers to comparative measurements of the body and is important indicator of child survival. the periodic measurement of anthropometric variables in diff erent populations and regions of a country refl ect changes in nutrition and health status of the country and is a reliable tool to evaluate social health2,3. the anthropometric measurements commonly used as indices of growth include length, weight, and head circumference. these measurements utilized at present time are based on the results obtained for more than fi ve decades, which are not able to determine a national pattern most likely due to some ethnic infl uences4 in addition genetic diff erences exist among races regarding growth and body composition3. some investigators have also argued for ethnic-specifi c standards 5,6. although the world health organization (who) advises a single international growth standard for developing countries7,8, the ideal is to establish local national growth charts refl ecting each country’s own genetic characteristics. hence this analytical descriptive study was carried out to observe the diff erences in these parameters in diff erent gestation age and ethnic groups. in this study, out of 600 newborns there were 326 (54%) males and 274 (46%) females. brahmin, gurung, dalit, chettri, magar and newar were the 6 major ethnic groups attending this hospital for delivery. there were 124(20.66%) sga {birth weight < 10th percentile for gestational age}, 109 (18.16%) preterms {born at < 37 completed weeks}, 463(77.16%). appropriate for age (aga) and 13 (2.16%) large for gestation age (lga weight 90th percentile for gestational age) babies using the reference ranges provided by alexander et al9. our fi nding was diff erent from another study10 where prematurity was observed in 25.5% and sga in 14.1% of the neonates. the reason for this may be that the adverse intrauterine environment and other risk factors vary in diff erent countries. highest number of sga (27.41%) and preterms (33.02%) were seen in brahmin compared to other ethnic groups. whereas highest number of large for date babies (lga) 9/13 was seen in gurung population. number of preterms was higher than sga in brahmin, newar and magar and sga was higher than preterms in dalit, gurung, chettri populations. prematurity and sga prevalence refl ects an adverse intrauterine environment; hence comparisons of risk factors at birth like primiparous vs multiparous mothers, smoker’s vs nonsmokers, twin’s vs singletons, in detail should be considered which was not seen in this study. if these factors were considered maybe we could explain why in some ethnic groups sga was common and preterm in others. the observed mean± sd of weight, length and ofc for term babies were 2.8177±0.61 gms, 47.68±2.48cm, 33.56±2.02cm respectively and for preterm babies it was 2.2153±0.41075 gms, 46.36±2.39 cm, 32.23±2.03 cm respectively. the fi ndings of term babies were comparable to study conducted by manandhar k, manandhar ds et al 11 in kathmandu and s muthayya, p dwarkanath et al12 in south india. this similarity could have been due to similar population and cultural backgrounds. in another study conducted by b. telatar, s. comert et al13 mean birth weight, height and head circumference was 3334 (sd 494) gm, 48.3 (sd 2.2) cm and 34.4 (sd 1.3) cm respectively which was greater than our study. yet another study by nahar et al14 reported a birth weight of 2690gm (sd= 0.36) which was fig 4: ofc in diff erent ethnic groups 90% 80% 70% 60% 50% 40% 30% 20% 10% 0% brahmin gurung newardalit chettri magar brahmin gururng dalit chettri magar newar <33 cm 20% 0% 80% 11% 24% 10% 33-35 cm 75% 90% 20% 89% 76% 74% >35 cm 5% 10% 0% 0% 0% 16% antropometric measurements in different ethnic groups of nepalese new borns <7>j. nepal paediatr. soc. lower than in our study. these two studies were carried out in iceland and bangladesh respectively and the diff erences in demographic factor, diff erent population and cultural backgrounds again could have made a diff erence in the results. a study by hickey states that sociocultural dimensions, ethnicity, social support etc can also aff ect the gestational weight gain15. the mean weight of preterm babies was much less (1780 gm) in another study16 compared to ours (2.2153gms). the reason for this is that in our study gestation age ≤32 weeks and weight ≤ 1500gms was not taken into consideration whereas all preterms was included in above mentioned study. for preterm comparable study was not available for length and ofc. these parameters when compared with diff erent ethnic groups showed that in term newborns weight, length and ofc was noted to be highest in gurungs (3.3004gms, 49.35cm, 34.72cm) and was statistically signifi cant (p<0.000).weight and length of brahmins was lowest (2.578 gms, 45.49cm) and ofc was lowest in dalits (30.88cm, statistically signifi cant<0.000). in case of preterms highest weight and length was seen in magars (2.387gms, 47.90cm) but ofc was highest in gurungs (34.18cm) whereas weight was lowest in chettri (2.1609gms), length in brahmin (44.61cm) and ofc in dalits (29.92cm). the diff erence between the diff erent ethnic groups could be due to varying, socioeconomic, nutritional and environmental factors of the underlying population as already mentioned earlier. factors other than ethnicity include altitude, maternal size, parity, smoking, parental social position which can aff ect fetal growth and could confound the ethnic diff erences which has been observed in numerous other studies5,6,17, 18. these factors were also present in our study but was not analyzed in detail. who defi nes low birth weight (lbw) as birth weight less than 2500gms. the defi nition of lbw does not take into account the gestation period.lbw observed in our study was 25% which is lower than that observed in another study which was 37.75 %10. even within the country 29.8% and 28.6% lbw was noted in two other studies carried out in western region hospital, pokhara in 2004 (7 years ago)19 and sarlahi in 2007(5years ago)20. the fi rst study was done in same city pokhara but it is observed that in present study the rate of lbw has decreased. the probable cause for this diff erence may be the low educational and socioeconomic status of the mothers in the previous study. as western region hospital is a government hospital, so mothers attending that hospital are probably from lower socioeconomic group. lower birth weight in this study indicates the poor socioeconomic status of the mothers. decrease in lbw cases in 7 years time may also indicate improvement in health status of the country, awareness in people regarding antenatal care. yet in another study21 carried out in same city pokhara 3 years ago (2008) out of 400 newborns only 8.5% was found to be lbw, which is much lower than our fi ndings. this diff erence may be due to the fact that their study included only term babies whereas our study has included term as well as preterm babies. highest number of low birth babies (lbw) were found in brahmins (27.15%) followed by magar (19.86%), newar (17.21%), dalit (14.56%), chettri (13.90%). only 7.28% lbw was seen in gurung population. the reason for the gurungs to be heavier, longer, and with larger heads than other groups may be due to the fact that genetically they are of heavy built and has a good nutrition status. a data analysis done in 200419 where the group was divided as indo-aryan (brahmin, chettris of our group) and lower caste (dalit of our group) ethnic groups had signifi cantly lower weight babies than tibeto-burman (gurung, magar of our group) and newar groups. this was consistent with our study. like their fi ndings brahmin and dalit had lower weight than other ethnic groups. again they found that tibeto – burman group and newars had higher birth weight, this was again similar with our study. this similarity in the fi ndings may support our fi nding in establishing ethnic specifi c standard. other two parameters of length and head circumference in diff erent ethnic groups were also compared in our study. a comparable study for this in diff erent ethnic groups of the country was not available. hence we can state that possibly this is the fi rst study of this kind, so it is not possible to conclude anything for these parameters. however irrespective of any ethnic groups the mean length and ofc of nepalese newborns noted by manandhar k, manandhar ds et al11 was 49cm and 33.8cm respectively and mean ofc noted by sreeramareddy ct, chuni n, patil r, singh d, shakya b21 was 33.5cm which was comparable to our study. correlation of gestation age with weight, length and ofc was also seen and these parameters were directly proportion to gestation age and was statistically signifi cant (p<0.000). similar correlation was also observed in another study22. conclus ion periodical updating of anthropometric variables in diff erent populations in a country is necessary to refl ect ongoing changes in local conditions. the limitation of this study is that the anthropometric values obtained refl ect the results of only one hospital and a limited population; hence generalization to the whole nepalese population cannot be made. furthermore our study is populationbased, rather than hospital-based, which makes the selection biased. other factors which could aff ect fetal malla k et al <8> j. nepal paediatr. soc. growth were not analyzed. despite these limitations, we believe that our results could provide a useful analytic comparison tool for future research. therefore we would reccomend parallel studies to be made in various regions of the country comprising multiple socio-economic, nutritional, cultural backgrounds, so that comparative analyses could be made with newfound results. the practical application of ethnic-specifi c standards requires further research in a larger scale. acknowledgements: none funding: none confl ict of interest: none permission from irb: yes references 1. ballard jl, khoury jc, wedig k, et al: new ballard score expanded to include extremely premature infants. j pediatr 1991;119:417-23. 2. tanner jm. growth as a mirror of the condition of society: secular trends and class distinctions. acta pediatric japonica 1987;29:96–103. 3. neyzi o, saka hn. anthropometric studies in turkish children. istanbul med faculty j 2002;65: 221–8. 4. k.  kalra, n.  kishore, r.  s.  dayal: anthropometric measurements in the newborn. indian j pediatr 1967:34:73-82. 5. davies dp, se nior n, cole g, blass d, simpson k: size at birth of asian and white caucasian babies born in leicester: implications for obstetric and pediatric practices. early hum dev 1982;6:257-63. 6. wang x, guyer b, paige dm: differences in gestational age-specific birth weight among chinese, japanese and white americans. int j epidemiol 1994;23:119-28. 7. ulijaszek sj. between population variation in pr-adolescent growth. european j clin nutrition 1994;48:5–13. 8. a growth chart for international use in maternal and child health care: guidelines for primary health care personnel. geneva, world health organization, 1978. 9. alexander gr, himes jh, kaufman rb, mor j, kogan m. a united states national reference for fetal growth. obstet gynecol 1996;87:163-8 10. gozal d, ndombo pk, ze minkande j, kago i, tetanye e, mbede j.anthropometric measurements in a newborn population in west africa: a reliable and simple tool for the identification of infants at risk for early postnatal morbidity. j pediatr 1991;118(5):800-5. 11. manandhar k1, manandhar ds2, baral mr3one year follow up study of term babies born 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badgley r, bain hw: birth weight, length, head circumference and bilirubin level in indian newborns in the sioux lookout zone, northwestern ontario. can med assoc j. 1984;131:453-56 19. pratima poudel acharya;  fiona alpass birth outcomes across ethnic groups of women in nepal. health care for women international 2004; 25(1)40–54. 20. l c mullany; g l darmstadt; s k khatry; s c leclerq; j m tielsch relationship between the surrogate anthropometric measures, foot length and chest circumference and birth weight among newborns of sarlahi, nepal. eur j clin nutr 2007;61:40-6. 21. sreeramareddy ct, chuni n, patil r, singh d, shakya b.anthropometric surrogates to identify low birth weight nepalese newborns: a hospital-based study. bmc pediatr 2008;25;8:16. 22. b. f. kalanda, s. van buuren, f. h. verhoeff , b. j. brabin. anthropometry of malawian live births between 35 and 41 weeks of gestation: annals human biol 2005;32(5):639–49. antropometric measurements in different ethnic groups of nepalese new borns how to cite this article ? malla k, mall t, rao s, gauchan e, basnet s, koirala dp. antropometric measurements in diff erent ethnic groups of nepalese new borns. j nepal paediatr soc 2012;32(1):1-8. j nepal paediatr soc | vol 42 | issue 02 |may-aug, 2022 71 original articlenasal anthropometry of indian neonates original article doi: 103126/jnps.v4113 adel moideen1, ayesha erum hadi2, apurv barche3, sneha jaganathan andrade3, aditya verma3, leslie edward lewis3, jayashree purkayastha3 1department of nephrology, university of toronto, toronto, ontario, canada 2department of radiodiagnosis, mcmaster university, hamilton, ontario, canada 3department of paediatrics, kasturba medical college manipal, manipal academy of higher education (mahe) karnataka 576104, india. role of neutrophil cd64 in the diagnosis of neonatal sepsis introduction: neutrophil surface cd64 (cluster of differentiation 64), the highaffinity fc receptor, is quantitatively up-regulated during infection and sepsis. the diagnostic utility of ncd64 as a reliable marker of neonatal sepsis has not been explored so far. hence this study has been conducted to compare ncd64 with other currently used infection markers including total leucocyte count, platelet count, absolute neutrophil count (anc), band:neutrophil ratio and highly sensitive c reactive protein (hs-crp). methods: consecutively born neonates between march 2014 to november 2014 were enrolled with documented sepsis (n = 81), clinical sepsis (n = 35), and no sepsis (n = 87). ncd64 was analyzed by flow cytometry. results: sepsis episodes had a higher median cd64 index of 10.35 (range: 15.88, 6.87) as against 2.97 (range: 5.53, 1.64) in the control group (p < 0.001). the percentage of ncd64 positive cells was also significantly higher in the sepsis group compared to the control group (63.90 ± 2.67 vs 15.07 ± 1.95; p = 0.001). in the roc curve analysis ncd64, percentage of ncd64 positive cells had the highest auc (auc-0.914) using a cutoff of 28.01%, followed by cd64 mean fluorescence intensity (mfi) with an auc of 0.850 using a cutoff of 5.54. ncd64 was significantly elevated in the groups with documented and clinical sepsis (p < 0.001). conclusions: ncd64 is a highly sensitive marker for neonatal sepsis. prospective studies incorporating ncd64 into a sepsis scoring system are warranted. abstract *corresponding author jayashree purkayastha professor, department of paediatrics, kasturba medical college manipal, manipal academy of higher education (mahe) karnataka 576104, india. email: jayashreepurkayastha@yahoo.com article history received on : 18 sep, 2021 accepted on : 17 aug, 2022 funding sources: none conflict of interest: none keywords: cd64; hs-crp; neonatal sepsis; neutrophil online access doi: https://doi.org/10.3126/jnps.v42i2.39821 introduction neonatal sepsis is a clinical syndrome of bacteremia with systemic signs and symptoms of infection with in the first 28 days of life. in a country like india and other developing countries, neonatal sepsis is the single most important cause of neonatal deaths in the community, accounting for over half of them. successful treatment depends on early initiation of appropriate antibiotic therapy, but early diagnosis of neonatal bacterial copyrights & licensing © 2022 by author(s). this is an open access article distributed under creative commons attribution license (cc by nc ) j nepal paediatr soc | vol 42 | issue 02 |may-aug, 202272 original article neutrophil cd64 in neonatal sepsis infections is difficult because clinical signs are non-specific and may initially be subtle.1,2 neonatal sepsis is categorized as early or late onset. although advances in neonatal intensive care have led to improved survival of very low birth weight (vlbw) infants, late-onset sepsis continues to be an important cause of morbidity and mortality.3-5 very few modalities are available for investigating neonatal sepsis. as such, positive blood cultures still remain the gold standard for the diagnosis of sepsis, but many times the cultures may be negative even in a symptomatic neonate. when blood culture is negative we have to depend on other parameters for the diagnosis of neonatal sepsis. these tests should be fast and reliable. one of these is neutrophil cd64 which is supposed to be very specific for neonatal sepsis. cd64 (cluster of differentiation 64) is a type of integral membrane glycoprotein known as an fc receptor that binds monomeric igg-type antibodies with high affinity; more commonly known as fc-gamma receptor 1 (fc≤r1).6 it is expressed at low concentration on the surface of nonactivated neutrophils making normal ranges easy to define and independent of ethnic background, genetic influences or gender.6 its up-regulation is induced by granulocyte colony stimulating factor (g-csf) and interferon gamma (inf-y) within four to six hours of stimulation, five to 10 fold increase during sepsis, under the influence of inflammatory cytokines; this increase in surface density occurs in a graded manner dependent on the intensity of the cytokine stimulus.6 the purpose of this study was to measure the neutrophil cd64 in blood as an adjunct to our previously validated hematologic scoring system for detecting neonatal sepsis. methods in this prospective observational study, all neonates presenting with clinical features suggestive of sepsis at the neonatal intensive care unit of a tertiary care hospital from march 2014 to november 2014 were eligible for inclusion. neonates who had received antibiotic therapy for < 72 hours were excluded. informed consent was taken from all parents prior to the inclusion in the study. this study was approved by the research ethics board of the institution. neonates were selected for the study if they had two or more of the following clinical features:7 respiratory changestachypnea, apnea, increased ventilatory support, or desaturation; cardiovascular changes heart rate variability, pallor, decreased perfusion or hypotension; metabolic changes hypothermia, hyperthermia, feed intolerance, glucose instability (hypo / hyperglycemia), or unexplained metabolic acidosis; neurologic changes lethargy, poor suck, convulsion, hypotonia or decreased activity; skin infections abscess, pyoderma, bruising. as part of the evaluation, detailed history with clinical examination was done and demographic, clinical and laboratory data were collected for all the neonates. in all the cases, neonatal details like date of birth, gestational age, gender and birth weight were collected. antenatal details including parity, gestational age, presence of maternal fever, prolonged rupture of membranes (prom), chorioamnionitis, urinary tract infection (uti) and pretreatment with antimicrobials whenever relevant were collected. mode of delivery, place of birth, resuscitation methods and apgar score were recorded. blood was drawn for a complete blood count along with blood culture, crp, ncd64 and cd64 index. neonatal age at the time of obtaining blood culture, physical examination details and antibiotic pretreatment were also recorded. all blood cultures were collected by using standard sterile techniques. the bactec microbial detection system (becton-dickinson, sparks, md) was used to detect positive blood cultures. the details of the organism isolated and the timing of the positive signal were obtained from the microbiology department. each positive blood culture result was grouped as either drawn < 72 hours (early onset) or ≥ 72 hours of life (late-onset). other investigations like urine routine, chest x-ray and cerebrospinal fluid (csf) study were done as and when required. accordingly, neonates were grouped as blood culture positive and negative sepsis, and no sepsis and were followed up. the following hematological criteria were used as indicators of sepsis: tlc: < 5,000 / cu.mm or < 20,000 / cu.mm, anc: considered low if below normal for age as per the manroe’s chart for term and mouzinho’s chart for vlbw infants, i:t ratio: < 0.3 in term and < 0.2 in preterms, platelet count: < 1,50,000/ cu.mm. measurement of ncd64 index: 50 µl of heparinised whole blood was incubated for 30 minutes at room temperature with a saturating amount of phycoerythrin conjugated ncd64 in dark condition. this was followed by ammonium chloridebased red cell lysis (for 12 15 mins). the counting beads were added to the sample and mixed thoroughly. sample was acquired in facs calibur using cell quest pro software. a minimum of 10,000 cells data was saved. the saved data was analysed. the study data was processed using spss v16.0. for the analysis of baseline characteristics of study group, descriptive statistics was used. median values were described with interquartile range of 75th percentile 25th percentile. for the variables following normal distribution curve, mean and standard error mean were computed. the chi-square test was used in the analysis of categorical variables between groups. receiver operating characteristic curves were generated and analyzed for the area under the curve (auc). significance was assessed at 5% level of significance. a p-value of < 0.05 was considered statistically significant. results we included 116 neonates who had clinical features of sepsis, after excluding 40 neonates who had received antibiotics for < 72 hours prior to admission. of the enrolled 116 neonates, blood culture was positive in 81 neonates (documented sepsis) and 35 were grouped as culture negative sepsis (clinical sepsis). the blood culture positive and negative groups were further subgrouped as early onset sepsis (eos) and late onset sepsis (los). healthy term and preterm neonates for whom blood was drawn for routine serum bilirubin estimation, with no evidence of sepsis were selected as controls. flowchart (figure 1) shows the study recruitment done. j nepal paediatr soc | vol 42 | issue 02 |may-aug, 2022 73 original articleneutrophil cd64 in neonatal sepsis table 1. comparison of auc values of various hematological parameters from receiver operating characteristic (roc) curves variable auc mean ± se (95% confidence interval) platelet 0.721 ± 0.036 (0.650 0.792) anc 0.645 ± 0.038 (0.570 0.720) i:t ratio 0.645 ± 0.038 (0.570 0.720) hs-crp 0.826 ± 0.028 (0.771 0.882) cd64 mfi 0.850 ± 0.039 (0.791 0.908) % ncd64 positive cells 1.914 ± 0.019 (0.878 0.951) figure 1. study flow chart table 2. baseline characteristics of study population variable sepsis (cases) n = 116 no sepsis (controls) n = 87 sex – male female 84 (72.4%) 32 (27.6%) 55 (63.2%) 32 (36.8%) singleton multiple 112 (96.6%) 4 (3.4%) 84 (96.6%) 3 (3.4%) age at sepsis evaluation: (mean ± sem, weeks) 35.99 ± 0.43 35.53 ± 0.39 inborn outborn 22 (19%) 94 (81%) 41 (47.1%) 46 (52.9%) gestation: preterm (≤ 33 weeks) late preterm (34 36 weeks) term (≥ 37 weeks) 26 (22.4%) 18 (15.5%) 72 (62.1%) 24 (27.6%) 19 (21.8%) 44 (50.6%) mode of delivery: vaginal assisted vaginal lscs (37.9%) 44 (3.4%) 4 (58.6%) 68 24 (27.6%) 3 (3.4%) 60 (69.0%) age at sepsis evaluation: < 72 hrs < 72 hrs (32.8%) 38 (67.2%) 78 72 (82.8%) 15 (17.2%) asphyxia: present absent (19%) 22 (81%) 94 10 (11.5%) 77 (88.5%) prom: present absent (16.4%) 19 (83.6%) 97 18 (20.7%) 69 (79.3%) blood culture yield in the early onset sepsis group was 60.5% as against the blood culture yield of 74.4% in the late onset sepsis group. of 116 septic neonates, pathogenic organisms could be isolated from 81 (70%) cases: 19 coagulase-negative staphylococcus (cons), 19 klebsiella pneumoniae, nine candida, seven enterobacter, six – escherichia coli, five burkholderia, three methicillin resistant staphylococcus aureus (mrsa), three streptococcus, two pseudomonas, one non fermenting gram negative bacilli (nfgnb), two serratia, two staphylococcus aureus, one acinetobacter and one kodamea spp. on the basis of the sepsis scoring system using hematologic variables, septic neonates (n = 116) were characterized by significantly higher white blood cell counts, anc’s and immature / total neutrophil ratios, compared with the control group (n = 87), as well as lower platelet counts (p < 0.05 for all comparisons) (table 3). table 3. haematological profile of cases and controls variable sepsis (cases) n = 116 median (75th, 25th iqr) no sepsis (controls) n = 87 median (75th, 25th iqr) p value hemoglobin (gm / dl) 14.7 (17.07, 12.55) 16.2 (18.2, 14.40) 0.006 total count (cu. mm) 9700 (16700, 5853) 12900 (18900, 9738) 0.001 anc (cu. mm) 3810 (8197.50,1795.50) 6365 (11300, 3870) 0.001 platelet count (lakhs / cu.mm) 1.03 (2.63, 0.34), 2.36 (2.86, 1.75) 0.001 i:t ratio 0.115 (0.33, 0.00) 0.00 (0.10, 0.00) 0.002 hs-crp (mg / l) 47.5 (120.82, 9.2) 4.5 (8.90, 1.1) 0.001 cd64 mfi 10.35 (15.88, 6.87) 2.97 (5.53, 1.64) 0.001 there was statistically significant difference in the hs-crp levels in the sepsis group compared to the control group (47.5 with range: 120.8 9.2 vs 4.5 with range: 8.9 1.1; p = 0.001). those with sepsis also demonstrated significantly higher cd64 mfi (mean fluorescence intensity) values than those with no sepsis (10.35 with range: 6.87-15.88 vs 2.97 with range 1.64 5.53; p = 0.001) (table 3). j nepal paediatr soc | vol 42 | issue 02 |may-aug, 202274 original article neutrophil cd64 in neonatal sepsis figure 2. roc curves for percentage ncd64 positive cells and cd64 index the mean time taken for hs-crp, ncd64 and blood culture positivity was analysed and it was observed that mean time taken for hs-crp positivity was 0.798 ± 0.33 hours which was the least, followed by ncd64 (2.802 ± 0.038 hours). mean time taken for blood culture positivity was 55.315 ± 3.022 hours which was significantly longer. as treatment cannot be withheld in a neonate with suspected sepsis waiting for blood culture report, the role of hs-crp and ncd64 becomes important as they give faster clue with regard to sepsis. in the receiver operating characteristic curves for the various hematologic parameters (hs-crp and ncd64) percentage of ncd64 positive cells had the highest auc (auc-0.914) using a cutoff of 28.01%, followed by cd64 mfi with an auc of 0.850 using a cutoff of 5.54. table 4. sensitivity, specificity, ppv and npv of various sepsis markers using optimal cutoff values a b c d e tlc 21 99 96 48 anc 25 93 83 48 platelet 62 83 83 62 i:t ratio 36 89 81 51 hs-crp 75 83 85 71 cd64 mfi 86 76 83 81 % ncd64 positive cells 85 81 85 81 note: a = variable, b = sensitivity (%), c = specificity (%), d = positive predictive value (%), e = negative predictive value (%) the assessment of individual markers indicated that ncd64 has the highest overall sensitivity and negative predictive value (npv) for the prediction of sepsis. we chose the calculated cutoff value of 28.01% ncd64 positive cells and cd64 mfi of 5.54 to be the optimal point, as it would permit a very high sensitivity (86%) and npv (81%) and simultaneously be able to maintain an acceptable specificity < 75%. the cutoff value crp ≥ 10 mg / l currently adopted in the neonatal unit provided only a sensitivity and specificity of 75% and 83% respectively. thus, neutrophil cd64 and hs-crp could be used in conjunction for early detection of sepsis owing to its faster result. ncd64 index at a cut-off value of ≥ 5.54 was positive in 86% of blood culture positive neonates (p value 0.001) while at a cut-off value of 28.01% ncd64 positive cells, blood culture positive neonates were 83% (p value 0.001). cd64 index at a cut-off value of ≥ 5.54 was able to detect 85% of eos and 87% of los neonates while at a cut-off value of 28.01% ncd64 positive cells,72% eos and 91% of los neonates were detected. discussion it is difficult to recognize the neonates with sepsis before receipt of the blood culture results and this remains a challenge. currently blood culture is the most effective method for diagnosing neonatal sepsis. however, the sensitivity of this method is low and using it as a gold standard in the diagnosis of septicemia is met with many difficulties. a study by magudumana et al showed that fewer than 10% of neonates evaluated and treated for sepsis had blood culture positive sepsis.8 as there is a possibility of sepsis even in the absence of negative blood culture results (for example, if the neonate had been exposed to antibiotics in utero), clinicians may opt to continue antibiotic treatment on the basis of the clinical profile. there are a variety of rapid tests which are helpful for screening of neonatal sepsis. white blood cell count and differential count, acute phase reactants like crp, micro esr, agglutination tests, pro-inflammatory and anti-inflammatory cytokine level estimation, dna sequence test like polymerase chain reaction (pcr), are used depending on their sensitivity and specificity. leucopenia (< 5000 / cu. mm) is more specific for neonatal sepsis.9 absolute neutropenia (< 1000 / cu.mm) is more predictive of neonatal sepsis than neutrophilia.10 a hematologic scoring system has been introduced by rodwell et al in 1988 by using seven hematological parameters.11 among the various cytokines studied, interleukin-6 (il-6) has emerged as an early marker of neonatal sepsis. the cutoff values for il-6 to diagnose sepsis ranges between 18 to 31 pg / ml.8,12-14 serial measurement of crp is probably of more value than expensive and time consuming determination of il-6 plasma concentration in the evaluation of neonatal sepsis.15 one of the newer acute phase markers of infection is procalcitonin (pct), the pro-hormone of calcitonin, which occurs in very low concentrations in the serum of healthy people.16 recently numerous cell surface antigens have been studied as potentially promising biomarkers of infection, including cd11b, cd69 and cd64.17 there is a significant increase in the cd64 expression on the surface of neutrophils in response to bacterial infection in neonates, similar to that seen in older children as well as adults.18 the quantification of ncd64 is rapid (< 60 minutes) and only minimal blood volume is used. for the present study, no additional blood was drawn from neonates. ncd64 is constitutively expressed on antigen presenting cells (monocytes, macrophages and dentritic cells), to a lesser extent to eosinophils, but only to a very low extent on resting neutrophils.6,19-21 several studies have indicated that quantification of the neutrophil / polymorphonuclear cell (pmn) ncd64 is a worthwhile candidate for evaluation as a more sensitive and specific indicator of sepsis than the other available diagnostics tests. majority of the studies have shown that ncd64 has high diagnostic specificity and sensitivity.22-24. therefore, we focused on ncd64 as a diagnostic adjunct to j nepal paediatr soc | vol 42 | issue 02 |may-aug, 2022 75 original articleneutrophil cd64 in neonatal sepsis standard hematologic indices in our study. a smaller study in critically ill neonates and infants by groselj-grenc et al used the standardized cd64 index for the first time and demonstrated its superiority over all other infection markers. they also pesented evidence that ncd64 was the best individual marker for bacterial sepsis in children.25 bhandari et al26 conducted the third largest study in neonates with suspected sepsis and also these authors found that cd64 index had the best diagnostic performance for culture-positive sepsis compared with all traditional hematologic assays. the study by bhandari et al showed a sensitivity of 70% and a specificity of 62% with a cutoff value of 2.30.26 in another study performed over 100 patients in nicu, the ncd64 index was found again to be the best with the highest sensitivity, with no difference between ncd64 and mfi.27 in the present study, we found that ncd64 positive cells and ncd64 index had the highest auc, compared to the other routinely used hematologic parameters. here, the cutoff cd64 index of 5.54 by itself yielded a sensitivity of 86% and a specificity of 76% for diagnosing sepsis. ncd64 index could detect 85% of early onset sepsis neonates and 87% of late onset sepsis neonates. in addition, the results of cd64 are available in a much shorter time than blood culture results which could reduce the injudicious use of antibiotics. however, ours is a relatively smaller study and hence larger, multicentric studies are warranted to implement it in neonatal sepsis. however critical issues like availability at all centers and cost must be evaluated carefully. conclusions the ncd64 index and percentage of ncd64 positive cells were significantly elevated during neonatal sepsis episodes and were the most diagnostic and faster measure of confirmed sepsis. it also additionally identifies a separate group among culturenegative sick neonates and may be useful to guide early antibiotic administration. references 1. gerdes js. clinicopathologic approach to the diagnosis of neonatal sepsis. clin perinatol. 1991; 18:pp361–81. pmid: 1879113 2. stoll bj, gordon t, korones sb, shankaran s, tyson je, bauer cr, et al. early-onset sepsis in very low birth weight neonates: a report from the national institute of child health and human development neonatal research network. j pediatr. 1996; 129: pp72–80. doi: 10.1016/s0022-3476(96)70192-0. 3. brodie sb, sands ke, gray je, parker ra, goldmann da, davis rb, et al. occurrence of nosocomial bloodstream infections in six neonatal intensive care units. pediatr infect dis j. 2000; 19: pp56–65. doi: 10.1097/00006454-200001000-00012. 4. berger a, salzer hr, weninger m, sageder b, aspock c. septicaemia in an austrian neonatal intensive care unit: a 7-year analysis. acta paediatr. 1998; 87: pp1066–69. doi: 10.1080/080352598750031392. 5. stoll bj, gordon t, korones sb, shankaran s, tyson je, bauer cr, et al. late-onset sepsis in very low birth weight neonates: a report from the national institute of child health and human development neonatal research network. j pediatr. 1996; 129:63–71. doi: 10.1016/s0022-3476(96)70192-0. 6. groselj-grenc m, ihan a, derganc m. neutrophil and monocyte cd64 and cd163 expression in critically ill neonates and children with sepsis: comparison of fluorescence intensities and calculated indexes. mediat. inflamm. 2008;2008:202646. doi: 10.1155/2008/202646. 7. ng pc, li k, wong rp, chui km, wong e, fok tf. neutrophil cd64 expression: a sensitive diagnostic marker for late-onset nosocomial infection in very low birthweight infants. pediatr res. 2002; 51:296–303. doi: 10.1203/00006450-200203000-00006. 8. magudumana mo, ballot de, cooper pa, trusler j, cory bj, vojoen e, et al. serial interleukin 6 measurements in the early diagnosis of neonatal sepsis. j trop pediatr, 2000. 46(5): p. 267-71. doi: 10.1093/tropej/46.5.267. 9. bhat r, rao ay. the performance of hematological screening parameters and crp in early onset of neonatal infections. j clin diagno res 2010;4:3331-6. 10. cockerill fr 3rd, wilson jw, vetter ea, goodman km, torgerson ca, harmsen ws, schleck cd, ilstrup dm, washington ja 2nd, wilson wr. optimal testing parameters for blood cultures. clin infect dis. 2004 jun 15;38(12):1724-30. doi: 10.1086/421087. epub 2004 may 25. pmid: 15227618. 11. rodwell rl, leslie al, tudehope dl. early diagnosis of neonatal sepsis using a hematologic scoring system. j pediatr 1988, 112(5):761–7. doi: 10.1016/s0022-3476(88)80699-1 12. berner r, niemeyer cm, leititis ju, funke a, schwab c, rau u, et al. plasma levels and gene expression of granulocyte colony-stimulating factor, tumor necrosis factor-alpha, interleukin (il)-1beta, il-6, il-8, and soluble intercellular adhesion molecule-1 in neonatal early onset sepsis. pediatr res. 1998;44: 469–477. doi: 10.1203/00006450-199810000-00002. 13. santana rc, garcía-muñoz f, reyes d, gonzález g, dominguez c, domenech e. role of cytokines (interleukin1beta, 6, 8, tumour necrosis factor-alpha, and soluble receptor of interleukin-2) and c-reactive protein in the diagnosis of neonatal sepsis. acta paediatr. 2003;92(2):221-7. d o i : 1 0 . 1 1 1 1 / j . 1 6 5 1 2 2 2 7 . 2 0 0 3 . t b 0 0 5 3 0 . x . pmid:12710650. 14. kroemer g, moreno de alborán i, gonzalo ja, martínez c. immunoregulation by cytokines. crit rev immunol. 1993;13(2):163-91. pmid: 8352909. 15. layseca-espinosa e, perez-gonzalezlf, torres-montes a, baranda l, de la feunte h, rosentstein y, et al. expression of cd64 as a potential marker of neonatal sepsis. pediatr allergy immunol, 2002. 13(5): p. 319-27. doi: 10.1034/j.1399-3038.2002.01064.x. https://doi.org/10.1034/j.1399-3038.2002.01064.x j nepal paediatr soc | vol 42 | issue 02 |may-aug, 202276 original article neutrophil cd64 in neonatal sepsis 16. ballot de, perovic o, galpin j, cooper pa. serum procalcitonin as an early marker of neonatal sepsis. s afr med j. 2004. 94(10): p. 851-4. pmid: 15532763. 17. ng pc, lam hs. diagnostic markers for neonatal sepsis. curr opin pediatr. 2006 apr;18(2):125-31. doi: 10.1097/01.mop.0000193293.87022.4c. pmid: 16601490. 18. fjaertoft, g, hakansson l, ewald u, foucard t, venge p. cd64 (fcgamma receptor i) cell surface expression on maturing neutrophils from preterm and term newborn infants. acta paediatr, 2005. 94(3): p. 295-302. doi: 10.1111/j.1651-2227.2005.tb03072. 19. masuda m, roos d. association of all three types of fc gamma r (cd64, cd32, and cd16) with a gamma-chain homodimer in cultured human monocytes. j immunol. 1993 dec 15;151(12):7188-95. pmid: 8258719.. 20. van vugt mj, kleijmeer mj, keler t, zeelenberg i, van dijk ma, leusen jh, geuze hj, the fcgammaria (cd64) ligand binding chain triggers major histocompatibility complex class ii antigen presentation independently of its associated fcr gamma-chain. blood. 1999 jul 15;94(2):808-17. pmid: 10397749. 21. ng pc, li k, wong rp, chui km, wong e, fok tf. neutrophil cd64 expression: a sensitive diagnostic marker for late-onset nosocomial infection in very low birth weight infants. pediatr res. 2002; 51:296–303. doi: 10.1203/00006450-200203000-00006. 22. danikas dd, karakantza m, theodorou gl, sakellaropoulos gc, gogos ca. prognostic value of phagocytic activity of neutrophils and monocytes in sepsis. correlation to cd64 and cd14 antigen expression. clin exp immunol. 2008, 154(1): p. 87-97. doi: 10.1111/j.1365-2249.2008.03737.x 23. allen e, bakke ac, purtzer m z, deodhar a. neutrophil cd64 expression: distinguishing acute inflammatory autoimmune disease from systemic infections. ann rheum dis. 2002. 61(6): p. 522-5. doi: 10.1136/ard.61.6.522. 24. cardelli p, ferraironi m, amodeo r, tabacco f. evaluation of neutrophil cd64 expression and procalcitonin as useful markers in early diagnosis of sepsis. int j immunopathol pharmacol, 2008. 21(1): p. 43-9. doi: 10.1177/039463200802100106. 25. groselj-grenc m, ihan a, pavnik-arnol m, kopitar n, gmeinerstropar t, derganc m. neutrophil and monocyte cd64 indexes,lipopolysaccharide-binding protein, procalcitonin and c-reactive protein in sepsis of critically ill neonates and children. intensive care med 2009;35:1950-8. doi: 10.1007/s00134-009-1637-7. 26. bhandari v, wang l, rinder c, rinder h. hematologic profile of sepsis in neonates: neutrophil cd64 as a diagnostic marker. pediatrics. 2008, 121(1): p. 129-34. doi: 10.1542/peds.2007-1308. 27. dilli d, oguz ss, dilmen u, köker my, kizilgün m. predictive values of neutrophil cd64 expression compared with interleukin-6 and c-reactive protein in early diagnosis of neonatal sepsis. 2010;24(6):363–370. doi: 10.1002/jcla.20370. https://doi.org/10.1111/j.1651-2227.2005.tb03072.x j nepal paediatr soc | vol 42 | issue 03 |sep-dec, 2022 43 original article doi: 103126/jnps.v4113 sensory profile of nepalese children with or without autism introduction: sensory integration dysfunction (sid) is common in children with autism spectrum disorder. if sid is detected early and intervened, there is a decrease in autistic mannerisms and an improvement in the areas of sensory processing and regulation, social-emotional function, and fine motor skills. thus, this study was conducted to compare the sensory profile of children with or without autism spectrum disorder (asd) in nepal. methods: thirty five parents of children with asd and 43 parents of children without asd (non asd) completed short sensory profile (ssp) questionnaire. results: definitive sensory issue was identified in 66% in asd and 23% in non asd group. common sensory issues in asd group were auditory filtering sensitivity (74.3%), movement sensitivity (37%), under responsiveness / seeks sensation (45.7%). in non asd group, common sensory issue was under responsive/seek sensation. the internal consistencies between the sub-scales ranged from 0.70 to 0.83. conclusions: two third of the children with asd have sensory issues. identifying different sensory problems with ssp would be helpful for further management of children with asd. abstract 1 associate professor, department of child health, tribhuvan university teaching hospital, maharajgunj medical campus, kathmandu, nepal. 2 assistant professor, padma kanya multiple campus, tribhuvan university, kathmandu, nepal. merina shrestha1, rena shrestha2 *corresponding author merina shrestha department of child health, tribhuvan university teaching hospital, maharajgunj medical campus, kathmandu, nepal. email: drmerinashrestha@gmail.com article history received on: 16 jan, 2022 accepted on: 15 dec, 2022 funding sources: none conflict of interest: none keywords: autism spectrum disorder, children, short sensory profile, nepal. online access doi: https://doi.org/10.3126/jnps.v42i3.42421 copyrights & licensing © 2022 by author(s). this is an open access article distributed under creative commons attribution license (cc by nc ) introduction sensory integration dysfunction (sid) is the condition in which the brain is unable to process the information that it receives through different senses. in sid, the brain has difficulty analyzing, organizing and connecting, or integrating sensory messages.1 this might lead to learning difficulties because of difficulty in using sensory information to plan and organize for the task to be completed.1 when there is delay and inefficient neurological processing of the sensory signals, they often manifest as inappropriate motor and behavior responses and are misinterpreted as behavioral problems.1 whenever the brain is unable to integrate sensory inputs like sights, sounds, touch, body position, or movements that it receives from sensory systems, effective responses cannot be generated.2 because of the altered responses and poor behavior, it creates difficulties with academic and motor learning as well.3 the sid may be tactile, olfactory hypersensitivity, and hypo or hyper-reactivity to sensory stimuli.4-7 when auditory or vestibular system dysfunctions are present, it affects speech development causing speech delay and articulation disorder.1 it is seen that young children with speech disorders have reduced functions in the vestibular, proprioceptive, and tactile sensory systems compared to original article doi: 103126/jnps.v4113 j nepal paediatr soc | vol 42 | issue 03 |sep-dec, 202244 original article sensory profile in children children without speech disorders.8 individuals with autism spectrum disorder (asd) often display atypical responses to sensory stimuli and this condition is considered one of autism’s defining features.9 different kinds of researches show that the prevalence of sid in children with autism ranges from 42% to 88%.10 when children with autism were compared with children without autism, there was a significant difference in their sensory profile with the greatest differences in under-responsiveness /seeking sensation, auditory filtering, and tactile sensitivity scales.11 symptoms of sid are categorized mainly into four patterns: “visual perception and auditorylanguage disorders,” “tactile defensiveness,” “disorders involving the vestibular system,” and “developmental dyspraxia”. children with asd may not display all the symptoms of a certain dysfunction but they usually have several signs of these four patterns.12 when sid is present in children with autism, it negatively impacts the performance of daily life activities.13,14 the recent global data shows the prevalence of asd is 1 in 100 children.15 when this data is projected in nepal’s population, there may be about three lakh children with asd. in these children along with diagnosis of asd if sid is detected early and intervened, there is a decrease in autistic mannerisms (a component of social responsiveness) and improvement in the areas of sensory processing and regulation, social-emotional function, and fine motor skills.16 to identify and detect sid in children, the sensory profile can be assessed through clinical examinations and parents’ interviews. the sensory profile is a parent-based questionnaire originally developed as a screening tool to identify children with sensory processing difficulties.13 it includes 125 items in three categories, namely sensory processing, modulation, and behavioral and emotional responses.17 the shorter version, short sensory profile (ssp) is mostly used for screening programs and research purposes.11,17 the ssp has good psychometric properties with a satisfactory internal consistency with cronbach alpha ranging from .68 to .92 and good convergent validity of over 95% in identifying children with and without sensory modulation differences.18 the tool has been translated into several languages such as spanish, arabic, turkish, indian, and chinese.19 the primary objective of the current study is to assess the sensory profile of children with asd and compare them with children without asd using ssp. we also aimed to measure the internal consistency of the questionnaire. methods for the current study, a convenient sample of parents of children with and without asd was selected from autism care nepal society (acns), a non-governmental organization that provides clinical and social services to families with children with autism, and tribhuvan university teaching hospital (tuth), a tertiary level hospital, both based in kathmandu, nepal. thirty-five parents of the asd group were identified from the acns registry and the first author’s neuro-developmental clinic at tuth. for comparison, children without asd (non-asd group) and any known physical, neurological, or behavioral disorders were identified from well-child visits and pediatric out-patient clinic register at tuth. other interested parents were also invited to participate in the study using the researcher’s social network. the inclusion criteria for enrollment were children below 16 years in both groups. in the asd group, children with autism but without any other associated comorbidities including any physical impairment or disability like cerebral palsy, down syndrome were excluded. altogether 48 parents in the non-asd group agreed to participate in the study. of them, five children were excluded as three had visual impairment, one child had a motor impairment and one child was recently diagnosed with asperger’s syndrome which was diagnosed after parents completed ssp and sought help. hence, a total of 43 parents was enrolled in the non-asd group. parents completed a demographic questionnaire that included information on the child’s age, gender, and any impairment or disability. for children with asd, an additional question on age at diagnosis of autism was also asked. the ssp is a questionnaire-based screening tool to identify sensory processing difficulties, patterns, and effects on functional performance.16,17 the ssp consists of 38 items organized into seven subscales; i) tactile sensitivity (7 items), ii) taste / smell sensitivity (4 items), iii) movement sensitivity (3 items), iv) under-responsive / seeks sensation (7 items), v) auditory filtering (6 items), vi) low energy / weak (6 items), and vii) visual / auditory sensitivity (5 items). each item is rated on a 5-point likert scale. the response options are from always =1 to never = 5. each item score is summed up to obtain the total score for each subscale which is then classified into one of the three possible ranges: typical performance corresponds to 1 sd below the mean (better than the lowest 16%), probable difference corresponds to 2 sd below the mean (performed like children in the lowest 14%), and definite difference corresponds to 3 sd below the mean (performed like children in the lowest 2%). for the ssp, a total score above 155 indicates typical performance, 142 to 154 indicates probable difference and below 141 indicates a definite difference. because there is no standardized and validated sensory profile assessment tool in nepal, the ssp english version was chosen as this tool is simple and easy for parents to respond to. the questionnaire along with the consent form was sent to the parents through closed social messaging tools such as whatsapp and viber chat services. participants were encouraged to reach out to the research team if they had questions about the survey. the contact information of the research team was provided j nepal paediatr soc | vol 42 | issue 03 |sep-dec, 2022 45 original articlesensory profile in children in the consent form. frequencies and descriptive statistics were performed to assess the demographic characteristics of the children. the internal consistency of the total score and subscales were measured using cronbach’s alpha (). the internal consistency is considered high when > 0.80; satisfactory when = 0.60 – 0.80; and moderate when = 0.40 – 0.59. in both groups, children were categorized in a typical performance, probable difference, and definite difference for each of the sensory scales. this was further analyzed in each item of the sensory scales. the analysis was performed in stata version 16. results a total of 78 parents, 35 in the asd group and 43 in the non-asd group participated in the study. the mean age of children was 7.8 (sd = 4.3) and 7.5 (sd = 3.6) years in the asd group and the non-asd group, respectively. majority of the children (80%, n = 35) were males in asd group while in non-asd group, 53% (n = 43) were males. children in the asd group had a clinical diagnosis as per the diagnostic and statistical manual of mental disorders, fifth edition (dsm-v; american psychiatric association 2013). the mean age of diagnosis of asd was 32.89 months ( range = 18 72 months, sd =14.61). more than half of the parents (57%, n = 35) in the asd group and only 2%, n =43) of parents in the nonasd group reported that their child may have sensory issues. almost a quarter of parents in the asd group were unsure about the presence of sensory issues of their child (table. 1). table1. demographic characteristics and presence of sensory issue in a child according parents asd group n = 35 non asd group n = 43 age in years (sd) 7.8 (4.3) 7.5 (3.6) gender male (%) 28 (80) 23 (53.4) sensory issue yes no may be 20 (57.2) 6 (17.1) 9 (25.7) 1 (2.3) 37 (86.0) 5 (11.6) table 2. standardized cronbach’s alphasa for the ssp total score and scores for each subscales for asd and nonasd group asd group non asd group tactile sensitivity 0.70 0.76 taste / smell sensitivity 0.75 0.80 movement sensitivity 0.72 0.81 under-responsive / seeks sensation 0.68 0.80 auditory filtering sensitivity 0.70 0.81 low energy / weak 0.72 0.83 visual auditory sensitivity 0.69 0.80 overall 0.74 0.83 a > 0.80 — high; = 0.60–0.80 — satisfactory; and = 0.40 – 0.60 — moderate internal consistency table 3: comparison of subscales of ssp between asd group and non asd group typical performance probable difference definite difference autism without autism autism without autism autism without autism n = 35 n = 43 n = 35 n = 43 n = 35 n = 43 (%) (%) (%) (%) (%) (%) tactile sensitivity 12 (34.3) 31 (72.2) 11 (31.4) 6 (13.9) 12 (34.2) 6 (13.9) taste / smell sensitivity 18 (51.4) 23 (53.5) 6 (17.1) 8 (18.6) 11 (31.4) 12 (27.9) movement sensitivity 12 (34.2) 30 (69.8) 10 (28.5) 8 (18.6) 13 (37.1) 5 (11.6) under responsive / seeks sensation 13 (37.1) 27 (62.8) 6 (17.1) 2 (4.6) 16 (45.7) 14 (32.6) auditory filtering sensitivity 7 (20) 32 (74.4) 2 (5.7) 7 (16.3) 26 (74.3) 4 (9.3) low energy / weak 21 (60) 39 (90.8) 2 (5.7) 2 (4.6) 12 (34.3) 2 (4.6) visual / auditory sensitivity 15 31 (72.2) 12 (34.3) 6 (13.9) 8 (16.3) 6 (13.9) total 9 (25.7) 23 (53.6) 3 (8.5) 10 (23.2) 23 (65.7) 10 (23.2) j nepal paediatr soc | vol 42 | issue 03 |sep-dec, 202246 original article sensory profile in children table 4: comparison of individual item in each subscale of ssp between asd and non asd group asd n = 35 (%) non asd n = 43 (%) tactile sensitivity a. expresses distress during grooming e.g. fights or cries during hair-cutting, face washing, finger nail cutting) 9 (25.7) 3 (6.9) b. prefers long sleeves when it is warm and short sleeves when it is cold 3 (8.5) 3 (6.9) c. avoids barefoot, specially in sand or grass 4 (11.4) 10 (23.2) d. reacts emotionally or aggressively to touch 4 (11.4) 3 (4.6) e. withdraws from splashing water 3 (8.5) 1 (2.3) f. difficulty in standing in line or close to other people 12 (34.3) 2 (4.6) g. rubs or scratches out on a spot that has been touched 4 (11.4) 1 (2.3) taste / smell sensitivity a. avoids certain tastes or food smells that are typically part of children’s diet 9 (25.7) 7 (16.2) b. will only eat certain tastes 13 (37.1) 13 (30.2) c. limits self to particular food textures/temperature 11 (51.4) 9 (20.9) d. picky eater, especially regarding food textures 8 (22.8) 10 (23.3) movement sensitivity a. becomes anxious or distressed when feet leave the ground 6 (17.1) 1 (2.3) b. fears falling or heights 11 (31.4) 3 (6.9) c. dislikes activities where head is upside down (e.g.; somersaults) 11 (31.4) 1 (2.3) under-responsive / seeks sensation a. enjoys strange noises/seeks to make noise for noise’s sake 11 (31.4) 7 (16.3) b. seeks all kinds of movement and this interferes with daily routines (figidity) 9 (25.7) 7 (16.3) c. becomes overly excitable during movement activity 12 (34.2) 9 (20.9) d. touches people and objects 8 (22.8) 7 (16.3) e. does not seem to notice when face or hands are messy 5 (14.2) 7 (16.3) f. jumps from one activity to another so that it interferes with play 15 (42.8) 5 (11.6) g. leaves clothing twisted on body 8 (22.8) (4.6) 2 auditory filtering a. is distracted or has trouble functioning if there is a lot of noise around 16 (45.7) 6 (13.9) b. appears to not hear what you say (seems to ignore) 15 (42.8) 3 (6.9) c. can’t work with background noise (fan, freeze) 4 (11.4) 1 (2.3) d. has trouble completing task when radio is on 4 (11.4) 2 (4.6) e. doesn’t respond when name is called but you know child’s hearing is ok 10 (28.6) 5 (11.6) f. has difficulty paying attention 20 (57.1) 2 (4.6) low energy / weak a. seems to have weak muscle 4 (11.4) 0 b. tires easily, especially when standing or holding particular body position 3 (8.5) 2 (4.6) c. has a weak grasp 5 (14.2) 0 d. can.t lift heavy objects (weak as compared to other children of that age) 5 (14.2) 0 e. props to support self (even during activity) 6 (17.1)) 4 (9.3) f. poor endurance/tires easily 6 (17.1) 0 visual/ auditory sensitivity a. responds negatively to unexpected or loud noises (cries or hides at noise from vacuum, dog barking etc) (20) 7 (4.6) 4 b. holds hands over ears to protect ears from sound 12 (34.2) 4 (4.6) j nepal paediatr soc | vol 42 | issue 03 |sep-dec, 2022 47 original articlesensory profile in children ?c. is bothered by bright lights after others have adapted to the light 3 (8.5) 0 d. watches everyone when they move around the room 8 (22.8) 12 (27.9) e. covers eyes or squints to protect eyes from light 3 (8.5) 9 (20.9) the cronbach’s alphas for the current sample on the ssp was .74 for the asd group and .83 for the non-asd group, indicating a satisfactory to high reliability. cronbach’s alphas on all scales for asd ranged between .76 .83 whereas it ranged between .68 -.75 for the non-asd group. in all the subscales, the internal consistency was low in the asd group as compared to the non-asd group (table 2). comparison of parent-reported sensory processing scores on the ssp for both groups is summarized in table 3. about 66% (n = 35) of the children in asd group had definitive sensory issues compared to 23% (n = 43) in non asd group. about half of the children (54%, n = 43) in the non-asd group had typical performance to sensory stimuli as compared to one-quarter (25%, n = 43) of children in the asd group. in the asd group, more than 30% (n = 35) had definitive sensitivity to tactile, taste, and smell, movement sensitivity, under-responsive or seek sensation, and low energy scales. almost 75% of the asd group had auditory filtering sensitivity. amongst the nonasd group, about 30% of them had a definitive sensory problem with taste/smell and under-responsive or seeking sensation scales. however, only a few children in both the groups (asd group: 16% (n = 35), the non-asd group: 14% (n = 43) had visual sensitivity issues. when individual items were explored in each sensory scale, about 30% of children in the asd group had a sensory issue when they come closer to another person in the queue and prefer only certain tastes; they were fearful of height and very excitable during movement. almost 50% (n = 35) of children in the asd group had inattention or difficulty in paying attention and jumping from one activity to another. similarly, about 50%(n = 35) of these children had auditory filtering issues in the form of getting distracted or trouble functioning with noise and sometimes like to produce noise. about 34% (n = 35) also had a habit of closing ears to protect themselves from noise. amongst the non-asd group, about 30% (n = 43) of the children eat only certain tastes and are aware of people around and watch them move. discussion to the best of our knowledge, this is the first study investigating and comparing the sensory profile of nepali children with or without asd. the mean age of children in both groups was about seven years. the mean age of diagnosis of asd was 36 months, indicating a decreased mean age of diagnosis of asd as compared to the previous findings of 56 58 months.20 the earlier age of diagnosis of autism in recent years might be because there has been an increase in the number of awareness programs conducted by acns as well as the increased knowledge about asd among health professionals and the general public. although general awareness about autism is improving, there remains a huge gap of knowledge on sensory processing disorders.21 although more than half of parents of the asd group knew that their child had sensory problems, about one-third of parents were unsure about it. in the non-asd group, only one-tenth of parents indicated that their child may have some sensory issues. ssp when applied by parents to their children, definite sensory issue was found in about three-quarters of the children in the asd group. the finding is consistent with many previous ssp studies that stated 42% to 95% of children with autism have a sensory issue reported by parents.11,22 in the non asd group, almost a quarter of the parents reported definitive sensory issues. this finding is comparable with the study from saudi arabia that showed similar proportions of their children without autism that displayed definite sensory issues, especially with tactile and under-responsiveness scales.23 in indian neuro-typical children also, the definite sensory issue was seen in about 30% of the children.24 the presence of sensory issues in neuro-typical children from nepal, india and saudi arabia is higher as compared to studies from developed western countries that ranged from 8.3% in population-based child survey 24 to 5 -13% in elementary school-aged children in the united states.25 about 16% of neuro-typical children from the us are over-responsive to certain auditory and tactile sensations as compared to nepalese neuro-typical children who were more sensitive to taste.26 this difference may be because of cultural differences in parenting style and perception of sensory issues of nepali parents. even health care professionals rarely discuss sensory issues as this issue is not integrated into the medical curricula. along with that, the sensory experiences felt by children are different from how parents perceive them in their children. parents often find it difficult to understand the actual sensory experiences of their children.27 in the current study, we found that about 65% of the children in the asd group had sensory issues as compared to 83% of the children with asd in the study from the usa.11 the most common sensitivity was seen in under-responsiveness and auditory filtering. according to parents’ report, children in this study within this group seemed to have problems in standing in a queue, aversion of certain tastes and texture of food, fear of height, inattention, easily distractible and negative response to unexpected sounds and sometimes appearing deaf. parents also reported that their children got more excited during activities and were easily distractible. however, the visual / auditory sensitivity was found to be in only 16% as compared to about 44% from a study among j nepal paediatr soc | vol 42 | issue 03 |sep-dec, 202248 original article sensory profile in children children in the us.11 the visual / auditory sensitivity in asd and non-asd was almost similar. children in the non-asd group seem to have more negative experiences with certain tastes, and textures of food. as many parents in the asd group are associated with the autism support group and might have received orientation regarding sid, thus they might have reported only extreme responses and unusual behaviors of autism. it is seen that many behavioral issues accompany sid and when together, it can cause a high level of stress level in parents with children having sid as compared to children without sid.28,29 however, when parents are educated regarding sid and when they have a better idea regarding the condition, families can adopt more meaningful family chores avoiding the sensory overload especially in cases of autism. the understanding of sensory issues helped parents to plan and prioritize the family occupations as well. in the case of neurotypical children, parents seem to emphasize more on unpleasant stimuli for the development of problemsolving and coping skills.30 thus, a better understanding of the sensory issues of the children and planning accordingly would be helpful for parents to reduce their stress. when internal consistency of ssp in the nepalese context (including children with or without autism) was explored, it was found to be above 0.6 in all the scales with satisfactory reliability. additionally, because ssp is simple and easy to administer, ssp can be filled in for each child with asd so that the sensory issues can be considered when planning different activities for children. this would also help physicians, teachers, and parents to understand their children better. the study presents some limitations. firstly, the findings of the study cannot be generalized. most of the parents of children with asd were associated with the autism support group where they have some orientation on sid and who are not associated with the support group, it is not known whether they have received any orientation on sid or not. secondly, the data was collected through an online portal. thus, only those parents who have access to the internet and knowledge on using such a portal participated in the study. thirdly, the data is based on a screening survey instrument. thus, it is not possible to determine the prevalence rate because of the lack of rigorous study assessing physiological and behavioral manifestations of sid in individuals through screening. it is recommended for more intensive diagnostic assessments for those who are identified to have sid through screening to validate the presence of a sensory processing disorder. fourthly, the study used a small sample size which was not able to evaluate the presence or absence of other co-morbidities like attention deficit hyperactive disorder or other pure sid. sensory over responsiveness is often associated with adhd and anxiety.24 when treatment is targeted towards sensory modulation associated with hyperactivity and anxiety, sensory over responsiveness might show improvements in function and behaviors. thus, when sid is explored, other associated co-morbidities should also be explored. the study excluded children with other disabilities like visual, hearing, and physical impairment. in such populations also, identifying and managing sid will help to manage their behaviors. as sid may rise or decline with age, conducting a longitudinal study would be helpful to find out the developmental trajectories of sensory processing disorders. conclusions sid is twice as common in children with asd as children without asd. the most common sensory problem seen in children with asd was auditory filtering sensitivity. amongst the non-asd group, the most common sensory problem was taste/smell and under-responsiveness. as ssp is an easy tool to identify the sensory issues, the nepali version of ssp could be explored for further extensive researches in nepal. references 1. ayres aj. sensory integration and learning disorders: western psychological services; 1972. 2. zimmer m, desch l. sensory integration therapies for children with developmental and behavioral disorders. pediatrics. 2012;129(6):1186-9. doi: 10.1542/peds.2012 0876 3. bundy ac, lane sj, murray ea. sensory integration: theory and practice: fa davis; 2002. 4. grandin t. an inside view of autism. high-functioning individuals with autism: springer; 1992. p. 105-26. 5. talay-ongan a, wood k. unusual sensory sensitivities in autism: a possible crossroads. intl j of disabil dev educ. 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durkin ms, saxena s, yusuf a, shih a, elsabbagh m. global prevalence of autism: a systematic review update. autism res. 2022 may;15(5):778-90. doi: 10.1002/aur.2696. 16. pfeiffer ba, koenig k, kinnealey m, sheppard m, henderson l. effectiveness of sensory integration interventions in children with autism spectrum disorders: a pilot study. am j occup therapy. 2011;65(1):76-85. doi: 10.5014/ajot.2011.09205. 17. dunn w. sensory profile: user’s manual: psychological corporation san antonio, tx; 1999. 18. mcintosh d, miller l, shyu v, dunn w. development and validation of the short sensory profile. sensory profile manual. 1999:59-73. doi: 10.1007/s10803-016-3018-8 19. jorquera-cabrera s, romero-ayuso d, rodriguez-gil g, triviño-juárez j-m. assessment of sensory processing characteristics in children between 3 and 11 years old: a systematic review. front pediatr. 2017; 5:57. doi:10.3389/fped.2017.00057 20. shrestha m, shrestha r. symptom recognition to diagnosis of autism in nepal. j autism dev disord. 2014; 44(6):1483-5. doi: 10.1007/s10803-013-2005-6 21. gee bm, peterson tw. changes in caregiver knowledge and perceived competency following group education about sensory processing disturbances: an exploratory study. occup ther int. 2016;23(4):338-45. doi: 10.1002/oti.1435. 22. liss m, saulnier c, fein d, kinsbourne m. sensory and attention abnormalities in autistic spectrum disorders. autism. 2006; 10(2):155-72. doi:10.1177/1362361306062021. 23. al-heizan mo, alabdulwahab ss, kachanathu sj, natho m. sensory processing dysfunction among saudi children with and without autism. j phys ther sci. 2015;27(5):1313-6. doi: 10.1589/jpts.27.1313 24. tripathi hj, dwivedi a. prevalence of sensory behaviors in normal indian children with short sensory profile. int j adv res innov ideas tech. 2019. 25. crasta je, salzinger e, lin m-h, gavin wj, davies pl. sensory processing and attention profiles among children with sensory processing disorders and autism spectrum disorders. front integr neurosci. 2020;14:22. doi:10.3389/fnint.2020.00022 26. ahn rr, miller lj, milberger s, mcintosh dn. prevalence of parents’ perceptions of sensory processing disorders among kindergarten children. am j occup ther. 2004;58(3):287-93. doi: 10.5014/ajot.58.3.287. 27. ben-sasson a, carter as, briggs-gowan mj. sensory over-responsivity in elementary school: prevalence and social-emotional correlates. j abnormal child psychol. 2009;37(5):705-16. doi: 10.1007/s10802-008-9295-8. 28. dickie va, baranek gt, schultz b, watson lr, mccomish cs. parent reports of sensory experiences of preschool children with and without autism: a qualitative study. am j occup ther. 2009; 63(2):172-81. doi: doi.org/10.5014/ajot.63.2.172 29. gourley l, wind c, henninger em, chinitz s. sensory processing difficulties, behavioral problems, and parental stress in a clinical population of young children. j child fam stud. 2013;22(7):912-21. doi: 10.1007/s10826-012-9650-9. 30. bagby ms, dickie va, baranek gt. how sensory experiences of children with and without autism affect family occupations. am j occup ther. 2012;66(1):78-86. doi: 10.5014/ajot.2012.000604 https://doi.org/10.5014/ajot.61.2.135 https://doi.org/10.1002/1097-0355(199324)14:4%3c330::aid-imhj2280140407%3e3.0.co;2-k https://dx.doi.org/10.1007%2fs10803-016-3018-8 https://doi.org/10.3389/fped.2017.00057 https://doi.org/10.1007/s10803-013-2005-6 https://dx.doi.org/10.1589%2fjpts.27.1313 https://doi.org/10.3389/fnint.2020.00022 https://doi.org/10.5014/ajot.63.2.172 https://dx.doi.org/10.5014%2fajot.2012.000604 403 forbidden forbidden you don't have permission to access this resource. apache/2.4.54 (ubuntu) server at www.nepjol.info port 443 403 forbidden forbidden you don't have permission to access this resource. apache/2.4.54 (ubuntu) server at www.nepjol.info port 443 nepas journal 32-1 cs5 final.indd original article <37>j. nepal paediatr. soc. risk factors of neonatal mortality in bangladesh kamal smm1, ashrafuzzaman m2, nasreen sa3 1dr. s. m. mostafa kamal, msc, phd, associate professor, department of mathematics, islamic university, kushtia, bangladesh, 2dr. md. ashrafuzzaman, mbbs, mph, senior medical officer, islamic university, kushtia, bangladesh, 3dr. syeda anjuman nasreen, mbbs, m.phil, lecturer, department of community medicine, mymensingh medical college, mymensingh, bangladesh. address for correspondence: dr. mostafa kamal, e-mail: kamaliubd@yahoo.com abstract introduction: to address united nations millennium develop ment goal 4 (mdg 4) on reducing childhood mortality rates by two-thirds by 2015, there is a need for better population-based data on the rates and causes of neonatal death. this study aims to identify the risk factors of neonatal mortality in bangladesh. materials and methods: the study used data from the nationally representative 2007 bangladesh demographic and health survey. the survey gathered information regarding socioeconomic, demographic, environmental and maternal and child health care of 10,996 ever married women and 6,058 children. both bivariate and multivariate statistical analyses were used to assess the relationship between neonatal mortality and contextual factors. results: the prevalence of neonatal mortality was 37/1,000. the statistical analyses yielded quantitatively important and reliable estimates of neonatal death. the multivariate logistic regression analysis yielded significantly increased risk of neonatal mortality for children with mother who had no formal education, the muslims, whose mother were adolescents of age 15-19, first ranked birth and twin babies. conclusion: emphasis should be given to improve female education in bangladesh for a better chance of satisfying important factors that can improve infant survival: the quality of infant feeding, general care, household sanitation, and adequate use of preventive and curative health services. key words: maternal and child health, female education, mdg 4, neonatal mortality introduction each year nearly four million newborns die during the fi rst 4 weeks of life and world-wide neonatal mortality makes up 40% of the total child mortality1,2,3,4. this means that the overall neonatal mortality rate is about 30/1,000 with an annual number of births in the world reaching 130 million1. whereas developed countries maintain a neonatal mortality rate of 2-3/1,000, it is not uncommon that this rate reaches over 60/1,000 in the poorer segments of the world5. of the deaths, 99% are accounting the developing countries, especially in subsaharan african and south asia1,5,6. during the last 30 years, the reduction in neonatal mortality rates has been slower, compared to both under-fi ve and child mortality rates after the fi rst month of life6,7,8. millennium development goal 4 (mdg 4) calls for reducing the under-fi ve mortality rate by twothirds between 1990 and 2015. as global momentum and investment for accelerating child survival grow, monitoring progress at the global and country levels has become even more critical. the most recent inter-agency group for child mortality estimation (igme) estimates show that nearly 8.1 million under-fi ve children died in 2009, implies that more than 22,000 children die per day. these fi gures refl ect substantial progress. globally, the under-fi ve mortality rate has fallen from 89 deaths per 1,000 live births in 1990 to 60 in 2009. but the rate of decline –a one-third reduction over 20 years –is insuffi cient to meet mdg 4, particularly in sub-saharan africa, southern asia and oceania.9 neonatal and postneonatal mortality rates declined less10, 3.0% and 2.5% decrease respectively. as a consequence there is an increasing proportion of infant deaths occurring in the neonatal period worldwide, which now accounts for two-thirds of deaths in children less than one year old, and nearly four-tenths of all deaths in children less than fi ve years of age8. infant and child mortality rates refl ect a country’s level of socioeconomic development and quality of life. they are used for monitoring and evaluating population january-april, 2012/vol 32/issue 1 doi: http://dx.doi.org/10.3126/jnps.v32i1.4845 <38> j. nepal paediatr. soc. and health programmes and policies. the rates are also important for monitoring progress towards the united nations mdg to reduce child mortality as expected by the year 2015. it would be diffi cult to achieve mdg without reducing child mortality by two-thirds by 201511. thus the study on infant and child mortality; in particular, neonatal mortality is of great importance to monitoring the progress of children’s health status. bangladesh has made substantial progress in the reduction of under-fi ve mortality. a recent estimate suggests that infant mortality reduced from 88/1,000 in 2000 to 55/1,000 in 2010. absolutely, in bangladesh, infant mortality reduced by 3.3% annually in the last decade. the neonatal and post neonatal mortality reduced from 65/1,000 and 38/1,000 in 1990 to 31/1,000 and 14/1,000 respectively in 2010.5 besides, the bangladesh demographic and health surveys (bdhss) reported that under-fi ve mortality reduced from 133/1,000 in 1993-1994 to 55/1,000 in 2007. in addition, the neonatal and post-neonatal mortality rates declined from 52/1,000 and 35/1,000 to 37/1,000 and 15/1,000 respectively during the same period12,13. the successive dhs surveys conducted in bangladesh since 1993-1994 confi rm a declining trend in childhood mortality. during 1989-1993 to 2002-2006, infant mortality declined by 40% from 87/1,000 live births to 52/1,000. even more impressive are the 72% decline in child mortality and the 51% decline in under-fi ve mortality over the same period. a comparison of mortality rates over the last three years shows that infant and child mortality declined by 20% and 42% respectively13. a recent study14 conducted on rural areas at matlab using the health and demographic surveillance system (hdss) data has shown gradual decline in under-fi ve mortality consistent with the reports of the bdhss. whereas literatures on neonatal, post-neonatal, infant and child mortality are enormous in other developed and developing countries, those are limited in bangladesh. many studies carried out elsewhere on risk factors of neonatal mortality attempt to show causal relationship between neonatal mortality and utilization of maternal health care facilities, such as receiving antenatal care (anc) services, place of delivery, skilled attendance during childbirth15,16. caste, religion and standard of living index are also identifi ed as infl uential factors of childhood mortality in india17. a number of studies showed signifi cant relationship between mother’s level of education6,16,18, maternal age19,20,21,22 and early childhood mortality. mother and child characteristics are analysed as determinants of child mortality and its components, socioeconomic characteristics, type of childbirth and birth defects have been extensively investigated22. regional and social diff erences may infl uence the composition of population groups and the success of local health promotion programmes. living in poor areas may have negative eff ects on children’s health23. bangladesh is still far away to achieve the mdg 4. to achieve the goal, the infant mortality rate should be reduced at 31/100024. it would be diffi cult to achieve mdg of reducing child mortality by two-thirds by 2015 without reducing neonatal mortality. despite impressive progress, bangladesh still has higher rate of infant and neonatal mortality. in bangladesh, very few studies have reported the causes of early and late neonatal deaths16. knowledge on some of the factors aff ecting child mortality is a fundamental requirement for devising appropriate policies and strategies to accelerate decline in neonatal mortality. this paper aims to investigate the risk factors aff ecting neonatal mortality in bangladesh. the fi ndings of this study may help the policy makers, programme managers and donors in driving up understanding about the risk factors of early childhood mortality, particularly neonatal mortality of bangladeshi children. materials and methods data for this study have been taken from 2007 bdhs. the bdhs is a nationally representative survey of 10,996 women age 15-49 and 3,771 men age 15-54 from 10,400 households and 6,058 under-fi ve children born during the last fi ve years preceding the survey date covering 361 sample points (clusters) throughout bangladesh, 134 in urban areas and 227 in the rural areas. this survey is the fi fth in a series of national-level population and health surveys conducted as part of the global demographic and health surveys (dhs) programme. it was designed to provide data to monitor the population and health situation in bangladesh as a follow up to the past four bdhs surveys. the survey utilised a multistage cluster sample based on the 2001 bangladesh census and was designed to produce separate estimates for key indicators for each of the six divisions of the country – barisal, chittagong, dhaka, khulna, rajshahi and sylhet. data collection took place over a fi ve-month period from 24 march to 11 august, 2007. the survey obtained detailed information on fertility levels, marriage, fertility preferences, awareness and use of family planning methods, breastfeeding practices, nutritional status of women and young children, childhood mortality, maternal and child health, and knowledge and attitudes regarding hiv/aids and other sexually transmitted infections (stis). the 2007 bdhs was conducted under the authority of the national institute for population research and training (niport) of the ministry of health and family welfare. it was implemented by mitra and associates, a bangladeshi risk factors of neonatal mortality in bangladesh <39>j. nepal paediatr. soc. research fi rm located in dhaka. technical assistance was provided by macro international inc. through the measure dhs programme. financial support for the survey was provided by the u.s. agency for international development (usaid/bangladesh). however, the details of the survey are given elsewhere13. conceptual framework many researchers such as mahmood25 and later on titaley and his colleagues6 adapted the conceptual framework proposed by mosley and chen26 with some modifi cations based on the limitations and structure of the dhs data. keeping in view the aforementioned frameworks, this study considered the “survival status” of children as the outcome variable. according to mosley and chen26 framework, the socioeconomic factors aff ect the outcome variable through the four proximate determinants namely, socio-demographic factors, environmental factors, nutritional factors and health seeking behaviour of mother. due to scarcity of information we also used a modifi ed model to investigate the risk factors for neonatal mortality in this study. the bdhs could not gather information of nutritional status of all living and death children such as height, weight or size of children at the time of their birth. rather, the survey gathered information regarding nutritional status for the living children at the time of survey. hence, we used mother’s body mass index (bmi) as the proxy determinant. study variables the primary outcome variable of this study is “neonatal death”, which is defi ned as the death of a live born infant in the fi rst four weeks of life. in the descriptive analyses, the neonatal mortality rate has been expressed as percentage. the outcome variable “neonatal death” was recorded as a binary variable. the explanatory variables included in this study have been defi ned in table 1. statistical analyses both bivariate and multivariate statistical analyses have been used in this study. bivariate analyses namely chi-square tests have been applied to examine the association of various independent variables and neonatal mortality. this was followed by multivariate logistic regression analysis. the logistic regression model used in this study is as follows: 1 1 e âx p − = + β where, p is the probability of death of a child within the fi rst four weeks of his/her born, β is a vector of unknown coeffi cients and x is the vector of covariates that aff ect the outcome variable. thus the general multivariate logistic regression model can be expressed as: elog 1 i j ji i p âx â x p = = − ∑β β which expresses the log odds of the outcome variable as a linear function of the independent or explanatory variables. the results of the logistic regression analysis have been presented by regression coeffi cient (β), standard error (se), odds ratios (or) (expβ) with 95% confi dence interval (ci). the data of this study have been analysed by means of spss v17 software. results background characteristics of the children the characteristics of the study children are presented in table 2 with the prevalence of neonatal mortality by socioeconomic, demographic, environmental and maternal health care services strata. as shown in the table, slightly over two-fi fths were from poor households and a slightly less than two-fi fths were from rich households. four in fi ve children were from rural areas. the more children were from dhaka division, followed by chittagong, rajshahi, khulna, sylhet and barisal divisions respectively. with regards to mother’s education, slightly over one-fourth had no formal education and over two-fi fths had at least secondary level of education. the vast majorities were muslims. 29% of the mothers were adolescents during childbirth, almost one-third were aged 20-24, one-fi fth were aged 25-29 and the rests were of age 30-49. one-third of the children were the fi rst ranked children, one-fi fth were the second ranked children and the rest were third or higher ranked children. half of the children were male and half were female. almost 99% of the children were singleton birth and slightly over 1% was twin or multiple in terms of foetus type. approximately one-third of the mothers were thin, three in fi ve were normal and 8% were overweight or obese. one-fourth of the children used to defecate in the unhygienic places, only 8% children were from households with less polluted cooking fuel and only 3% had no facilities of safe drinking water. over half of the mothers received anc services, whereas less than one-fourth sought skilled birth assistance (sba) and only 15% births were delivered in medical facility places. prevalence of neonatal mortality the third broad column of table 2 shows the percentage of neonatal mortality by various characteristics under socioeconomic, demographic, environmental and maternal health care factors. primarily sixteen variables were included in the analyses to examine their association with neonatal mortality. overall, the neonatal mortality was 37/1000. of the variables included in the study wealth index, region, kamal smm et al <40> j. nepal paediatr. soc. table 1: operational defi nition and categorization of the variables used in the analysis variables defi nition and categorization socioeconomic factors wealth index household wealth index: (1) poorest; (2) poorer; (3) medium; (4) richer; and (5) richest. residence place of residence: (1) urban; and (2) rural. region administrative regions: (1) barisal; (2) chittagong; (3) dhaka; (4) khulna; (5) rajshahi; and (6) sylhet. maternal education mother’s level of education: (1) no education; (2) primary; (3) secondary; and (4) higher. religion religion of mother: (1) islam; and (2) others. demographic factors maternal age mother’s age at child birth: (1) 15-19; (2) 20-24; (3) 25-29; (4) 30-34; (5) 35-49. birth order birth rank of child: (1) first; (2) second; (3) third; (4) fourth; and (5) fifth or above sex of child sex of child: (1) male; and (2) female. number of foetus whether child is twin: (1) single; and (2) twin. mother’s bmi mother’s body mass index: (1)thin (bmi < 18.5); (2) normal (bmi = 18.5-24.5); and over weight/obese (bmi > 24.5). sex of child, mother’s bmi, drinking water facility and maternal health care factors did not show to have signifi cant association with neonatal mortality. the variables identifi ed to have signifi cant association with neonatal mortality were place of residence, maternal education, religion, maternal age, type of foetus, toilet facility and cooking fuel. as expected, the prevalence of neonatal mortality was 1.1% higher in rural than urban areas (urban 2.8% vs. rural 3.9%). although, the administrative regions did not show signifi cant association with neonatal mortality, the prevalence varied 30/1,000 in khulna division to 52/1,000 in sylhet division. maternal education and maternal age showed negative association with neonatal mortality. the muslims had higher rate of neonatal mortality than their peer non-muslims. the prevalence of neonatal mortality was higher for the fi rst and fi fth or higher ranked births than that of the the second, third or fourth. the prevalence of neonatal mortality among twin or multiple births was 325/1,000 as against of 33/1,000 for singleton birth. households with unhygienic toilet facility and polluted cooking fuel showed higher prevalence of neonatal mortality than those with hygienic toilet facility and less polluted cooking fuel. results of multivariate logistic regression analysis the results of the multivariate logistic regression analysis have been shown in table 3. all the variables identifi ed to have signifi cant association with neonatal mortality were included in the multivariate analysis to assess their net eff ect. after controlling for other confounding factors, the environmental factors and maternal health care services appeared to have no signifi cant eff ect on neonatal mortality. the variables showed to have net eff ect on neonatal mortality were maternal education, religion, maternal age, birth order and type of foetus. maternal education showed negative relationship with neonatal mortality. for instance, the odds of neonatal death was signifi cantly 28%, 33% and 85% lower among children whose mother had primary, secondary and higher education than those who had mothers with no formal education. the non-muslim children had 40% lower risk of neonatal death as compared to the muslim children. maternal age showed negative relationship with neonatal mortality of the children. as compared to fi rst ranked birth, the second ranked birth had signifi cantly 50% lower risk of neonatal death, whereas the diff erence of likelihood of neonatal death between third and higher ranked births and the fi rst ranked had no signifi cant eff ect. the twin or multiple births were at 15.2 times as likely as to be dying at neonatal period than that of the singleton birth. risk factors of neonatal mortality in bangladesh <41>j. nepal paediatr. soc. kamal smm et al variables defi nition and categorization environmental factors drinking water sources of drinking water: (1) safe water (piped, tube well bottle); and (2) unsafe water (well, pond, others) toilet facility type of toilet facilities: (1) hygienic (flush, pit latrine); and (2) unhygienic (open fi eld, jungle etc.) cooking fuel type of cooking fuel: (1) less polluted (gas, kerosin); and polluted (dried cow dung, leaves, wood etc.) maternal health care behavioural factors anc seeking sought antenatal care services during pregnancy: (1) no; and (2) yes. sba seeking sought skilled birth assistance during child delivery: (1) no; and (2) yes. place of delivery medically facilitated place of child delivery: (1) no; and (2) yes. table 1 continued... table 2: background characteristics of under-fi ve children and prevalence of neonatal death, bdhs-2007 background characteristics no. of children % of neonatal death chi-square n % no yes wealth index 5.07 poorest 1367 22.6 95.8 4.2 poorer 1312 21.7 96.3 3.7 middle 1173 19.4 95.9 4.1 richer 1149 19.0 96.5 3.5 richest 1056 17.4 97.3 2.7 place of residence 2.94† urban 1249 20.6 97.2 2.8 rural 4809 79.4 96.1 3.9 region 5.87 barisal 383 6.3 96.6 3.4 chittagong 1337 22.1 96.5 3.5 dhaka 1908 31.5 96.7 3.3 khulna 578 9.5 97.0 3.0 rajshahi 1306 21.6 95.8 4.2 sylhet 547 9.0 94.8 5.2 maternal education 11.26** no education 1658 27.4 95.6 4.4 primary 1910 31.5 96.3 3.7 secondary 2122 35.0 96.4 3.6 higher 366 6.0 99.3 0.7 religion 3.35* islam 5558 91.8 96.2 3.8 other 499 8.2 97.8 2.2 maternal age 16.65*** 15-19 1769 29.2 94.8 5.2 20-24 1977 32.6 96.6 3.4 25-29 1254 20.7 97.2 2.8 30-34 714 11.8 96.9 3.1 35-49 343 5.7 98.1 1.9 <42> j. nepal paediatr. soc. table 3: multivariate logistic regression analysis showing the risk of neonatal mortality among under-fi ve children of bangladesh, bdhs-2007 background characteristics β se or 95% ci maternal education no education reference ------primary -0.33 0.18 0.72* 0.50-1.03 secondary -0.41 0.19 0.67* 0.46-0.97 higher -1.93 0.65 0.15** 0.04-0.52 religion islam reference ------other -0.51 0.32 0.60† 0.32-1.12 background characteristics no. of children % of neonatal death chi-square n % no yes birth order 16.12** first 2050 33.8 95.2 4.8 second 1568 25.9 97.7 2.3 third 1009 16.7 96.5 3.5 fourth 648 10.7 96.6 3.4 fifth+ 782 12.9 95.9 4.1 sex of child 0.62 male 3021 49.9 96.1 3.9 female 3036 50.1 96.5 3.5 number of foetus 197.50*** single 5975 98.6 96.7 3.3 twin 83 1.4 67.5 32.5 mother’s bmi 1.75 thin (bmi <18.5) 1935 32.3 96.1 3.9 normal (bmi 18.5-24.9) 3593 60.0 96.4 3.6 over weight (bmi =>25.0) 462 7.7 97.5 2.5 drinking water§ 0.01 safe 5251 96.6 96.2 3.8 unsafe 185 3.4 96.3 3.7 toilet facility§ 4.08* hygienic 4309 79.3 96.5 3.5 unhygienic 1127 20.7 95.2 4.8 cooking fuel§ 4.97* less polluted 436 8.0 98.2 1.8 polluted 5000 92.0 96.0 4.0 sought anc services‡ 1.97 no 2365 48.3 97.9 2.1 yes 2535 51.7 98.5 1.5 sought sba‡ 0.38 no 4957 81.9 96.2 3.8 yes 1093 18.1 96.6 3.4 delivery setting‡ 0.13 home 5164 85.3 96.3 3.7 hospital 887 14.7 96.5 3.5 total 6058 100.0 96.3 3.7 note: level of signifi cance *** p<0.001; ** p<0.01; * p<0.05; and † p<0.10. § the fi gures do not round to 6,058 due to exclusion of non-dejure residents. ‡ the fi gures do not round to various missing information. table 2 continued … risk factors of neonatal mortality in bangladesh <43>j. nepal paediatr. soc. kamal smm et al table 3 continued … maternal age 15-19 reference ------20-24 -0.35 0.20 0.70* 0.48-1.03 25-29 -0.62 0.28 0.54* 0.31-0.93 30-34 -0.80 0.35 0.45** 0.23-0.89 35-49 -1.40 0.50 0.25** 0.09-0.66 birth order first reference ------second -0.70 0.22 0.50*** 0.32-0.76 third -0.25 0.26 0.78 0.47-1.29 fourth -0.28 0.33 0.75 0.40-1.42 fifth+ 0.14 0.35 1.14 0.58-2.26 number of foetus single reference ------twin 2.72 0.26 15.20*** 9.16-25.22 note: level of signifi cance *** p<0.001; ** p<0.01; * p<0.05; and † p<0.10. discussion to address united nationas mdg 4 on reducing childhood mortality, there is a need for better populationbased data on the rates and causes of neonatal death27. in this study it was our aim to identify the risk factors of neonatal death in bangladesh using the nationally representative 2007 bdhs data. both bivariate and multivariate statistical analyses were employed to examine the factors aff ecting neonatal death. findings showed that the prevalence of overall neonatal death was 37/1,000. our analyses revealed that socioeconomic and demographic factors rather than environmental factors and maternal health care services had signifi cant net eff ect on neonatal death. the variables infl uencing neonatal death are maternal education, religion, maternal age, birth order and type of foetus. consistent with earlier studies18,28,29,30, the fi ndings of this study revealed that the higher the mother’s education the lower the risk of neonatal mortality. a review of the literature shows that while the higher socioeconomic status of better educated women explains about half of the magnitude of the relationship between maternal education and child survival31, the domestic health practice of individual women is probably the new most salient mechanism in the maternal education child mortality relationship. the fact that mother’s education is a more important determinant of child survival than father’s education: explains that there is greater maternal involvement in child-health related care32. the mother’s education infl uences her choices and skills in health care practices32,33. for instance, both educated and illiterate mothers recognize when their child is sick but an educated mother more frequently will take action “without waiting for (her) husband or mother-in-law to notice the child’s condition too” 32. this is partly because illiterate women do feel a lack of capability when dealing with the modern world. we found that being born to a mother who practiced islam was signifi cantly associated with an increased risk of neonatal death. the infl uence of religion on neonatal mortality may be linked to the beliefs and myths they may have concerning child birth. this was the case with the faith assembly people in indiana, u.s., who believed that child bearing was an act of god not to be interfered with34. it is evident that muslim women have higher fertility, lower use rate of contraceptive method, early age at marriage and motherhood and lower access to maternal health care services than their peer non-muslim sisters in bangladesh35,36. hence, we explored this association further by introducing an interaction term between religion and maternal age (results not shown). we found that children who were born to muslim adolescent mothers had a higher risk of neonatal deaths compared to children born to nonmuslim mothers (results not shown). despite this, it is diffi cult to say whether or not this fi nding refl ects the early motherhood among the subgroup of traditional muslim women and thus further qualitative research may be useful. our fi ndings showed signifi cant increased risk between adolescent motherhood and neonatal mortality. this result is comparable to numerous studies conducted elsewhere which found higher risk of neonatal mortality in younger adolescents than older mothers19,20,22. this is partially explained by diff erences in socioeconomic factors in younger versus older women and is mediated primarily through preterm delivery, small for gestational age (sga) and low birth weight (lbw) or some interaction of these variables37. the signifi cance of young and old maternal ages at childbirth <44> j. nepal paediatr. soc. as risk factors for adverse neonatal health outcomes remains largely socioeconomic, environmental and cultural context dependent. previous research that aimed at providing an explanation to the frequently observed association between mother’s age and various quality-of-life measures, including perinatal, neonatal and infant mortality38,39, preterm delivery38,40, and low birth weight41,42 have yielded inconsistent results. birth order of a child showed controversial results for neonatal mortality. some studies showed that fi rst or lower ranked births were at higher risk of neonatal mortality, whereas some others showed that higher ranked births were at increased risk of neonatal mortality. for instance, in a study to determine the impact of maternal and child health (mch) services on child survival in a socio-economically poor rural pondicherry, india, infants of fi rst birth had higher risk of neonatal mortality than fourth or higher ranked births. the study further showed that second ranked births had the least mortality risk42. a study conducted in taiwan44 showed that children with fi rst and fi fth ranked births were at higher risk of early neonatal deaths, while in nigeria45, the children with sixth or higher order births were at increased risk of neonatal mortality. it is argued that, in most of the developing countries, higher mortality risks were found for the fi rst-born children compared to birth order two through six46. our study showed that children with fi rst ranked birth were signifi cantly at increased risk of dying at early infancy than the second or higher ranked births. social and cultural contexts may be partly attributed to the infl uence on birth order on neonatal mortality. however, our studies are consistent with most of the earlier studies as mentioned above. multiple births are relatively rare events, but contribute substantially to mortality in both neonatal and post-neonatal periods28. our study showed a reduced risk of neonatal mortality for singleton baby compared to twins or multiples. twins are more likely to be born with low birth weight and biological immaturity. possibly, the use of the curative services, which are needed in emergency situations, could be underutilized by mothers of twins47. however, one possible reason for this observed association is that multi-foetal pregnancy and multiple births including twins and higher order multiples such as triplets and quadruplets are high-risk pregnancy and birth. these high-risk births are frequently accompanied by a number of associated foetal and neonatal complications that require special and expensive medical care48. in addition, multiplebirth children are at greater risk of birth defects and/ or disabilities and accounted for larger percentage of prenatal deaths49. therefore, mortality of these highrisk groups contributes to the higher rate of childhood mortality especially during the early period of life. this fi nding is consistent with earlier studies conducted elsewhere28,47,48,49,50. it is important to discuss the limitations of this study as well as its strengths. the fi rst potential limitation of this study is the cross-sectional nature of the analyses. second, the information used in this study is based on the retrospective birth history of children and reported characteristics of mothers and households by the mother’s of the children, which may cause underreporting in age. third, only surviving women were interviewed, which may have lead to an underestimate of the neonatal mortality rate, because of the association of neonatal deaths with maternal deaths. this could also have lead to an underestimate of the eff ect of some of the associated factors, such as delivery complications6. fourth, as a secondary data set, we could not include weight of child at the time of birth which is an important predictor in the study of neonatal mortality. another limitation with household wealth indices derived from dhs is that those are based on current status data so that the variable might not capture the true level of household wealth during the infancy of children born several years before the survey. however, since these analyses are restricted to births within fi ve years of the surveys, this biasness will not be substantial. despite these limitations, the strength of the study is that it dealt with a large nationally representative sample size with stratifi ed random sampling gathered by international standardized method, which are mostly used in public health research. conclusion in summary, we found that twin or multiple births are more likely to be dying during the fi rst four weeks of life as compared to singleton children. maternal education, religion, maternal age and birth order are also important determinants of neonatal mortality in bangladesh. this evidence suggests that improving maternal education may be key factor to improving child survival in bangladesh. a well educated mother has a better chance of satisfying important factors that can improve infant survival: the quality of infant feeding, general care, household sanitation, and adequate use of preventive and curative health services. adolescents should be discouraged to initiate childbearing at the teen ages not only for the reduction of neonatal mortality, also for their physical and psychological maturity that may bring overall wellbeing to mother and child health. acknowledgements: none funding: nil confl ict of interest: none permission from irb: data used in this study have been taken from a nationally representative survey. the risk factors of neonatal mortality in bangladesh <45>j. nepal paediatr. soc. survey was conducted by a bangladeshi government run institution. it followed all criteria and ethical issues prescribed by world health organization. this data set is widely used for public health research in home and abroad. references 1. malqvist m. neonatal mortality: an invisible and marginalized trauma. global health action 2011;4:5724. 2. black re, cousens s, johnson hl, lawn je, rudan i, bassani dg, jha p, cambell h, walker cf, cibulskis r, eisele t, liu l, mathers c. global, regional, and national causes of child mortality in 2008: a systematic analysis. lancet 2010;375:1969-87. 3. lawn je, kerber k, enweronu-laryea c, massee bateman o. newborn survival in low resource settings –are we delivering? bjog 2009;116:49-59. 4. world health organization (who). the world 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uthman oa, uthman mb and yahaya i. a populationbased study of eff ect of multiple birth on infant mortality in nigeria. bmc pregnancy and childbirth 2008;8:41. risk factors of neonatal mortality in bangladesh how to cite this article ? kamal smm, ashrafuzzaman m, nasreen sa. risk factors of neonatal mortality in bangladesh. j nepal paediatr soc 2012;32(1):37-46. final nepas journal no.28-2.indd ���� collodion baby gurubacharya sm1, subedi k2, aryal dr3 1����� �� � ��������� ��������� �������2������������������ ��������������3������ ����� �!������������������"#�$�� ������ ���%��� �����& ���� �� ����� ������"����� address for correspondence:������ �� � ���������� ������'� � �(�� )���*��� ++ � ���� !�+������� ���!������,��������������������, ����� �������� �������������, � ����� ����� -�� � ������ �.��� � �� , ���� ������ ������ ���� ����� � �� ����� ������ ����������������� ����� � ��� �.� ������& �� � ������ �������� ������� ��� � �� ������� ���� �+� � +�� �� � ++������ �������+�� 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original article distribution of serotypes and antimicrobial resistance of streptococcus pneumoniae in a children’s hospital in nepal sherchand jb1, joshi ar2, gauchan p3, amatya j4 ����� ��� �� ��� ��������� ��� ���� ��� ��� �� � �� ���������� ��� �� �!���"!�#�$% ��� &�!�� �� '��# � (������� )�"��� ��% *+��!�� (�, ����! ���� ���!��# �!���"!�#�$%�� &�##�$� �� ��!���� ��� ������#�$%� ��#!-� !� .�/�! � 0�/����� ���� ���!��# �!���"!�#�$%� (������� 1��!���2 ��!"�/ �� ��! ���! % 3�� ! / � �� ���!�!��� '��# � (������� )�"��� ��%� ����� -�� �� &#!�!��# �!���"!�#�$%� ������,$/�,� �� �-���/� 4���#� ��%� ! +-� %�� ���� ���!��# �!���"!�#�$%�� +��!� �� ��������� !� �!���"!�#�$%2 &�##�$� �� ��!���� ��� ������#�$%� ��#!-� !� 4���# address for correspondence: ���� � � ��������� 5�-�!#2 ! ���6���# ��� ��$ �� abstract introduction: streptococcus pneumoniae !� #�7 �/-"�� !� � ��� �� ���-�# ��������%�$��# ��� �������%�$��# �#��� �� -��% ���# �% ������� ��� �#�� ��!#����� 7�!�� $�����##% ��-�!�� ���-#��� /�#��� ��� �8�� "% !��# !���� !��� �/�� �� ��--�� ��#�� !��#/��9� � � 3� �!� �! /� !��� ���/-�����/� !� ��������% �� ��$�� "/ -�% "� ��!-��% �� ��$�� !� !--/����-���-!��� ����#� ��� ��!�# ��#��!9� !�� �� ��������%�: � �� � !--��!� �#% �� �� "!� � 7! ��/ �� �#��-�� �� �!����� objectives: ��� � /�% 7�� ����/� �� � �� ��-!�� �� ��� �#���� �� ��������%�$��# ��#��!9� !�� 7! � s pneumoniae� � �� ��-!�� ��!� �� !"!� !� �/���� !"!#! % �� ��� ��� � �� ��-!�� �!� �!"/ !�� �� �!������ ���� %��� methods: '���! �# "���� ������� ! � � /�% 7�� ����/� �� ��� ;�"�/��% *<<= � ��� �-"�� *<<= +## �� ����!-��� 7��� !���/#� �� !� � => ����� "#��� �$�� ��� !��/"� �� !� � ? �<> &1 * � -������� � .= @& ��� *� ��/�� s pneumoniae 7�� !��� !�!�� "% ��#��% -�����#�$%� 0��-a� � �!�� �� ���!� �/���� !"!#! % �� ��� "!#� ��#/"!#! % �� +� !"!� !� �/���� !"!#! % �� 7�� ������-�� "% -��!�!�� �!�"%���/�� �!�� �!��/�!�� -� ��� ��� in vitro �/���� !"!#! % �� �� 1:��!##!� ���!� �� !��#� �� � ���!�!##!� 7�� �� ��-!��� "% �� 5� �� ���� %��� 7��� �� ��-!��� "% "�� ��!�# ����$$#/ !�� !�� /�!�$ �� ���/-� �� �! �� � ��� ���/3�� ! / � ���-��8� 7! � �* ���#�� �� !���� results: 3� �� � /�%� .� b> �� �� ��!#���� 7��� ��/�� � "� ��������%�$��# ����!��� �� s pneumoniae �-��$ 7�!�� �< c> 7��� -�#� ��� �d *> 7��� ��-�#� &���� �:!-� ��<<>�� &�#���-����!��# ��<<>� ��� 5�% ���-%�!� �dc �>� 7��� -�� ����� ! � �� !"!� !� �$�!�� s pneumoniae ��� �� #��� ����� ! � 7�� &� �!-�:�9�#� �b<>� �� �> 7��� ��/�� � "� ���!� �� � 1:��!##!� 1� ������-!�$ 5� �� �� 1:��!##!� ���!� �� � ��!�� �$�!�� ���!�!##!�� �## 7��� ��/�� � "� �/���� !"#� � ���!�!##!� ��� !��#� �� � ��!�� 7��� ��/�� � "�#��$ � �b �!������ ���� %��� 7������ c> 7��� 4��� %���"#� conclusions: &���!�$� �� � !� �!-!#�� "� � !� -�#� ��� ��-�#� ���!�!##!� ���!� ���� ��� �� ��!��� !� s pneumo�!�� !��#� �� �����������%�: 7�!�� !� ��/�!�$ ���"#�7��#� 7!�� key words: &�!#����� 4�������%�$��# �7�"� s pneumoniae� ���� %��� 5� �� introduction (���!�� ��% ��� !���� !�� !� �� -�,�� ���# � ���"#� !� �� �#��!�$ ��/� �!�� 3���� !�� �� �� ����!�� ��% ��� !� �� -�� ���e/�� ��� !-��� �� ��/�� �� ���� ��!##���� !� �� ���/#� !�� 3 !� ���e/�� #% �� � �� !���� !�� � ���/� �� �� "!� �� ��� �� �� �� �� � ��# !##���� "����� ��� �� +# ��/$�� -�,��! % �� ���� ����!�� ��% ��� !���� !��� ��� !��# !� ��!$!�� ��������% "�� ��!�# !���� !�� !� �#�� ��--��� ��� !�/#��#% !� -�#��/�!���� ��� ��% %�/�$ (��!��� "�� ��!� �� /���� ����!�� ��% ��� �/�� �� s pneumoniae� h infl uenzae� s pyogenes ��� �� -�� ��--�� ��/�� ! � �$�� � �� ����!�� ��% ��� !���� !��� s pneumoniae !� ��!�� % !� �# �� !� !���� !�� �� /���� ��� #�7�� ����!�� ��% ��� ��-� !-��� �� ����!�� ��% ��� !���� !��� �� ��� ��/��� "% s pneumo�!�� ��� �!�/�! !�� � ! !� -��!�� "�����! !�� ���/-��!� � �* +����:!-� �#% * b -!##!�� ��!#���� /���� � %���� �� �$� �!� ���/�##% �� ���/-��!� �����-!��� #% !� j. nepal paediatr. soc. vol 28, no. 2 ��b� �� �� �#��!�$ 7��#�� �����:!-� �#% ��� ��#� �� ���� ��� �� ��� � �!"/ �"#� � s pneumoniae 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20 40 60 80 100 120 140 160 180 200 number percentage number 188 65 33 32 percentage 34.6 50.8 49.2 total subjects enrolled total positive male positive female positive ��=� 100.0 100.0 60.0 98.5 78.5 84.6 0.0 20.0 40.0 60.0 80.0 100.0 cephotaxime chloromphenicol cotrimoxazole erythromycin tetracycline oxacillin p er ce nt ag e antibiotics fig. 22 ���7!�$ �� !"!� !� �/���� !"!#! % �� ��� �� ��������%�$��# ���/-������# !��#� �� &���� �:!-�� &�#���-����!��# ��� 5�% ���-%�!� 7��� ��/�� � "� -�� ����� ! � ��/$� �$�!�� �� !��#� �� &� �!-�:�9�#� 7�� �� #��� ����� ! � ��/$ �� �> �� �� !��#� �� 7��� ��/�� � "� 1:��!##!� ���!� �� �;!$/�� *� 1� ������-!�$ 5� �� �� 1:��!##!� ���!� �� � ��!�� �$�!�� ���!�!##!�� �!�!-/ 3��!"! ��% &����� �� !�� ��3&� �� ���!�!##!� 7�� ��/�� � #!� "�#�7 * g$h-# !�� �!�� !�$ ���� !��#� �� � "� �/���� !"#� � ���!�!##!� ��� !��#� �� � ��!�� �� s pneumo�!�� 7��� ��/�� � "�#��$ � �b �!������ ���� %��� 7������ c> �� �� !��#� �� 7��� 4����%���"#� ���� %��� �d� b� �� ��� c 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d��)������1�=�� ��� ���,��)�� ���+������)� !�� signifi cant culture/ culture positive &�� d����� � ��� � ��� ��� ������� �)������ � �� 0���� �) �)���� 0���� e� &5�� �� ���� ��� ��!� )����� ��� + ����� c � ��� )����� ��� ��)�!� ��� ���� ��� �� �������� ��� ������ ������)���0�����)�� �)+��� ���� ����� ��� ��� ���� observation and results =������&.-���� �����29� �%�� ��f��%&4��) �� ��!� ������� )������������ ��6.�2�'34�� �� �������,���) �)������ )���������������'��28��634�0������������) �)�����! ��,�� ��������� ���� ������)���g�&5��29�!�&4�� table 1:��!�� ��� ��/� ������+)����� �� ������� ����� 2#f&.-4 sex age <1yr >1-5yr 6-10yr >10yr male = 55 (32.7%) &� �5 &5 &5 female = 113 (67.2%) &6 85 :8 �5 total :8 .5 88 :5 57% 43% culture positive uti culture negative uti fig. 1:�1����� ) �)���d����� ���� �� ��� � ���� � ������� ������ 0���� ��,��� 2-.�634��� ��� +�� ���28.�834� ���,� ����!�2:6��-34�� <������� ��� ����� )������� �����!��������)�� ��+)��� ��!� ���)�������� ����� ���� )����� ���>)������ �� �)� �� ��� �!)�� ��� �� ��)���������, !�� ������ �!��� ����� ���� ��� ��+�� �� ���*)����� ����� �� ��� ������ ����� �� ��� ������������������ ��������� ���� ��� �! �������� + ���� 2� + ���4� table 2:���!��� ����� ��� �� ��������� ���� clinical picture number (n=168) percentage fever &8. -.�63 pain abdomen '8.�83 vomiting .. :6��-3 dysuria 85 �:�-3 chills and rigors :5 &'�-3 burning micturition �. &��83 loose stool �8 &8��-3 increased frequency �� &:�53 febrile seizures �� &:�53 concomitant respiratory tract infection �� &:�53 haematuria &&5�'3 facial puffi ness &� '�&3 hepatosplenomegaly &� '�&3 constipation &� '�&3 ��8� oliguria &� '�&3 vaginal discharge &5 ��6�3 irritability &5 ��6�3 failure to thrive &5 ��6�3 decreased appetite . :��'3 bed wetting 8 ��:-3 phimosis 8 ��:-3 recurrent pain abdomen 8 ��:-3 encopresis & 5��63 ���� ������ �����! ��� ���� ����0 �� e.coli� 2�534��� �0���+�� klebsiella pneumoniae 2&.�..34�� proteus vulgaris� 2&���534�� enterococcus� 2.���34�� citrobacter species, enterobacter� ���staphylococcus aureu��0������� �������8�&53�� ����pseudomonas aerogenosa 0 ����� ��������� ����5-3����� �����29�!��4�� #�� � ���+�����! ��� �����)�!� � �0 ������������ � ��!� ����� ���! ����������� �" ����� �� ���� ��!������������,����26.34�0�� ��8�&.3����enterococcus� ��� pseudomonas� 0���� ������ ���� #��� � ����� ��� 7��� ������ ���������,�������'5�-3� ���.8�-.3�������� ��,� ���������������! ��� ����� �������� ������7��� ����� 0���� citrobacter, enterobacter� ��� staphylococcus�� �������/ ���� ���#�����/ ����0������)������+�������� ��,����� ���� �+ ����� ��/�����pseudomonas�0����� 0 ��&553������� �������#�����/ �����# ���/��� ���� ��� ����� �/ *� �� ���0��� , �� + �� ������ ���� ��!��� ���� ��! ��� �� ��� ����� �������,���� ���� ; ��� ����� 2&5534�� ��� �/��� 2�534�� <���/ ���� 2::�::34� ��� ����� /���� 2::�::34� 0 �� ������� �� �� ��� &�� � ����� � ��!���� �; ��� �����0 ����)������+��&553����� ����,�� to enterococcus, staphylococcus, e.coli� ��� klebsiella���!�������!������������� ����0 ����������0���� � �/��� ���26�34��� ���� ���2-��:.34�� ��� *��� �� 2-��'&34�� � �/��� ,� 2-:�::34�� ���� /� �� 2'.�8'34�� �� *� ���2.��-�34��<���/ ����2..�..34� ��� ����� /���2..�..34�� �����������,����� ���������, ���)����! ��� ��0 �� ��� ��)������e.coli ����������+������0����� ���! ���������� 9 )���>)��� ����� ��� #�����)� ������ +)�� ���� ���� � ��!�� ������ ���� ��� ���� ��� ���� ��� ���������� 1�����)� �� �� �������,���� 0���� ��)���� !���� ����� ����� ��0 ���������������� ���� �����d����� ����0 �� �����������0����� ���� ����� �/��� ��� ���� �/�� � ,�������������,����� ���������klebsiella ��������)�� 0 �� �� � �� 0���� ��0� ����� ������������ �"�� �����)�� ���0��!���!�����������,����� ��������0 ����#�����/ ���� 2�534�� #�����)� ������ 2::�::34�� ����� �/ *� �� 28&�..34� ���� � �������� �+��� ����0��!��������,� �������#�����/ ����2:&���34�#�����)� �����2&���34� ��� ����� �/ *� ��2&-�'�34��� ��!�������!� ���! ��,�� + ����� � ��)����� �� �� enterococcus� 0 �� &553� ������ � ������� �" ��������)!�� ���� ���! ��� �����0��� -5�&553� ������ ���� ��� ������ ����� enterococcus� 0 �� &553� �������,�� ��� �/��� ,�� � ������� ��� ���� ��� ���������0�������������,���������+ ����� ���� �� ����� ���������� ����+ ����� ��/����� enterobacter�0 �� �������,�� ��� #��� �)� ������� citrobacter, enterobacter� ��� staphylococcus� ���0��� �� �� ��������� ��� ���� � ���! ��������� �/����� 7��� ������ ; ��� ����� ���0������������ ����+)�������������,��������; ��� ����� 0 ����������� �����&8�� ����2&8��34� 50% 16.66% 12.50% 6.25% 4.10% 4.10% 4.10% 2.08% 0% 10% 20% 30% 40% 50% tage bacterias series1 50% 16.66 12.50 6.25% 4.10% 4.10% 4.10% 2.08% e.coli klebsi prote enter staph enter citrob pseud fig. 2:�b ����� ���� ��������) �)���������,��� ��� percentage ���� antibiotic sensitivity tested sensitivity not tested sensitive intermediate sensitive resistant no % no % no. % amikacin �5 8. 86.�5 5� 5 �� 8�� netilimycin 88:8 '5�8� -�: &5� �5�gentamycin '8 �� 8.8�6 5� 5 �.� :��& amoxy-clav :. .5 . &.�' 5� 5 :5� -:�: ampicillin -� &8 &� &8�. 5� 5 '5� -��8 amoxycillin �5 8. 8 -�5 5� 5 8.� 6��5 cefotaxime :8 .� �:�� 5� 5 �.� '.�� cefazoline '5 �. �8 :8�: �� ��6 88� .��ceftazidime &8 -� � &8�: 5� 5 &� -��' ceftriaxone &� -8 8 ::�: 5� 5 -� ..�' cephalexin &� -8 . �5�5 5� 5 .� �5�5 ciprofl oxacin 8� �8 :8� -5�6 �� 8�.� &8�: nalidixic acid :� .8 &8 8:�5� 5 &-� �.�� norfl oxacin '8 �� �'-�: 5� 5 &.� �&�. co-trimoxazole .. :5 : �'�. 5� 5 �� 8��8 nitrofurantoin �8 8� 8--�6 5� 5 .� &&�& vancomycin &8 -� &8 &55�5 5� 5 5� 5 ofl oxacin &� -8 8 ::�: 5� 5 -� ..�' table 3:�����+��!� ���� ) �)���������,�� ����2#f6.4 ��.� table 4a:���������+�������������,�����������<�! ��� ��=�� ������� ����� ����� organisms (% sensitive) drugs e .c ol i [n =4 8] (% ) k le bs ie lla p ne um on ia [n =1 6] (% ) p ro te us [n = 12 ]( % ) e nt er oc oc cu s [n =6 ]( % ) c itr ob ac te r [n =4 ]( % ) e nt er ob ac te r [n =4 ] ( % ) st ap h ae ru s [n =4 ]( % ) p ae ru gi no sa [n =2 ] ( % ) amikacin (n=50) is=0 �f�8 100% df5� �f100% df5� �f. 100% df5 �f5 df� 100% �f8 100% df5 �f8 100% df5 �f� 100% df5 #� netlimycin (n=48) �f&69.2% df8 &��:-3 =�f8 &��:-3 �f8 100% df5 =�f5 �f8 66.66% df� ::�::3 =�f5 #� �f� �53 df� �53 =�f5 �f� 100% df5 =�f5 �f5 df� 100% =�f5 �f� 100% df5 =�f5 gentamycin (n=74) is=0 �f�. 72.22% df&5 �'�''3 �f66.66% df8 ::�::3 �f80% df� �53 �f8 100% df5 53 �f5 53 df8 100% �f5 53 df8 100% �f5 53 df� 100% �f� 100% df5 53 ampicillin (n=82) is=0 �f�. 2&��'-34 df:� 84.2% �f� 2&8��-34 df&� 85.71% �f� 2&.�.34 df&5 83.33% �f� �53 df� �53 �f5 2534 df8 100% �f5 �2534 df8 100% �f5 2534 df8 100% �f5 2534 df� 100% amoxycillin (n=50) is=0 �f� -�::3 df�� 91.66% �f5 53 df100% �f� �53 df80% �f5 2534 df� 100% �f5 53 df� 100% �f5 53 df� 100% �f5 53 df� 100% #� amox-clav (n=36) is=0 �f5 53 df&100% �f5 53 df� 100% �f5 53 df. 100% �f8 100% df5 53 �f5 2534 df8 100% �f5 53 df8 100% #� #� r= resistant s=sensitive is=intermediate sensitive nd=sensitivity not tested ��'� organisms (% sensitive) drugs e .c ol i [n =4 8] (% ) k le bs ie lla pn eu m on ia [n =1 6] (% ) p ro te us [n = 12 ]( % ) e nt er oc oc cu [n =6 ]( % ) c itr ob ac te r [n =4 ]( % ) e nt er ob ac te r [n =4 ] ( % ) st ap h. a er us [n =4 ]( % ) p se ud om on as [n =2 ] ( % ) cephalhexin (n=12) is=0 �f�� 100% df5 53 �f� 100% df5 53 �f� �53 df� �53 #� �f5 �3 df� 100% #� �f5 53 df� 100% #� cefotaxime (n=34) is=0 �f8 �����3 df&8 77.77% �f� ::�::3 df8 66.66% �f5 53 df� 100% #� �f� �53 df� �53 �f5 53 df8 100% #� #� ceftriaxone (n=12) is=0 �f� ::�::3 df8 66.66% #� #� #� #� �f5 53 df� 100% �f5 53 df� 100% �f� 100% df5 53 cephazolin (n=70) �f&� :&��'3 df�8 63.15% =�f� &5���3 �f. 75% df� ��3 =�f5 53 �f8 ::�::3 df66.66% =�f5 53 #� �f5 53 df8 100% =�f5 53 �f5 53 df8 100% =�f5 53 �f5 53 df� 100% =�f5 53 �f5 53 df� 100% =�f5 53 ceftazidime (n=14) is=0 �f� ::�::3 df8 66.66% #� �f5 53 df� 100% #� �f5 53 df� 100% �f5 53 df� 100% #� �f5 53 df� 100% ofl oxacin (n=12) is=0 �f� ::�::3 df8 66.66% #� #� �f� 100% df5 53 #� �f5 53 df� 100% �f5 53 df� 100% #� table 4b:���������+�������������,�����������<�! ��� ��=�� ������� ����� ����� r= resistant s=sensitive is=intermediate sensitive nd=sensitivity not tested ��-� organisms (% sensitive) drugs e .c ol i[ n= 48 ] (% ) k le bs ie lla p ne um on ia [n =1 6] (% ) p ro te us [n = 12 ]( % ) e nt er oc oc cu s[ n= 6] (% ) c itr ob ac te r [n =4 ]( % ) e nt er ob ac te r sp p. [n =4 ] (% ) st ap h. au re us [n =4 ]( % ) p se ud om on as [n =2 ] ( % ) ciprofl oxacin (n=42) �f&� 60% df. :53 =�f� &53 �f8 100% df5 53 =�f5 53 �f8 100% df5 53 =�f5 53 �f8 100% df5 53 =�f5 53 �f� 100% df5 53 =�f5 53 �f8 100% df5 53 =�f5 53 �f� 100% df5 53 =�f5 53 �f� 100% df5 53 =�f5 53 norfl oxacin (n=74) is =0 �f�73.68% df&5 �.�:&3 �f&5 83.33% df� &.�..3 �f&� 100% df5 53 �f� 100% df5 53 �f� �53 df� �53 �f� 100% df5 53 �f� 100% df5 53 �f5 53 df� 100% nalidixic acid (n=32) is=0 �f. �'��'3 df&. 72.72% �f� 100% df5 53 �f8 100% df5 53 #� #� �f� 100% df5 53 #� �f5 53 df� 100% nitrofurantoin (n=54) is=0 �f:5 100% df5 53 �f8 �53 df8 �53 �f100% df5 53 �f� 100% df5 53 �f� 100% df5 53 �f5 53 df� 100% #� �f� 100% df5 53 co-trimoxazole (n=66) is=0 �f&. 88�883 df�5 �����3 �f. �53 df. �53 �f&5 100% df5 53 #� �f� 100% df5 53 �f� 100% df5 53 �f� �53 df� �53 #� vancomycin (n=14) is=0 r=0 �f� 100% �f� 100% #� �f. 100% #� #� �f8 100% #� table 4c: ��������+�������������,�����������<�! ��� ��=�� ������� ����� ����� r= resistant s=sensitive is=intermediate sensitive nd=sensitivity not tested ��6� discussion 1��� ����� �������������� ����� ��,� ���� ������ ��� ����� ��� ��� ��� ���� )��������,���0���� +������ ���������)��� ����� ��� ��&������/ ���� �������1�=� ��� )�"��0��+�� )��� �� ��� ��853��������������������� � ���� ������ ����� ��� �� ��� �����=��� ��+��������� ������ �� ����/� �� ��:3����!�� �� ���&3����+���� � ,���� ��� ����1�=�+���������� !�����&&��� �����h��� ��������)������� ����� ��)�����'������������!�� �� ����� �����������+����0� �� ,�� ��� ��� ������) �)������� ��� ���)��� ����� �������������+����/��� ������ !��:� =���������)������2:��'34� ��� ���&&:�2.'��34� �� ���0���� ������� ��� ����&%��0������ !������ ���� ,��1�=��� ��!������������ ������-.�63����������� 0������+�� ��1�=� ��������� ����!�&:�&3�� �������� �� ��� ��� 1��� ��� �� ��� �� �"�� ���)�� � 2�:�-34�� +)����!� )����2&��834�� ����� ���� ���>)����� 2&:34�� � � �)�� �2&534��� �!)�� �2'�&34��+���0�����!�2��:-34� 0���� ����������<������� ��� ��0����� ��� +�� ��� 28.�834��,� ����!�2:6��-34����� �� �����!����2&'�-34�� ��������� �2&8��-34����+�� �����*)����2&:34������� �� ��� ������ ������� ��������������2&:34��'�&3�� �������������� 0����� �� ��)���������������� ��������� ���� ��� �! 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