key: cord-017248-a37t31u1
authors: nan
title: Alphabetic Listing of Diseases and Conditions
date: 2010-05-17
journal: Handbook of Autopsy Practice
DOI: 10.1007/978-1-59745-127-7_17
sha: 
doc_id: 17248
cord_uid: a37t31u1

Part II begins with a list of special histologic stains, their for use and their corresponding references. At the end of this list is a procedure for removal of formalin precipitate from tissue sections.

diseases. There may also be a list of Possible Associated Conditions. These entities are generally linked pathogenetically to the main disease entry. Any asterisk after a related disease indicates that that disorder is also listed as a disease entry.

Many disease entries will be followed by a three-column table that provides the reader with a listing of the pathologic findings to be expected with the disease as well as the prosection and dissection procedures necessary to demonstrate those findings. It is expected that routine hematoxylin-eosin stains will be done on all sections submitted for histologic examination. Special stains will be recommended in the Procedures column of the tables, when indicated. Any table immediately following the two columns of disease entries always refers to the disease in the right column.

Prepare smears of undiluted blood. Obtain blood for molecular studies For preservation of small intestinal mucosa and for preparation for study under dissecting microscope, see Part I, Chapter 2. Submit sample for histologic study. Submit stool for chemical analysis. Record weight and submit sample for histologic study. Freeze liver for molecular studies Record appearance of spine (see also chest roentgenogram). For removal and specimen preparation, see Chapter 4. Request Luxol fast blue stain.

For removal and specimen preparation, see Chapter 5.

Below-normal weight in infants. Kyphoscoliosis.

Very low concentrations of cholesterol and decreased triglycerides; serum~-lipoprotein or absent; a.-lipoproteins present. Acanthocytosis (spiny red cells). Gene mutations (4) . Abnormal shape of villi; vacuolation of epithelial cells.

Fatty stools Fatty changes.

Gene mutations (4) Systemic manifestations of malabsorption syndrome* and of vitamin A deficiency. * Kyphoscoliosis.

Axonal degeneration of the spinocerebellar tracts; demyelination of the fasciculus cuneatus and gracilis (2) . Possible involvement of posterior columns, pyramidal tracts, and peripheral nerves. Atypical retinitis pigmentosa (2) with involvement of macula. Angioid streaks (3) .

Synonym: Cerebral abscess. NOTE: For microbiologic study of tissues and abscesses, see Part I, Chapter 7. Include samples for anaerobic culture. It is best to study the brain after fixation but if specimen is examined fresh, aspirate and prepare smears of abscess content. Photograph surface and coronal slices of brain. Request Giemsa stain, Gram stain, PAS stain, and Grocott's methenamine silver stain for fungi.

External examination If there is evidence of trauma, see also under "Injury, head." Prepare roentgenograms of chest and skull. Submit for microbiologic study. For removal and specimen preparation, see Chapter 4. For microbiologic study, photography, and special stains, see under "Note." For exposure of venous sinuses, see Chapter 4. Sample walls of sinuses for histologic study. For exposure of paranasal sinuses, mastoid cells, and middle ears, see Chapter 4.

For removal and specimen preparation, see Chapter 5.

Procedures depend on suspected lesions as listed in right-hand column.

Skin infections in upper half of face. Edema of forehead, eyelids, and base of nose, proptosis, and chemosis indicate cerebral venous sinus thrombosis. * Trauma; craniotomy wounds. Skull fracture and other traumatic lesions. For possible intrathoracic lesions, see below under "Other organs."

The National Transportation Safety Board (NTSB)* has authority over aircraft wreckage and the legal authority to investigate and to determine the cause of air crashes. (1) The dead are the responsibility of the medical examiner or coroner. Local police will seal off the area of the crash. Other than for the purpose of determining that death has occurred, no one should be allowed to approach the bodies or any objects until the identification teams and the medical examiner or coroner have taken charge.

The sudden influx of bodies after a commercial air carrier accident and the request for speedy identification of the victims would overburden almost any institution. Managing such a disaster is eased by writing a contingency plan beforehand. Temporary morgue facilities may have to be established near the scene of the crash. Refrigerated trucks may serve as storage space. A practical approach is to deal first with those bodies that seem to be the easiest to identify, in order to narrow the field for the more difficult cases.

If bodies are scattered, their locations can be referenced to stakes in the ground or spray paint on pavement; only then should these bodies (or parts) and personal effects be collected. For large-scale crashes a locations can be referenced to a string-line grid benchmarked to GPS coordinates. Records and diagrams of the relative positions of victims are prepared during this phase. If bodies are still within the airplane, their positions are recorded, and photographed.

The personnel of the medical examiner or coroner can augmented by D-MORT team staffed by forensic pathologists, anthropologists, dentists, morgue technicians, and investigators supplied by the National Disaster Medical System. ** The airline will provide a list of the passengers and the Federal Bureau of Investigation (FBI) disaster team will make itself available to take and identify fingerprints and aid in the acquisition of other identifying data such as age, race, weight, height, and hair color and style. If dental records can be obtained, this provides one of the most certain methods of identification. A medical history indicating amputations, internal prostheses, or other characteristic surgical interventions or the presence of nephrolithiasis, gallstones, and the like will be helpful. Fingerprints (and footprints of babies) should be taken in all instances. Wallets with identification cards,jewelry, name tags in clothing, or other personal belongings may provide the fastest tentative identification.

The medical examiner may elect to autopsy only the flight crew but not the passengers of an aircraft crash. However, the grossly identifiable fatal injuries should be described, photographed, and x-rayed. This may reveal identifying body changes. If comparison of somatic radiographs, dental records, fingerprints, or photographs do not identify the victim, DNA comparison must be considered. Burned or fragmented bodies of passengers and the bodies ofcrew members, and particularly the pilots, must have a complete autopsy, including roentgenographic and toxicologic examinations, which must always include alcohol and carbon monoxide determinations. Internal examination might reveal a coronary occlusion, or roentgenograms may disclose a bullet as evidence that violence preceded the crash. In some airplane crashes, particularly in light airplane accidents, suicide must be considered. In such cases police investigation is required to determine if the pilot exhibited suicidal ideation in the recent past.. When resources permit, autopsies should be performed on all deceased occupants of aircraft crashes, including passengers, in order to distinguish among blunt impact trauma, smoke inhalation, and flash fires as causes ofdeath, and to answer future questions concerning pain and suffering, intoxication, and sequence of survivorship.

After a crash victim has been identified, the coroner or medical examiner will issue a death certificate. If remains of a decedent cannot be found, a judge can, upon petition, declare a passenger dead and sign a death certificate prepared by a medical examiner.

*Phone # ofNTSB Command Center: 202-314-6000 **Phone # of DMORT: 800-872-6367. entry should be followed. Usually, the circumstances that led to drowning are not apparent from the autopsy findings but can be reconstructed from reports of witnesses and the police. Because the reflex drive to seek air is triggered by hypercarbia, not hypoxia, loss of consciousness and drowning can ensue after hyperventilation and breath-holding by experienced swimmers who then drown without a struggle. There are no specific autopsy findings. A search for trauma, including a posterior neck dissection, should be made in all instances. Head and cervical injuries may be responsible for loss of consciousness and drowning, usually in individuals diving into shallow water. Toxicologic examination as described below for scuba diving accidents is always indicated.

With scuba diving fatalities, investigation of the equipment and circumstances is usually more important than the autopsy. Scuba fatalities should be studied by or with the aid of diving experts-for instance, members of a diving club or shop (not the one providing the gear used by the decedent) or the U.S. Navy. (1) Careful investigation of the scene and study of reports of witnesses and the police are essential. The investigation should ascertain the site of diving (currents and other underwater hazards), the estimated depth, the water temperature (exposure to cold), and a description of water clarity. Electrocution should be considered if the site has electric underwater cables (see "Injury, electric"). Cerebral concussion should be considered if explosives were used in the vicinity. Knowledge of the method of recovery of the body and the type of resuscitation efforts can aid in the interpretation of apparent wounds. The medical history of the diving victim should be sought, as it may lead to a diagnosis for which the autopsy is typically silent, such as seizure disorder, or may reveal asthma, emphysema, or chronic bronchitis, all of which increase the risk of air trapping and arterial air embolism.

Although drowning may be the terminal event in some scuba deaths, the investigation should be focused on the adverse environmental and equipment factors that place a capable swimmer at risk of drowning (see "Embolism, air" and "Sickness, decompression"). Because scuba divers risk arterial air embolism if they ascend with a closed glottis, on can attempt to document gas bubbles at autopsy, but their interpretation is problematic: Bodies recovered immediately are subjected to resuscitation efforts, which can by themselves produce extra-alveolar air artifacts, and bodies not recovered immediately tend to be found in a putrefied condition, full of postmortem gas. In the remaining cases, the pathologist must consider the potential of introducing artifactual gas bubbles by the forcible retraction of the chest plate and by sawing the calvarium. The following procedures apply primarily to scuba diving accidents. Interrogation of witnesses is important; the behavior and complaints of the decedent, if any, might help distinguish between a natural death by heart disease and an unnatural death by air embolism.

External examination Eyes and ears Head (skull and brain)

Chest Blood (from heart and peripheral vessels) Heart Tracheobronchial tree and lungs A Procedures Photograph victim as recovered and after removal of wet suit and other diving gear. Record condition of clothing and gear. Impound all diving equipment for study by experts, particularly scuba tank, breathing hoses, and regulators. Residual air in tank should be analyzed.

Record color of skin (including face, back, soles, palms, and scalp). Palpate skin and record presence or absence of crepitation. Record extent and character of wounds. Prepare histologic specimens.

Record appearance of face (including oral and nasal cavities) and of ears.

Prepare roentgenograms. If air embolism must be expected, as in the presence of pneumomediastinum, follow procedures described under "Embolism, air." For evaluation of findings, see also above under "Note." If decompression sickness (Caisson Disease) is suspected, also prepare roentgenograms of the elbows, hips, and knees. Otoscopic examination. Funduscopic examination. Save vitreous for possible toxicologic and other studies. For removal of brain, see Chapter 4. Record contents of arteries of the circle of Willis and its major branches and basilar artery.

Strip dura from base of skull and from calvarium. For removal and specimen preparation, see Chapter 4.

For demonstration of pneumothorax, see under "Pneumothorax". If gas is visible in coronary arteries, photograph. Photograph and aspirate gas in heart chambers. Submit samples of heart blood and peripheral blood for toxicologic study and drug screen.

Examine lungs in situ. Save bronchial washings for analysis of debris. Fresh dissection is recommended. If decompression sickness is suspected, prepare Sudan stains from fresh-frozen lung sections.

Complete toxicologic sampling should be carried out (see Chapter 13). Record nature of gastric contents. Remove neck organs toward end of autopsy. For posterior neck dissection, see Chapter 4. Incise tongue.

For removal, see Chapter 4.

For removal, see Chapter 4.

For removal, prosthetic repair, and specimen preparation, see Chapter 2. Consult roentgenograms.

In decompression sickness, fatty change of liver, and ischemic infarctions of many organs.

Interstitial emphysema. Aspiration (see above). Trauma to cervical spine. Mottled pallor of tongue after air embolism. Contusion of tongue after convulsive chewing. Nitrogen bubbles in spinal cord arteries may occur after rapid ascent. Air embolism;' cerebral edema in decompression sickness. Aseptic necroses (infarcts, "dysbaric osteonecrosis"), most often in head of femur, distal femur, and proximal tibia. Infarcts indicate repeated hyperbaric exposures. Nitrogen bubbles in and about joints and in periosteal vessels ("bends") occur during rapid ascent.

Related Terms: Automobile accident; motorcycle accident. NOTE: A visit to the scene can make the interpretation of the autopsy findings easier. The vehicle can also be inspected in a more leisurely fashion at the impound lot. This is particularly useful for correlating patterned injuries with objects in the vehicle. Most vehicular crashes occur as intersection crashes or because a vehicle with excessive speed left a curved road.

The medical examiner or coroner should gain a basic understanding of the crash mechanism so that informed descriptions can be rendered, e.g., "Impact to the B pillar of the decedent's automobile by the front of a pickup truck which failed to stop for a stop sign at an intersection, resulting in a 2-feet intrusion into the cabin; restraint belts not employed; air bag deployed; extrication required which took 15 minutes."

Police are responsible for determining mechanical and environmental risk factors for the crash and for determining some human risk factors such as suicidal or homicidal intent. The pathologist determines other risk factors for crashes such as heart disease, a history of epilepsy, and intoxication by carbon monoxide, drugs, and alcohol.

Suicide as a manner of death should be considered when a single-occupant vehicle strikes a bridge abutment or a large tree head-on, with no evidence of evasive action or braking. In such a situation, the standard police traffic investigation should be supplemented of interviews of the victim's family and friends.

The ambulance run sheet is an invaluable source of observations that often are not available from the police. This document should be acquired in all instances, even if the paramedics determined that death occurred and did not transport.

The basic autopsy procedures are listed below. Most traffic victims who die at the scene or who are dead on arrival at the hospital died from neurogenic shock caused by wounds of the head or vertebral column, or from exsanguination from a tom vessel or heart. As such, they have little lividity, and little blood is found in the vehicles. Presence ofintense lividity may indicate suffocation or heart disease as a cause of death.

If postural asphyxia is suspected, the first responders to the scene should be interviewed to determine the position of the decedent in the vehicle, and the vital signs, ifany, ofthe decedent from the time of the crash to the time of extrication. Posterior neck dissection is indicated in these instances.

If manifestations of heart disease, intense lividity, and absence oflethal wounds suggest that a crash occurred because the driver was dead, other drivers on the road may have observed that the victim was slumped at the wheel before the crash. The determination of heart attack at the wheel is usually simple, because most such victims realize that something is wrong, and bring the vehicle to a stop at the side of the road, or coast gently into a fixed object. In such instances, damage to the vehicle is minor, and wounds to the decedent are usually trivial.

While pattemed wounds can often be matched to objects (see below), patternless wounds usually cannot be visually matched to specific objects, although an opinion can sometimes be given as to what object was struck, based on the direction of motion and position ofthe body with respect to the vehicle. Impacts with the A-pillar produce narrow vertical zones of facial laceration and fractures extending from forehead to jaw. Tempered glass shatters into small cubes on impact, and leaves so-called "dicing" wounds, which are abraded cuts arranged in a somewhat rectilinear pattern. Windshield glass leaves shallow, abraded, vertically oriented cuts on the face or scalp.

With pedestrians, the lower extremities are of particular forensic interest, to determine the height and direction of impact from vehicles that left the scene. Scalp hair and blood should be collected from such "hit and run" victims and from occupants of a suspect car if police have a question as to which occupant was the driver; these exemplars can be compared to fibers and tissue recovered from the vehicle in question. Likewise, foreign material in wounds can sometimes be matched to suspect vehicles, and should be sought and retained as evidence. For pedestrians, the distance between the impact point on the lower extremities and the soles of the feet should be recorded. The legs should be opened to inspect tibial fractures; cortical fractures initiate propagation opposite to the side of impact, where they usually have a pulled-apart appearance, and then splinter the cortex at the side of impact. Abrasions are better impact markers than contusions, because subcutaneous blood extravasation can be caused not only by impact to the skin, but also from blood extravasating from underlying fractures. If no cutaneous abrasions or fractures of the leg bones are found, the skin of the legs should be incised to expose contusions.

Fracture descriptions should include location in the bone (e.g., proximal metaphysis or shaft), whether the fracture is complete or incomplete, and whether the fracture is displaced or distracted. Lacerations of intervertebral disks, facet joint capsules, and ligamenta flava should not be loosely termed "fractures." The presence or absence of blood extravasation in soft tissue adjacent to the fractures should be recorded, and its volume estimated if it appears severe enough.

Venous air embolism from tom dural sinuses cannot be diagnosed without a pre-autopsy chest radiograph or an in situ bubble test. If an X-ray machine is readily available, an anterior-posterior chest radiograph should be obtained in every traffic victim who dies at the scene or after a failed resuscitation attempt.

If a hemothorax is suspected, the rib cuts should be placed further lateral and the chest plate reflected so that the internal mammary vessels can be inspected before the chest plate is removed. After measuring and removing the bloody effusion, the underlying serosal surfaces should be inspected for defects. Lacerations of the heart and aorta will be obvious. Tamponaded lacerations of the aorta, around which the adventitia still holds, must be noted as such. If no lacerations are found at the usual sites, lacerations of the azygous veins must be considered, especially in association with fracture dislocations of the thoracic vertebral column; other sites are the internal mammary arteries, especially with fractures of ribs I and 2 or of the sternum, and intercostal arteries with displaced rib fractures. Only after the serosal defect is identified should the organs be removed, because that procedure creates many more holes in the serosa. For that reason, as much information as possible should be gained by in situ observation.

The only evidence of concussion of the heart may be a cardiac contusion or a sternal fracture. The usual clinical history suggests cardiovascular instability that is not associated with craniocerebral trauma and which does not respond to the infusion of intravenous volume agents.

The autopsy assistant may saw but should not retract the skull cap and remove the brain. The pathologist should observe in situ whether shallow lacerations of the pontomedullary junction with stretching of the midbrain are present. These lesions cannot be distinguished from artifact by examining the brain later. Thus, only after appropriate in situ inspection should the pathologist remove the brain.

A posterior neck dissection is required if no lethal craniocerebral or cardiovascular trauma is found, or if suffocation is suspected; neck trauma must be ruled out to diagnose suffocation in a traffic fatality. Sudden death in a patient with seemingly trivial wounds may be caused by undiagnosed trauma of the craniocervical articulation. A posterior neck dissection is required in these instances.

The diagnosis of diffuse axonal injury of the brain in victims with no appreciable survival interval requires that suffocation be ruled out and that no resuscitation from a cardiac arrest has been attempted. Clinicians are quick to apply the label "closed head injury" when a victim of a traffic crash has cerebral edema on a computerized axial tomogram of the head, even if no cerebral contusions, scalp contusions, or skull fractures are evident. This may be a misinterpretation, because cerebral edema can be caused by hypoxic encephalopathy made evident after resuscitation from a cardiac arrest, or from hypoxia caused by suffocation.

Procedures Possible or Expected Findings

Record presence of lividity.

Photograph all external wounds; measure all lacerations and any abrasions or contusions with a pattern.

Collect scalp hair and blood (see below) from victims of hit and run accidents. Collect foreign material in wounds.

Intense lividity and absence of lethal wounds may indicate that the crash occurred because the driver was dead from heart disease or suffocation. Wound documentation. Patterned injuries often sometimes be matched to objects in or about the vehicle (the most common patterned wound is that from tempered glass; see above under "Note"). Impact patterns in pedestrians may help to reconstruct the accident.

Hair and blood of the victim may be matched to transfer evidence on a vehicle suspected of having left the scene.

PART II / DISEASES AND CONDITIONS

Internal examination of body cavities Heart and great vessels Abdomen Skull and brain; neck

Soft tissue compartments at any location Prepare roentgenograms of chest is cases with head impact and skull fractures. Collect samples for toxicologic study from all victims, including passengers.

Create pleural window to detect pneumothorax.

If blood is seen, examine internal mammary vessels (see under "Note"). Measure volume of blood in cavity bleeds, and note whether chambers of heart and great vessels are collapsed or filled. Record evidence of cardiac contusion, sprain of intracardiac inferior vena cava, laceration of pericardial sac, and fracture of sternum. Laceration of heart or great vessels (measure volume of blood). Follow routine procedures for dissection of heart and great vessels (see Chapter 3) .

In situ bubble test to confirm venous air embolism.

Record evidence of trauma and volume of blood in peritoneal cavity; estimated volume of blood in retroperitoneal soft tissues. Autopsy assistant may saw the skull but pathologist should inspect brain in situ and remove it personally. For removal and specimen preparation of brain, see Chapter 4. Record brain weight. Posterior neck dissection is indicated if there is no craniocerebral or cardio-vascular trauma, or if suffocation is suspected. Record evidence of trauma and estimate volume of blood.

Venous air embolism.'

Evidence of alcohol or drug intoxication.

Pneumothorax, hemothorax, e.g., after laceration of internal mammary vessels.

Evidence of significant hemorrhage.

Indirect evidence of cardiac concussion.

Evidence of exsanguinating wounds. Evidence of cardiovascular disease that may have felled the driver before the crash. In European countries, the concentration is expressed in promille (grams per liter). In the United States, it has become customary to refer to concentration by percentage (grams per deciliter), and values in these units have been written into legislation and included in the uniform vehicle codes. Unless qualified, the use of promille or percentage does not indicate whether the result of the analysis is weight/weight, weight/ volume, orvolume/volume. Another common way ofexpressing concentration, milligrams per deciliter, has also been used to indicate alcohol concentrations. The method ofexpressing concentration must be clearly specified whenever the alcohol level is mentioned. The desired expression canbe derived from the toxicologic report by using the following equation: I,000~g/mL =100mg/dL =0.10g/dL =21.74 mmollL =1.0 promille =0.10%

What Is the Legal Interpretation of Alcohol (Ethanol) Intoxication?

Objective impairment of driving ability is observed at threshold blood alcohol concentrations of .035-.040 g/dL. As of August 2005 all states and the District of Columbia have adopted laws that make it criminal offense for a driver to operate a motor vehicle with a blood alcohol concentration of 0.08 g/ dL or greater. Many states have an enhanced penalty for high concentrations such as 0.15 g/dL or above. Several states have zero tolerance laws, under which drivers who are minors are legally operating only if their blood alcohol concentration is 0.02 g/dL or less, and in some states, not detectable at all.

Blood alcohol concentrations obtained at autopsy are valid until putrefaction begins. Specimen tubes with sodium fluoride should be used, and the specimen should be stored in the refrigerator. If the air space above the blood samples in the container is large, alcohol can evaporate and a falsely low blood alcohol level can result. Putrefactive changes before autopsy or during storage may cause a falsely high blood alcohol concentration. Ethanol can be produced in the specimen container; this is more likely in the absence of a preservative. Because fluoride inhibits bacteria far more than fungi, higher fluoride concentrations are required for the inhibition of fungal growth (4) .

Although there is no major difference in the alcohol concentrations ofblood samples from the intact heart chambers and the femoral vessels (5), autopsy samples from pooled blood in the pericardial sac or pleural cavity are unsatisfactory. We therefore recommend that blood be withdrawn from peripheral vessels. Is There Normal "Endogenous" Blood Alcohol (Ethanol) in a Living Person?

Blood alcohol concentrations are generally believed to be negligible in the absence of ingested alcohol. "Endogenous" ethanol in human blood exists at a concentration of about 0.0002 g/dL, which is below the limit of detection for most methods (6) . First in such a list would be postural asphyxia, for example, in drunks who fall asleep face down. Also, depressant drugs in the tricyclic, analgesic, barbiturate, and benzodiazepine classes all potentiate the effect of alcohol (7) . Also included in such a list would be infancy and childhood; ischemic heart disease;' chronic bronchitis and emphysema;' other chronic debilitating diseases; poisoning with carbon tetrachloride' or carbon monoxide;' and other causes of hypoxia.' How Can One Estimate Blood Alcohol (Ethanol) Concentrations From Vitreous, Urine, or Tissue Alcohol Levels and From Alcohol in Stomach Contents?

The ratio of serum, plasma, urine, vitreous, and various tissues has been compiled by Garriot (8) . The values may vary considerably. For vitreous, the ratios varied from 0.46-1.40. These variations may depend on whether blood alcohol concentrations were increasing or decreasing at the time of death. Most other body fluids and tissues showed ranges closer to 1.

Most urine values were above the blood alcohol concentrations. In another study (9) , the blood/vitreous (BN) ratio in the early absorption phase was 1.29 (range, 0.71-3.71; SD 0.57) and in the late absorption and elimination phase, the BN ratio was 0.89 (range, 0.32-1.28; SD 0.19). Blood ethanol concentrations probably can be estimated using B =1.29V for early absorption and B = 0.89V for later phases. A urinelblood ethanol ratio of 1.20 or less indicates that the deceased was in the early absorption phase.

How Can One Use Alcohol (Ethanol) Concentrations in Postmortem Specimens to Estimate the Blood Alcohol Concentration at Various Times Before Death?

With certain limitations, one can base calculations of this kind on the assumption that the blood alcohol level decreases from its peak at a fairly constant rate of 0.015-Q.018g/dL/h until death (10) . If blood is not available, conversion factors (see above) must be used. Alcoholics have been reported to metabolize at a rate of up to 0.043 g/dL/h (6) .

Example: The driver of an automobile drinks at a party until midnight. He leaves his host at about 1:30 a.m. and is involved in a head-on collision at 2:15 a.m. He dies in the emergency room at 6:35 a.m. There are multiple injuries and the patient exsanguinates. The autopsy is done at 1:30 p.m. Although this appears quite unlikely, let us assume that no satisfactory blood sample was obtained before death and that no blood or plasma expanders were given. If under such circumstances the alcohol concentration in the vitreous was found to be 0.157 g/dL, what was the alcohol concentration in the blood at the time of the accident?

Vitreous and blood alcohol concentrations may be assumed to have remained unchanged after death. Therefore, the blood alcohol level at the time of death must have been approx 0.157 (vitreous humor alcohol) x 0.89 (conversion factor, see above) = 0.14g/dl. The time interval between the accident (2:15 a.m.) and death (6:35 a.m.) is 4 hand 20 min or 4 1/3 h. If we assume that the decedent was not an alcoholic and that the blood alcohol concentration was decreasing from its peak at a constant rate of 0.015 g/dL/h, then the concentration at the time ofthe accident is estimated to have been 0.14 (concentration at time of death) + (4 1/3 x 0.015) = 0.140 + 0.065 = 0.205 g/dL or 0.2%.

The blood alcohol concentration at the time of the accident could have been lower if the victim stopped drinking later than 1h or 1 1/2 h before the accident. In the latter case, the peak alcohol level would have occurred after the accident, reflecting the time to absorb the latest drink.

The blood alcohol concentration at the time of the accident could have been lower or higher if the time when the patient stopped drinking, the time of the accident, or the time of the death is uncertain.

The blood alcohol concentration at the time of the accident could have been higher if the victim was a chronic alcoholic. The elimination rate in such persons may be as high as 0.040 mg/dL, which would change the figures in our example above to 0.140 + (4 1/3 x .040) =0.140 + 0.173 = 0.313 g/d1 or 0.3%.

Only rough estimates are possible. First, the peak blood alcohol level must be determined or calculated, as described in the previous paragraphs. Tables (see below) are available that relate blood alcohol level to the minimal amounts of whiskey, wine, or beer that must have been consumed (10) . However, tables of this type are often based on the minimum amount of alcohol circulating in the body after specific numbers of drinks; such tables do not yield reliable results if used conversely. Furthermore, inasmuch as drinking and elimination of alcohol may take place concomitantly, over a longer period the total amount of alcohol consumed may have been much greater than the tables would indicate. It cannot be lower. According to these tables, 6 pints of ordinary beer or 8 fl oz of whiskey would be the minimal amounts needed to produce a blood alcohol level of about 200 mg/dL in a person weighing 140-180 pounds. The total body alcohol can be calculated from the blood alcohol level by using Widmark's formula:

Average concentration of alcohol in entire body = .68 Concentration of alcohol in the blood In a person weighing 70 kg, the blood alcohol concentration would be increased 50 mg/dL (0.05%) by the absorption of 1oz of ethanol (20z of 100-proof whiskey).

Strength of alcohol is measured in "proof'; absolute alcohol is 200 proof. Therefore, in the United States, alcohol content as volume percent is half the proof (for example, 100-proof whiskey contains 50% alcohol by volume). The alcohol content of various beverages is shown in the following table.

Approximate Alcohol Content in Various Beverages t tOata from Glaister, Rentoul E. Medical Jurisprudence and Toxicology, 12th ed. E & S Livingstone, Edinburgh, 1966 with permission. tWithin 1 h after consumption of diluted alcohol (approx 15%) on an empty stomach, assuming body weight of 140-180 pounds (63.6-81.7 kg) reproduced from (11) with permission.

*One ounce (about 30mL) of whiskey or 120z (about 355mL) of beer.

What Is the Toxicity of Alcohol Other Than Ethanol?

In general, the toxicity increases as the number of carbon atoms in the alcohol increases. Thus, butyl alcohol is two times as toxic as ethyl alcohol: but isopropyl alcohol is only twothirds as toxic as isobutyl alcohol and one-half as toxic as amyl alcohol. Primary alcohols are more toxic than the corresponding secondary isomers (10) . Anemia, Hemolytic Synonyms and Related Terms: Acquired hemolytic anemia; extracorpuscular hemolytic anemia; hereditary hemolytic anemia (hereditary elliptocytosis, pyropoikilocytosis, stomatocytosis. spherocytosis); immunohemolytic anemia; intracor-puscular hemolytic anemia; microangiopathic hemolytic anemia; spur cell anemia.

Possible Associated Conditions: Disseminated intravascular coagulation;* eclampsia;* glucose-6-phosphatase deficiency (G6PD); hemolytic uremic syndrome;* malignant hypertension; lymphoma* and other malignancies; paroxysmal nocturnal hemo-globinuria; sickle cell disease;*thalassemia;* thrombotic thrombocytopenic purpura.* (See also below under "NOTE.") NOTE: Hemolysis also may be caused by conditions such as poisoning with chemicals or drugs, heat injury, snake bite,* or infections or may develop as a transfusion reaction* or be secondary to adenocarcinoma, heart valve prostheses (see below), liver disease (see below), renal disease, or congenital erythropoietic porphyria. *

Procedures Prepare skeletal roentgenograms.

Jaundice; skin ulcers over malleoli. In young patients: thickening of frontal and parietal bones with loss of outer table ("hairon-end" appearance); paravertebral masses caused by extramedullary hematopoiesis; deformities of metacarpals, metatarsals, and phalanges. Osteonecrosis* of femoral heads.

Remove and place in fixative as early as possible in order to minimize autolysis (alternatively, formalin can be injected in situ; see below). Samples should include oxyntic corpus and fundus mucosa.

Record weights. Submit tissue samples for histologic study. Record weight of thyroid gland. For removal and specimen preparation, see Chapter 4. Request Luxol fast blue stain.

For removal and specimen preparation, see Chapter 5. If there is a clinical diagnosis of anemia-related amblyopia, follow procedures described under "Amblyopia, nutritional." Jaundice. Manifestations of malnutrition. * Stomatitis with cheilosis and perianal ulcerations due to folic acid deficiency. Chronic exfoliative skin disorders. Vitiligo. Macrocytosis; poikilocytosis; macroovalocytes; hypersegmentation of leukocytes; abnormal platelets. Atrophic glossitis with ulcers. Pharyngoesophagitis (folic acid deficiency). Previous total or subtotal gastrectomy. Carcinoma of stomach.

Autoimmune gastritis (diffuse corporal atrophic gastritis) with intestinal metaplasia. Crohn's disease;* sprue;* other chronic inflammatory disorders; jejunal diverticula; intestinal malignancies; fish tapeworm infestation; previous intestinal resection or blind intestinal loop; enteric fistulas. Hepatosplenomegaly. Alcoholic liver disease. * Giant epithelial cells. Hyperthyroid goiter; thyroiditis. Demyelination of cerebral white matter (in advanced cases). Demyelination in posterior and lateral columns of spinal cord, most frequently in thoracic and cervical segments. Demyelination of peripheral nerves. Retinal hemorrhages; demyelination of optic nerves.

Hypercellular; megaloblastic. Myeloproliferative disorder.

Brain Other organs

If mycotic aneurysms are expected and microbiologic studies are intended, follow procedures described below under "Aneurysm, mycotic aortic." Request Verhoeff-van Gieson, Gram, and Grocott's methenamine silver stains. For cerebral arteriography, see Chapter 4. If arteriography cannot be carried out, rinse fresh blood gently from base of brain until aneurysm can be identified. Record site of rupture and estimated amount of extravascular blood. For paraffin embedding of aneurysms, careful positioning is required. Expected findings depend on type of aneurysm.

Mycotic aneurysms are often multiple and deep in brain substance.

Berry aneurysms are the most frequent types and often are multiple. Most frequent sites are the bifurcations and trifurcations of the circle of Willis. Saccular atherosclerotic aneurysms are more common than dissecting aneurysms, which are very rare. With congenital cerebral artery aneurysm: coarctation of aorta;* manifestations of hypertension;* and polycystic renal disease. With mycotic aneurysm: infective endocarditis;* pulmonary suppurative processes; and pyemia.

Aneurysm, Dissecting Aortic (See "Dissection, aortic.") Aneurysm, Membranous Septum of Heart NOTE: For general dissection techniques, see Chapter 3. Most aneurysms ofthe membranous septum probably repre-sent spontaneous closure of a membranous ventricular septal defect by the septalleafiet of the tricuspid valve.

Aneurysm, Mycotic Aortic NOTE: (I) Collect all tissues that appear to be infected. (2) Request aerobic, anaerobic, and fungal cultures. (3) Request Gram and Grocott methenamine silver stains. (4) No special precautions are indicated. (5) No serologic studies are available. (6) This is not a reportable disease.

Chest and abdominal organs Aorta Other organs Submit blood samples for bacterial culture. En masse removal of adjacent organs is recommended. Photograph all grossly identifiable lesions. Aspirate material from aneurysm or para-aortic abscess and submit for culture. Prepare sections and smears of wall of aneurysm and of aorta distant from aneurysm. Request Verhoeffvan Gieson and Gram stains.

Septicemia and infective endocarditis. * Streptococcus, staphylococcus, spirochetes, and salmonella can be found in mycotic aneurysm. Para-aortic abscess.

Septic emboli with infarction or abscess formation.

Aneurysm, Syphilitic Aortic PART II / DISEASES AND CONDITIONS

Heart and aorta

Other organs

En masse removal of organs is recommended. For coronary arteriography, see Chapter 10.

Request Verhoeff-van Gieson stain from sections at different levels of aorta, adjacent great vessels, and coronary arteries. See also under "Syphilis."

Aneurysm usually in ascending aorta. May erode adjacent bone (sternum). Syphilitic aortitis may cause intimal wrinkling, narrowing of coronary ostia, and shortening of aortic cusps. Disruption of medial elastic fibrils.

Aortic valvulitis and insufficiency;* syphilitic coronary arteritis; syphilitic myocarditis.

External examination Aorta

Prepare chest and abdominal roentgenograms. Open aorta along line of blood flow, or bisect into anterior and posterior halves. Photograph tear(s). Measure bloody effusions in body cavities. Measure or estimate amount of blood in mediastinum.

Request Verhoeff-van Gieson stain.

Cutaneous impact trauma.

Mediastinum widened by hemorrhage in case of tarnponaded dissection. A bleed into a body cavity of less-thanexsanguinating volume should point to an alternate mechanism of death such as neurogenic shock or lethal concussion; a posterior neck dissection may be required in such instances. Microscopy may show transmural rupture, false aneurysm, or localized dissection.

Angiitis (See "Arteritis, all types or type unspecified.") Angina Pectoris NOTE: See under "Disease, ischemic heart" and Chapter 3.

Angiokeratoma Corporis DitTusum (See "Disease, Fabry's.") Angiomatosis, Encephalotrigeminal (See "Disease, Sturge-Weber-Dimitri.") Angiopathy, Congophilic Cerebral Synonyms and Related Terms: Beta amyloid angiopathy due to~-amyloid peptide deposition (~A4) (associated with Alzheimer's disease; hereditary cerebral hemorrhage with amyloid angiopathy of Dutch type; or sporadic beta amyloid angiopathy); hereditary cerebral amyloid angiopathy, due to deposition of other amyloidogenic proteins such as cystatin C (Icelandic type) and others (e.g., transthyretin, gelsolin) (1).

Procedures Possible or Expected Findings

Request stains for amyloid, particularly Congo red, and thioflavine S (examine with polarized and ultraviolet light, respectively). Request immunostain for~A4. Some tissue should be kept frozen for biochemical studies.

Multiple recent cerebral cortical infarctions or small cortical hemorrhages, or both, or massive hemispheric hemorrhages, both recent and old. Amyloid deposition in leptomeninges and cortical blood vessels. Senile plaques are usually present. In some cases, angiopathy is part of Alzheimer's disease. *

Other organs A

Prepare material for electron microscopy.

Electron microscopic study permits definite confirmation of diagnosis. Organs and tissues may be minimally affected by amyloidosis. Anomaly, Coronary Artery Possible Associated Conditions: With double outlet right ventricle; persistent truncal artery; tetralogy of Fallot;* and transposition of the great arteries.* NOTE: Coronary artery between aorta and pulmonary artery, often with flap-valve angulated coronary ostium. Coronary artery may communicate with cardiac chamber, coronary sinus, or other cardiac veins, or with mediastinal vessel through pericardial vessel. Saccular aneurysm of coronary artery with abnor-mal flow, infective endarteritis of arteriovenous fistula, and myocardial infarction may be present. Ifone or both coronary arteries originate from pulmonary trunk, myocardial infarction may be present.

Heart Perform coronary angiography.

If infective endarteritis is suspected, submit blood sample for microbiologic study.

Ectopic origin of coronary arteries or single coronary artery. Sudden death. For a detailed description of possible additional findings, see above under "Note."

Anomaly, Ebstein's (See "Malformation, Ebstein's") Anorexia Nervosa NOTE: Sudden death from tachyarrhythmias may occur in advanced cases and thus, autopsy findings may not reveal the immediate cause of death.

External examination All organs

Record height and weight, and prepare photographs to show cachectic features. Record abnormalities as listed in righthand column. Follow procedures described under "Starvation." Record weight of endocrine organs and submit samples for histologic study.

Cachexia, often with preserved breast tissue; hirsutism; dry, scaly, and yellow skin (carotenemia). Mild edema may be present. Parotid glands may be enlarged. Manifestations of starvation.* Ovaries tend to be atrophic; other endocrine organs should not show abnormalities.

Synonyms: Cutaneous anthrax; gastrointestinal anthrax; pulmonary (inhalational) anthrax.

NOTE: (1) Collect all tissues that appear to be infected. This is a reportable disease. Bioterrorism must be considered in current cases.

External examination and skin

Blood Photograph cutaneous papules, vesicles, and pustules. Prepare smears and histologic sections. Submit samples for bacteriologic study.

Submit sample for serologic study.

Disseminated anthrax infection may occur without skin lesions. Edema of neck and anterior chest in nasopharyngeal anthrax. Anthrax septicemia. See above under "Note."

PART II I DISEASES AND CONDITIONS

Lungs Gastrointestinal tracts and mesentery

Neck organs

Record character and volume of effusions. After sampling for bacteriologic study (see above under "Note") perfuse one or both lungs with formalin. Extensive sampling for histologic study is indicated.

Extensive sampling for histologic study is indicated.

Photograph meningeal hemorrhage in situ.

Pleural effusions;* hemorrhagic mediastinitis; anthrax pneumonia (inhalational anthrax; Woolsorter's disease). Histologic sections reveal hemorrhagic necrosis, often with minimal inflammation and gram-positive, spore-forming, encapsulated bacilli. Gastrointestinal anthrax with mucosal edema and ulcerations. Hemorrhagic mesenteric lymphadenitis. Tongue, nasopharynx, and tonsils may be involved.

Hemorrhagic meningitis (hemorrhage tends to predominate). 

External examination Distal colon and rectum

Photograph perineum. Measure depth of anal pit, if any. Dissect distal colon, rectum, and perirectal pelvic organs in situ (as much as possible). Search for opening of fistulous tracts from lumen. Use roentgenologic study or dissection, or both, to determine course of tract.

Absence of normally located anus; anal dimple. Abnormal termination of the bowel into the trigone of the urinary bladder, the urethra distal to the verumontanum, the posterior wall of the vagina, the vulva, or the perineum.

Aortitis NOTE: See also under "Arteritis" and "Aneurysm, ascending aortic."

Heart and aorta

Other organs and tissues

Remove heart with whole length of aorta and adjacent major arteries. Record width and circumference of aorta at different levels.

Describe and photograph appearance of intima and of orifices of coronary arteries and other aortic branches. Submit multiple samples for histologic study and request Verhoeff-van Gieson stain. Procedures depend on expected findings or grossly identified abnormalities as listed in right-hand column.

Secondary aortic atherosclerosis or intimal fibroplasia. Widening of aorta; syphilitic aneurysm. * Giant cell aortitis; rheumatoid aortitis; syphilitic aortitis; Takayasu's arteritis.*

Manifestations of rheumatoid arthritis, * syphilis,* systemic sclerosis,* Hodgkin's lymphoma, and many other diseases associated with vasculitis.

External examination Brain Spine and spinal cord

Other organs

Prepare roentgenogram of spine.

For removal and specimen preparation, see Chapter 4. For removal of spinal cord and specimen preparation, see Chapter 4. Expose nerve roots. Record appearance and photograph spinal cord in situ. Submit samples of spinal cord and inflamed tissue for histologic study. Request Gram, Gomori's iron, and Grocott's methenamine silver stains. Procedures depend on expected findings or grossly identified abnormalities as listed in right-hand column.

Signs of previous spinal surgery or lumbar puncture (myelography). Evidence of previous trauma or previous myelography. Cerebral arachnoiditis.

Fibrous arachnoidal adhesions and loculated cysts.

Tuberculosis;* syphilis;* fungal or parasitic infection.

Systemic infection (see above). Ascending urinary infection or other manifestations of paraplegia.

Arch, Aortic, Interrupted Synonym: Severe coarctation. NOTE: The basic anomaly is a discrete imperforate region in the aortic arch, with a patent ductal artery joining the descending thoracic aorta. Type A interruption is between the left subclavian and ductal arteries; type B between the left subclavian and left common carotid arteries; and type C (rare) between the left common carotid and brachiocephalic (innominate) arteries. For general dissection techniques, see Part I, Chapter 3.

Possible Associated Conditions: Bicuspid aortic valve (with type A); di George syndrome* with thymic and parathyroid aplasia (with type B); hypoplasia of ascending aorta (with all types); persistent truncal artery (truncus arteriosus); ventricular septal defect.

Arrhythmia, Cardiac NOTE: See also under "Death, sudden cardiac." Toxicologic studies may be indicated, for instance, if digitalis toxicity (see "Poisoning, digitalis") is suspected. If a cardiac pacemaker had been implanted, the instrument should be tested for malfunction. Arteriosclerosis (See "Atherosclerosis.") Arteritis, All Types or Type Unspecified Synonyms and Related Terms: Allergic angiitis and granulomatosis (Churg-Strauss);* allergic vasculitis; anaphylactoid purpura* and its synonyms; angiitis; Buerger's disease;* cranial arteritis; giant cell arteritis;* granulomatous arteritis (angiitis); hypersensitivity angiitis; infectious angiitis; necrotizing arteritis; polyarteritis nodosa;* rheumatic arteritis; rheumatoid arteritis, syphilitic arteritis; Takayasu's arteritis;* temporal arteritis; thromboangiitis obliterans; and others (see also below under "Note").

NOTE: Autopsy procedures depend on (1) the expected type of arteritis, such as giant cell arteritis,* polyarteritis nodosa,* or thromboangiitis obliterans (Buerger's disease*); and (2) the nature of suspected associated or underlying disease, such as aortic arch syndrome,* Beh~et's syndrome,* Cogan's syndrome, Degos' disease,* dermatomyositis,* erythema nodosum and multiforme,* Goodpasture's syndrome,* polymyositis, rheumatic fever, * rheumatoid arthritis,* syphilis,* and other nonspecific infectious diseases, systemic lupus erythematosus,* systemic sclerosis (scleroderma),* or Takayasu's disease. For histologic study of blood vessels, Verhoeff-van Gieson stain or a similar stain is recommended. Temporal and ophthalmic arteritis.

Arteritis of ciliary and retinal vessels. Clinically, polymyalgia. Anemia.

Arteritis, Takayasu's Synonyms: Aortic arch syndrome; pulseless disease.

External examination Heart, aorta, and adjacent great vessels Kidney Eyes and optic nerve Brain

For in situ aortography, clamp distal descending thoracic aorta and neck vessels as distal as possible from takeoff at aortic arch. Remove heart together with aorta and long sleeves of neck vessels. For coronary arteriography, see Chapter 10 (method designed to show coronary ostia). Test competence of aortic valve. Open aortic arch anteriorly and measure (with calipers) lumen at origin of great neck vessels. Photograph aorta and neck vessels and submit samples for histologic study. Request Verhoeffvan Gieson stain. Submit tissue for histologic examination.

For removal and specimen preparation, see Chapter 5. For removal and specimen preparation, see Chapter 4.

Facial muscular atrophy and pigmentation. Narrowing at origin of brachiocephalic arteries.

Dilated ascending aorta. Narrowing of coronary arteries at origins. Myocardial infarction.

Aortic insufficiency. * Aortic atherosclerosis. Thromboses of brachiocephalic arteries. Giant cell arteritis. * Diffuse mesangial proliferative glomeulonephritis (1) . Atrophy of optic nerve, retina, and iris; cataracts; retinal pigmentation. Ischemic lesions.

Artery, Patent Ductal Synonym: Patent ductus arteriosus. NOTE: The basic anomaly is persistent postnatal patency of the ductal artery, usually as an isolated finding (in 75% of cases in infants, and in 95% in adults). It is more common in premature than full-term infants and at high altitudes than at sea level. Possible complications in unoperated cases include congestive heart failure, * plexogenic pulmonary hypertension,* ductal artery aneurysm or rupture, fatal pulmonary embolism,* or sudden death. In some conditions, such as aortic atresia* or transposition with an intact ventricular septum,* ductal patency may be necessary for survival.

Possible Associated Conditions: Atrial or ventricular septal defect;* coarctation ofthe aorta;* conotruncal anomalies; necrotizing enterocolitis in premature infants; postrubella syndrome; and valvular or vascular obstructions. Artery, Persistent Truncal Synonym and Related Terms: Type I, pulmonary arteries arise from single pulmonary trunk (in 55%); type 2, pulmonary arteries arise separately but close-by (in 35%); type 3, pulmonary arteries arise separately but distal from one another (in 10%).

NOTE: The basic anomaly is a common truncal artery, with truncal valve, giving rise to aorta, pulmonary arteries, and coronary arteries, usually with a ventricular septal defect. Interventions include complete Rastelli-type repair, with closure of ventricular septal defect, and insertion of valved extracardiac conduit between right ventricle and detached pulmonary arteries.

Possible Associated Conditions: Absent pulmonary artery (in 15%); atrial septal defect (in 15%); absent ductal artery (in 50%); coronary ostial anomalies (in 40%); Di George syndrome;* double aortic arch; extracardiac anomalies (in 25%); interrupted aortic arch* (in 15%); right aortic arch (in 30%); truncal valve insufficiency (uncommon) or stenosis (rare); trun-cal valve with three (in 70%), four (in 20%), or two (in 10%) cusps.

Heart and great vessels

If infective endocarditis is suspected, follow culture procedures for endocardial vegetation described in Chapter 10.

Request Verhoeff-van Gieson stain.

Infective endocarditis,* usually of truncal valve. Late postoperative conduit obstruction. Postoperative late progressive truncal artery dilation with truncal valve insufficiency.

Hypertensive pulmonary vascular disease. Cerebral abscess,* if right-to-Ieft-shunt was present.

Arthritis, All Types or Type Unspecified NOTE: For extra-articular changes, see under the name of the suspected underlying conditions. Infectious diseases that may be associated with arthritis include bacillary dysentery, * brucellosis, * gonorrhea, rubella,* syphilis, * tuberculosis, * typhoid fever, * and varicella. * Noninfectious diseases in this category include acromegaly,* Beh<;et's syndrome,* Felty's syndrome,* gout,* rheumatoid arthritis,* and many others, too numerous to mention.

Remove synovial fluid and prepare smears. Submit synovial fluid for microbiologic and chemical study. For removal of joints, prosthetic repair, and specimen preparation, see Chapter 2. For removal and specimen preparation, see Chapter 5.

In the polyarticular variant, facial asymmetry may be noted. Rheumatoid factor positive in some cases.

Pericarditis.* Interstitial pneumonitis; pleuritis. (See also under "Arthritis, rheumatoid.") Lymphadenopathy.

Splenomegaly.

Monarthritis or severe, erosive polyarthritis; see also under "Arthritis, rheumatoid" and above under "Externalexamination and skin." Ankylosing spondylitis* may be present. Chronic iridocyclitis. See "Arthritis, rheumatoid."

Arthritis, Rheumatoid Synonyms and Related Terms: Ankylosing spondylitis;* Felty's syndrome;* juvenile rheumatoid arthritis* (Still's disease); rheumatoid disease; and others.

Possible Associated Conditions: Amyloidosis;* polymyositis (dermatomyositis*); psoriasis;* Sjogren's syndrome;* systemic lupus erythematosus;* systemic vasculitis, and others. 

Subcutaneous rheumatoid nodules on elbows, back, areas overlying ischial and femoral tuberosities, heads of phalangeal and metacarpal bones, and occiput. Deformities and subluxation of peripheral joints (see also below under "Joints"). Subaxial dislocation of cervical spine may be cause of sudden death. Pneumothorax;* pleural empyema.* T-cell abnormalities (1) .

Bacteremia. Positive rheumatoid factor.

Rheumatoid granulomas in myocardium (septum), pericardium, and at base of aortic and mitral valves; constrictive pericarditis;* aortic stenosis;* coronary arteritis. Systemic vasculitis (arteritis*). Rheumatoid granulomas in pleura and lung (with pneumoconiosis*); bronchopleural fistula; rheumatoid pneumonia with interstitial pulmonary fibrosis and honeycombing; bronchiectasis;* bronchiolitis with cystic changes; pulmonary arteritis. Pneumoconiosis* in Caplan 

Arthrogryposis (2) may be a primary muscle disease, or it may involve abnormalities of the brain, spinal cord, and/or peripheral nerves. Etiologies are numerous, as are the modes of inheritance. Critical to making the appropriate diagnosis is the collection of muscles from various sites for routine histology, muscle histochemistry, and electron microscopy. Portions of peripheral motor nerves must also be prepared for histology and electron microscopy.

Abdominal cavity Intra-abdominal lymphatic system

Puncture abdominal cavity and submit fluid for microbiologic study. Record volume of exudate or transudate and submit sample for determination of fat and cholesterol content. Prior to routine dissection, lymphangiography (see below) may be indicated. Possible Associated Conditions: With pulmonary aspergillosis-bronchiectasis; * bronchocentric granulomatosis;* sarcoidosis;* tuberculosis. * With systemic aspergillosisleukemia;* lymphoma;* and other conditions complicated by immunosuppression (l, 2) .

Other organs A

Carefully make multiple parasagittal sections through the unperfused lungs. Culture areas of consolidation. If diagnosis was confirmed, perfuse lungs with formalin. Prepare histologic sections from walls of cavities, cavity contents, and pneumonic infiltrates. Procedures depend on expected findings or grossly identified abnormalities as listed in right-hand column.

Assault NOTE: All procedures described under "Homicide" must be followed. Asthma NOTE: Spray death* may occur in asthma sufferers from pressurized aerosol bronchodilators. Record thickness and position. Perfuse one lung with formalin. Because mucous plugs may block bronchial tree, attach perfusion apparatus to pulmonary artery or to bronchus and pulmonary artery. Monitor perfusion to ensure proper inflation. Prepare photograph of fixed cut section. Submit samples of pulmonary parenchyma and bronchi for histologic study. Request azure-eosin and Verhoeff-van Gieson stains.

Record weight and thickness of walls. Leave attached to stomach.

Photograph and submit samples for histologic study.

Eczema. Conjunctival hemorrhages and subcutaneous emphysema may be present after fatal attack. Pneumothorax;* mediastinal emphysema. Low diaphragm (see below). Increased IgEconcentrations in fatal asthma; postmortem tryptase determination is of doubtful value in this regard (1) . Hypertrophy. Low position of diaphragm. Hyperinflated lungs.

Thick-walled bronchi with prominent viscid mucous plugs.

Typical microscopic inflammatory changes (2) . Asthmatic bronchitis with eosinophilic infiltrates. Bronchocentric granulomatosis.* Pulmonary atherosclerosis with breakup of elastic fibers. Paucity of ecosinophils in mucous (6) . Cor pulmonale. Refl ux esophagitis (3) . Peptic ulcer. * Pneumatosis of small intestine; emphysema of colon. Centrilobular congestion and necrosis.

Petechial hemorrhages in hypothalamus; necrosis of cerebellar folia; anoxic changes in cortex, globus pallidus, thalamus, Sommer's sector of hippocampus, and Purkinje cells of cerebellum. Suspected changes in anterior hom cells of spinal cord in patients with asthma-associated poliomyelitis-like illness (Hopkins syndrome) (4). Allergic polyps and other allergic inflammatory changes (5) .

Increased erythropoiesis. Atresia, Aortic Valvular Synonym: Aortic atresia; aortic atresia with intact ventricular septum; hypoplastic left heart syndrome.

NOTE: The basic anomaly is an imperforate aortic valve, with secondary hypoplasia ofleft-sided chambers and ascending aorta. For possible surgical interventions, see two-stage Norwood and modified Fontan procedures in Chapter 3.

Possible Associated Conditions: Atrial septal defect* (or patent foramen ovale, usually restrictive); dilatation of myocardial sinusoids thatcommunicate with coronary vessels; dilatation of right atrium, right ventricle, and pulmonary trunk; fibroelastosis ofleft atrial and left ventricular endocardium; hypertrophy of ventricular and atrial walls; hypoplastic left atrium, mitral valve, left ventricle, and ascending aorta; mitral atresia* with minute left ventricle; patent ductal artery (ductus arteriosus); small left ventricle with hypertrophic wall; tubular hypoplasia of aortic arch, with or without discrete coarctation.

Synonyms and Related Terms: Congenital biliary atresia; extrahepatic biliary atresia; infantile obstructive cholangio-pathy; syndromic (Alagille's syndrome) or nonsyndromic paucity of intrahepatic bile ducts ("intrahepatic" biliary atresia).

Possible Associated Conditions: Alpha]-antitrypsin deficiency;* choledochal cyst;* congenital rubella syndrome;* polysplenia syndrome* (1); small bowel atresia; trisomy 17-18; trisomy 21; Turner's syndrome;* viral infections (cytomegalovirus infection;* rubella*). Dissect extrahepatic bile ducts in situ or leave hepatoduodenalligament intact for later fixation and sectioning (see below). Record appearance and contents of gallbladder and course of cystic duct.

In postoperative cases, submit sample of anastomosed hepatic hilar tissue for demonstration of microscopic bile ducts. Remove liver with hepatoduodenalligament. Prepare horizontal sections through ligament and submit for histologic identification of ducts or duct remnants. Prepare frontal slices of liver and sample for histologic study. Request PAS stain with diastase digestion.

Procedures depend on expected findings or grossly identified abnormalities as listed in right-hand column.

Jaundice. Congenital rubella and other viral infections.

Alpha]-Antitrypsin deficiency;* defects in bile acid synthesis. Chromosomal abnormalities.

In atresia of the hepatic duct, the gallbladder will be empty. In isolated atresia of the common bile duct, the gallbladder contains bile but it cannot be squeezed into the duodenum. Atresia or hypoplasia of bile duct(s); choledochal cyst(s).

Biliary drainage created by Kasai operation.

Obliterative cholangiopathy (2) .

Intrahepatic cholelithiasis; postoperative ascending cholangitis; secondary biliary cirrhosis; giant cell transformation; paucity of intrahepatic bile ducts. PAS-positive inclusions in alphal-antitrypsin deficiency.* Polysplenia syndrome* (1) with malrotation, situs inversus, preduodenal portal vein, absent inferior vena cava, anomalous hepatic artery supply, and cardiac defects. For other abnormalities outside the biliary tree, see under "Possible Associated Conditions"). Nephromegaly (3) .

Atresia, Cardiac Valves (See "Atresia, aortic valvular," "Atresia, mitral valvular," "Atresia pulmonary valvular, with intact ventricular septum," "Atresia, pulmonary valvular, with ventricular septal defect," and "Atresia, tricuspid valvular.") Atresia, Duodenal

Possible Associated Conditions: With membranous obstruction of the duodenum-annular pancreas; atresia of esophagus* with tracheoesophageal fistula; congenital heart disease; cystic fibrosis;* Down's syndrome;* Hirschsprung's disease; imperforate anus* or other congenital obstructions of the intestinal tract (1); intestinal malrotation; lumbosacral, rib-, and digitllimb anomalies; single umbilical artery; spinal defects; undescended testis (1).

See also under "Atresia, small intestinal." The basic anomaly is an imperforate pulmonary valve, with a hypoplastic right ventricle. In unoperated cases, ductal closure is the most common cause of death. For possible surgical interventions, see modified Blalock-Taussig shunt, mod-ified Fontan procedure, and pulmonary valvulotomy in Chapter 3. For general dissection techniques, see Chapter 3.

Possible Associated Conditions: Dilated myocardial sinusoids that may communicate with epicardial coronary arteries or veins; patent ductal artery (ductus arteriosus); patent oval foramen (foramen orale); tricuspid atresia with minute right ven-tricle; tricuspid stenosis with hypoplastic right ventricle (in 95%); tricuspid insufficiency with dilated right ventricle (in 5%).

Synonym: Tetralogy of Fallot with pulmonary atresia. NOTE: The basic anomaly is atresia of the pulmonary valve and ofvariable length ofpulmonary artery, and ventricular septal defect (membranous or outlet type), with overriding aorta, and with pulmonary blood supply from ductal or systemic collateral arteries. For possible surgical interventions, see Rastelli-type repair and unifocalization of multiple collateral arteries in Chapter 3.

Possible Associated Conditions: Right ventricular outflow tract a short blind-ended pouch (70%) or absent (30%); atresia of pulmonary artery bifurcation, with nonconfluent pulmonary arteries; right aortic arch (40%); atrial septal defect (50%); persistent left superior vena cava; anomalous pulmonary venous connection; tricuspid stenosis or atresia; complete atrioventricular septal defect; transposed great arteries; double inlet left ventricle; asplenia, polysplenia, or velocardiofacial syndromes; dilated ascending aorta, with aortic insufficiency.

Related Term: Jejuno-ileal atresia.

Possible Associated Findings: Esophageal atresia* with tracheoesophageal fistula; lumbosacral, rib-, or digit/limb anom -alies; undescended testes (l) .

NOTE: See also under "Atresia, duodena1."

Fascia lata, blood, or liver These specimens should be collected using aseptic technique for tissue culture for chromosome analysis (see Chapter 9) . Intestinal tract For mesenteric angiography, see Chapter 2.

Leave mesentery attached to small bowel, particularly to the atretic portion.

Trisomy 21.

Multiple atresias; proximal dilatation; volvulus; malrotation; meconium impaction; other evidence of cystic fibrosis. Anorectal malformation (l) . Annular pancreas (1). Atresia, Tricuspid Valvular NOTE: The basic anomaly is an absent right atrioventricular connection (85%) or imperforate tricuspid valve (15%), with a hypoplastic right ventricle (100%), muscular ventricular septal defect (90%) that is restrictive (85%), and a patent oval Atresia, Urethral foramen (80%) or secundum atrial septal defect (20%). For possible surgical interventions, see modified Fontan or Glenn procedures in Chapter 3. For general dissection techniques, see Chapter 3.

Possible Associated Conditions: Juxtaposed atrial appendages; large left ventricular valvular orifice; large left ventricular chamber; persistent left superior vena cava; pulmonary atresia; transposition of the great arteries (25%), with aortic co-arctation (35% of those); anomalies of musculoskeletal or digestive systems (20%); Down's,* asplenia, or other syndromes. 

Heart Aorta and cervical arteries Brain

If infective endocarditis* is suspected, culture using the method described in Chapter 7. For dissection of carotid and vertebral arteries, see Chapter 4.

For removal and specimen preparation, and cerebral anteriography, see Chapter 4. 

If a foreign body is discovered during a medicolegal autopsy or if the discovery of a foreign body may have medicolegal impli-cations (e.g., presence of a surgical instrument in the abdominal cavity), the rules of the chain of custody apply. For the handling of bullets or bullet fragments, see "Injury, firearm." If analysis offoreign material is required, commercial laboratories may be helpful.

Bolus (See "Obstruction, acute airway!') Burns NOTE: Fatal bums should be reported to the medical examiner's or coroner's office. The questions to be answered by the pathologist depend on whether the incident was accidental, sui-cidal, or homicidal, and whether the victim survivied to be treated in the hospital. A pending death certificate should be issued if the fire and police investigators are not sure of the circumstances at the time of the autopsy. For electrical bums, see under "Injury, electrical."

For victims who were treated at the hospital, autopsy procedures should be directed toward the discovery or confirmation of the mechanism of death, such as sepsis or pulmonary embolism.* Death can be caused primarily by heart disease, with other-wise minor bums and smoke inhalation serving as the trigger that leads to lethal ventricular arrhythmia. Because carbon monoxide concentrations are halved approx every 30 min with 100% oxygen therapy, the pathologist must obtain the first clinical laboratory test results for CO-hemoglobin. Soot can be detected with the naked eye 2 or 3 d after inhalation of smoke. Ambulance records should be examined to determine whether a persistent coma might have been caused by hypoxic encephalopathy following resuscitation from cardiac arrest at the scene.

Admission blood samples should be acquired to test for COhemoglobin and alcohol. This may not have been done in the emergency room. Persons suffering from chronic alcoholism succumb to fire deaths more often than persons who do not drink. A very high initial serum alcohol concentration suggests a risk factor for the fire and presence of chronic alcoholism. Patients with chronic alcoholism typically are deprived of alcohol when they are in the bum unit and this can cause sudden, presumably cardiac, death,just as it occurs under similarcircum-stances, not complicated by bums. Under these circumstances, the heart fails to show major abnormalities. This mode of dying seems to have no relationship to the presence or absence of liver disease. 

If the body is found dead and charred at the scene, prepare whole body roentgenograms, before and after removal of remanants of clothing. See also under "Identification of the body" and "External examination" in Chapter 13). One or two fingerpads may yield sufficient ridge detail for identification. If this is not possible, ante-and postmortem somatic and dental radiographs must be compared for identification, or DNA comparison must be used. 

External examination, heart and lungs Abdominal cavity and liver

See below under "Cardiomyopathy, dilated."

Record volume of ascites. Record actual and expected weight of liver. Request iron stain.

See below under "Cardiomyopathy, dilated."

Alcoholic cirrhosis and alcoholic cardiomyopathy rarely coexist. However, in genetic hemochromatosis,* cirrhosis and heart failure are common findings.

Cardiomyopathy, Dilated (Idiopathic, Familial, and Secondary Types) NOTE: For general dissection techniques, see Chapter 3.

External examination 

Heart Other organs and tissues

Record actual and expected weights. Record ventricular thicknesses and valvular circumferences. Evaluate relative atrial and ventricular chamber sizes.

Procedures depend on expected findings or grossly identified abnormalities as listed in right-hand column. NOTE: Huntington's disease maps to the short arm of chromosome 4. The gene is widely expressed but of unknown function; it contains a CAG repeat sequence, which is expanded (range, 37 to 86) in patients with Huntington's disease. A sensitive diag-nostic test is based on the determination of this CAG sequence, which can be done on fresh-frozen tissue or blood (1) . In the absence of genetic confirmation, sampling of organs and tissues cannot be excessive because a complex differential diagnosis must be resolved. NOTE: Disseminated intravascular coagulation (DIC) often is a complication of obstetrical mishaps such as abruptio placentae or amniotic fluid embolism,* or it complicates malignancies (such as adenocarcinomas or leukemia*) or bacterial, viral, and other infections. Other conditions such as aortic aneurysm* or hemolytic uremic syndrome* are known causes also. Ifthe nature of the underlying disease is known, follow the procedures under the appropriate heading also. NOTE: This is a cause of diarrhea. Microscopic colitis is associated with older age; collagenous colitis is associated with female sex (1). The colon is grossly normal but microscopically, increased lymphocytes in the lamina propria and a subepithelial band of collagen is found. If only the lymphocytic infiltrate is found, the term "lymphocytic colitis" or "microscopic colitis" should be applied. A trichrome stain should be ordered in all instances, because the collagen band may be difficult to see without the special stain. I Death, Anaphylactic Synonym: Generalized anaphylaxis. NOTE: Autopsy should be done as soon as possible after death. Neck organs should be removed before embalming. If death is believed to be caused by drug anaphylaxis, inquire about type of drug(s), drug dose, and route of administration (intravenous, intramuscular, and oral or other). This will determine proper sampling procedures-for instance, after penicillin anaphylaxis. Allergy to bee stings, wasp stings, fire ants, and certain plants may also be responsible for anaphylaxis. However, envenomation also can be fatal in the absence of anaphylaxis.

External examination Search for injection sites or sting marks. If such lesions are present, photograph and excise with 5-cm margin.

Freeze excised tissue at -70°C for possible analysis. Prepare chest roentgenogram.

Foam in front of mouth and nostrils. Swelling of involved tissue.

Antigen-antibody reaction in involved tissues. Antibodies against suspected antigen.

Laryngeal edema may recede soon after death.

Foamy edema in trachea and bronchi; diffuse or focal pulmonary distention ("acute emphysema") alternating with collapse; pulmonary edema and congestion; accumulation of eosinophilic leukocytes.

Eosinophilic leukocytes in red pulp. Death, Anesthesia-Associated. NOTE: There are many possible causes of anesthesiaassociated death that are not drug-related, such as acute airway obstruction* by external compression, aspiration, arrhythmia of a heart not previously known to be diseased, tumor, or an inflammatory process. Some ofthe complications are characteristically linked to a specific phase of the anesthesia, and many are not revealed by customary morphologic techniques.

The task for the pathologist charged with investigating an anesthesia-associated death is to reconstruct the chain of physiologic events culminating in cessation of vital signs. Autopsy morphology plays a supporting role; the main investigations center around the record left by the anesthesiologist, testing of anesthesia equipment, and toxicological testing. A consulting anesthesiologist can divine much more information from the anesthesia and recovery room records than can the pathologist, and can suggest avenues of further investigation. Therefore, the most important step in these autopsies is to obtain the anesthesiaassociated records and to secure the consulting services of an independent anesthesiologist. The changes in the vital signs during and after anesthesia will help to focus the investigation toward a cardiac mechanism ofdeath or depression ofbrainstem function as a terminal mechanism.

When information is gathered about drugs and chemical agents that have been administered or to which the victim may have had access, the pathologist must keep in mind that some non-medical chemicals and many drugs are known to affect anesthesia. Drugs and their metabolic products, additives, stabilizers, impurities, and deterioration products (one of which can be carbon monoxide) may be present and can be identified in postmortem tissues. Therefore, all appropriate body fluids and solid tissue should be submitted for toxicological examination. If the anesthetic agent was injected into or near the spinal canal, spinal fluid should be withdrawn from above the injected site into a standard toxicologist's collection tube with fluoride preservative. If the anesthetic agent was injected locally, tissue should be excised around the needle puncture marks at a radius of 2-4 em. Serial postmortem analysis of specimens may permit extrapolation to tissue concentration at the time of death. The time interval between drug administration and death sometimes can be calculated from the distribution and ratio ofadministered drugs and their metabolic products. For a review of anesthetic death investigation, see ref. (1) .

Halothane anesthesia and some other anesthetic agents may cause fulminant hepatitis and hepatic failure. The autopsy procedures suggested under "Hepatitis, viral" should be followed. NOTE: For special autopsy procedures in postoperative deaths, see Chapter 1. In some instances, procedures described under "Death, anesthesia-associated" may be indicated. For a review of investigational procedures and autopsy techniques in operating-room-associated deaths, see ref. (1) . If the autopsy will involve anatomy or dissection techniques that are unfamiliar, the pathologist should not hesitate to invite the surgeon to the autopsy. In patients who develop a cerebral infarction after open heart surgery, arterial air embolism should be considered as a possible cause. The diagnosis often must be based on excluding other causes because the air has been absorbed prior to death. If a patient dies rapidly, the hospital records may be incomplete or scanty. For example, if a patient bleeds to death despite attempted repair of hepatic lacerations, hospital records may not suffice to reach the correct cause-of-death opinion; personal accounts from the surgeon and anesthesiologist may be needed. Autopsy data on patients dying following thoracic surgery may be found in ref (2) . D Death, Restaurant (See "Obstruction, acute airway.") Death, Sniffing and Spray Related Terms: Glue sniffing; sudden sniffing death syndrome.

NOTE: No anatomic abnormalities will be noted at autopsy. Sudden death may occur after cardiac dysrhythmia or respiratory arrest.

Procedures Possible or Expected Findings Lungs Brain

If poison had been inhaled at the time when death occurred, tie main bronchi. Submit lungs in glass container for gas analysis.

Submit samples of small bronchi for histologic study.

For removal and specimen preparation, see Chapter 4. Submit samples of fresh or frozen brain for toxicologic study.

Submit samples in glass containers (not plastic) for toxicologic study.

Trichloroethane, fluorinated refrigerants, and other volatile hydrocarbons are most often involved in the "sudden sniffing death syndrome." Spray death may occur in asthma sufferers using pressurized aerosol bronchodilators. Freons and related propellants may also be responsible for sudden death. Toxic components of glue-such as toluene-accumulate in the brain of glue sniffers. Also present in various glues are acetone, aliphatic acetates, cyclohexane, hexane, isopropanol, methylethyl ketone, and methylisobutyl ketone. Aerosols may occlude the airway by freezing the larynx. Carbon tetrachloride sniffing may cause hepatorenal syndrome (see also under "Poisoning, carbon tetrachloride").

Death, Sudden Unexpected, of Adult NOTE: Medicolegal autopsies are usually indicated, and appropriate procedures should be followed. Ifanaphylactic death is suspected, see also under that heading. For all unexpected deaths, the pathologist should learn the circumstances of the death, in order to determine whether the mechanism of death was rapid or slow, and to guide the selection of ancillary tests. Whenever paramedics attended a person, the run sheet should be obtained to look for a history of recent drinking or ofchronic alcoholism may be an important clue. The combination of a history ofalcoholism, a negative test for ethanol, and absence ofcardiovascular disease, should suggest alcohol withdrawal as the cause ofa sudden death. The list of"Possible or Expected Findings" below is not complete. For general toxicologic sampling, see Chapter 13. Possible Associated Conditions: Atrial septal defect;*bicuspid aortic valve;* coarctation,* hypoplasia, or interruption (type A) of aortic arch; coronary artery from main pulmonary artery; right atrial arch; patent ductal artery;* right pulmonary artery from ascending aorta; subaortic stenosis;* tetralogy of Fallot;* ventricular septal defect. * (In approx 50% of the cases, one or more of these associated conditions are found.) Defect, Atrial Septal NOTE: The basic anomaly is a defect of the atrial septum, usually at the oval fossa (in 85%). Possible complications in unoperated cases include atrial arrhythmias, congestive heart failure; paradoxic embolism; plexogenic pulmonary hypertension «10%), and pulmonary artery aneurysm. Possible surgical interventions include surgical and transcatheter closure of defect. For Deficiency, Vitamin C Synonyms: Hypovitaminosis C; scurvy.

External examination and skin

Other organs

Bones, joints, and soft tissues

Record extent and character of skin lesions; prepare sections of skin.

Describe appearance of gums, and prepare sections.

Record evidence of bleeding.

For removal, prosthetic repair, and specimen preparation of bones and joints, see Chapter 2.

Hyperkeratotic hair follicles with perifollicular hemorrhages (posterior thighs, anterior forearms, abdomen); petechiae and ecchymoses (inner and posterior thighs); subcutaneous hemorrhages. Gingivitis.

In rare instances, gastrointestinal or genitourinary hemorrhages. Hemorrhages into muscles and joints. Subperiosteal hemorrhages occur primarily in distal femora, proximal humeri, tibiae, and costochondral junctions (scorbutic rosary).

Deficiency, Vitamin D Synonyms: Hypovitaminosis D; rickets. NOTE: Features or rickets may be found in familial hypophosphatemia (vitamin D-resistent rickets; Fanconi syndrome).

Vitreous or blood (serum)

Other organs

Prepare skeletal roentgenograms.

In infants with suspected rickets, record size of anterior fontanelle and shape of head; state of dentition; and shape of costochondral junctions, wrists, long bones, and spine. Submit samples for calcium, magnesium, and phosphate determination. Procedures depend on expected findings or grossly identified abnormalities as listed in right-hand column.

Weigh parathyroid glands and submit samples for histologic study.

Submit samples of intestine for histologic study.

For removal, prosthetic repair, and specimen preparation, see Chapter 2.

In infantile rickets, diagnostic sites for histologic sampling are costochondral junctions, distal ends of radius and ulna, and proximal ends of tibia and humerus. For adults, see under "Osteomalacia."

In infants, rachitic changes at costochondral junctions; in adults, osteoporosis* and osteomalacia*-with or without pseudofractures (Milkman's syndrome (1) . NOTE: The term spinocerebellar degeneration encompasses a variety of lesions whose classification is controversial. A new approach has come from linkage analysis and molecular biology. For instance, Friedreich's ataxia, the classic form of hereditary ataxia, is due to an intronic expansion of a GAA tri-nucleotide repeat. Other forms are also identified by their specific gene loci. Neuropathologic examination still is important and ample sampling is suggested, which should include cerebral cortex, basal ganglia (caudate nucleus, putamen, and globus pallidus), thalamus, subthalamic nucleus, midbrain (red nucleus and substantia nigra), pons (pontine nuclei), spinal cord (at cer-vical, thoracic, and lumbar levels), optic tract, optic nerves with lateral geniculate nucleus, and sensory and motor peripheral nerves. For removal and specimen preparation, see Chapter 5.

Enlargement of head.

Poor demarcation between cortex and gelatinous white matter. Extensive demyelination and vacuolation of white matter, particularly subcortically. Optic atrophy.

Degeneration, Striatonigral (See "Atrophy, multiple system.")

Related Term: Thirst. NOTE: Possible underlying conditions not related to inaccessibility of water include bums, exposure to heat, gastrointestinal diseases, recent paracentesis, renal diseases, and use of diuretic drugs. See also under "Disorder, electrolyte(s)."

External examination Vitreous Urine

Prepare histologic sections of blisters, ulcers, or skin abrasions.

Submit sample for sodium, chloride, and urea nitrogen determination.

Skin turgor may be decreased and eyes may be sunken. Microscopic changes help to decide whether skin lesions are antemortem or postmortem. Sodium concentrations more than 155 meqlL, chloride concentrations more than 130 meq/ and urea nitrogen concentrations between 40 and 100 meq/dL indicate dehydration. Absence or minimal amount of urine.

Dementia (See "Disease, Alzheimer's.") Drug abuse, Amphetamine(s) NOTE: Methamphetamine abuse may be suggested by poor condition of the dentition. Methylenedioxymethamphetamine ("Ecstasy") abuse is often suggested by friends with whom the decedent was abusing drugs. Follow procedures described under "Dependence, drug(s)." Drug abuse, Cocaine NOTE: Cocaine is spontaneously hydrolyzed by blood esterases, even after death. However, one of its major metabolite, benzoylecgonine, is routinely identifiable by immunoassay screening tests. When cocaine is abused concurrently with heroin or other depressant drugs, it may be difficult to ascribe deth to a single agent, unless circumstances clearly point to a rapid cardiac mechanism or a slow brainstem depression mechanism. NOTE: If narcotic paraphernalia and samples of the drug itself are found at the scene of the death, they should be submitted for analysis. Helpful information about the nature of a drug may be obtained from witnesses. State crime laboratories may provide much assistance. If name of drug is known, see also under "Poisoning,..." The slang name of a drug may be insufficient for identification because these names often are used for different compounds at different times of places.

Opoid narcotics can be injected intravenously, or subcutaneously, or snorted. Death may occur with such speed that the bodies may be found with needles and syringes in the veins or clenched in the hands. Drug abuse may be associated with a multitude of local (see below) or systemic complications, including malaria* and tetanus. * As stated in Chapter 13, for a growing number of analytes, most notably tricyclic antidepressants, peripheral blood is preferred over central blood. Peripheral blood is aspirated by percutaneous puncture before autopsy, from the femoral vein or the subclavian vein. The authors prefer the femoral approach in order to avoid any question of artifact in the diagnosis of venous air embolism. It may be pru-dent to add NaF to some of the samples. 

Related Term: Childhood dermatomyositis (or polymyositis) associated with vasculitis; dermatomyositis (or polymyositis) associated with neoplasia or collagen vascular disease; primary idiopathic dermatomyositis; primary idiopathic polymyositis.

Possible Associated Conditions: Carcinoma (lung, stomach, intestine, and prostate in males; breast, ovary, and uterus in females; miscellaneous sites in both sexes); lymphoma* (rare) and other malignancies (1); lupus erythematosus;* mixed connective tissue disease; progressive systemic sclerosis;* rheumatoid arthritis;* Sjogren's syndrome;* and others. Vasculitis of childhood polymyositis (dermatomyositis).

External examination and skin Heart Lungs Esophagus and gastrointestinal tract

Photograph grossly involved skin.

Prepare sections of involved (anterior chest, knuckles, knees) and grossly uninvolved skin and subcutaneous tissue.

Prepare roentgenograms.

Submit samples from myocardium for histologic study.

Perfuse one lung with formalin.

Submit samples from all segments for histologic study. 

Arteritis* and phlebitis* with thrombosis, fibrosis, and infarctions. Steatohepatitis and manifestations of diabetes mellitus* may be found (2) .

Myositis with muscular atrophy and fibrosis; vasculitis in childhood cases.

Polyneuropathy (rare) (5).

Arthritis. Diabetes Mellitus Synonyms: Type I (insulin-dependent or juvenile-onset) diabetes mellitus; type II (insulin-independent or adult onset) diabetes mellitus; secondary diabetes mellitus (e.g., due to drugs or pancreatic disease).

NOTE: In infants of diabetic mothers, macrosomia and congenital malformations must be expected. Record size and weight of placenta and total weight and length, crown to rump length, and crown to heel length of infant. Compare with expected measurements (see Part III). Expected histologic finding in-clude hyperpla-sia with relative increase ofB cells of the islands of Langerhans with interstitial and peri-insular eosinophilic infiltrates, decid-ual changes of the endometrium, enhanced follicle growth in the ovaries, and Leydig cell hyperplasia.

Possible Associated Conditions: Acanthosis nigricans; acro-megaly;* amyotrophic lateral sclerosis; * ataxia telangiectasia;* Fanconi's anemia;* Friedreich's ataxia;* gout;* hemochro-matosis; *hyperlipoproteinemia; * hyperthroidism;* obesity;* Turner's syndrome;* and many others, too numerous to mention. NOTE: The term "Caroli's syndrome" often is used for cases that also show histologic features of congenital he-patic fibrosis or other manifestations of fibropolycystic liver disease,* whereas the name "Caroli's disease" refers to idiopathic dilatation of intrahepatic bile ducts, without associated abnormalities.

Possible Associated Conditions: Choledochal cyst* and related extrahepatic biliary abnormalities (1); congenital hepatic fibrosis; * cysts of kidneys (renal tubular ectasia or medullary sponge kidney; autosomal-recessive polycystic kidney disease, and rarely, autosomal-dominant polycystic kidney disease [2] )* and of pancreas. Record volume of effusions. Prepare smears of fresh blood or of buffy coat, or make thick-drop preparation. Submit sample for xenodiagnosis or animal inoculation and for serologic study.

Record weight. In chronic Chagas' disease, perfuse intact heart with formalin (Chapter 3) and slice fixed heart in a frontal plane so as to create anterior and posterior halves. Prepare photographs. Histologic samples should include conduction system.

Include several sections of atrial (auricular) walls for histologic study of autonomous ganglia.

Perfuse at least one lung with formalin.

Leave affected hollow viscera intact and fill with formalin. Cut fixed organs in half, photograph, and cut histologic sections on edge.

Record liver weight and submit samples for histologic study.

Record weight.

Prepare photographs of abnormalities.

Weigh and examine. Prepare histologic sections.

For removal and specimen preparation, see Chapter 4. Autopsy is desirable in suspected cases because the diagnosis can only be firmly established after neuropathologic examination. Serologic studies are not available. Unfortunately, all tissues (not just the brain and spinal cord) may remain infectious even after prolonged fixation and histologic processing. Thus, the autopsy recommendations for most other infectious diseases do not apply here. This is a reportable disease in some states. Special precautions are indicated and therefore, the procedures described here should be followed strictly (1) (2) (3) (4) :

All persons in the autopsy room must wear disposable long-sleeved gowns, gloves, and masks. Contamination of the autopsy table should be prevented by covering it with a disposable, non-permeable plastic sheet. Autopsy generally should be restricted to the brain. If organs in the chest or abdomen need to be examined, this is best done in situ. To prevent aerosolization of potentially infectious bone dust, a hood or other protective device should be used while opening the skull with a Stryker saw. After completing the autopsy, instruments and other potentially contaminated objects should be autoclaved in a steam autoclave (1 h at 134°C). Porous load is considered more effective than gravity displacement autoclaves. Immerse autopsy instruments in distilled water before and during autoclaving, in order to protect them from corrosion. Ifno autoclave is available, chemical disinfection (see below) is a satisfactory alternative. Disposable items should be put in a container for infectious hospital waste and ultimately incinerated. Contaminated objects not suitable for autoclaving (such as the Stryker saw) should be soaked with a2NNaOH solution for 1 h (alternatively, 1 NNaOH may be used for 2 h). Contaminated surfaces should be thoroughly washed with the same solution. Aluminum should be treated for 2 h with a fresh 5% NaOCI (sodium hypochlorite) solution with at least 20,000 ppm free chloride. Wash waters should be collected; if no autoclave is available, 2 N NaOH or >4 volumes of 5% sodium hypochlorite bleach should be added to the water and left for a minimum of 2 h before being discarded. Before removing the body from the autopsy room, it should be sponged with 5% sodium hypochlorite.

To deactivate CJD infectivity, tissue blocks, 5 mm or less in thickness, should be fixed in formalin in a formalin-totissue ratio of at least 20: 1 for at least 48 h and then soaked in concentrated formic acid (95-100%) for I h, followed by another 48 h of formalin fixation. The fixation fluid should be collected and decontaminated, as described earlier for wash water. Glassware and tissue carriers should also be decontaminated as previously described. After this deactivation, the tissue blocks can be processed in a routine fashion. At any stage of these procedures, special care must be taken to avoid cuts with potentially contaminated glassware, blades, or other objects. Parenteral exposure to potentially contaminated material also should be avoided.

Remains of patients who have died of the disease should not be accepted for anatomy teaching for students. If specimens are prepared for pathology collections, they should be handled with great caution. Morticians and mortuary workers should be warned of possible hazards posed by tissues of patients with transmissible spongiforme encephalopathies; they should be advised about proper use of disinfectants. Clinical laboratories that receive autopsy tissues or fluids must be warned about the infectious nature of the material. If possible, decontamination should be done at the site where the autopsy was done. For the shipping of potentially infected material, see Chapter 15.

Increased concentrations of NSE (5).

Spongiforme changes, astrocytosis, neuronal loss, amyloid plaque formation, PrP deposition, and proliferation of activated microglia (6).

Cerebrospinal fluid Brain Submit sample for neuron-specific enolase (NSE). For removal and specimen preparation, see Chapter 4 and above under "Note." Submit fresh-frozen material for confirmation of diagnosis by histoblot technique on protease K-digested frozen tissue or Western blot preparations on brain homogenates. Immunohistochemical localization ofPrP and HLA-DR protein on paraffin-embedded tissue is possible. Disease, Demyelinating (See "Degeneration, spongy, of white matter," "Encephalomyelitis, all types or type unspecified," "Leukodystrophy, globoid cell," "Leukodystrophy, sudanophilic," "Sclerosis, multiple;' and "Sclerosis, Schilder's cerebral.") Disease, Diffuse Alveolar Synonym: Diffuse pulmonary disease. NOTE: Autopsy procedures are listed under the more specific diagnoses, such as "Hemosiderosis, idiopathic pulmonary," "Lipoproteinosis, pulmonary alveolar," "Microlithiasis, pulmonary alveolar," "Pneumonia, lipoid," and "Syndrome, Goodpasture's." Glycosphingolipid storage in cornea; lens opacities; dilated vessels in conjunctiva and lens; thrombi in blood vessels (5).

Disease, Fibropolycystic, of the Liver and Biliary Tract NOTE: "Fibropolycystic disease of the liver and biliary tract" comprises a group of well defined conditions, which may occur together and hence need a collective designation. The conditions include autosomal-recessive (infantile) and auto-somal dominant (adult) polycystic disease of the liver; Caroli's disease or syndrome;* choledochal cyst,* congenital hepatic fibro-sis,* multiple biliary microhamartomas, and related disorders. For autopsy procedures, see also under more specific designations. Disease, Glycogen Storage Synonyms: Andersen's disease or brancher deficiency (glycogenosis, type IV); Cori's or Forbes' disease (glycogenosis, type Ill); cyclic AMP dependent kinase (type X); glycogen synthetase deficiency (type 0); Hers' disease (glycogenosis, type VI); McArdle's disease (glycogenosis type V); phosphorylase B kinase deficiency (types IXa, b, and c); Pompe's disease (glycogenosis, type IT); Tarui disease (glycogenosis type VII); von Gierke's disease (glycogenosis, type Ia); X-linked glycogenosis (type VIll).

NOTE: If the diagnosis had not been confirmed prior to death, samples of liver, skeletal muscle, blood, and fascia (for fibroblast culture, see below) should be snap-frozen for enzyme assay, which will determine the specific deficiency. Types Ia and b, III, VI, and hepatic phosphorylase B kinase deficiency (types IXa, b and c) are hepatic-hypoglycemic disorders, whereas types V and VII affect muscle energy processes. Type II also affects the musculature, whereas type IV may cause cirrhosis and death in infancy from extreme hypotonia.

Determination of type of glycogenosis usually can be based on (I) pattern of glycogen storage in liver, (2) presence or absence of nuclear hyperglycogenation in liver, (3) cytoplasmic lipid in liver, (4) presence or absence of liver cirrhosis, and (5) presence or absence of glycogen and basophilic deposits in skeletal muscles.

Possible Associated Conditions: Fanconi syndrome* or gout* with type Ia glycogenosis; neutropenia, recurrent infections, and Crohn's disease with types Ib or Ie. Glycogen primarily in retinal ganglion cells and ciliary muscle. Glycogen in sympathetic nerve ganglia and neurons of cranial nerves in type VII.

Gouty arthritis.

Disease, Graft-Versus-Host NOTE: This disease occurs most commonly after bone marrow transplantation. The disease has also occurred after transfusion of viable lymphocytes, for example, to patients with cancer or leukemia. * In patients with graft-versus-host disease (GVHD), autopsy also may reveal recurrence of the underlying disease such as leukemia. Possible Associated Conditions: Alphal-antitrypsin deficiency;* amyloidosis;* ankylosing spondylitis;* primary sclerosing cholangitis;* Sjogren's syndrome. * See also below under "Possible or Expected Findings." NOTE: In many instances, either chronic ulcerative colitis or Crohn's disease* had been diagnosed clinically, but sometimes, the distinction is difficult to make, even at autopsy. Many features described below occur in chronic ulcerative colitis but some manifestations of Crohn's disease or conditions that may occur in all types of inflammatory bowel disease also are listed so that both positive and negative findings can be recorded properly. Osteoporosis;* ankylosing spondylitis;* arthritis of peripheral joints; periarthritis; hypertrophic osteoarthropathy;* tendinitis (particularly of ankle and Achilles tendons).

Disease, Iron Storage (See "Hemochromatosis.")

Related Terms: Atherosclerotic heart disease. NOTE: The most common anatomic finding at autopsy in subjects older than 30 yr is coronary atherosclerosis. Unusual under-lying or associated conditions include chronic aortic stenosis or regurgitation; coronary artery anomalies; coronary artery dissection; coronary embolism; coronary ostial stenosis (due to calcification of aortic sinotubular junction or, rarely, to syphilitic aortitis); coronary vasculitis (for instance, in polyarteritis nodosa* or acute hypersensitivity arteritis); hyperthyroidism,* gastrointestinal hemorrhage; * hypothyroidism, * idiopathic arterial calcification of infancy; intramural coronary amyloidosis; pheochromocytoma, polycythemia vera; * pseudoxanthoma elasticum,* radiationinduced coronary stenosis; severe pulmonary hypertension (with right ventricular ischemia); sickle cell disease;* and others. If bypass surgery had been performed, see "Surgery, coronary bypass." 

Macular rash (4).

Multifocal fibrinopurulent pneumonia with sparing of the bronchi and bronchioles. Exudate is rich in phagocytes, fibrin, and karyorrhectic debris.

Synonym: Lyme arthritis NOTE: This infection is caused by the spirochete, Borrelia burgdoiferi, which is transmitted from rodents to human by the hard deer ticks, Ixodes dammini, 1. ricinus, and others. 

Brain and spinal cord

For removal and specimen preparation, see Chapter 4. Request Luxol fast blue stain for myelin.

Symmetric and zonal demyelination in corpus callosum, anterior commissure, optic chiasm, optic tracts, and white matter of frontal lobes. 

External examination and skin; oral cavity Lungs Aorta

Record distribution of skin lesions and submit tissue samples for histologic study.

For preparation of angiograms of the pulmonary arterial and venous vasculature, see Chapter 2.

If aneurysm or dissection is present, follow procedures described under those headings.

Telangiectatic (often papular) lesions most commonly found in cheeks, scalp, nasal orifices, oral cavity, ears, neck, shoulders, fingers, toes, and nail beds. Cyanosis and clubbing may be prominent. Arteriovenous malformations/fistulas.

Aneurysm; * aortic dissection. * If cirrhosis is present, prepare angiograms of hepatic arteries and veins (Chapter 2). Photograph and prepare sections of angiomatous lesions. NOTE: Parkinson's syndrome is caused by conditions that may simulate Parkinson's disease; these include carbon monoxide* and manganese poisoning, corticobasal degeneration, druginduced parkinsonism, Huntington's disease, multiple system atrophy,* progressive supranuclear palsy* (Steele-Richardson-Olszewski syndrome), space-occupying lesions (rare), trauma (dementia pugilistica), and causes related to tumors and vascular diseases.

Brain For removal and specimen preparation, see Chapter 4. Histologic sections should include midbrain (substantia nigra), upper pons (locus ceruleus), medulla, nucleus basalis (substantia innominata), and basal ganglia.

If Parkinsonian syndrome was diagnosed, follow procedures described under the name of the suspected underlying condition (see above under "Note").

Depigmentation of substantia nigra and locus coeruleus; neuronal loss and reactive gliosis; eosinophilic intracytoplasmic inclusion bodies (Lewy bodies) in some of the surviving neurons; no significant changes in basal ganglia.

Disease, Pelizaeus-Merzbacher Synonyms: Sudanophilic (orthochromatic) leukodystrophy.

Brain and spinal cord

For removal and specimen preparation, see Chapter 4. Request Luxol fast blueIPAS stain for myelin and Bielschowsky's stain for axons. Prepare frozen sections for Sudan stain.

Brain generally atrophic. Myelin loss in centrum ovale, cerebellum, and part of brain stem, with a tigroid pattern of residual myelin near vessels. Axons are preserved. Diffuse gliosis with relatively few lipoid-containing macrophages, compared to the myelin loss. Lipoid material stains with Sudan. 

Brain and spinal cord

For removal and specimen preparation, see Chapter 4. Request silver stains (Bielchowsky or Bodian stain). Histochemical stains in Pick's cells and bodies reveal phosphorylated neurofilaments, ubiquitin, and tubulin. Some tissue should be kept frozen for biochemical studies.

Severe cerebral atrophy, involving primarily frontal and anterior temporal lobes (knifeblade atrophy; walnut brain). Microscopically, severe neuronal loss accompanied by astrocytosis. Characteristic argyrophilic, intracytoplasmic inclusions (Pick's bodies), particularly in hippocampus and swollen, distended "ballooned" neurons (pick's cells). These changes are not always present. 

External examination, skin, and adipose tissue Blood Cerebrospinal fluid Heart Liver and kidneys Brain, spinal cord, and peripheral nerves Eyes

Submit sample for determinaion of phytanic acid concentration and for molecular studies. For obtaining a sample, see Chapter 7.

Sample for histologic study. For removal and specimen preparation, see Chapter 4. For removal and specimen preparation, see Chapter 5.

Ichthyosis. Phytanic acid accumulation in adipose tissues. Phytanic acidemia, mutation of PHYH or PEX 7 (2). Increased protein concentrations. Cardiomyopathy.* Phytanic acid accumulation.

Axonal neuropathy. Retinitis pigmentosa. 

Hypoalphalipoproteinemia.

Lymphadenopathy with diffuse deposition of cholesterol esters.

Premature atherosclerotic cardiovascular disease (1).

Hepatosplenomegaly with foam cells.

Enlarged tonsils with characteristic orange discoloration.

Polyneuropathy (2) .

In adults, corneal infiltrates.

Foam cells. Request PAS stain. In granulomas, bacilli are not always PAS positive (2) . Section all grossly involved tissues for histologic examination. Submit section for electron microscopy.

Emaciation. Hyperpigmentation, particularly of exposed skin and in scars. Hyperkeratosis. Arthritis involving ankles, knees, shoulders, and wrists.

Ascites; fibrinous peritonitis. * Nodules in peritoneum containing sickle-form particlecontaining cells (SPC cells Submit sample for determination of sodium, potassium, chloride, glucose, urea nitrogen, and creatinine concentrations. Calcium and phosphate concentrations can also be tested.

If sample is small, indicate priority for testing.

If indicated, submit sample for chemical study. Submit tissue samples for histologic study.

Considerably increased or decreased values for sodium (more than 155 meqlL or less than 130 meqlL) and chloride (more than 135 meqlL or less than 105 meqlL) indicate that changes were present before death. For further interpretation, see Chapter 8. Postmortem electrolyte concentrations are quite unreliable. May be useful for calcium determination. Vacuolar nephropathy (vacuolar changes in proximal convoluted tubules) in potassium deficiency (may also occur after infusion of hypertonic solutions).

Disorder, Hemorrhagic (See "Coagulation, disseminated intravascular," ''Disease, Christmas:' ''Disease, von Willebrand's," "Hemophilia," and "Purpura,.��")

Disorder, Inherited, of Phagocyte Function NOTE: Several conditions represent phagocyte function disorders. Autopsy procedures for one of these disorders can be found under "Disease, chronic granulomatous." Consult this entry for other phagocyte function disorders.

Synonyms and Related Terms: Fabry's disease* (angiokeratoma corporis diffusum); gangliosidosis;* Gaucher's disease;* glycogenosis,* type II; leukodystrophies (Krabbe's or globoidcell,* metachromatic leukoencephalopathy*); mucopolysaccharidoses* (Hunter, Hurler, Morquio, and Sanfilippo disease); mucolipidosis; Niemann Pick disease* (type A, B, C, or sphingomyelinase deficiency); neuraminidase deficiency; neuronal ceroid lipofuscinosis (Batten's disease or Kufs' disease). 

Hypopharyngeal pulsion diverticulum (Zenker's diverticulum) at lower margin of inferior constrictor muscle of pharynx. Traction diverticulum at midesophagus after an inflammatory process-for instance, tuberculous lymphadenitis. Epiphrenic diverticulum may also occur. luxtacardiac or juxtapyloric diverticulum. Heterotopic tissue in Meckel's diverticulum, with or without peptic ulceration. Colonic muscular hypertrophy and stenosis, usually in sigmoid colon. Diverticulitis with perforation, fistulas, or peritonitis. *

Diving (See "Accident, diving (skin or scuba).")

Related Terms: Dry drowning; fresh-water drowning; near-drowning; salt (sea)-water drowning (see the following table).

Primary Drowning ("Immediate Drowning") Deaths occurring within minutes after immersion, before or without resuscitative measures

Deaths from hypoxia and acidosis caused by glottal spasm on breath holding. There may be no evidence of water entering stomach or lungs and no appreciable morphologic changes at autopsy. NOTE: The diagnosis is one of exclusion. The pathologist should help the police to determine: I) How did the person (or dead body) get in the water, and 2) why could that person not get out of the water? It is not enough to ask if a person could swim but investigators should find out how well (what strokes did the victim know?) and how far he or she could swim. The inquiry must include the depth of the water and must address hazards such as undertow or underwater debris, and the behavior

Deaths occurring from within 30 min to several weeks after resuscitation, because of metabolic acidosis, pulmonary edema, or infective or chemical pneumonitis

Deaths from hypoxia and acidosis caused by obstruction of airway by water related to: Hypervolemia Hemolysis Hyponatremia Hypochloremia Hyperkalemia of the victim immediately before submerging. Deaths of adults in bathtubs and swimming pools are usually from natural, cardiac causes, or they are suicides, unless the victim was drunk.

Diatom tests (1) have not proven useful in the United States but there is enthusiasm for such tests among European pathologists. The distinction between hyponatremic deaths in fresh water and hypernatremic deaths in salt water derives from experimental studies; in practice, one cannot reliably predict the salinity of the immersion medium from autopsy studies. Because many bodies of drowning victims are recovered only after the body floats to the surface, decomposition will often obscure even the nondiagnostic findings such as pleural effusions, which are often associated with drowning.

External examination and skin (wounds)

Organ samples for diatom search Serosal surfaces and cavities

If identity of drowning victim is not known, record identifying features as described in Chapter 13. Prepare dental and whole-body roentgenograms. Submit tissue samples for histologic study of wounds.

Inspect inside of hands.

Collect fingernail scrapings.

Record appearance and contents of body orifices.

Record features indicative of drowning.

Photograph face from front and in profile. Take pictures of all injuries, with and without scale and autopsy number.

Remove vitreous for analysis.

If diatom search is intended, clean body thoroughly before dissection to avoid contamination of organs and body fluids with algae and diatoms (see below). Submit sample for toxicologic study.

Sample early during autopsy, before carrying out other dissections. Use fresh instruments for removal of specimens to avoid contamination. Submit subpleural portion of lung: subcapsular portions of liver, spleen, and kidneys; bone marrow; and brain. Store samples in clean glass jars. For technique of diatom detection, see below.

Record volume of fluid in pleural spaces. Photograph petechial hemorrhages.

Photograph layerwise neck dissection if strangulation* is suspected. Open airways posteriorly, and photograph, remove and save mud, algae, and any other material in tracheobronchial tree. Record size and weight of lungs.

There may be wounds that were inflicted before drowning occurred-for instance, in shipwrecks or vehicular and diving accidents.

Other wounds may be inflicted after deathfor instance, from ship propellers or marine animals. Sometimes, premortem and postmortem wounds can be distinguished histologically.

Object (hair?) held by hands in cadaveric spasm. Cutis anserina and "washerwoman" changes of hands and feet are of no diagnostic help.

Foreign bodies; semen (see also under "Rape"). Foam cap over mouth and nose.

In the autopsy room, water running from nose and mouth is usually pulmonary edema or water from the stomach.

High concentrations of alcohol indicate intoxication (see under "Alcoholism and alcohol intoxication").

Evidence of alcohol intoxication may be found. Diatoms may occur in the liver and in other organs of persons who have died from causes other than drowning. Comparison with diatoms in water sample from area of drowning may be helpful.

Penny-sized or smaller hemorrhages may indicate violent respiratory efforts or merely intense lividity. Presence of pleural fluid suggests drowning. 

For diatom detection (l) , boil 2-5 g oftissue for 10--15 min in 10 rnL of concentrated nitric acid and 0.5 rnL of concentrated sulfuric acid. Then, add sodium nitrate in small quantities until the black color of the charred organic matter has been dispelled. It may be necessary to warm the acid-digested material with weak sodium hydroxide, but the material must soon be washed free from alkali to avoid dissolving the diatoms. The diatoms should be washed, concentrated, and stored in distilled water. For examination, allow a drop of the concentrate to evaporate on a slide, and then mount it in a resin of high refractive index. All equipment must be well-cleaned, and distilled water must be used for all solutions. There are several variations and adaptations of this method. Drug Abuse, Amphetamine(s) NOTE: Methamphetamine abuse may be suggested by poor condition of the dentition. Methylenedioxymethamphetamine ("Ecstasy") abuse is often suggested by friends with whom the decedent was abusing drugs. Follow procedures described under "Dependence, drug(s)." Ductus Arteriosus, Patent (See "Artery, patent ductal.")

Synonyms and Related Terms. Achondroplastic dwarf; asexual dwarf; ateliotic dwarf; micromelic dwarf; normal dwarf; pituitary dwarf; true dwarf; and many other terms, too numerous to mention. 

External examination

Bones and joints

Record height and weight. Prepare skeletal roentgenograms.

For removal, prosthetic repair, and specimen preparation, see Chapter 2.

Growth retardation. Abnormal growth of epiphyseal cartilage with enlargement of metaphysis. Long bones and pelvis most commonly affected. Cavernous hemangiomas (Maffucci's syndrome). See above under "External examination." Chondrosarcoma.

Dyscrasia, Plasma Cell NOTE: These conditions are characterized by abnormally proliferated B-immunocytes that produce a monoclonal immunoglobulin. Multiple myeloma, * plasma cell leukemia, plasma-cytoma, and Waldenstrom's macroglobulinemia* as well as heavy-chain diseases and monoclonal gammopathies of unknown type belong to this disease family. Amyloidosis* is closely related to these conditions. For autopsy procedures, see under "amyloidosis," "macroglobulinemia," or "multiple myeloma" and under name of condition that may have caused the plasma cell dyscrasia. Such conditions include carcinoma (colon, breast, or biliary tract), Gaucher's disease,* hyperlipoproteinemia, * infectious or noninfectious chronic inflammatory diseases, and previous cardiac surgery.

Synonym: Shigella dysentery. NOTE: (I) Collect all tissues that appear to be infected. 

Blood Bowel Eyes Joints Submit sample for culture and for serologic study. Submit sample of feces or preferably bloodtinged mucus for culture. If bacteriologic diagnosis has already been confirmed, pin colon on corkboard, photograph, and fix in formalin for histologic study. Submit sample of vitreous for study of sodium, potassium, chloride, and urea nitrogen concentrations.

For removal and specimen preparation of eyes, see Chapter 5. For removal, prosthetic repair, and specimen preparation, see Chapter 2.

Escherichia coli septicemia.

Colitis with microabscesses; transverse shallow ulcers and hemorrhages, most often in terminal ileum and colon.

Dehydration* pattern of electrolytes and urea nitrogen.

Serous arthritis* of knee joints is a late complication. 

External examination

Record extent of pigmentation, facial features, and primary and secondary sex characteristics.

Prepare skeletal roentgenograms.

For removal, prosthetic repair, and specimen preparation, see Chapter 2.

Record size of apertures of cranial nerves in base of skull.

Unilateral skin pigmentation and precocious puberty in females (Albright's syndrome), less commonly in males. Synonyms and Related Terms: Becker's muscular dystrophy; congenital muscular dystrophy; Duchenne's progressive muscular dystrophy; dystrophinopathy; Em-ery-Dreifuss mucular dystrophy; facioscapulohumeral dystrophy; limb girdle dystrophy; myotonic muscular dystrophy.

External examination

Record pattern of scalp hair.

Record status of skeletal musculature.

Obtain sections for histologic examination. Dystrophin staining of the sarcolemma is absent in Duchenne's muscular dystrophy and patchy in Becker's dystrophy.

Frontal baldness (in myotonic muscular dystrophy). Atrophy and wasting of muscles (generalized or local: predominantly distal in myotonic muscular dystrophy). Pseudohypertrophy of calf muscles in Duchenne's muscular dystrophy. Dystrophic changes include variations in fiber size, fiber degeneration and regeneration, peri-and endomysial fibrosis, and fatty replacement of muscle. The liver, especially the right lobe, is the most common site of involvement. Secondary infection or calcification may be present.

The lung is the second most common site of involvement. Fluid and air may be visible on the roentgenogram.

Cysts may be present in the abdominal cavity, muscles, kidneys, spleen, bones, heart, and brain. Eosinophilia.

Edema, Angioneurotic Synonym: Angioedema. NOTE: Possible causes and suggested autopsy procedures are described under "Death, anaphylactic."

Related Term: Silo-filler's disease. N<YfE: This condition is causedby inhalationoftoxic gases, such as oxides of nitrogen (silo-filler's disease) and phosgene (COCI 2 ). See also "Bronchitis, acute chemical" and "Poisoning, gas." (1) gunshot and shotgun wounds of the head involving dural sinuses; (2) injury to large veins, particularly cranial sinuses (during neurosurgical procedures) and veins ofthe neck (knife wounds, surgery) or uterus (criminal abortion); (3) insufflation offallopian tubes (particularly in pregnancy or during menstrual period); (4) infusion of blood components or crystalloid; (5) malfunctioning of dialysis machines; (6) subclavian vein catheterization in the semi-Fowler position; and (7) fracture in the hub of a central venous catheter used for parenteral nutrition.

Possible causes of arterial air embolism include:

(1) open heart surgery involving the aorta, left atrium, or left ventricle;

(2) positive-pressure ventilation in newborn infants; and (3) underwater ascent with closed glottis (see Accident, Diving)

Ifair embolism is suspected, the autopsy should be performed as soon after death as possible. Decomposition gases may be produced within a few hours. Roentgenography of the whole body may detect large quantities of air, and the roentgenograms may serve as a guide to the most advantageous way of dissection.

The postmortem diagnosis of arterial air embolism is made largely on the basis of medical history and circumstances. The autopsy serves to rule out competing conditions.

The diagnosis of venous air embolism is made by chest roentgenography before the autopsy (1). The air in the right chambers of the heart can be confirmed at autopsy by aspiration into a syringe. The formerly recommended procedure of clamping the internal mammary vessels below the sternoclavicular joints, and then cutting across the sternum distal to these clamped vessels so that the sternoclavicular joint area remains intact, is designed to prevent the production of a vacuum that pulls air into the veins. The procedure is not necessary if the air is welldocumented by roentgenography before autopsy. At autopsy, a large fatal pulmonary air embolism is readily apparent. The right atrium and ventricle are distended with fine, frothy, brightred blood, which also may distend the pulmonary arteries and superior vena cava. Microbiologic examination ofblood and pericardial sac contents may help to rule out the presence of gas-forming bacteria that may simulate air embolism (Fig. II-I). However, the presence of putrefactive emphysema in any part of the body points toward a bacterial origin of the gas. Differentiation of air and decomposition gases at the autopsy table with the pyrogallol test is described below.

A 2% pyrogallol solution is prepared (it should be waterclear). Two lO-mL syringes (syringe A and syringe B) are loaded with 4 mL of the pyrogallol solution in each, without permitting any air to enter the system. Immediately before the solution is used, 4 drops of 0.5 N NaOH is aspirated through the needle of syringe A to adjust the pH to about 8 (1 drop per 1mL of solution); the mixture will turn faint yellow. Six mL of gas is then aspirated from the heart or blood vessels. The needle is immediately sealed with a cork or replaced by a cap, and the syringe is vigorously shaken for about 1 min. In the presence of air, the pyrogallol solution will turn brown. If the solution remains clear, decomposition gases were present. In the latter instance, 4 drops of 0.5 N NaOH and 6 mL of room air should be aspirated into syringe B, which is then also sealed and shaken for 1 min. The mixture should turn brown, thus serving as a Fig. 11·1 . Gas-forming bacteria simulating air embolism. The pericardial sac is opened and filled with water. The heart is kept submerged with a pair of scissors. The coronary arteries have been incised with a scalpel. Note gas bubbles and foam on the water surface. No discoloration of 2% pyrogallol was noted. Blood cultures were positive for Enterococcus organisms. Microscopically, gram-negative rods were found in most tissues.

control that the pyrogallol solution had been properly prepared. Syringe B may also serve as a reserve. If only one syringe is used, the decomposition gas can be expel1ed and room air can be aspirated for the control test.

If only small amounts of gas can be aspirated, the volume of the pyrogallol solution should be decreased so that the gas-fluid volume ratio is at least 3:2.

If no bacterial gas formation is present, the edges of the pericardial incision are elevated and the pericardial sac is fil1ed with water. Clamping of the ascending aorta and venae cavae prevents the escape of gas into these vessels. The heart is held under water while the coronary arteries are incised, and the escape of bubbles is recorded. When the right coronary artery is opened, care must be taken that the right atrium is not incised. Air in the coronary arteries indicates systemic embolism. The heart chambers are then incised.

When there is gas in any of the arteries or heart chambers, gas bubbles rise to the surface of the water in the pericardial sac ("bubble test"). Sometimes the vessels have to be somewhat compressed in order to cause the gas to escape. Because large amounts of air or other gases cause the heart to float, it must be kept submerged before the vessels and chambers are incised. Basically the same procedure is used for demonstrating the presence of gas in the superior or inferior vena cava and the pelvic veins (for example, in cases of criminal abortion). In this situation, the abdominal cavity is filled with water and the inferior vena cava and its tributaries are incised.

In support of the diagnosis of systemic arterial air embolism, the skull vault may be removed without puncturing the meninges, so that the cerebral arteries can be inspected for gas bubbles. The demonstration of gas bubbles in the meningeal vessels and in the circle of Willis is meaningful only when the neck vessels are still intact and the internal carotid artery and basilar artery have been clamped before the brain is removed. In acute cases, gas bubbles will be visible within the cerebral vessels. They are released under water when the clamps are removed and the vessels are slightly compressed.

For the collection of gas from blood vessels or cavities, a system oflittle quantitative reliability is an air-tight, water-filled glass syringe with a needle. The needle is inserted into the vessel or cavity in question and gas is carefully aspirated.

A combined qualitative and quantitative method has been described by Kulka (2,3) (see Fig. 11 -2). He devised an apparatus for gas collection and described it as shown in Fig. 11 -2 and caption.

The entire system is filled with mineral oil so that, when the funnel is level with the upright bottle, the oil fills only about half of the funnel. In operation, the funnel is first raised to a position 30-40cm above the level of the upright bottle. All the cocks are opened and the position is held until every trace of gas has been driven from the system through the needle, which is thereby coated on the inside by a film of oil. After all air has been expelled, the cocks are closed and the funnel is lowered to its original position.

As a precautionary measure and control, the air-tightness of the whole system should be tested before operation. This is done by inserting the needle into musculature or skin and attempting aspiration in the manner described in the next paragraph.

To make the test, the bottle is inverted and the needle is inserted into the cavity in question. When the needle is in position, all cocks are opened. The funnel is lowered about 70-90 cm, or until adequate suction is created. This aspirates the contents of the cavity, which may consist of air or other gases, either pure or mixed with blood or other liquid. Any gas or liquid entering this system may be observed through the wall of the short bent glass tubing. In a positive test, gas bubbles will collect in the bottle above the level of the oil. If desired, this gas can be saved for further examination by closing all the cocks and returning the bottle to its upright position (4).

The volume ofintravenous air needed to cause death in adults depends of the cross sectional area of the cardiac cavity or great vessel that is the site of the gas lock, which in tum depends on the position of the decedent at the time of the embolism (5). 100 mL of gas can pass through an intravenous hub in just a few seconds. Small amounts of air entering the systemic circulation may cause death within minutes. Delayed air embolism with fatal outcome may also occur. Other organs

If purpura is present, prepare photographs and record extent. Submit sample (from right atrium) for microbiologic study. Collect blood from right atrium and right ventricle. After the heart has been removed, allow blood in pulmonary vessels to pool in the pericardial sac.

Centrifuge this blood and submit sample of flocculent layer above buffy coat for microscopic study (1) . Submit a section of lung for bacteriologic study. Dissect pulmonary arteries; prepare histologic sections of all lobes; request mucicarmine stain and the aldan blue and phloxinetartrazine stain of Lendrum. Also request Sudan stain on frozen sections.

Skin purpura.

Vernix, lanugo hairs, and meconium can be found in pericardial blood pool.

Meconium-type material in blood vessels.

In histologic sections, squamous epithelium, meconium, and fat from vernix caseosa.

Complete or incomplete lower uterine tear; chorioamnionitis.

Manifestations ofdisseminated intravascular coagulation* and fibrinolysis. Intrauterine pneumonia.

Large amounts of debris in the blood vessels of all sections of the lungs may be considered to be lethal if there is no other cause of death. Small amounts in one or more blocks of pulmonary tissue are more likely incidental (1). A small uterine tear is more likely followed by a fatal amniotic fluid embolization than is a large tear, which may result in fatal hemorrhage or fibrinogen depletion. Chorioamnionitis, intrauterine pneumonia, and positive lung cultures of the mother indicate infection of the amniotic fluid. Record weight and sample for histologic study.

For removal and specimen preparation, see Chapter 4. Photograph horizontal sections through brain, brain stem, and spinal cord. Prepare frozen sections and request Sudan stain. Prepare frozen sections of one-half of gland and paraffin sections of the other half.

Petechial hemorrhages of skin (chest, neck, and face). Wounds; other traumatic lesions. Bone fractures. Petechiae of conjunctivas and retinas. Fat may accumulate on surface ofblood pool. No useful technique is available for estimating the amount of fat globules in the blood.

Fat emboli in lumen of small vessels and in pulmonary air spaces.

Severe fatty changes may be the cause offat embolism. Fractures are the most common cause of fat embolism. Petechial hemorrhages, fat emboli (2). 

Abdominal cavity

Submit sample of subphrenic exudate for gram stain and cultures. Record location and volume of subphrenic exudate.

Possible causes of subphrenic empyema include appendicitis, cholecystitis, * diverticulitis, intrahepatic abscess, pancreatitis,* ruptured viscus; penetrating abdominal wound(s), perforated ulcer of stomach or duodenum,* and other conditions.

Pleural cavities and lungs E Procedures Record volume of effusion or exudate in pleural space.

Basal pleuritis and pneumonia, adjacent to empyema.

Encephalitis, AU Types or Type Unspecified Synonyms and Related Terms: Acute disseminated encephalomyelitis;* acute hemorrhagic encephalitis; acute infective encephalitis or encephalomyelitis; acute poliovirus encephalitis or encephalomyelitis; amoebic encephalitis; Arbovirus encephalitis (Japanese encephalitis; eastern encephalitis, western encephalitis, venezuelan equine encephalitis, St. Louis encep-halitis); bulbar encephalitis;* brain stem encephalitis;* herpes encephalitis (cytomegalovirus encephalits, Epstein-Barr virus encephalitis, varicella-zoster encephalitis); herpes simplex ence-phalitis; HIV encephalitis; measles encephalitis; measles inclusionbody encephalitis; postinfectiousencephalitis; postvac-cinal encephalitis; progressive multifocal leukoencephalitis or leukoencephalopathy; rabies* encephalitis; subacute encephalitis; subacute sclerosing panencephalitis; viral encephalitis, west nile encephalitis and other terms (1), too numerous to mention. See also under "Note" and under "Possible or expected findings." NOTE: If the condition that caused the encephalitis is known, see also under that heading. If the cause of the encephalitis is unknown, submit samples of tissue for microbiologic and toxicologic study, particularly if there is a suspicion of lead poisoning.* See also under "Encephalitis, brain stem," "Encephalomyelitis,...," "Encephalopathy" and "Myelopathy, Myelitis." Encephalitis, Brain Stem Synonyms and Related Terms: Brain stem abscess; infectious brain stem encephalitis; Listeria monocytogenes brain stem encephalitis; viral brain stem encephalitis.

NOTE: See also under "Encephalitis, limbic."

Brain

For removal and specimen preparation, see Chapter 4.

Necrotizing encephalitis, with or without abscess formation (1 Enterocolitis, Other Types or Type Undetermined NOTE: A multitude of infectious and noninfectious agents may cause inflammation of the small bowel, large bowel, or both. If the condition is not listed under "Colitis," "Enteritis," or "Enterocolitis" or under another specific heading such as "Dysentery, bacillary," obtain sufficient material for microbio-logic and histologic study to identify organisms such as Clos-tridium, Chlamydia, Shigella, Salmonella, Yersinia, Helicobacter, verotoxic E. coli, and others. If lymphogranuloma venereum,* or tuberculosis* are suspected, see also under these headings. See also under "Disease, inflammatory bowel" and "Disease, Crohn's."

Synonyms and Related Terms: C. difficile colitis; Darmbrand; hemorrhagic necrosis (gangrene; infarction) of intestine; ischemic enteritis or enterocolitis;* neutropenic enterocolitis;* pseudomembranous colitis. NOTE: The name "Pseudomembranous enterocolitis" is descriptive; the condition may be infectious, ischemic, or both. If the intestinal changes are clearly ischemic, see above under "Enterocolitis, ischemic." If the cause is in doubt and if pseudomembranes can be identified, follow the procedures described here.

(l) Collect all tissues that appear to be infected. For other infectious intestinal diseases, see under specific names, such as "Enterocolitis, neutropenic" or "Enterocolitis, staphylococcal."

Intestinal tract and mesentery

Other organs

Collect material from pseudomembranes for culture and for C. difficile toxin assay.

Sample intestinal wall with pseudomembranes for histologic study.

If the condition is suspected to be caused by ischemia, follow procedures described above under "Enterocolitis, ischemic." NOTE: Myoclonic seizures also have been described in a number of progressive encephalopathies with complex neurological symptoms, such as GMI and GM2 gangliosidosis,* and Niemann-Pick* and Krabbe's disease but also acquired disorders, including Alzheimer's disease,* Creutzfeldt-Jakob Epilepsy, Myoclonus (continued) disease,* posthypoxic encephalopathy, and subacute sclerosing panencephalitis. Mitochondrial encephalomyopathy also can present with myoclonus epilepsy and a mitochondrial myopathy with ragged red fibers (MERRF syndrome) in skeletal muscles. Lafora-body-type material in the heart, liver, retinas, peripheral nerves, skeletal muscles, and sweat gland ducts (especially axillary).

Ragged red fibers in mitochondrial myopathies.

Epilepsy, Symptomatic NOTE: Possible causes or underlying conditions include cerebrovascular diseases, congenital malformations ofthe brain, degenerative and demyelinating diseases of the brain, head injury,* intracranial and cerebral infections, toxic or metabolic disorders (alcoholism,* barbiturate,* carbon monoxide,* and lead poisoning,* hemodilution, hypocalcemia, or hypoglycemia),* and tumors of the brain. * F Failure, Congestive Heart NOTE: Coronary atherosclerosis and manifestations of ischemic heart disease, * valvular heart disease, congenital cardio-vascular diseases, and manifestations of systemic or pulmonary hypertension are the most frequent findings in patients dying of or with congestive cardiac failure. Other causes include cardiomyopathies* and secondary myocardial disease (such as amyloid or pericardial constriction). If the cause of the congestive cardiac failure is unknown or not immediately evident after dissection of the heart and of the great vessels, myocardium and other appropriate tissues may be submitted for microbiologic study-including viral cultures-and for electron microscopy. Specimens can also be snap-frozen for possible immunofluorescent, biochemical, or histochemical studies, particularly of the myocardium. Possible causes of congestive cardiac failure are too numerous to mention. Dilatation of heart, with or without mural thrombosis. Myocardium may be soft, normal, or firm. Pulmonary congestion, with or without hemosiderosis; congestion of viscera with organomegaly. Other organ manifestations include bowel edema or hemorrhagic enteropathy (without mechanical vascular occlusion) and zonal hepatic steatosis, fibrosis, or necrosis, with or withoutevidence of liver failure. Acute renal tubular necrosis may be present also.

Synonyms and Related Terms: Acute kidney failure; chronic kidney failure; renal failure; uremia. NOTE: If acute kidney failure had been diagnosed, the autopsy procedures will depend on the expected causes, such as poisoning with ethylene glycol,* lead,* mercury,* or methyl alcohol;* disseminated intravascular coagulation* and its various underlying conditions; glomerulonephritis* and its various underlying conditions; diabetes mellitus;* or multiple myeloma. * The procedures described below deal primarily with chronic renal failure. If the patient had had dialysis, see also under "Dialysis (for chronic renal failure )." If transplantation had been carried out, see also under "Transplantation, kidney." 

Lassa fever is a highly communicable disease and autopsy studies are not recommended in the usually available surround-ings. If Lassa fever is suspected, contact the state health department and the Centers for Disease Control and Prevention, Atlanta, GA, for disposition and further studies (1).Ifan autopsy is done, disinfection can be accomplished by washing instruments with 0.5% phenol in detergent (i.e., Lysol), 0.5% hypochlorite solution, formalin, or paracetic acid. For shipping procedures, see Chapter 15. This is not a reportable disease. 

Synonyms: Borreliosis; louseborne (epidemic) relapsing fever; tickborne (endemic) relapsing fever. NOTE: (I) Collect all tissues that appear to be infected. (2) Rat/mouse inoculation with infected blood is the most sensitive method for the detection of the organism. Consult the state health department. (3) Before the autopsy, consultation with the microbiology laboratory is advised. (4) Request direct dark-field examination and Giemsa or Wright stain. (4) No special precautions are indicated. (5) Serologic studies are of questionable value due to the antigenic variability of the organism. (6) Fever, Scarlet NOTE: Usually, this disease is a nonfatal group A streptococcal tonsillitis and pharyngitis with skin rash. Potentially fatal complications include suppurative otitis media,* mastoiditis, and pharyngeal abscess (1), with or without septicemia.

(1) Collect all tissues that appear to be infected. (2) atresia, but may also be seen with viral myocarditis, type II glycogen storage disease (Pompe disease) and carnitine deficiency. Thus, a metabolic disorder must be considered. It is ideal to perform the autopsy as soon after death as possible (1) .

Urine Plasma For removal, prosthetic repair, and specimen preparation, see Chapter 2.

Malnutrition (1) Muscle necrosis (Clostridial myonecrosis) and accumulation of gas; little leukocytic infiltration.

Other organs G

Procedures depend on expected findings or grossly identified abnonnalities as listed in right-hand column. See also above under "Note" and under "Skeletal muscles." 

Synonyms and Related Terms: Acute postinfectious glomerulonephritis (nonstreptococcal postinfectious glomerulonephritis;*minimalchangedisease;mesangialproliferativeglomerulonephritis;* focal and segmental glomerulosclerosis with hyalinosis (focal sclerosis); poststreptococcal glomerulonephritis; idiopathic nephrotic syndrome; IgA nephropathy (Berger's disease); membranous glomerulonephritis; membranoproliferative glomerulonephritis; mesangial proliferative glomerulonephritis; rapidly progressive glomerulonephritis (associated with systemic infectious or immunologic multisystem diseases; drug idiosyncrasy; or as primary crescentic glomerulonephritis or superimposed on another primary glomerular disease).

Possible Associated Conditions: Acquired immunodeficiency syndrome (AIDS);* Alport's syndrome;* amyloidosis; anaphylactoid purpura; bee stings; chronic allograft rejection; dennatomyositis;* dennatitis herpetiformis; diabetes mellitus;* drug dependence;*Fabry's disease;* Goodpasture's syndrome;* Guillain-Barre syndrome;* Henoch-Schonlein purpura;* hemolytic uremic syndrome;* infective endocarditis;* leprosy;* malig-nancies;mixedconnectivetissuedisease;myxedema;polyarteritis nodosa;* rheumatoid arthritis;* preeclamptic toxemia; renovascular hypertension; sarcoidosis;* serum sickness;* Sjogren's syndrome;*syphilis;*systemiclupuserythematosus;*systemic sclerosis;* thrombotic thrombocytopenic purpura;* thyroiditis;* vasculitis; viral hepatitis;* Wegener's granulomatosis;* and many other conditions. Urate deposits in scleras and corneas.

Granulocytopenia NOTE: Midline granulomas may belong to the angiocentric immunoproliferative lesions, which are related to lymphomatoid granulomatosis* and malignant lymphoma. The name "idiopathic midline granuloma" should be reserved for the few cases without evidence of malignant lymphoma or Wegener's granulomatosis* (1). The diagnosis of idiopathic midline granuloma also can be ruled out if studies reveal fungal organisms or features of leishmaniasis;* leprosy,* rhinoscleroma, pseudotumor of the orbit or tuberculosis. * Complications of nasal cocaine abuse also may mimic midline granuloma (2). 

Eosinophilic pneumonitis (degenerating eosinophils with Charcot-Leyden crystals) and granulomas. Angiitis (mostly arteritis), typically with giant cells in tunica media (1) . Findings resemble those in polyarteritis nodosa. * Heart (2), grastrointestinal tract (3), skin, muscles, and joints are commonly involved. However, renal disease is often (but not always) mild or absent.

Necrotizing vasculitis of small arteries and veins is present, with extravascular granulomas and eosinophilic infiltration of vessels and perivascular tissues. Optic neuritis may be found (4). May be affected by the vasculitis (4). PCR studies on paraffin sections via RNA in situ hybridization may confirm presence of Epstein-Barr virus-positive B-cell proliferations combined with dense T-cell accumulations (3) (4) (5) . The condition closely resembles angiocentric T-/NK cell lymphoma (3). Infiltration of lymphocytoid cells, plasma cells, and macrophages with necroses and granulomatous features, which are found primarily in the vicinity of blood vessels. Special stains may reveal evidence of infection.

Lymphoreticular and granulomatous infiltrates (see "Lung"). Lymphoreticular and granulomatous infiltrates (see "Lung"). Characteristic infiltrates may be present in all organs and tissues. Involvement of spleen, lymph nodes, and bone marrow is uncommon. In rare instances, the disease is confined to the abdomen.

In most instances, characteristic infiltrates are present (6). 4 

Septicemia; circulating immunoglobulin complexes, and antiendothelial antibodies (7). Angiocentric granulomatosis (4) Hemorrhage, Intracranial (See under "Hematoma, subdural" and under name of suspected underlying condition, such as "Aneurysm, cerebral artery," "Infarction, cerebral," "Injury, head," and "Tumor of the brain."

Hemorrhage, Subarachnoid (See under name of suspected underlying condition, such as "Aneurysm, cere-bral artery," "Infarction, cerebral," "Injury, head," and "Tumor of the brain."

Hemosiderosis, Idiopathic Pulmonary NOTE: This diagnosis is made by exclusion; involvement of organs other than the lungs (see below under "Kidneys") suggests another disease. Hepatitis, Chronic Synonyms and Related Terms: Autoimmune hepatitis; chronic viral hepatitis B (with or without D) and C. NOTE: The term "chronic hepatitis" is not a complete etiologic diagnosis and related names such as chronic active (aggressive) hepatitis, chronic active liver disease, and chronic per-sistent hepatitis are obsolete. Most cases in these categories represent autoimmune hepatitis or chronic viral hepatitis B or C. Many other liver diseases, including drug-induced hepatitis, inborn errors ofmetabolism (e.g., alphal-antitrypsin deficiency* or Wilson's disease*), developmental disorders, and chronic biliary diseases such as primary biliary cirrhosis or primary sclerosing cholangitis also may present as chronic hepatitis.

Ifchronic hepatitis was the cause ofdeath, submassive hepatic necrosis or cirrhosis* is usually present, often with manifestations ofportal hypertension,*hepatic encephalopathy, hepatorenal syndrome,* and with ascites or spontaneous bacterial peritonitis.

Possible Associated Conditions: See also below under "Possible or Expected Findings." Conditions that may be associated with hepatitis C include (1): autoimmune hepatitis; Behcet's disease; diabetes mellitus (type 2);* glomerulonephritis;* Guillain-Barre syndrome;* idiopathic pulmonary fibrosis; idiopathic thrombocytopenic purpura; IgA deficiency; lichen planus; mixed essential cryoglobulinemia; Mooren's corneal ulcers; polyarthritis; porphyria cutanea tarda;* thyroiditis. * NOTE: Coinfection with other hepatitis viruses (e.g., C and G) and/or systemic viruses such as the immunodeficiency virus are common, particularly in drug addicts (1, 2) . In many cases of fulminant hepatitis, tests for known hepatitis viruses are negative (3, 4) . Homicide NOTE: Not all of the following procedures can be carried out or will be required in all cases, nor will this checklist be sufficient in all instances. Consult also the entries indicating the cause of death, such as "Injury, firearm" or "Injury, stabbing." If the victim is an infant, see all under "Infanticide."

Before the body arrives or before autopsy is begun:

1. Investigate the scene where the body was found and where the crime may have been committed (these may be two separate locations). If this is not possible, study the report and photographs submitted by the investigator or the police. Study all available information. Request previous medical records and roentgenograms of the victim. 2. Emphasize to first responders and autopsy personnel that the body of the victim should not be undressed, washed, or otherwise disturbed until it has been inspected by the pathologist. Embalming is not permitted before completion of the autopsy. Advise first responders or transporters to put paper bags over the hands of the victim. This will protect possible evidence, such as hair from the assailant. 3. Prepare record sheets to document the time of arrival and release of the body; chain of custody for the body and specimens; the name, age, sex, and other information about the victim; the names ofthe technician, pathologist and guests; and the specimens taken. 4. Prepare roentgenographic and photographic equipment.

After Body Arrives but Before Autopsy Is Begun:

1. If the body is left unguarded-for instance, overnight-a lock should be placed on the refrigerator or cool room where the deceased is kept, and the key should be retained by the technician. 2. If the pathologist is not accustomed to a busy autopsy room, he or she should feel free to ask all persons other than the pathologist(s), technicians, and law enforcement personnel who are not immediately involved in the actual performance of the autopsy to leave the morgue. 3. The body temperature of the victim can be recorded at the autopsy facility, but this is rarely useful, particularly if the victim seems to have been deadfor longer than a day ortwo or ifthe body had previously been stored in arefrigeratoror cool room. Insert thermometer deep into the anus (about 7-8cm). Record temperature and manner and time of procedure. 4. Aspirate vitreous from one or both eyes and store the specimen in a refrigerator. 5. Prepare roentgenograms. In each case, a decision must be made whether roentgenograms should be made of the entire body or only of parts of it-or not at all-and whether they should be made before the victim is undressed, after the victim is undressed, or at both times.

1. Take overall survey photographs that depict the anterior surfaces of the body as it is on arrival to the facility, before any undressing, manipulation for roentgenographs, or cleaning. 2. Take close-up photographs of any trace evidence such as fibers or flakes of gunpowder before undressing or cleaning. 3. Photograph the face of the victim for identification purposes after it has been cleaned of any blood and foreign matter. To avoid perspective distortion from wide angle lenses, the lens should be about 4 ft from the face. 4. Photograph all injuries and other forensically significant findings after blood and foreign matter have been cleaned off the body. A moist towel is useful; brushes are too abrasive. Two photographs should be taken of each finding. One should include the autopsy number and a scale. A second photograph should be taken without any extraneous objects.

Collection and Documentation of Evidence:

1. Fingerprinting can be done before or after the autopsy but should be done in all homicide cases. In cases involving close contact between assailant and victim, fingernail scrapings or clippings (use a new clipper) and hair with roots should be collected, and its source should be identified (hair pulled from scalp, axillae, and pubis is identified as that of the victim; hair in hands or under fingernails or on clothing of victim may be from the assailant). Hair exemplars can be collected in cases of homicide by firearm but are rarely of use.

If the body is decomposed or mutilated or for any other reason has not been identified, prepare roentgenograms ofthe head, neck, and torso before the autopsy (the radiographic appearance is altered by the autopsy). These can be used for comparison purposes if antemortem films are located. If films of the extremities are needed they can be prepared after the autopsy. Dental roentgeno-.grams are taken, usually after the autopsy, when investigators have located antemortem dental records. Detailed descriptions and sizes of clothing, with photographs of clothing, can be useful then there is no putative identity. 3. In cases in which sexual assault may have been involved, collect pieces of clothing that may contain seminal fluid stains. Follow procedures described under "Rape." 4. Preserve clothing or part of clothing as evidence. Wet clothing should be dried before it is stored in labeled and sealed bags.

The Autopsy:

1. The measured length and weight, extent of rigor, and color and distribution of livor should be recorded, along with the state of preservation, nutrition, and hydration. Do not omit examination of the hands, particularly of the volar surfaces. 2. If air embolism is suspected, see procedure described under Part II "Embolism, Air". If pneumothorax is suspected, see procedure described under Part II "Pneumothorax". If there is evidence of strangulation or other neck injury, perform a layerwise dissection of the neck that includes the hyoid bone and tongue. 3. Prepare diagrams of wounds and identify their location by anatomic region. For wounds of the torso, state the distance from the soles of a foot and the distance laterally from to the right or left of the midline. 4. Submit samples of wounds for histologic study when there is any question of the age of the wounds. 5. The records should show when body fluids and tissues were collected for laboratory analysis, the volume and appearance of such specimens, and what was done with them. If most of the toxicology specimens are collected immediately after the internal examination has commenced, the time of the internal examination serves as the collection time. In all instances, the following items should be collected: blood ofvictim for DNA comparison and toxicologic study (determination of alcohol, carbon monoxide, and drug concentrations will be requested most frequently); vitreous; urine; gastric contents; at least one solid organ specimen for toxicologic study (liver and brain have the most comparison data If an extracerebral congenital malformation is suspected, follow procedures described under "Malformation, Arnold-Chiari." If cerebrospinal fluid is aspirated with a syringe, record volume. Record weight of brain; record size of brain in relation to inner dimensions of skull. Describe size of ventricles.

Other procedures depend on expected findings or grossly identified abnormalities as listed in right-hand column. If a surgical shunt is present, its location should be recorded and both ends of the implanted specimen that was used for shunting may be submitted for microbiologic study. If the shunt is not patent, record site and nature of obstruction. 

Related Terms: Antibody-mediated hydrops fetalis; erythroblastosis fetalis;* nonimmune hydrops fetalis. NOTE: The classic example of antibody mediated (Rh incompatibility) hydrops fetalis is hemolytic disease of the newborn (erythroblastosis fetalis*). However, nonimmune hydrops fetalis also may be caused by hematologic disorders, e.g., alpha-thalassemia* (1) or it may have no known cause. Infec-tions, e.g., with human parvovirus Bl9 (2), cytomegalovirus, or syphilis;* heart and vascular diseases (3) (cardiac tumors, cardi-omyopathy,* myocarditis,* arterial calcification, and others); storage disease (4); tumors (including neonatal leukemia*); and many other fetal (e.g., congenital chylothorax* or lymphatic dysplasia; pulmonary sequestration and cystic adenomatoid malformation) or maternal conditions, e.g., maternal thyrotoxicosis, also may cause nonimmune hydrops fetalis. If coronary insufficiency or myocardial infarction is suspected, follow procedures described under "Disease, ischemic heart."

For coronary arteriography, see Chapter to.

Record distribution of atherosclerotic lesions in aorta. Samples for histologic study should include aorta, coronary arteries, and peripheral arteries. See also above under "Heart." Submit samples of head, corpus, and tail for histologic study. If applicable, see also under "Diabetes mellitus." For special procedures and stains, see above under "Heart." Other procedures depend on expected findings or grossly identified abnormalities as listed in right-hand column.

For removal and specimen preparation, see Chapter 4.

Obesity* is a common finding. Eruptive xanthomas (palms, elbows, knees) in some but not all types of hyperlipoproteinemia. Most prominent in apoprotein CII deficiency. Xanthomas of tendons (knees, elbows, dorsum of hands), xanthelasmas, and arcus comeae in familial hypercholesterolemia. Gangrene of lower extremities (see below under "Arteries").

Severe coronary atherosclerosis and myocardial infarcts in type 3 hyperlipoproteinemia and multiple lipoprotein-type hyperlipidemia.

Atherosclerosis of abdominal aorta and its branches and of carotid arteries. Coronary atherosclerosis (see above).

Pancreatitis* in familial apoprotein CII deficiency.

Foam cells with triglycerides in liver, spleen, and bone marrow in familial lipoprotein lipase deficiency. Manifestations of diabetes mellitus* and hypothyroidism* in some cases of type 3 hyperlipoproteinemia. Cerebral infarct (stroke*) in type 3 hyperlipoproteinemia.

Hypernatremia (See "Disorder, electrolyte(s).") Hyperoxaluria Synonyms and Related Terms: Oxalosis; primary hyperoxaluria type I (a1anineglyoxylate aminotransferase deficiency); primary hyperoxaluria type II (D-glyceric acid dehydrogenase deficiency); secondary hyperoxaluria (see under "Note").

NOTE: In ethylene glycol poisoning,*oxalatecrystals in media ofsmall arteries, with associated ischemic lesions. Similardeposits may occur after long-term hemodialysis (1). These conditions must be distinguished from the genetic disease. Also, oxa-Iate nephropathy may be a complication of short bowel syndrome.

If patient with congenital hyperoxaluria underwent liver or combined liverlkidney transplantation (2) , see also under these headings. Remove vitreous for biochemical evalvation.

Submit tissue (i.e., fascia lata) for karyotype analysis.

Polycystic ovaries, testicular adrenal rests (4 NOTE: There are no diagnostic autopsy findings for hypoxia. Possible causes of acute hypoxia include anesthesia-associated death,' compression of the torso by a vehicle, diving accident,' exposure to toxic gases-forinstance, carbon monoxide poisoning;' mechanical failure ofan oxygen supply system, as in an airplane; and sudden mechanical airway obstruction,' as in aspiration ofa foreign body orstrangulation. Causes ofchronic asphyxia include prolonged exposure to high altitude and chronic pulmonary disease. Profound medial hypertrophy of small pulmonary arteries and of pulmonary veins from chronic exposure to hypoxia. Acute pulmonary edema may also occur in chronic hypoxia. Procedures depend on expected findings or grossly identified abnormalities as listed in right-hand column.

Malnutrition.

Empty and collapsed colon; small amounts of gray and dry meconium in terminal ileum; meconium masses in dilated and hypertrophied mid-ileum; liquid contents in proximal intestine (1); appendicitis; bowel perforation. Glandular inspissation and atrophy of intestinal mucosa. Volvulus, infarction, necrosis, perforation, peritonitis, and acquired intestinal atresia may be present. Absence of neural plexuses in congential megacolon* (Hirschsprung's disease).

Manifestations of cystic fibrosis, with or without cirrhosis;* biliary atresia* (3).

Immunodeficiency (See "Syndrome, acquired immunodeficiency" and "Syndrome, primary immunodeficiency.") Impression, Basilar NOTE: Basilar impression constitutes an upward bulging of the margins of the foramen magnum. When occipital condyles are displaced above the plane of the foramen magnum, basilar invagination is present.

Possible Associated Conditions: Klippel-Feil syndrome;* osteogenesis imperfecta;* osteomalacia;* Paget's disease of bone;* rheumatoid arthritis; rickets; syringobulbia; syringomylia. * Compression and secondary ischemic injury to lower brain stem and spinal cord. See also above under "Skull and spine" and under "Possible Associated Conditions."

Incompetence,��� (See "Insufficiency,..�")

Related Terms: Battered-child syndrome; child-abuse or child-neglect death. NOTE: Vitreous should not be aspirated until the skull has been opened and trauma ruled out, because there is a risk of artifactual damage to the retina. In the presence of craniocerebral trauma the pathologist can opt to examine the eyes by removal, fixation, and histologic study; or retinal photography.

Follow general procedures described under "Homicide" and, if necessary,. the procedures described under "Rape."

External examination See above under "Note." Prepare roentgenograms of entire body.

See above under "Note." In other situations, or after study of the fundus, submit sample of vitreous for chemical and possible toxicologic study. Umbilical cord attachment Prepare histologic sections of umbilicus. and end of umbilical cord Blood Submit sample for toxicologic study. Consider retaining dried blood on filter paper for possible DNA testing for paternity investigation.

If there is a possibility that the cadaver represents a stillbirth, see Part II, "Stillbirth".

If infant is thought to have died shortly after birth, determine the location of air in the intestinal tract; roentgenograms may be helpful.

Save stomach contents and record amount.

Other organs See also under "Homicide."

In the abused child, bruises, hematomas, burn marks, or other patterned injuries.

In any child, diaper rash, Mongolian spots, self-inflicted fingernail scratches, skin infections, and emaciation. In the previously battered child, fractures in various states of healing. In hypertonic dehydration,' sodium concentrations more than 150meq/L. This may be caused by organic disease, improper medical treatment, or physical neglect. If infant was born alive, inflammatory changes may be found, depending on survival interval.

For the hydrostatic lung test, see "Stillbirth." The test is unreliable. Extraneous material in air passages indicates that child was alive. Air reaches the stomach after 15 min, the small intestine after 1-2 h, the colon after 5-6h, and the rectum after l2h. There is no difference in the speed of gas propulsion between full-term and premature infants. Bacterial gas production and previous resuscitation attempts are potential sources of errors. In questionable stillbirth, presence of milk proves that infant was alive and had been nursed.

Traumatic lesions and signs of neglect may be present. Unilateral ulcers in visceral zoster.

In rare instances, diffuse meningoencephalitis may occur. Limited necrotic and inflammatory lesions in the cord or brain stem at the level of the affected ganglion are common. Posterior hom myelitis (3). Ganglion cell necrosis; lymphocytic infiltration, hemorrhage, and, later, fibrosis. As a rule, only one ganglion is severely involved, but less severe lesions may occur in the ganglia that are immediately adjacent. Diffuse lymphocytic infiltration; demyelination; axonal destruction; fibrosis. Conjunctivitis; keratitis; iridocyclitis; retrobulbar neuritis; neuroretinitis; occlusion of retinal vessels.

Evidence of hematologic malignancies (2) or other conditions, as listed above under "Note."

Infection, Middle Ear (See "Otitis media.") Infection, Pneumocystis carinii Synonym: Pneumocystosis. NOTE: (1) Collect all tissues that appear to be infected. (2) This organism cannot be cultured at present but is frequently associated with viral, bacterial, or fungal infections that can be diagnosed by culture. (3) For rapid staining of aspirates, use Gram-Weigert stain, which will stain cysts of Pneumocystis carinii and also fungi and bacteria. Pneumocystis carinii is best demonstrated with Grocott's methenamine silver stain. (4) No special precautions are indicated. (5) Usually, no serologic studies are available. (6) This is not a reportable disease. Injury, Fire (See "Burns.") Injury, Firearm NOTE: Protect all drains of autopsy table, which will prevent accidental loss of bullets or bullet fragments. Recovery of bullet fragments and pellets can also be improved by passing tissue fragments, blood, and other appropriate materials through a fine nonmetallic sieve. Caution must be exercised because sharp edges andjagged projections ofbullets and bullet fragments may cause injury (1). This applies particularly to the Black Talon bullet. Bullets and bullet fragments should not be touched with forceps or other metal instruments that may produce artifactual markings; they must be placed in properly labeled evidence containers, and the chain of custody must be preserved. From a birdshot wound, at least 10 pellets should be recovered. The firearms examiner will divide the total pellet weight by 10 and derive median pellet weight. From a buckshot wound, all pellets should be recovered. In many of these cases, procedures described under "Homicide" must also be followed.

Do not excise wounds before completion of autopsy (see below). As an academic exercise, excised firearm wounds may be used later for analysis of metal traces by neutron activation or for tests for carboxyhemoglobin. In practice, however, a high quality photograph is adequate to establish the presence or absence of gunpowder stippling or soot deposition toward the end of establishing the range of fire. The dimensions of the stippled areas and soot deposits should be recorded.

After completion of external examination, dry the wet clothing and keep as evidence. Clothing may also be used for possible test firing or for stain, powder, or particle analysis.

External examination

If identity of victim is unknown, follow procedures described in Chapter 13. Prepare initial roentgenograms of the body regions that have been shot, before disrobing of body, and then lateral films of body regions with bullets.

Follow up with films of other body regions as needed in the course of autopsy. If the body is decomposed to the extent that the external examination cannot be relied upon to determine cutaneous gunshot perforations, prepare roentgenograms of the entire body before autopsy. Record location and number of bullet holes in all layers of garments, indicating whether the involved area is bloodstained or shows gunpowder or soot.

If there are several cutaneous perforations, number or letter each consecutively and refer to these numbers in all records. Location of bullet holes should be described by recording distance from soles of feet or top of head, and from midline of body. Prepare diagrams and photograph holes, with and without labels. Photographs should show surrounding garments and extent of blood stains. In hairy areas, shave around bullet wounds for better photographic documentation.

For evaluation of distance from where a shot had been fired, see Fig. 11 Additional roentgenograms during the autopsy may be needed to find bullets embolized to the femoral veins or down the spinal canal. Systemic roentgenograms may reveal bullets from obscure entrance wounds, particularly in decomposed bodies, old bullets, bullets in the skin flaps reflected at autopsy, or bullets external to the body in clothing. Bullet(s) may have been arrested in hollow viscus or blood vessel and may have been transported to distant sites by peristalsis or bloodstream ("bullet embolus"). Bullets may be deflected in unexpected directions from bony surfaces. Soot ("smudging") and gunpowder stippling may occur around entrance wound, depending on the muzzle-to-skin distance. There may also be an impression from a recoiling handgun or from a power piston.

Wounds may be stellate, round, jagged, or slit-like, with or without wide margins of abrasion.

Primer residues on hand of suicide victim after use of a handgun. Fatal secondary injuries, particularly hemorrhages.

Alcohol intoxication is a frequent finding. Fig. 11·3 . Bullet wounds. Differences in the appearance of cutaneous wounds of entrance and of exit and differences in wounds of entrance according to the distance between muzzle and skin at the moment of fire. Entrance wounds often differ from exit wounds in that the former are usually surrounded by a narrow zone of abrasion. If the muzzle of a gun is in close contact with the skin at the moment of fire, the combustion products will be blown into the wound and will not be visible on the surface. For close-range wounds, the stippling of the skin around the wound, produced by particles of burned and unburned powder, becomes progressively more dispersed as the range of fire increases and is ordinarily not perceptible if the range has been greater than 24 inches ( 

Synonyms and Related Terms: Acute radiation syndrome; chronic (delayed) radiation injury; radiation enterocolitis; radiation nephritis; radiation pneumonitis. NOTE: Procedures and expected findings depend on type of radiation damage, whether acute or chronic, localized or whole-body irradiation. If suspected radiation injury was associated with the administration of radionuclides such as 32p, 131 1, or 198Au, follow procedures suggested in Chapter 11. In fatal 355 whole-body irradiation, findings are most likely related to bone marrow injury, and the suggested procedures and expected findings are those described under "Pancytopenia." Record extent of oropharyngeal and intestinal ulcerations and hemorrhages. Late complications include malignant tumors (carcinoma, leukemia,* lymphoma*), manifestations of hypothyroidism,* and cataracts. Organ changes in localized radiation injury depend on site of irradiation (lung, brain, kidney, intestine). The skin is involved in both acute (erythema) and chronic (atrophy, epilation) radiation injury.

Related Terms: Cutting injury; knife injury. NOTE: In many of these cases, procedures described under "Homicide" must also be followed. Insuffiency, Adrenal Synonyms and Related Terms: Adrenocortical insufficiency; polyglandular autoimmune syndrome (type I, usually in childhood, with parathyroid insufficiency and chronic mucocutaneous moniliasis; type II, usually in adults, with two or more autoimmune endocrine disorders such as thyroiditis and diabetes mellitus*); primary adrenal insufficiency (Addison's disease); secondary adrenal insufficiency (in hypothalamic-pituitary disease or steroid induced); X-linked adrenal hypoplasia.

is caused by anatomic changes (see below under "Adrenal glands"), drugs (enzyme inhibitors or cytotoxic drugs), or ACTH-blocking antibodies.

Possible Associated Conditions: AIDS with systemic infections (1); antiphospholipidantibody syndrome (2); autoimmune hepatitis; chronic lymphocytic thyroiditis; type I diabetes mellitus;* hypoparathyroidism;* hypothyroidism;* megaloblastic anemia;* surgical procedures, such as heart surgery or orthopedic procedures. Large cell lymphoma (5). Should be removed as soon as possible (before embalming), either from cervical midline incision or from chest (neck of cadaver must be well-extended). Photograph obstruction before larynx is opened, and inside of larynx and epiglottis after larynx is is opened in posterior midline. Make Gram-stained touch preparations.

Make histologic sections of larynx and epiglottis.

Hemophilus injluenzae (cannot always be isolated from larynx 

Synonyms and Related Terms: Acute or chronic lymphocytic leukemia, acute or chronic myelogenous leukemia, and hairy cell leukemia. Multiple subtypes have been identified; they are characterized by cell surface markers, chromosomal abnormalities, staining reactions, and morphologic features. NOTE: At the time of autopsy, most leukemias have been properly classified and often treated. In these cases, the goal of the autopsy is to document the extent of the disease and the presence of complications. If the leukemia had not been classified or if the features might have changed from the time of the last work-up (e.g., in suspected cases of superimposed diffuse large cell lymphoma [Richter's syndrome]), material should be snap-frozen and studied in more detail. If the patient was treated by bone marrow transplantation,* see also under that heading.

Possible Associated Conditions: Agnogenic myeloid metaplasia with myelofibrosis; Bloom's syndrome;* cutaneous mastocytosis; Down's syndrome;* immunodeficiency syndromes* (Bruton's disease; Louis-Bar syndrome; Wiskott-Aldrich syndrome); infantile genetic agranulocytosis; Klinefelter's syndrome;* lymphoma;* multiple myeloma;* polycythemia;* and many others. For sampling and specimen preparation, see Chapter 4.

Increased protein concentration.

Material in sediment stains red with toluidine blue. Metachromatic material. Arylsulfatase-A deficiency.

Excessive loss of myelin with large amounts of metachromatic material (see above under "Urine") in white matter and also in some neurons. Demyelination with metachromatic material (see above under "Urine").

Leukoencephalopathy, Progressive Multifocal Possible Associated Conditions: AIDS* and other immunosuppressed states; carcinoma; malignant myeloproliferative or lymphoproliferative disorder; sarcoidosis;* tuberculosis. *

Procedures Submit portion of fresh brain for viral culture. Fix remainder of brain and spinal cord in formalin and submit samples for histologic study. In situ hybridization for IC virus is available on paraffin-embedded tissue.

Small patches of demyelination with tendency to form confluent areas in the cerebral white matter. White matter necrosis may be present. Eosinophilic intranuclear inclusions occur in affected oligodendroglia cells. Subsequently, large, bizarre astrocytes develop. No inflammatory (lymphocyte) cell reaction is present.

Lightning (See "Injury, lightning.") Lipoproteinemia (See "Hyperlipoproteinemia.") Lipoproteinosis, Pulmonary Alveolar Synonym: Idiopathic alveolar lipoproteinosis. NOTE: The condition has been described in allografts (1); in such cases, see also under "Transplantation, lung." Elephantiasis of penis, scrotum, or vulva (in chronic cases); skin rash (l) and conjunctivitis (in acute cases); genital ulcers.

Suppurative or fibrosing inguinal, iliac, or pelvic lymphadenitis, with or without sinus tracts. Rectal strictures and fistulas in chronic cases (2) .

Systemic involvement in the acute stage with pericarditis,* and arthritis,* and meningitis,* proctitis (3) Lymphoma Synonyms and Related Terms: AIDS-related lymphoma; adult T-cellieukemiallymphoma; angioimmunoblastic lymphadenopathy; B-celllymphoma; Burkitt's lymphoma; cutaneous T-cell lymphoma (includes mycosis fungoides and Sezary syndrome); Hodgkin's disease; non-Hodgkin's lymphoma (low grade, intermediate grade, high grade, all with multiple subtypes too numerous to list, which vary in natural history); natural killer cell neoplasms; post-transplant lymphoproliferative disorders (PTLD); T-cell lymphoma.

NOTE: At the time of autopsy, most lymphomas have been properly classified and often treated. In these cases, the goal of the autopsy is to document the extent ofthe disease and the presence of complications. If the lymphoma had not been classified or if the features might have changed from the time of the last work-up, material should be snap-frozen and studied in more detail. If the patient was treated by bone marrow transplantation, * see also under that heading.

For current terminologies of Hodgkin's disease and non-Hodgkin lymphomas as well as for staging criteria, appropriate hematologic textbooks should be consulted.

Possible Associated Conditions: Acquired immunodeficiency diseases (e.g., after organ transplantation; human immunodeficiency virus-l infection); autoimmune disease (e.g., celiac sprue,*rheumatoid arthritis,* systemic lupus erythematosus,* or Sjogren's syndrome*); chemotherapy with or without radiation treatment; Epstein-Barr virus infection; human T-cell leukemia virus infection; inherited diseases with immunodeficiency (e.g., Klinefelter syndrome; * common variable immunodeficiency disease, Wiskott-Aldrich syndrome); radiation. The lesions are found most commonly in the urinary bladder and other parts of the urinary tract, but may also occur at many other sites, including skin (1) and upper respiratory tract (2). NOTE: (I) Collect all tissues that appear to be infected. (2) Usually, cultures are not indicated. (3) Request Giernsa or Wright stain. Tissue blocks should be rinsed of blood and be as thin as possible before fixation in refrigerated buffered and neutral 10% formalin solution. This is to avoid precipitationofformalin pigment that may be confused with malaria pigment. The formalin solution should be used in a ratio of 100 parts formalin to one part tissue and should be agitated every few hours. If one is dealing with tissues that contain formalin pigment, this pigment may be removed with a bleaching solution. (4) Usually, no special precautions are indicated. (5) Possible Associated Conditions: Histoplasmosis* and other chronic fungal infections; immune connective tissue diseases such as rheumatoid arthritis* (l) ; malignancies (l); sarcoidosis;* silicosis;* tuberculosis* (l) . NOTE Record location and extent of skin changes or skin defects on back. Prepare skeletal roentgenograms. If meningitis is suspected, submit sample of cerebrospinal fluid for culture. Dse the posterior approach to remove the spinal cord.

Atrophic skin over meningocele, lacking rete pegs and skin appendages; skin defect in complete rachischisis. Bony defects of spine.

In meningocele and spina bifida occulta, arachnoid and dura herniate through the vertebral defect. Spinal cord and roots are generally not involved. Lumbosacral mass in meningomyelocele, with a highly vascular mass (area medullovasculosa) in spina bifida aperta. Neural defects in complete rachischisis. Diastematomyelia, hydrocephalus (1) . Pyelonephritis;* enlarged urinary bladder ("neurogenic bladder").

External examination Heart Lungs Procedures Prepare chest roentgenogram. Procedures depend on expected findings or grossly identified abnormalities as listed in right-hand column.

Record weights. Prepare photographs and roentgenograms of fresh and fixed lung specimens. Perfuse one lung with formalin. For preparation of paper-mounted sections, see Chapter 2. Submit one lobe for microbiologic and chemical study. Decalcify tissue for histologic study.

Request van Gieson's, Hale's colloidal iron, PAS, and Sudan stains.

Miliary mottling in lower lung fields. Mitral stenosis* may be a cause of microlithiasis (mostly intra-alveolar ossification). * Cor pulmonale and heart failure (1) (l) , see also under that heading.

(1) Collect all tissues that appear to be infected. (2) Viral isolation, especially with EBV, is not diagnostically useful, due to long incubation periods. (3) NOTE: These diseases are characterized by a deficiency of a variety of hydrolases, resulting in accumulation of gly-cosaminoglycans (mucopolysaccharides) and glycolipids within lysosomes of fibroblasts, macrophages, white cells, and parenchymal cells of many organs (1, 2) . Mucopolysaccharides are also excreted in the urine. The general approach is similar in all types. Formalin may dissolve all of the stored material and leave empty vacuoles in the involved cells. Therefore, frozen sections should be utilized or tissues should be fixed in absolute alcohol. The accumulated material will show intense metachromasia if stained with toluidine blue. It will also stain with PAS, alcian blue, and colloidal iron. Oil red a will also stain the material from frozen sections. For specimen preparation for elec-tron microscopy, see Chapter IS.

Characteristic external features include dwarfism; thickened long bones; coarse facial features; coarse hair; macrocephaly; prognathism; hypertelorism; malformed teeth, and scaphocephalic skull with hyperostosis of sagittal suture; short neck; chest deformity; umbilical and inguinal hernias; lower thoracic and lumbar gibbus; genu valgum and coxa valga; pes planus and other joint deformities; wide hands (clawhands) and feet. Coarse thickened skin, covered with lanugo-like hair. Hyperlordosis; ovoid deformities of vertebrae; odontoid hypoplasia; large, shoe-shaped sella turcica; kyphosis; hypoplasia of femoral heads; osteoporosis.* If patient underwent bone marrow transplantation (3), see also under that heading. Chronic upper respiratory infections.

Large cytoplasmic granules in neutrophils.

Storage of mucopolysaccharides in osteocytes, chondrocytes, and fibroblasts of periosteum, tendons, fasciae, and other connective tissues; dysostosis multiplex.

Other tissues Histologic sampling cannot be excessive.

(1) Collect all tissues that appear to be infected. (1), see also under that heading.

Blood; other organs and tissues

Record extent of skin lesions and prepare photographs; prepare sections of affected and unaffected skin. Request Gram stain of sections and smears. Submit samples of blood and grossly affected organs or tissues for microbiologic study. Other procedures depend on expected findings or grossly identified abnormalities as listed in right-hand column.

Extensive epidermal necrosis. Shedding of granular and horny layers of epidermis.

Septicemia; staphylococcal infection of nose, throat, ears, eyes, heart valves, urogenital tract, and other sites. Bronchial epithelial detachment and bacterial pneumonia (2).

Synonyms and Related Terms: Acute kidney failure; *acute tubular necrosis; lower nephron nephrosis. NOTE: The morphologic diagnosis of this condition may be difficult to discern from autolysis. The features that suggest tubular necrosis are: tubular dilation with epithelial flattening, intertubular edema and necrotic epithelial cells in the collecting ducts. The autopsy should be performed as soon as possible. Needle specimens of the kidneys obtained in the immediate postmortem period may yield acceptable material. Ifnephrotoxic drugs or chemicals are thought to be responsible for tubular necrosis, submit samples for toxicologic study (see also under 

Synonyms and Related Terms: Multiple endocrine neoplasia (MEN), type 1 (parathyroid hyperplasia or adenoma; pancreatic islet cell hyperplasia, adenoma, or carcinoma; pituitary hyperplasia or adenoma); or type 2A (medullary thyroid carcinoma, parathyroid hyperplasia or adenoma; and pheochromocytoma); or type 2B (medullary thyroid carcinoma, pheochromocytoma, mucosal and gastrointestinal neuromas, and marfanoid features). NOTE: In MEN, type 1, foregut carcinoids and subcutaneous and visceral lipomas also may be found. In type 2A, cutaneous lichen amyloidosis may be observed. Mixed syndromes include (I) familial pheochromocytoma and islet cell tumor, (2) von Hippel-Lindau syndrome, pheochromocytoma and islet cell tumor, (3) neurofibromatosis* with features of MEN1 or 2, and myxomas, spotty skin pigmentation, and generalized endocrine overactivity (Carney complex).

In all instances, the autopsy should be done as soon as possible so that tissues for biochemical study can be frozen without delay. Nephritis NOTE: See under specific designation, such as "Glomerulonephritis" and "Pyelonephritis," or under name of suspected underlying condition, such as "Gout" or "Lupus erythematosus, systemic." Nephroblastoma (See "Tumor of the kidney(s).")

Synonyms and Related Terms: Renal stones; urolithiasis. Possible Associated Conditions: Carcinomatosis; Cushing's syndrome;* Cystinuria;* Fanconi syndrome;* hyperoxaluria;* hypervitaminosis D;* gout;* multiple myeloma;* osteoporosis;* polycystic renal disease;* primary hyperparathyroidism;* rheumatoid arthritis* (1); sarcoidosis* (2).

Kidneys Ureters, urinary bladder, and urethra Other organs

Remove kidneys, ureters, and urinary bladder en bloc. Excise and bivalve kidneys to reveal renal pelves. Open ureters. Record size, number, and appearance of stones, and save for chemical analysis (4).

Record heart weight. Dissect and record weights of all parathyroid glands. Other procedures depend on suspected underlying conditions, as listed above under "Possible Associated Conditions." Nephropathy NOTE: See under name of suspected underlying condition, such as "Diabetes mellitus," "Disorder, electrolyte(s)" (hypercalcemia, potassium depletion), "Gout," "Hypertension (arterial), all types or type unspecified," or "Poisoning,.. ." (heavy metal). If kidney failure was present, procedures under that heading should also be followed. Renal tissue may need decalcification or fixation in water-free solution. (2) NOTE: In this condition, lesions in the brain and cranial nerves, spinal cord, and spinal roots may be schwannomas (including bilateral vestibular schwannomas); multiple meningiomas; gliomas (generally of spinal cord), mostly ependymomas (75%) and pilocytic astrocytomas; or schwannosis of spinal dorsal root entry zones. Intracortical meningioangiomatosis; glial hamartia (intracortical, basal ganglia, thalamus, cerebellum, and dorsal horns of spinal cord) and cerebral calcifications also may be found. Cutaneous abscess (J).

Acute necrotizing nocardial pneumonia with abscesses or sinus formation into surrounding tissues; empyema.

Pulmonary and mediastinallymph nodes Other organs Submit samples for culture and histologic study. Submit samples from all organs and tissues with suspicious gross lesions for culture and histologic study.

Poorly encapsulated abscesses of any organ; endocarditis (2) .

Related Term: Total parenteral nutrition.

NOTE: Air embolism* or blood loss may have occurred if line became detached from the catheter hub. Metabolic complications such as fluid overload or disturbances of acid-base and electrolyte balance often cannot be diagnosed reliably at autopsy.

The disease(s) that may have necessitated parenteral nutrition therapy are not considered here; they include the acquired immunodeficiency syndrome (AIDS);* cancer cachexia; inflammatory bowel disease; liver or kidney failure;* severe pancreatitis;* short bowel syndrome, and others. Obstruction, Acute Airway Synonyms and Related Terms: Aspiration; bolus death; "cafe coronary"; croup; restaurant death. NOTE: Ifpermission has been obtained, remove neck organs through straight incision from chest to chin to avoid dislodging a foreign body. Ifdissection has to be accomplished from chest, neck of unembalmed cadaver should remain well extended during procedure. Inspect larynx and trachea from above and below, respectively, before opening them carefully along the posterior midline. For removal, prosthetic repair, and specimen preparation, see Chapter 2. Submit samples of osteocartilaginous and synovial tissues for histologic study; sagittal or frontal saw section through spine provides for best routine evaluation.

Heberden's nodes at interphalangeal joints of fingers. Degenerative changes, primarily of spine, hip joints, and knee joints. Histologic and macroscopic degeneration of cartilage; exposure of subchrondral bone; formation of marginal osteophytes; bone cysts; synovial fibrosis; hypertrophic synovitis.

o Procedures If artificial joints (e.g., hip or knee) had been implanted, record state of implant site.

Loose joint prostheses.

Synonyms: Hypertrophic pulmonary osteoarthropathy; pac-hydermoperiostosis (idiopathic hypertrophic osteoarthropathy [1J).

NOTE: In all instances, an underlying disease must be identified; they include cardiac disease (cyanotic congenital heart dis-ease with right-to-left shunt; infective endocarditis*); gastroeso-phageal reflux (3); neoplasms of lungs, esophagus, intestine, and liver; chronic liver disease; inflammatory bowel disease;* pulmonary disease (e.g., abscess;* bronchiectasis;* cystic fibrosis;* emp-yema;* emphysema;* lipoid pneumonia* (4); Pneumocystis pneumonia; sarcoidosis;* tuberculosis*); hyperthyroidism. *

External examination

Elastic arteries

Other organs

Prepare roentgenograms of extremities.

For removal, prosthetic repair, and specimen preparation, see Chapter 2.

Record presence of aneurysms (thoracic aorta, subclavian) or arteriovenous fistula of brachial vessels.

If abdominal aortic aneurysm had been surgically repaired, search for evidence of graft infection (2) . Procedures depend on expected findings or grossly identified abnormalities as listed above under "Note."

Clubbing of fingers or toes (see below under "Elastic arteries"); swelling of extremities.

Periosteal new bone formation in distal shafts of bones of forearms and legs. All bones of extremities may be involved. See above under "External examination."

Unilateral clubbing.

Clubbing of toes but not of fingers. 

Swelling; fistulas. Bone defect(s) and focal osteosclerosis. Bacterial or fungal infections, most common in metaphyseal region of bones; paravertebral or psoas abscess in osteomyelitis of spine. Bacterial or fungal osteomyelitis, with or without cavitation and fistulas.

Trauma, surgery, insertion of prosthesis, generalized (1) or contiguous infection, inflammatory bowel disease (2), diabetes mellitus,* and peripheral vascular diseases, deep vein thrombosis (3).

Synonyms and Related Terms: Aseptic necrosis of bone; avascular necrosis of bone; idiopathic osteonecrosis; Perthes' dis-ease; postfracture osteonecrosis; renal transplant associated osteonecrosis.

NOTE: Possible underlying conditions include chronic alcoholism,* decompression sickness,* diseases that have been treated with high doses of corticosteroids (1), Gaucher's disease,* human immunodeficiency virus infection* (2), tuberculosis* (1), sickle cell disease, * systemic lupus erythematosus,* tuberculosis* (2), and bisphosphonate therapy (3).

Other organs Submit sample for biochemical study. For removal, prosthetic repair, and specimen preparation, see Chapter 2. If osteonecrosis is suspected because one of the possible underlying conditions (see above) is present, prepare saw sections through femoral heads, medial femoral condyles, and heads of humeri. Procedures depend on suspected underlying conditions, as listed above under "Note."

Osteopetrosis Synonymsand Related Terms: Albers-SchOnberg disease; autosomal-dominantosteopetrosis; carbonic anhydrase-IT deficiency; malignant infantile osteopetrosis (1); marble bone disease.

NOTE: Manifestations of renal tubular acidosis may have been a clinical complication.

Possible Associated Conditions: Bone marrow transplantation* (for infantile osteopetrosis). Anemia, recurrent infections, bleeding, and bruises may have resulted from myelophthisis associated with osteopetrosis. Upper airway obstruction in malignant infantile osteopetrosis may have necessitated tracheostomy (1). In heritable osteoporotic disorders of connective tissue (osteogenesis imperfecta,* Marfan' s syndrome,* and others) are pre-sented separately. For"osteomalacia," see above. For "osteodystrophy," see under "Failure, kidney." Possible Associated Conditions: Acromegaly;* chronic alco-holism;* chronic obstructive pulmonary disease; chronic kidney failure* (1); Cushing's syndrome;* debilitating disease (various kinds, often with immobilization); diabetes mellitus* (2); epilepsy;* hyperthyroidism (2);* hypogonadism; malabsorption syndrome;* malnutrition;* primary biliary cirrhosis;*rheu-matoid arthritis;* scurvy;* steroid therapy or anticonvulsant medication. 

Normal parathyroid glands in uncomplicated cases. Hyperparathyroidism* (1) (without osteitis fibrosa) may be present, however. Features of osteitis fibrosa (osteoclastic osteoporosis; see "Hyperparathyroidism") or osteomalacia* exclude the diagnosis of uncomplicated osteoporosis. Osteoporosis, which may be localized, occurs in various neoplastic (e.g., mastocytosis) and inflammatory diseases.

External examination

Brain and base of skull with middle ears

Examine external ear canal.

For removal and specimen preparation, see Chapter 4. Culture any lesion appearing to be infectious.

Draining, foul-smelling greasy material associated with acquired cholesteatoma.

Bacterial or viral infection, with or without mastoid osteitis. Neck abscess, sinus thrombosis (1) and brain abscess* and meningitis* may complicate otitis media. Otosclerosis (1,3) NOTE: Otosclerosis may be a measles-virus-associated disease (1) and therefore, studies to rule out a past infection may be indicated.

For removal and specimen preparation, see Chapter 4. For current methods of temporal bone studies, see ref. (2) .

Spongy bone in the capsule of the labyrinth. Trabeculae of woven bone show pagetoid changes. If stapedectomy had been done, a prosthesis may be in place. 

Brain is externally normal or mildly atrophic. Characteristic is midbrain atrophy with aqueduct dilatation and depigmentation of the substantia nigra. Neuronal loss with reactive gliosis, associated with neurofibrillary tangles (globose tangles). Primarily affected are globus pallidus, subthalamic nucleus, red nucleus, substantia nigra, tectum, periaqueductal gray matter, and dentate nucleus (grumose degeneration).

Palsy, Pseudobulbar (See "Disease, motor neuron.")

Related Terms: Acute pancreatitis; alcoholic pancreatitis; chronic (or chronic fibrosing) pancreatitis; interstitial pancreatitis.

NOTE: Some causes of pancreatitis such as adverse drug reactions (e.g., due to azathioprine, furosemide, or estrogens) cannot be diagnosed at autopsy.

Possible Associated Conditions: Acute fatty liver of pregnancy; apolipoprotein ClI deficiency; cystic fibrosis; *hyperparathyroidism;* kidney transplantation. * Status post endoscopic retrograde cholangiopancreatography. 

Synonyms and Related Terms: Calcifying panniculitis; histiocytic cytophagic panniculitis; mesenteric panniculitis (mes-enteric lipodystrophy); "sclerema neonatorum." NOTE: The term Weber-Christian disease described systemic panniculitis with fever, bleeding, pulmonary and pancreatic lesions, and other abnormalities also involving lungs and pancreas, among others. However, this condition is not a specific entity and thus, the name has become obsolete. The term histiocytic cytophagic panniculitis is more descriptive and preferred.

Leukemia* and subcutaneous T-celllymphoma* may closely simulate panniculitis.

Possible Associated Conditions: Alphaj-antitrypsin deficiency;* dermatomyositis;* rheumatoid arthritis;* scleroderma and morphea; Sjogren's syndrome;* systemic lupus erythematosus.*

External examination, skin, subcutaneous tissue, and breasts Chest Heart Lungs Liver and spleen

Record extent and character of skin lesions. Record size, location, and gross appearance of subcutaneous nodules. Submit tissue samples for histologic study of grossly unaffected skin, skin lesions, and subcutaneous nodules. Request Verhoeff-van Gieson, Gram, and Grocott's methenamine silver stains.

Request S-l 00 protein stain. Ascertain that cell infiltrates are not leukemic or lymphomatous (1) . Dimpling of skin; necroses; fistulas. Panniculitis with subcutaneous nodules in trunk, breasts, and thighs (5) . Calcification may occur in breast tissue but also at other sites (e.g., in kidney failure*). Pancreatic enzymeinduced fat necroses associated with severe pancreatitis. Widespread hardening of fat tissue with rupture of fat cells and crystal formation in "sclerema neonatorum." Focal traumatic panniculitis also may occur in newborns. S-IOO stain negative in panniculitis but positive in Rosai-Dorfman disease (sinus histiocytosis with massive lymphadenopathy). Mediastinal panniculitis.

Pericarditis;* interstitial myocarditis. * Pneumonia;* pleuritis. Fat embolism. * Features of alphal-antitrypsin deficiency* (2) . Fatty changes of liver; splenitis.

Intestinal mucosal erosions and ulcers, with or without perforation. Massive gastrointestinal hemorrhage in histiocytic cytophagic panniculitis. Blind intestinal loop (3). 

For sampling and specimen preparation, see Chapter 4. Prepare samples for electron microscopy.

Vacuolar myopathy. (1) .

External examination, skin, and mouth

Record extent of skin lesions; photograph; submit multiple samples for histologic study, preferably of areas that are free of secondary infection.

Prepare sections for immunofluorescent staining.

Bullous and other lesions of scalp, eyelids, nose, axillae, umbilicus, inframammary areas, back, hands, groins, genitalia, anus, knees, and feet. Similar lesions may occur in the mouth. Acantholysis is suprabasal or near granular layer. IgG deposits on the surfaces of keratinocytes. NOTE: (I) Collect all tissues that appear to be infected. Collect inguinal, popliteal, axillary, and supraclavicular lymph nodes for aerobic culture. Photograph enlarged lymph nodes, and prepare smears of fresh cut surfaces. Submit consolidated areas for microbiologic study (see above under "Note"). Perfuse both lungs with formalin. Submit samples of pulmonary tissue and bronchial lymph nodes for histologic study. Submit samples of grossly abnormal organs, exudates, or drainage fluids for culture and Gram stain.

Hemorrhagic necrosis; variable suppuration.

Severe hemorrhagic edema; pneumonia with lobular to lobar features.

Large areas of necrosis teeming with organisms and minimal to suppurative inflammation.

Plasmacytoma (See "Myeloma, multiple.") Platybasia NOTE: Possible causes or associated conditions include Arnold-Chiari malformation;* basilar impression* orinvagination; fusion ofatlas to the foramen magnum; Klippel-Feil syndrome;* malpositioning of odontoid process; osteitis deformans (Paget's disease of bone*); osteogenesis imperfecta;* osteomalacia;* rickets; syringobulbia, syringomyelia. *

Skull and spine Prepare roentgenograms of skull and cervical spine.

Flattening of the base of the skull (angle formed bythe planeoftheclivus andtheplane of the anterior fossa exceeds 135°).

Pleuritis (See "Effusion(s) and exudate(s), pleural.") Pleurodynia, epidemic Synonyms: Bornholm disease; devil's grip; epidemic myalgia; epidemic myositis.

Blood Heart and pericardium

Submit samples for viral culture. Sample for histologic study and for viral cultures (Coxsackievirus A and B; echoviruses). If pericardia! fluid can be obtained, submit for viral culture also. Cultures may be negative and search for viral RNA by in situ hybridization may be indicated.

Chest organs

Other organs

Dissect thoracic duct and its tributaries (see Chapter 3). Perfuse one or both lungs with formalin.

Procedures depend on expected findings or grossly identified abnormalities as listed in right-hand column.

Submit samples of cystic lesions for histologic study.

Submit samples of cystic lesions for histologic study.

Gas in thoracic duct. Emphysema* of lungs.

Necrotizing enterocolitis in premature infants. Pyloric stenosis, colitis (5), redundant sigmoid colon, ischemic bowel disease. Mucosal pseudolipomatosis (6) . Usually, cysts are subserosal in adults and located between muscularis mucosae and muscularis propria in infants and children. Mucosa may be inflamed. Extraintestinal cysts, as listed above under "Abdomen." Cysts may also occur in vaginal mucosa.

include bright-field and polarized light microscopy, transmission electron microscopy, scanning or transmission electron microscopy with energy dispersive X-ray analysis (2), ion beam instrumentation, and secondary ion mass spectrometry. The last three methods are time-consuming, require much skill and expensive equipment, and do not lend themselves well to quantitation. Ideally, bulk analysis and in situ microanalysis should be used in combination. Analyses can be conducted in specialized laboratories at the following centers (for charges and other information, consult the appropriate laboratories):

Prepare chest roentgenogram. Submit sample for microbiologic study. Refrigerate sample for possible immunologic study.

Submit one lobe or samples of all lobes for bulk analysis and for in situ microanalysis (see above under "Note"). Microincineration may permit preliminary dust analysis. For demonstration of asbestos fibers, see Chapter 2 and ref. (4) . Submit one lobe or segment for microbiologic study. For pulmonary arteriography and bronchography, see Chapter 2. Prepare roentgenograms of entire lungs and slices. Perfuse one lung (or both, if only routine studies are intended) with formalin. Submit samples for histologic study of nodular dust lesions, diffuse fibrotic lesions, grossly uninvolved areas, bronchi, and bronchopulmonary lymph nodes. For preparation of paper-mounted sections, see Chapter 2. Prepare samples for transmission and scanning electron microscopic study. For removal, prosthetic repair, and specimen preparation, see Chapter 2. alveolar rupture with dissection of air into the mediastinum in neonates with respiratory distress (see "Syndrome, respiratory distress, of infant") and in adult patients ventilated on volume respirators.

External examination

Prepare chest roentgenogram.

Photograph mediastinum. Explore major veins and record compression in cases of tension pneumomediatinum.

Roentgenograms provide the best permanent record of a pneumomediastinum. Air bubbles in mediastinal soft tissues.

PART II / DISEASES AND CONDITIONS

Abdomen and neck organs Perfuse one lung with formalin. Other procedures depend on expected findings or grossly identified abnormalities as listed in right-hand column.

Search for evidence of trauma of other lesions that may have allowed air to enter soft tissues.

Intubation injury (J); perforation or rupture of major bronchus, trachea, or esophagus. Alveolar rupture, e.g., in asthma* (2), may not be discernible. Bronchiolitis obliterans (3), interstitial pneumonia* (4) and other lung conditions also may lead to pneumomediastinum. Dissection of air from lesions in abdomen (e.g., retroperitoneal colonic perforation [5] ) or in neck area.

Pneumonia, All Types or Type Unspecified NOTE: If the type of underlying infection is known, follow procedures suggested under the name of the infectious disease. If the etiologic agent of the pneumonia is unknown, proceed as follows: (l) Collect all tissues that appear to be infected. and Grocott's methenamine silver stains. (4) Special precautions may be required (see Chapter 6). (5) Serologic studies may be helpful once a specific etiologic agent is suspected. Thus, collect serum at the time of autopsy or procure serum that was collected prior to death. (6) This may be a reportable disease. Related Terms: Acute interstitial pneumonia (Hamman-Rich syndrome); desquamative interstitial pneumonia (DIP); idiopathic organizing pneumonia (or "bronchiolitis obliterans with patchy organizing pneumonia [BOOP]" or "cryptogenic organizing pneumonitis"); idiopathic pulmonary fibrosis; lymphoid interstitial pneumonitis (LIP); nonspecific interstitial pneumonia (NSIP) (or "cellular interstitial pneumonia" [eIP]); usual interstitial pneumonia (UIP); fibrosing alveolitis; pulmonary alveolitis; and many others.

NOTE: The conditions listed under "Related Terms" are histologic variants of idiopathic interstitial pneumonia. VIP is synonymous with idiopathic pulmonary fibrosis (IPF) (1). Diffuse alveolar damage is the histologic finding in patients with the adult respiratory distress syndrome* (ARDS) causing interstitial and intra-alveolar fibrosis. This is an acute condition, referred to as acute interstitial pneumonia when it occurs as an idiopathic form of rapidly progressive interstitial pneumonia.

Possible Associated Conditions: Acquired immunodeficiency syndrome* (AIDS) in patients with with LIP or nonspecific interstitial pneumonia (2,3); trauma and shock in ARDS. Secondary pulmonary fibrosis from inhalants (extrinsic allergic alveolitis or pneumonia; pneumoconiosis*), in drug-induced pneumonia, hypersensitivity pneumonitis (microgranulomatous hypersensitivity reaction of lung), rheumatoid arthritis,* Sjogren's syndrome,* and other collagen-vascular diseases; primary biliary cirrhosis in patients with nonspecific interstitial pneumonia (2).

External examination 

Postmortem chest roentgenograms provide the only reliable and permanent record of a pneumothorax and its main complication, mediastinal shift due to a tension pneumothorax (Fig.  11-4) . If roentgenograms cannot be prepared, a reasonably reliable diagnosis still can be made if the prosector inserts a needle through the lateral chest wall. The needle should be connected to a water-filled flask. Ifa pneumothorax is present, gas bubbles appear in the flask as shown in Fig. 11 -5. One can also expose the intact parietal pleura and observe if the lung tissue is separated from the parietal pleura by gas. Incising the thorax at the base of a water-filled skin pocket is the least reliable method.

Infants can be totally submerged under water before the chest cavity is incised. However, care must be taken not to make an accidental incision into the underlying lung tissue.

Poisoning, All Types or Type Unspecified NOTE: If a specific substance is suspected-for instance, arsenic or ethylene glycol-follow procedures described under the appropriate entry. Similar entries can be found for poisons whose general character or source is known-for instance, gas or mushroom poisoning. For some substances, the appropriate entry can be found under "Abuse,...," "Death,...," or "Dependence, ..." In all instances, routine sampling of toxicologic material should be done as described in Chapter 13. If no specific substances can be incriminated, the toxicologist must be provided with all available clinical information, as shown in Chapter 13. In most cases of fatal poisoning, the coroner or medical examiner must be notified.

Poisoning, Alkaloid NOTE: See under specific name of alkaloid-for instance, "Dependence, cocaine," "Poisoning, atropine," "Poisoning, digitalis," or "Poisoning, strychnine." Only a fraction of this large group of plant poisons has been listed. Whether the specific name of the alkaloid is known or unknown, complete toxicologic sampling is recommended. Poisoning, Antimony OTE: Toxicologic material should be submitted for anal ysis, as suggested under "Poisoning, arsenic." Iatrogenic antimony toxicity may occur after treatment with antimony compounds for conditions such as filariasis, fungal infections, and schistosomiasis.*

External examination and eyes Pharynx and gastrointestinal tract

Heart, liver, and kidneys

Record skin changes and eye abnormalities.

For toxicologic sampling of contents, see Chapter 13. Submit tissue samples for histologic study.

Request frozen sections for Sudan stain.

If exposure was from du t in smelting work, dermatitis and conjunctivitis may be present. Severe gastroenteritis in acute poisoning.

If victim drank antimony trichloride, ulcerative pharyngitis and gastritis may be present. Fatty changes of myocardium and hepatic and renal parenchyma.

Poisoning, Arsenic NOTE: Toxicologic material will be contaminated by bringing it in contact with fluids. Keratinized tissues take up arsenic from solutions. Put plastic bags over hands of victim.

If exhumed bodies are investigated for arsenic poisoning, include material from surrounding soil and coffin along with tissues submitted for chemical analysis.

Interpretation of toxicologic findings: After fatal poisoning, arsenic concentrations in the liver tend to exceed 0.5-1.0 mg/IOO g wet tissue. In acute poisoning, arsenic concentrations in hair may reach 3 !1g/g (1) and in nails 8 !1g/g. Organs Collect all contents, particularly in accidental poisoning in children.

Body dry and warm after death. Mydriasis.

Iatrogenic atrioventricular block (1) . Fruits of Atropa belladonna or seeds of Datura stramonium may be found. No characteristic findings.

Poisoning, Barbiturate(s) NOTE: This type of poisoning has become uncommon. Barbiturates may cause sudden death. In all instances, concomitant alcohol intoxication* must be ruled out. Standard toxicologic sampling is sufficient.

Blood Bile Urine Esophagus and stomach Liver and brain Procedures Submit samples of blood from portal vein and peripheral veins or heart for toxicologic study. Refrigerate for possible toxicologic study. Record total volume and pH value. Request tests for protein, glucose, and ketones; request drug screen. Submit all contents and record their character. Analyze for barbiturates and alcohol.

Submit samples for toxicologic study.

Evidence of alcohol intoxication.* Poisoning by other addictive drugs.

Gritty residues of unabsorbed tablets, powder, or capsules. Mucosal corrosion, ulceration, and discoloration from capsules may occur. Concentration of barbiturate in parenchyma important for interpretation.

Poisoning, Bismuth NOTE: Accidental poisoning is common (industrial exposure or drugs with soluble bismuth compounds). Search also for other heavy metals. Acute kidney failure* may be the cause of death.

External examination and oral cavity 

Stomatitis with bluish black discoloration of gums; loose teeth; sticky white membranous patches in mouth and throat. Jaundice.

Protein casts and tubular epithelial cells in sediment. Gray or black mucosal membranes; swelling of mucosa; intestinal ulcers that may be perforated. Hemosiderosis. Fatty changes; hemosiderosis.

Fatty changes; renal tubular degeneration with amorphic basophilic deposits in epithelium of convoluted tubules. Hemosiderosis.

See above under "External examination." For removal and specimen preparation, see Chapter 4. Peripheral neuritis.

Synonyms: Bromine poisoning; bromism. NOTE: The lethal dose is about 0.2 g in children and I g in adults (ingested). After fatal methyl bromide poisoning, headspace gas chromatography revealed a subclavian blood concentration of 3.0 microgram/mL whereas inorganic bromide concentrations were 530 micrograms/mL in the blood (1). Tissue concentrations were lower than those in the blood. Toxicologic samples should include liver and kidneys.

Chemical bums on face and conjunctivitis indicate direct exposure; skin pustules over body and nodose bromoderma of the legs indicate bromism. Blood is best suited for bromide determination.

After ingestion of bromide, necrosis with brown discoloration of mucosa of upper gastrointestinal tract may be present. After inhalation of bromide, swelling and inflammation ofmucous membranes in upper and lower respiratory tracts may be present. There may be pulmonary edema. Pneumonia occurs in bromism.

Organs to be analyzed for Cd should have no contact with water or be contaminated with blood; they should be sealed in polyethylene bags. Cd leaks into fixation fluid. Postmortem blood concentrations are very high and no indicator of the antemortem values (1).

External examination and oral cavity Blood Urine

Gastrointestinal tract

Other organs Procedures Submit sample for toxicologic study. Submit sample for determination of cadmium concentration. Submit sample for toxicologic study and a portion of one lobe for microbiologic study. Perfuse one lung with formalin. Fix bowel as soon as possible.

Collect renal tissue for light microscopic and electron microscopic study.

Sample for toxicologic study. Submit samples for histologic study also.

Poisoning, Carbon Monoxide NOTE: If the victim had been in a fire, see also under "Bums." Carbon monoxide poisoning may rarely be responsible for automobile accidents. Relatively low carboxyhemoglobin concentrations may contribute to death if there is concomitant poisoning-forinstance, with alcohol or drugs, particularly sedatives. Anemia, atherosclerotic heart disease, and chronic pulmonary disease also increase sensitivity to carbon monoxide.

Ifblood had been withdrawn at time of hospital admissionfor instance, for crossmatching-submit this for carboxyhemoglobin determination. If no blood can be obtained, see under "Heart, kidneys, and other organs." For quick-orienting qualitative tests, for quantitative methods of carbon monoxide determi-nation, and for interpretation of toxicologic findings, see below. Request also determination of hemoglobin concentrations and of blood alcohol. Request drug screen. For shipping of blood and tissues for carbon monoxide determination, see Chapter 15. It should be noted that losses of up to 60% of the original saturation occurred when blood was kept in uncapped container at room temperature for 2 Yz wk or at 4°C for 3 wk.

External examination Blood Heart, kidneys, and other organs Brain Record color of fingernails, particularly in heavily pigmented persons in whom lividity is difficult to discern. Record appearance of blood and submit sample of postmortem blood for toxicologic study. See also above under "Note."

If no blood can be obtained, prepare water extract of spleen, kidneys, or other organs. Request determination of carbon monoxide content and of carbon monoxide-binding capacity of this mixture. Submit tissue samples for histologic study.

For removal and specimen preparation, see Chapter 4.

Pink skin and fingernails; bullous edema of skin; decubital ulcers.

Blood tends to be cherry red. For interpretation of toxicologic findings, see below.

Necrosis of papillary muscles in the heart or myocardial infarction may occur. Renal tubular degeneration may also be found. Acute kidney failure* has been observed after rhabdomyolysis complicated by compartment syndrome (2) .

Hemorrhagic necrosis of basal ganglia (lenticular nucleus in globus pallidus); diffuse petechial hemorrhages in white matter; cerebral edema. Acute hydrocephalus in infants (3) .

Many methods of carbon monoxide determination have been described. Currently, carboxyhemoglobin is detected in most medical examiner toxicology laboratories by visible spectrophotometry or gas chromatography. In hospitals, carboxyhemoglobin is frequently detected and reported in the course of routine arterial blood gas analysis.

Pink discoloration of skin and organs usually indicates the presence of more than 30% carboxyhemoglobin (but rule out cyanide poisoning* and exposure to cold*).

In a healthy, middle-aged person, a carboxyhemoglobin concentration greater than 50-60% is usually fatal. If the victim was anemic or suffered from chronic lung disease, particularly emphysema* or atherosclerotic heart disease, the concentration may be lower. In association with alcohol, sedatives, and other drugs, carboxyhemoglobin levels may also be much lower and yet fatal.

A heavy cigarette smoker may have a carboxyhemoglobin concentration of 8-10%, and higher levels may occur in police officers and other persons exposed to automobile exhaust in dense traffic.

If the victim survived the carbon monoxide poisoning for several hours, postmortem blood samples usually will fail to show the presence of carboxyhemoglobin. In these instances, blood taken at the time of admission to the hospital may still be available and of particular value. If the victim had spent 1 h in fresh air before death, 40-50% of the carbon monoxide will have been removed, and 8-10% will have been removed during each subsequent hour. Even though clearance may be complete, death may still occur-primarily from brain damage and infectious complications in prolonged coma. or delayed by only a few hours, particularly after inhalation of carbon tetrachloride. Sudden death probably is caused by cardiac dysrrhythmia.

Alcohol concentrations should be determined in all cases or, if death was delayed, evidence of drinking at the time of exposure should be sought. Alcohol considerably increases the hazards of carbon tetrachloride. NOTE: See also under "Bronchitis, acute chemical" and under "Poisoning, gas." Hydrochloric acid is sold by plumbing supply houses and pool supply companies as muriatic acid. It is a liquid. Chlorine is a water-soluble gas. As supplied for pool sanitation, liquid chlorine is usually acidic. For convenience, chlorine and hydrochloric acid are discussed here together.

Prepare photographs of face.

Conjunctivitis and cyanosis in chlorine gas poisoning; burns of lips from hydrochloric acid. See also above under "Larynx and trachea." Photograph opened esophagus and stomach.

Sample for histologic study, particularly if there is doubt whether a perforation was antemortem or postmortem.

For removal and specimen preparation, see Chapter 4.

Severe pulmonary edema, bronchopneumonia, and swelling of mucous membranes in chlorine poisoning. Arterial thrombosis may occur. Pulmonary fibrosis may develop after prolonged survival. Swelling and ulceration of mucous membranes in chlorine poisoning; acute laryngotracheitis. Tracheoesophageal perforation.

Corrosion of mucosa with thickening, hemorrhage, and blackish discoloration after ingestion of hydrochloric acid. Antemortem and postmortem perforation may occur.

Glomerular capillary thromboses. Hemorrhages in white matter (1). Poisoning, Cyanide Synonym: Hydrocyanic acid (hydrogen cyanide) poisoning. NOTE: Hydrocyanic acid (hydrogen cyanide, HCN) is a water-soluble gas. Its salts, sodium cyanide and potassium cyanide are sold as "eggs" to the jewelry industry. Hydrocyanic acid is formed when cyanide salts are dissolved in acidic solutions. Containers from which the poison might have been ingested or inhaled should also be submitted for toxicologic examination. For cyanide screening tests in the autopsy room and for interpretation offindings, see below. Caution: Stomach may still contain cyanide gas, formed by acidic reaction of cyanide salt. It may be best to open the stomach under a hood (1). The odor is quite characteristic for cyanide poisoning but most persons are unable to smell this odor. It is helpful to know in advance if any person in an office or laboratory can smell cyanide. Forensic pathologists who can smell the compound state that it has its own specific odor, which differs from the often quoted smell of bitter almonds. Autopsies also can be done in a negatively pressured isolation room (1). For odor, see above under "Note." Bright red skin color is not always present. Corrosion around mouth and in oral cavity may be found after ingestion of potassium or sodium cyanide. Blood is fluid and sometimes bright red.

If potassium or sodium cyanide was ingested, brown-red mucosal corrosion may be present in stomach or in upper digestive tract.

Pulmonary edema.

For odor, see above under "Note." If death was not instantaneous, there may be hyaline thrombi in small blood vessels, minute hemorrhages, and necroses of lenticular nuclei.

This test can be used for blood and gastric contents (2) . Dip squares of filter paper in a small amount ofsaturated picric acid. Let these squares dry until barely moist. Place a drop ofthe material to be tested-e.g., blood or gastric contents--on a piece of paper. Let material dry for a moment, and then place one drop of 10% sodium carbonate in the center of the material to be tested. Ifcyanide is present, a reddish purple color will chromatograph out from the material. The higher the concentration of cyanide the more blue the color will be. It is possible to recognize whole blood because the blood turns a rather dark brown and the reddish to purple color is clearly visible. High concentrations of sulfide interfere by giving a false-positive test.

Another screening test is done as follows (3) . Dip filter paper into normal blood. Then treat the paper with potassium chlorate, whereupon brown methemoglobin forms. Place this preparation into the fluid suspected of containing cyanide (e.g., blood, gastric contents, pulmonary edema fluid). If bright red cyanmethemoglobin forms, the reaction is positive.

If the concentration of cyanide in the stomach is high and the concentration in the lungs is low, cyanide was ingested. Alternatively, if the pulmonary cyanide concentration is high and the concentrtaion in the gastric contents is low, hydrogen cyanide most likely was inhaled. Occasionally, minimal cyanide levels will be present in decomposed bodies.

Related Term: Digoxin toxicity. NOTE: Certain drugs may interfere with correct digitalis determination.

Blood Heart Vitreous Procedures Submit sample of peripheral blood for digoxin radioimmunoassay. Freeze fresh myocardium for digitalis extraction. Submit sample for digoxin radioimmunoassay.

Poisoning, Drug(s) (See "Dependence, drug(s), all types or type unspecified" or under "Poisoning,���" followed by specific name of drug.)

Poisoning, Ethanol (Ethyl Alcohol) (See "Alcoholism and alcohol intoxication," "Cardiomyopathy, alcoholic," "Disease, alcoholic liver," "Syndrome, fetal alcoholic," and Syndrome, Wernicke-Korsakorr.")

Poisoning, Ethylene Glycol Related Term: Antifreeze poisoning. NOTE: Pulmonary and cerebral manifestations are the main findings in acute poisoning, and renal tubular necrosis is the primary finding in chronic poisoning. For general toxicologic sampling, see Chapter 13. Calcium oxalate crystals can be demonstrated in routine histologic sections but also in scanning electron micrographs of thick deparaffinized sections.

Eyes Blood Urine For removal and specimen preparation, see Chapter 5. In acute cases, submit sample for ethylene glycol determination; in chronic cases, request determination of calcium concentrations. Prepare sediment.

Papilledema; optic nerve atrophy.

Ethylene glycol in serum (I) .

Protein casts; calcium oxalate crystals (2) . Crystals are light yellow and birefringent, arranged as sheaves, rhomboids, or prisms. Poisoning, Food Related Terms: Bacillary dysentery* (Shigella food poisoning); botulism;* Clostridium perfringens food poisoning; favism; mushroom poisoning;* Salmonella food poisoning; staphylococcal food poisoning.

NOTE: If cause of food poisoning is unknown, submit suspected food for aerobic and anaerobic cultures, Gram stain of smears, and routine toxicologic study. This should include tests for heavy metals (antimony, cadmium, and lead) that may have leaked from old cooking utensils. Test for the presence of staphylococcal enterotoxin are done only in specialized laboratories. If botulism is suspected, follow procedures described under that heading. Mushroom poisoning also is listed as a separate entity. If Salmonella food poisoning is suspected, see under "Fever, typhoid." See also under "Enteritis" or "Enterocolitis" or under another specific heading such as "Dysentery, bacillary." Obtain sufficient material for microbiologic and histologic study to identify organisms such as Chlamydia, Clostridium (type F strains), Salmonella, Shigella, verotoxic E. coli, Yersinia, and others.

External examination Gastrointestinal tract Submit contents for aerobic and anaerobic cultures (see above under "Note"); prepare smears of contents for Gram stain. Submit samples for histologic study.

Debilitated states; patients in extremes of life. Enteritis or enterocolitis.

Poisoning, Gas NOTE: Anesthesia-associated death, * carbon monoxide poisoning,* and sniffing and spray death* are presented under the appropriate headings. Procedures discussed here deal with other volatile substances, including chemical irritants such as ammonia (NH 3 ), chlorine or hydrochloric acid poisoning (see also under that heading); methylene chloride, phosgene (COCI 2 ), sulfurous acid (H 2 S0 3 ), or sulfur dioxide (S02) ' Gases from body cavities, heart chambers, or blood vessels can be removed as described under "Embolism, air." Gases can also be trapped with a rubber dam after cutting organs under water. Samples from various organs should be shipped in hermetically sealed nonplastic containers or in analyzing solutions.

External examination, and oral cavity Blood Larynx and trachea Record extent of chemical bums.

Submit sample for gas analysis. In many instances, inhaled gases can be demonstrated chromatographically in gas from head space above sealed blood specimen.

Chemical bums in and around mouth or conjunctivas.

Chemical bums.

Other organs

If gas was inhaled and is to be analyzed, submit intact lungs with bronchi ligated in airtight, nonplastic container to laboratory that can conduct gas analysis. If survival was short, formalin perfusion of lungs is not recommended; it may cause artifactual ballooning and internal ruptures of organ. Submit samples of tissue for routine histologic study. See above under "Note."

Chemical pneumonia; pulmonary edema. After longer survival, obliterating fibrous bronchiolitis, chronic bronchitis, and saccular bronchiectasis* may occur.

Poisoning, Glycol (See "Poisoning, ethylene glycol.") Poisoning, Halogen (See "Fluorosis," "Poisoning, bromide," "Poisoning, chlorine or hydrochloric acid," "Poisoning, gas?, and "Poisoning, iodine!')

Poisoning, Heavy Metal (See "Poisoning, antimony," "Poisoning, arsenic," "Poisoning, cadmium," "Poisoning, lead," Poisoning, mercury," "Poisoning, thallium!')

Poisoning, Insecticide (See "Poisoning, organophosphate(s)*)

Poisoning, Iodine Related Terms: Lugol's solution; tincture of iodine. For toxicologic sampling, see Chapter 13.

External examination and eyes

Urine, blood, and parenchymal organs Lungs and upper respiratory tract Stomach

Record color of skin and extent of corrosive lesions.

Submit samples for toxicologic study.

Submit samples for histologic study.

Submit contents for toxicologic study; photograph mucosa; prepare histologic sections.

See above under "Stomach."

Perioral corrosive lesions; yellow discoloration of skin; conjunctivitis after exposure to vapors.

Acute inflammation of respiratory tract after inhalation of vapors. Corrosive gastritis; if starch was used as antidote, gastric lining will be bluish. Histologically, well-preserved mucosa is present because of in vivo fixation. Mucosa may show same changes as stomach. Swelling of tubular epithelium.

Synonyms: Propanol; rubbing alcohol.

Procedures See under "Alcoholism and alcohol intoxication."

Nonspecific autopsy findings: visceral congestion; pulmonary and cerebral edema.

Poisoning, Lead NOTE: Lead-free syringes and lead-free polyethylene containers should be used. Blood lead concentrations can be determined by inductively coupled plasma mass spectrometry (1). For screening methods, see ref. (2) . This is a reportable disease in some states.

External examination, oral cavity, and hair

Bluish lead line at gingival margin in victims with poor oral hygiene.

External examination, oral cavity, and hair For diagnosis of chronic plumbism, analysis of scalp hair may be useful. Analysis is done by neutron activation (see also under "Poisoning, arsenic").

Remove samples with lead-free syringe (see above under "Note") or 20-mL Vacutainer tubes (Becton, Dickinson and Company). Do not add anti-coagulant or preservative. For colIection procedures, see above under "Blood" and under "Note." Request also determination of coproporphyrin concentrations.

Submit sample for histologic study; submit remaining tissue for toxicologic analysis. Submit with contents for toxicologic study, particularly in acute poisoning. Submit sample from each kidney for histologic study; submit remaining tissue of both kidneys separately for toxicologic study. Submit at least 10 g of fresh bone for toxicologic study.

For removal and specimen preparation, see Chapter 4.

Poisoning, LSD (d-Lysergic Acid Diethylamide) (See "Abuse, hallucinogen(s).")

Poisoning, Lye Related Terms: Ammonium hydroxide poisoning; calcium oxide or quicklime poisoning; poisoning by alkaline corrosives; potassium hydroxide poisoning; sodium hydroxide poisoning.

External examination and oral cavity Blood Neck organs, esophagus, trachea, and lungs

Record extent of oral, perioral, and other facial corrosive injuries. Photograph lesions. Prepare histologic sections of tissue from inside of lips or mouth. Submit sample for toxicologic study. After removal of heart, remove neck organs with hypopharynx, esophagus, larynx, and trachea.

Leave stomach attached to esophagus. Open pharynx and esophagus along posterior midline. In acute cases, formalin perfusion of lungs is not recommended; it may cause artifactual balIooning and internal ruptures of organ.

Lye bums on face and chest in acute cases; scars and manifestations of malnutrition* in chronic cases.

SwelIing, edema, and necrosis of mucous membranes in acute poisoning. Fibrosis and strictures in chronic cases. Bronchitis* and bronchopneumonia. Submit specimen from pharynx for histologic study.

For removal and specimen preparation, see Chapter 4. Submit sample of brain for toxicologic study.

Exfoliative dermatitis.

Blue line at gingival margin; hypertrophy of gum; acute and chronic gingivitis; exfoliation and loss of teeth (2) .

Degeneration of myocardium.

Increased concentrations of mercury (1) .

Congestion.

Erosive gastritis and colitis.

Increased concentrations of mercury (1) . Degeneration of proximal tubules; calcifications. Chronic kidney failure* may be the cause of death. Induration of mucosa.

Increased concentrations of mercury (1) . Cortical hemorrhages. Cholinesterase activity will be low.

Pulmonary edema if poison was inhaled. Airways may contain aspirated material.

Cholinesterase activity can be determined reliably even after decomposition and embalming. Clinically, Guillain-Barre syndrome has been observed after poisoning with organophosphate.

In acute poisoning, the cholinesterase levels may be 25% of the normal values (see below). The cholinesterase levels in the blood are not affected by the duration of the postmortem interval; measurement may be attempted even on decomposed or exhumed bodies. Severe fatty changes; periportal necroses.

Fatty changes in myocardium, skeletal muscles, and other organs. For toxicologic sampling (gastric contents, urine, blood, brain, and other organs), see Chapter 13.

Rigor mortis after fatal strychnine poisoning may occur very soon after death and may be very severe (opisthotonos); it may persist until decomposition sets in. Congestion of viscera; no characteristic morphologic autopsy findings. Acute pancreatitis has been observed (1). High strychnine concentrations (also demonstrable in exhumed bodies [2] ).

Poisoning, Thallium NOTE: For toxicologic sampling, see below and Chapter 13. Thallium is used as a rodenticide and pesticide, and has some industrial applications. Retrobulbar neuritis. Chapter 4 and 5. Submit brain for toxicologic study. Submit sections of brain and optic nerves for histologic study. Submit specimens for toxicologic study (take from lower extremity). For removal, prosthetic repair, and specimen Osteomalacia;* osteomyelofibrosis. preparation of bones, see Chapter 2. For preparation of sections and smears of bone marrow, see Chapter 2. Submit samples for toxicologic study.

Synonym: Acute anterior poliomyelitis. NOTE: The disease has been nearly eliminated in the USA but not in many other countries.

(1) Collect all tissues that appear to be infected. ( For removal and specimen preparation, see Chapter 4. In acute cases, submit portions of brain and spinal cord for viral culture.

Neurogenic atrophy of skeletal muscles in areas of paralysis.

Electrolyte disorder. * Hypertensive heart disease; myocarditis.* Aspiration or bronchopneumonia (or both); edema; atelectasis; embolism;* alveolar wall necrosis (acute or organizing diffuse alveolar damage) after oxygen toxicity. Acute ulcers. Acute gastric dilatation; acute gastroduodenal ulcers; gastrointestinal erosions and hemorrhages. Dilatation of colon; perforation of cecum. Urolithiasis and nephrolithiasis,* pyonephrosis and pyelonephritis. * Phlebothrombosis of legs, most commonly on left side. Necrosis of anterior hom cells of spinal cord, with neuronophagia and perivascular inflammatory reaction. Old lesions show neuronal loss and gliosis. Medulla ("bulbar polio") and other areas of brain stem, cerebellum, and cerebrum, particularly the motor cortex, may be affected in various degrees.

PART II / DISEASES AND CONDITIONS Pregnancy NOTE: In some instances, procedures described under "Death, abortion-associated," "Embolism, amniotic fluid," or "Toxemia of pregnancy" may be indicated.

Synonym: Werner syndrome.

External examination and skin 

Abdomen Procedures Record volume of intraperitoneal fluid; prepare smears; submit samples of peritoneum for histologic study.

Colloid carcinoma of stomach or colon. Well-differentated adenocarcinoma of ovary or, rarely, of appendix; ruptured appendiceal mucinous adenoma is the most common cause of peritoneal adenomucinosis.

Pseudotumor Cerebri Synonym: Benign intracranial hypertension; meningeal hydrops.

NOTE: This condition, which generally affects young, obese females, is characterized by symptoms and signs of increased intracranial pressure without a demonstrable cause. Hence, intra-cranial mass lesions, infections, and related conditions should be excluded. Such conditions include adrenal insufficiency;* Guillain-Barre syndrome* (increased colloid-osmotic pressure); hyperadrenalism; hypervitaminosis A* (e.g., after treatment of acne); hypoparathyroidism;* hypothroidism;* infectious mono-nucleosis;* Lyme disease; pregnancy; Sydenham's chorea; throm-bus ofthe lateral or superior sagittal sinus (otitic hydrocephalus); and Wiskott-Aldrich syndrome. Submit samples of all accessible organs and tissues, with or without gross lesions. Submit material for electron microscopy.

For removal and specimen preparation, see Chapter 5.

For removal, prosthetic repair, and specimen preparation, see Chapter 2.

Heart Small bowel Liver

Procedures depend on expected findings or grossly identified abnormalities as listed in right-hand column.

Fix the bowel as soon as possible. Record weight. Submit samples for histologic study.

For removal, prosthetic repair, and specimen preparation, see Chapter 2.

Aortitis involving ascending aorta and aortic valve with regurgitation; mitral valve and adjacent myocardium and conduction system also may be involved. Sprue-like changes with loss of villi. Fibrosis or cirrhosis and other abnormalities, particularly in patients who had been taking methotrexate. Psoriatic arthritis and spondylitis. 

Synonyms and Related Terms: Allergic purpura; anaphylactoid purpura; hypersensitivity vasculitis.

External examination and skin Rabies NOTE: (I) In most instances, an autopsy limited to the brain is sufficient to confirm the diagnosis. (2) Rabies virus is especially infectious and thus, universal precautions should be strictly followed (see Chapter 6) . Avoid the use of scalpels whenever possible. The generation ofaerosols should be assidu- See above under "Note." If a complete autopsy is done, sample heart, lungs, kidneys, pancreas, submaxillary salivary glands, and adrenal glands; freeze or refrigerate sampled tissues and submit for viral study (see below under "Brain and spinal cord").

Obtain serum for serologic studies. Freeze or refrigerate cerebral tissue immediately after removal and submit frozen to special laboratory for fluorescent antibody staining. Take these sections from hippocampus or brain stem. Place the refrigerated tissues in 50% neutral glycerol saline solution for preservation. Fix remaining brain and spinal cord tissue in 15% formalin and submit for histologic study. As an alternative to freezing of tissue, immunoperoxidase methods for detection of rabies viral antigens can now be applied to formalin-fixed tissue (1 In almost all instances, the procedures described under "Assault" and under "Homicide" must also be followed.

See also refs. (1) and (2) . For the evaluation of bite marks, photographs with and without scales and black and white film should be used. A forensic dentist is required to compare the victim's bite marks to the teeth of suspects. Swabs of possible sperm or other fluids must be sent to the crime lab for proper evaluation.

External examination

Other organs and tissues Genital organs

Comb pubic hair over a towel and then pull sample; also, pull samples of hair from head. Collect fingernail clippings and place in containers marked "right" and "left." Collect blood, hair, fibers, and residues of urine or saliva and/or semen that may have stained the victim's clothing or that may be found on the skin of the victim. For study of stains and scrapings, see below.

Introduce sterile dry cotton swab into the posterior vault of the vagina or-preferablyinto the cervical canal. Prepare smear on glass slide and send to crime laboratory for identification of sperm (see also above under "Note" Using a sterile cotton swab moistened with sterile saline, lift suspected dried spermatic fluid from hair or skin of external genitalia, perineum, buttocks, or other sites. Stains on clothing will be extracted by forensic laboratory personnel. Let specimens air dry in absence of sunlight and then place in paper bags or envelopes and seal. Smears are made for the demonstration of spermatozoa, cytologic changes, bacteria, and other possible findings (Some crime labs prefer to make their own smears from the swabs). Other samples are used for the determination of acid phosphatase, as described below.

Usually, the survival time of morphologically recognizable spermatozoa in the vagina is less than 24 h, but on occasion this may be much longer. Thus, within the first 24 h after coitus, 64% of cervical smears have been found to be positive for spermatozoa. At day 10, spermatozoa have been found in 13% of the smears. Obviously, if the assailant had had a vasectomy, spermatozoa will not be found at any time.

Spermatozoa have been found in the vagina of some women several days or weeks after death. Dried stains (see above) may give positive results after longer intervals. Acid phosphatase activities greater than 5 Bodansky units (for method, see above) indicate that probably less than 12 h have elapsed since the time of intercourse (I) . In another study, vaginal swab specimens showed acid phosphatase activities of more than 2,000 King If there is evidence of parotid involvement, other salivary glands should also be studied. Parotid gland can be biopsied from scalp incision. Submaxillary gland can be removed with floor of mouth. For sampling and specimen preparation, see Chapter 4. For removal, prosthetic repair, and specimen preparation, see Chapter 2.

Chronic meningitis; space-occupying lesions (submeningeal nodular granulomas). Granulomatosis.

Iridocyclitis; chorioretinitis; papilledema; posterior uveitis; keratoconjunctivitis sicca; conjunctival follicles; cataracts. Involvement of lacrimal glands and other orbital tissues (2) . Sarcoidosis of parotid gland is common.

Sarcoid neuropathy and sarcoid myopathy.

Cystic changes, primarily of small bones of hands and feet. Sarcoidosis of joints (3).

Synonyms and Related Terms: Bilharziasis; Schistosoma haematobium infection; Schistosoma japonicum infection; Schis-tosoma mansoni infection. NOTE: Unless specifically stated, the changes listed below refer to chronic schistosomiasis mansoni and japonica.

(1) Collect all tissues that appear to be infected. (2) Request direct examination for Schistosoma. The following procedures have been described and recommended (1). For demonstration of Schistosoma eggs, compress 4-mm tissue fragments-for instance, mucosa of the urinary bladder-between glass slides. If this gives negative results, digest a 5-g portion of tissue in potassium hydroxide.

For the recovery of adult worms of Schistosoma mansoni and Schistosoma haematobium, remove the viscera en bloc and rinse with water. Separate the intestines from the mesentery. Subsequently, perfuse the portal vein system, the liver, and one lung with saline (2) . Then pass the perfusion fluid through a monofilament nylon cloth with an aperture size of 180 11m. Submerge the cloth in water and examine with a dissecting microscope. Fix worms in formalin solution. Examine the intestinal mucosa directly.

From the urinary bladder, ureters, and surrounding connective tissue, worms can be recovered as follows. Inject water into the tissue until the tissue increases 2 or 3 times in thickness. Then compress the tissue gently with a glass plate. Cut slices 0.1-0.2 cm in thickness. Compress these slices between the glass plate and the stage of a dissecting microscope and examine for the presence ofadult worms. Many worms also will be present in the fluid expressed from the tissue as it is cut. For the counting of eggs in tissues, urine, and feces, see ref. (1) . Immunodiagnostic methods (ELISA and imrnunoblot) also have been developed Prepare skeletal roentgenograms. There are no diagnostic laboratory tests but it still is advisable to save a blood sample.

Record heart weight. Test competence of valves (Chapter 3). Open heart in line of blood flow. Photograph and measure valvular lesions. Prepare sections of valves, myocardium, conduction system (if there was a history of heart block), and ascending aorta. Perfuse at least one lung with formalin. Submit any consolidated area for microbiologic study.

Fix intestines as soon as possible. abnormalities as listed in right-hand column. Sample for histologic study and request amyloid stains or renal parenchyma. Prepare roentgenograms of spine, sacroiliac joints, symphysis ossium pubis, and manubriosternal, sternoclavicular, and humeroscapular joints.

If spine cannot be removed in its entirety, it can be split in midline and one half can be removed, with costovertebral and costotransversal joints. Hip joints should be exposed.

Histologic sections should include synovia and periarticular tissue. Secondary and peripheral osteoarthritis* may be present. Leukemia* developed in some patients who had had radiation treatment (1950 or earlier). Acute anterior uveitis.

(2) Request fungal culture. (3) Request Grocott's methenamine silver stain. (4) Record weight of all organs (for expected weights, see Part III). Submit samples of all major organs, including endocrine glands, lymphatic and fat tissue, bone, and bone marrow for histologic study.

Hunger edema; skin changes secondary to vitamin deficiencies. Hypoproteinemia. Atrophy; hemosiderosis of spleen. Degree of atrophy varies from organ to organ; atrophy of fat tissue, lymphoid tissue, and gonads usually is most pronounced. Severe infections, such as tuberculosis,* may be present without having been apparent clinically.

Steatohepatitis, Nonalcoholic (NASH) NOTE: The morphologic findings in the liver are indistinguishable from those in alcoholic liver disease* (1). Follow autopsy procedures described under "Disease, alcoholic liver." If the patient had received a liver transplant, procedures described under "Transplantation, liver" should be followed also.

Possible Associated Conditions: Celiac sprue (rare); diabetes mellitus* (type 2); hyperlipidemia; malnutrition* from bulemia (rare); morbid obesity with liver failure particularly after episodes of rapid weight loss (e.g., after recent gastroplasty); lipodystrophy; mild obesity;* short bowel syndrome (rare); total parenteral nutrition. * Stenosis, Congenital Valvular Pulmonary Related Terms: Isolated (pure, simple, or dome-shaped) pulmonary stenosis. NOTE: The pulmonary valve is usually acommissural in isolated congenital pulmonary stenosis. With other coexistant congenital heart disease (such as tetralogy), the pulmonary valve is usually bicuspid and hypoplastic, but may be unicommissural or dys-plastic and tricuspid.

Heart and great vessels; peripheral pulmonary arteries Brain Procedures Culture any grossly infected valve. For general dissection techniques, see Chapter 3.

Record weight of heart, thickness of ventricles, and annular circumferences.

For removal and specimen preparation, see Chapter 4.

Infective endocarditis* of pulmonary valve and tricuspid valve; infective endarteritis at bifurcation of pulmonary trunk. It is preferable to have autopsies of fetuses and stillborns performed by pathologists experienced in perinatal pathology. If such personnel are not immediately available, the attending pathologist may nevertheless collect important information. If attempts to induce abortion appear to have caused the death of the mother, see "Death, abortion-associated."

The placenta should be immediately procured from the delivery room should it not arrive in the laboratory with the fetus. This will avoid the possibility of the placenta being discarded by the delivery room staff. No fetal autopsy is complete without a careful examination of the placenta (see Part I, Chapter 2). Pathologic changes of placenta that are causative of stillbirth are summarized in Ref. 1 .

Fascia lata or other aseptically obtained tissue should be collected for tissue culture for karyotype analysis. If the fetus is at all autolyzed, then the fascia lata cells may not grow in tissue culture. Therefore, if the fetus shows any evidence of autolysis, tissue should be taken aseptically, from placenta immediately beneath the chorionic plate. A portion ofplacenta and liver should also be snap-frozen for possible molecular analysis.

The initial stage of the autopsy should include photography and radiography, taking anterior-posterior and lateral views. The photographs will record the degree of maceration, which can be roughly correlated with the duration of fetal demise before delivery [1] External measurements should include body weight, circumference ofhead, chest and abdomen, crown-rump length, crown-heel length, and foot length. These measurements are compared with Tables of standards for normal fetuses (see Part III of this book). Assessment of growth retardation may be based on these data. A careful external examination should search for abnormalities such as jaundice, bulging fontanel, cranial bone softening, hyper-or hypotelorism, choanal atresia, external ear anomalies, cleft lip, cleft palate, macroglossia, micrognathia, colobomata, cystic hygroma, shortened neck, contractures, omphalocele, gastroschisis, abnormal external genitalia, anal atresia, absent vagina, sacral pits, open neural tube defects, hemihypertrophy, syndactyly, clinodactyly, transverse palmar creases, or incomplete descent of testes.

The organ bloc may be removed in the manner similar to the adult, using the technique of Letulle (see Chapter 2) . If the thyroid gland is noted to be in its usual location and if it appears normal, then the tongue may be left in the body. In macerated fetuses, it is suggested that the organ bloc be fixed overnight in formalin solution prior to dissection. To aid adequate fixation, the following simple steps may be done: I) wash the organ bloc thoroughly prior to fixation; 2) place multiple transverse cuts through the liver and lungs; 3) dissect the posterior leaves of the diaphragm away from the adrenal glands and kidneys; 4) bivalve the adrenal glands and kidneys in the coronal plane; 5) instill formalin in the lumen of the intestine, using a syringe; and 6) gently instill formalin into both ventricles of the heart, being careful to avoid the ventricular septum.

The procedure for dissection of the organs is similar to that of the adult except: 1) The venous and arterial connections of the heart, including the patency of the ductus arteriosus must be determined before the heart is removed; 2) The esophagus must be opened posteriorly prior to its complete removal so that esophageal atresia or tracheo-esophageal fistula may be recognized and photographed prior to further dissection; 3) The location of the appendix and of the testes should be recorded. Until the intestine has been examined for stenosis or atresia, the mesentery should be left attached.

The degree of autolysis as seen grossly and with histologic examination of both the fetus and the placenta can be used to estimate the duration of time between fetal death and delivery (2, 3, 4) . Trichrome stain is useful for better visualizing histologic features in severely autolyzed tissue.

To remove the brain, Benecke's technique of one of its modifications may be used.

Stillbirth vs Livebirth. A decision has to be made whether the infant was born alive or was stillborn. The hydrostatic lung test, described in the previous edition, appears unreliable. The presence of gas in the lungs does not rule out stillbirth. After death, air can be introduced into the lungs, or putrefaction gases might be present. However, air artificially introduced after death will not distend the alveoli and can be squeezed out, whereas this does not seem to be the case after active ventilation. The distribution of fat in the fetal zone of the adrenal cortex may indicate whether intrauterine death was acute, more prolonged, or chronic (3). This is particularly helpful if the stillborn baby is macerated. If the mother died also, see under "Death, abortion-associated" Strangulation NOTE: In many instances, procedures described under "Hom-icide" must also be followed. If a rope or some other material had been used (see also under "Hanging"), leave ligature in place until autopsy can be done. See also under "Hypoxia." Toxicologic sampling, particularly for alcohol, should be done in all instances (Chapter 13). results than body fluids. (2) Universal precautions should be strictly followed. The generation of aerosols should be minimized. To sterilize tissue surfaces in preparation for culture, swab the surface with povidone iodine. Avoid searing the tissue surface since this will produce an aerosol. Keep no more than one scalpel in the dissecting area at anyone time. Have an assistant available with clean, gloved hands to receive specimens in containers, so as to minimize the degree of contamination on the outside of the containers. For cleaning procedures and related information, see Chapter 6. (3) HIV-l in tissue may be demonstrated using polymerase chain reaction (PCR), immunohistochemistry, in situ hybridization, or immunofluorescence.

(4) For immunocytochemical and molecular studies, fix tissue in ethanol or Carnoy's solution. The virus can be identified using PCR in most tissues, whether fresh, frozen, or fixed in ethanol. (5) Serologic tests as well as direct fluorescent antibody tests are avail-able for many of the expected infections. (6) This is not a reportable disease.

External examination and skin

Record body weight and evidence of lipodystrophy following AIDS medications.

Record and photograph any skin lesions. Examine oral cavity.

If the diagnosis is in doubt, samples can be submitted for enzyme immunoassay.

Cachexia. Severe loss of subcutaneous fat in face and extremities, associated with large fat deposits on upper back and upper abdomen ("protease paunch"). Cutaneous Kaposi's sarcoma (l) .

Test may be positive for many weeks after death (2) . Syndrome, Myelodysplastic Synonyms and Related Terms: Chronic myelomonocytic leukemia;* refractory anemia; refractory anemia with excess of blasts; refractory anemia with excess of blasts in transformation; refractory anemia with ringed sideroblasts; refractory dysmyelopoietic anemias.

NOTE: The myelodysplastic syndromes are represented by a heterogeneous group of normocytic anemias, often with neutropenia, thrombocytopenia, and monocytosis. For expected bone marrow changes, see above under "Synonyms and Related Terms." Autopsy procedures are similar to those recommended for most cases of leukemia, with particular attention paid to intercurrent infections and thrombocytopenic hemorrhages. In all instances, material should be collected using aseptic technique for tissue culture for chromosome analysis. Common findings in these conditions are deletion of the long arm ofchromosome 5, deletion of chromosome 5 or 7, or trisomy 8. Syndrome, Nephrotic NOTE: See under name of suspected underlying condition, such as amyloidosis,* anaphylactoid purpura,*diabetes mellitus,* glomerulonephritis,* Goodpasture's syndrome,* heavy metal poisoning, hemolytic uremic syndrome,* infective endo-carditis,* polyarteritis nodosa,* syphilis, * or systemic lupus erythematosus. * If accelerated hypertension or constrictive pericarditis is the suspected underlying condition, see under "Hypertension (arterial), all types or type unspecified" or "Pericarditis," respectively.

In all instances, the renal veins and the inferior vena cava should be opened in situ. "En masse" removal of organs is recommended for this purpose. If thrombosis is found, record exact location and size of clot and submit sample of clot with wall of veins for histologic study. See also under "Thrombosis, venous." Coronary atherosclerosis and its complications seem to be increased in patients with the nephrotic syndrome. Submit sample for bacteriologic, viral, and serologic study. Record weight and submit samples for histologic study. If heart block was present, submit samples of conduction system for histologic study (Chapter 4). Submit consolidated areas for microbiologic study. Perfuse at least one lung with formalin. For removal of urethra, see Chapter 2.

Procedures depend on expected findings or grossly identified abnormalities as listed in right-hand column. For removal and specimen preparation, see Chapter 5. Consult roentgenograms (see above under "External examination, skin, and oral cavity").

Keratoderma (keratosis blennorrhagica) with vasculitis (2) of sole of feet, of palms, and of circumcised glans penis.

Arthritis* of knees, ankles, and metatarsal and midtarsal joints, with or without ankylosis. Osteoporosis.* Usually, sterile culture.

Pericarditis; * myocarditis.* Aortic valve lesions.

Pleuritis and pneumonia.* Pulmonary fibrosis involving upper lobes. Erosions, papules, and plaques in urethral or bladder mucosa. Enteritis. Thrombophlebitis.

Conjunctivitis; multifocal choroiditis; acute anterior uveitis; iritis; keratitis. Arthritis* (see above under "External examination, skin, and oral cavity") resembling rheumatoid arthritis. * Nonspecific synovitis. For removal and specimen preparation, see Chapter 5.

Syndrome, Reye's NOTE: Hypoglycemia* may have been present, but that condition is difficult or impossible to confirm after death. An immediate autopsy is indicated (see below under "Liver" and "Pancreas").

Vitreous Blood Urine Heart Lungs Submit sample for sodium, chloride, and urea nitrogen determination. Submit sample for microbiologic (viral) and serologic study, for ammonia and bilirubin determination, and for determination of salicylate levels if there is a history of treatment with this drug. Obtain sample for biochemical and toxicologic analysis.

Record weight. Request frozen sections for Sudan stain. Submit fresh tissue for bacterial and viral culture.

Request paraffin sections and frozen sections for fat stain (see above under "Heart").

Influenza;* varicella.* Manifestations of liver failure. Evidence of salicylate administration.

Assay for organic acids should rule out acylcoenzyme A dehydrogenase deficiency, and toxicity, e.g., of acetaminophen and valproic acid (l) . Cardiomegaly; fatty changes of myocardium and patchy myocytolysis. Viral pneumonia rarely present.

Acute interstitial pneumonia;* bronchitis;* hemorrhages. Lipid-laden histiocytes in alveoli. Record body weight and length, stature, and distribution of head, axillary, and pubic hair. Record and prepare photographs of features of face and neck. Prepare skeletal roentgenograms.

Submit samples of breast tissues for histologic study. These specimens should be collected using aseptic technique for tissue culture for chromosome analysis (see Chapter 9) . Procedures depend on expected findings or grossly identified abnormalities as listed in right-hand column. Submit for histologic study. Submit samples of all portions for histologic study. If biliary atresia is suspected, follow procedures described under that heading. Dissect kidneys and ureters in situ and record findings. Submit samples of gonads or-if gonads cannot be identified-equivalent ridges on mesosalpinx for histologic study. Also submit samples of endometrium, cervix, and vagina. Record weight and submit sample for histologic study. Procedures depend on expected findings or grossly identified abnormalities as listed in right-hand column. For removal and specimen preparation, see Chapter 5.

Procedures depend on expected findings or grossly identified abnormalities as listed in right-hand column.

For sampling and specimen preparation, see Chapter 4. Peroxidase-labeled gastrin antibodies seem to react with cells in all gastrinomas.

Aberrant gastrinoma may occur at hilus of spleen. Metastases are found in regional lymph nodes and liver. There may be manifestations of multiple endocrine neoplasia. * secondary syphilis. Levaditi's stain or Warthin-Starry stain is recommended for paraffin sections, and labeled fluorescent antibody techniques are recommended for frozen sections. India ink preparations or the Fontana-Masson silver stain has been used for the study of fresh lesions, and electron microscopy has also been employed. (4) In adult autopsies, no special precautions are indicated (see below under "Liver"). (5) Serologic studies are available from local and state health department laboratories. (6) This is a reportable disease.

External examination Record and prepare photographs of all abnormalities listed in right-hand column. Prepare smears and sections of acute lesions; prepare sections of older skin lesions or anogenital mucosal lesions.

Hunterian chancre in primary syphilis; condylomata lata. Noduloulcerative gummas and scarring in later stages.

Cerebrospinal fluid

Lymph nodes Heart and aorta

Other organs and tissues Prior to 20 wk gestation, the destructive effects of syphilis may not be seen. Gummata are rare in neonates. Tabes dorsalis is also uncommon. Serologic diagnosis is difficult in the neonate because of transplacental transfer of maternal IgG antibodies. Acquired syphilis (syphilis in adulthood) is presented above under a separate heading.

(1) Collect all tissues that appear to be infected. (2) Culture methods are not available, but animal inoculation can be performed. Consultation with a microbiology laboratory is recommended. (3) Special stains for Treponema pallidum rarely are positive except with material from fresh lesions of primary or secondary syphilis and of syphilitic hepatitis of the newborn. Levaditi's stain or Warthin-Starry stain is recommended for paraffin sections, and labeled fluorescent antibody tech-niques are recommended for frozen sections. India ink preparations or the Fontana-Masson silver stain has been used for the study of fresh lesions, and electron microscopy has also been employed. (4) In neonates, special precautions are indicated (see below under "Liver") (5) Serologic studies are available from local and state health department laboratories. (6) This is a report· able disease. (I) Collect all tissues that appear infected. (2) Request aerobic and anaerobic cultures. However, the presence of tetanus bacilli established in culture is not diagnostic, since spores of C. tetani frequently contaminate wounds. 

Record body weight and length and appearance of wound(s); photograph and excise wound(s) for histologic study. Record evidence of parenteral drug abuse, especially subcutaneous injection (i.e., "skin popping"). Prepare chest roentgenogram.

Evidence of weight loss; subcutaneous abscesses.

Tetanus may occur in drug addicts.

Tension pneumothorax* after mechanical ventilation. 

Submit sample for microbiologic study. Submit any consolidated area for microbiologic study.

Bacterial meningitis must be ruled out. Aspiration and bronchopneumonia; embolism;* atelectasis.

Tetany NOTE: See under name ofsuspected underlying conditions, such as hyperparathyroidism,* malabsorption syndrome,* or vitamin 0 deficiency. * Respiratory or metabolic alkalosis* cannot be confirmed after death; determination of blood pH is not helpful because acidity increases rapidly after death. The concentration of serum phosphates also increases after death.

Synonym: Large ventricular septal defect with pulmonary stenosis* or atresia.* NOTE: The basic anomaly consists of subpulmonary stenosis, ventricular septal defect, overriding aorta, and secondary right ventricular hypertrophy. For general dissection techniques, see Chapter 3. Surgical interventions include modified Blalock-Taus-sig subclavian-to-pulmonary arterial shunt; complete repair with patch closure of ventricular septal defect, and reconstruction of right ventricular outflow tract (with a patch or with an extracardial conduit).

Possible Associated Conditions: Origin of left pulmonary artery from aorta; minor abnormalities of the tricuspid valve; absent ductal artery (25%); atrial septal defect* (in 20%; pentalogy of Fallot); bicuspid pulmonary valve;* dextroposition of aorta; double aortic arch; hypoplastic pulmonary arteries; patent ductal artery;* patent oval foramen; complete atrioven-tricular septal defect* (usually with Down's syndrome*); persis-tent left superior vena cava; pulmonary valve atresia (see "Atresia, pulmonary valve, with ventricular septal defect"); right aortic arch (25%); second ventricular septal defect; origin ofleft anterior descending (LAD) or right coronary artery (RCA) from contralateral aortic sinus or coronary artery (5%); syndrome with absent pulmonary valve and massively dilated pulmonary arteries (rare).

Chest cavity Heart Lungs Other organs

Record course of superior vena cava and of its tributaries.

Record course of thoracic aorta and of its main branches.

Record origin of pulmonary arteries.

If infective endocarditis is suspected, follow procedures described under that heading (Chapter 7). For coronary arteriography, see Chapter 10.

Perfuse both lungs with formalin. Request Verhoeff-van Gieson stain. Procedures depend on expected findings or grossly identified abnormalities as listed in right-hand column.

Persistent left superior vena cava.

Right aortic arch with or without right-sided ductal artery; double aortic arch; absent ductal artery. Origin of the left pulmonary artery from descending aorta. For possible additional findings, see above under "Note" and under "Possible Associated Conditions." Infective endocarditis* (usually of pulmonary or aortic valve). See also above under "Possible Associated Conditions." Marked right ventricular hypertrophy. Right ventricular dilatation and fibrosis with late postoperative right-sided heart failure or sudden death due to arrhythmia. Old in situ thrombosis of small pulmonary artery branches. Paradoxic embolism; cerebral abscess. * Thalassemia Synonyms and Related Terms: Congenital hemolytic anemia; alpha-thalassemia; beta-thalassemia major (Cooley's anemia); beta thalassemia minor (beta-thalassemia trait). NOTE: The changes described below are observed primarily in beta-thalassemia major. Unless the cause of the renal vein thrombosis and the nature of the complicating or underlying renal disease are known, follow procedures described under "Glomerulonephritis."

Procedures depend on expected findings or grossly identified abnormalities as listed in right-hand column.

In infants, manifestations of dehydration. * Pulmonary embolism. * Tumor of the kidney* (renal cell carcinoma); aortic aneurysm; lymphadenopathy; other mass lesions. Inferior vena cava thrombosis involving orifice or whole length of renal veins. Tumor thrombus (e.g., from renal cell carcinoma). Femoral vein thrombosis. Rare venous malformations also may cause renal vein thrombosis (l) . Hemorrhagic infartion. Parenchymal renal disease with nephrotic syndrome,* including amyloidosis* and vasculitis* (these last conditions may be associated with renal vein thrombosis). Acute gastroenteritis in infancy.

Hyperparathyroidism,* pregnancy,* trauma, and other conditions. Thrombosis, Venous NOTE: For peripheral venous thrombosis, the autopsy procedures are essentially similar to those described under "Phlebitis." See also under "Hypertension, portal," "Syndrome, Budd-Chiari," "Thrombosis, cerebral venous sinus," "Thrombosis, lateral sinus," and "Thrombosis, renal vein."

Thymoma (See "'fumor of the thymus.") Thyroiditis

Synonymsand Related Terms: Chronic fibrosing thyroiditis (Riedel's struma); chronic thyroiditis with transient thyrotoxicosis; Hashimoto's thyroiditis; pyogenic thyroiditis; subacute (granulomatous, giant cell, de Quervain's) thyroiditis.

PossibleAssociatedConditions: WithHashimoto's thyroiditisautoimmune (chronic) hepatitis,*megaloblastic anemia,*rheumatoid arthritis,*Sjogren's syndrome,*and systemic lupus erythematosus.* With Riedel's struma-retroperitoneal fibrosis,* sclerosing cholangitis,* sclerosing mediastinitis,*and other conditions with idiopathic fibrosis. With subacute thyroiditis-viral infection. Submit sample for determination of protein concentration; record appearance of sediment.

For removal and specimen preparation, see Chapter 4.

Thromboses of small vessels. Glomerulonephritis.* Hemorrhagic necroses. Proteinuria; abnormal sediment.

Abruptio placentae, small placenta with accelerated maturation of villi; infarcts; fibrinoid necrosis of decidual arterioles.

Thrombotic occlusion of small vessels with hemorrhagic necroses; anterior lobe of pituitary gland may contain necroses (see also "Syndrome, Sheehan's"). Submit samples for histologic study.

If infection is expected, submit material for microbiologic study. Other procedures depend on expected findings or grossly identified abnormalities as listed in right-hand column.

Transposition, Complete, of the Great Arteries NOTE: The basic anomaly is the origin of the aorta from the right ventricle, and of the pulmonary artery from the left ventricle, with a shunt, and usually with a right anterior aorta. There are four major types: (1) with an intact ventricular septum (65%), (2) with a ventricular septal defect* (20%), (3) with a ventricular septal defect and subvalvular pulmonary stenosis* (10%), and (4) with an intact ventricular septum and subvalvular pulmonary stenosis* (5%). For general dissection techniques, see Chapter 3. Interventions include atrial septostomy or septectomy; Mustard or Senning atrial switch procedure; Rastelli-type repair with a valved extracardiac conduit; and Jatene arterial switch procedure with LeCompte maneuver.

Possible Associated Conditions: Abnormal origin ofcoronary arteries (10%); atrial septal defect* (5%); coarctation of the aorta;* interrution of the aortic arch;* juxtaposition of atrial appendages (4%); overriding aorta (5%); overriding pulmonary artery (10%); patent ductal artery;* patent oval foramen; subvalvular pulmonary stenosis* (15%); tubular hypoplasia ofthe aortic arch;* ventricular septal defect* (30%), often mal-alignment type (50%). Synonyms: Atrioventricular and ventriculoarterial discordance; L-transposition. NOTE: The basic anomaly is a mirror-image ventricular inversion, with a left anterior aorta, and with blood flow from right atrium to left ventricle to pulmonary artery, and from left atrium to right ventricle to aorta. For general dissection techniques, see Chapter 3. Interventions include patch closure of the ventricular septal defect; relief of pulmonary stenosis; relief of tricuspid insufficiency; and insertion of pacemakers.

Possible Associated Conditions: Anterior and posterior AV nodes (100%), prone to develop complete heart block; dysplasia or Epstein's malformation* ofleft-sided tricuspid valve (40%); mirror-image epicardial coronary artery distribution (100%); right-sided mitral valve anomalies; subvalvular pulmonary stenosis* (40%); ventricular septal defect* (65%). Kinyoun's, or other acid-fast stains. Polymerase chain reaction for mycobacterial DNA may be helpful in the differential diagnosis ofgranulomas (1). (4) Universal precautions should be strictly followed and aerolization should be avoided. (5) Reliable serologic studies are not available. A definite diagnosis requires isolation of the organism. (6) This is a reportable disease. Cavitary, fibrocalcific, miliary, bronchial, and other types of pulmonary tuberculosis; granulomas in hilar lymph nodes.

Most organs and tissues may be involved, including liver, pancreas, spleen, kidneys, adrenal glands and other endocrine glands, gonads, and lymph nodes. Tuberculous meningitis; tuberculoma.

Iridocyclitis or panophthalmitis. Tuberculous arthritis and synovitis (hips, spine, knee); tuberculous osteomyelitis (anterior aspect of vertebrae; metaphysis of long bones).

PART II / DISEASES AND CONDITIONS 

Procedures depend on expected findings or grossly identified abnormalities as listed in right-hand column. Procedures depend on expected findings or grossly identified abnormalities as listed in right-hand column.

Chronic ulcerative colitis may be associated with carcinoma of bile ducts, typically arising in PSc.* Metastases common in regional lymph nodes and lungs. I For removal, prosthetic repair, and specimen preparation and decalcification procedures, see Chapter 2. If osteoblastic metastases appear to be present, search for primary tumor in prostate and breast, carcinoid tumors and Hodgkin's lymphoma. Procedures depend on expected findings or grossly identified abnormalities as listed in right-hand column.

Cachexia. Evidence of hypercalcemia (particularly in presence of osteolytic metastases).

Metastases to bone (e.g., from carcinoma of prostate, breast, bronchi, thyroid gland, kidney, or urinary bladder) usually involve red bone marrow. Therefore, distal extremities are rarely involved by metastatic tumors in adults.

Metastases, commonly in lungs. Metastatic calcification in patients with hypercalcemia. Eosinophilia. Myopathy.* Metastases in liver and regional lymph nodes. See also above under "Possible Associated Conditions." Malakoplakia (rare) (2) .

PART II / DISEASES AND CONDITIONS

Other organs and peripheral arteries tumor(s). If tumor is within a heart chamber (usually a myxoma), record extent of mobility and capability of tumor to obstruct a valvular orifice. Submit samples of tumor(s) for histologic study and, particularly if the tumor is of unknown type, for immunohistochemical study and electron microscopy (Chapter 15). Procedures depend on expected findings or grossly identified abnormalities as listed in right-hand column.

metastatic carcinoma or melanoma. Secondary cardiac effects by tumors include carcinoid heart disease, amyloid heart disease in multiple myeloma, and lymphocytic myocarditis in pheochromocytoma. Treatment effects such as adriamycin cardiotoxicity may be noted also. Tumor (myxoma) emboli in pulmonary or peripheral vessels (1) . Multiple aneurysms* of cerebral and other arteries may rarely be associated with atrial myxomas. Procedures depend on expected findings or grossly identified abnormalities as listed above and in right-hand column.

PART II / DISEASES AND CONDITIONS If there was evidence of endocrine activity (see above), snap-freeze portion of fresh tumor for hormone assay. Perfuse lungs with formalin. Sample neoplastic and non-neoplastic tissue for histologic study.

Procedures depend on expected findings or grossly identified abnormalities as listed above and in right-hand column.

For removal and specimen preparation, see Chapter 4. For removal and specimen preparation, see Chapter 4.

For removal and specimen preparation, see Chapter 4.

For removal and specimen preparation, see Chapter 4.

For removal and specimen preparation, see Chapter 2.

See above under "Possible Associated Conditions." Note that hypoglycemia* generally cannot be confirmed at autopsy. Nonbacterial thrombotic endocarditis.* Carcinoma may be associated with asbestosis or other types of pneumoconiosis,* chronic bronchitis,* emphysema,* interstitial pneumonia,* and many other bronchopulmonary diseases.

See above under "Possible Associated Conditions." Metastases (regional lymph nodes, liver, bones, brain, and many other sites) and metastatic calcification. Migratory thrombophlebitis.* Encephalomyelitis;* cerebellar corticoid degeneration;* subacute spinocerebellar degeneration. * Crooke cell hyperplasia.

Myasthenic syndrome (Eaton-Lambert syndrome); myopathy;* dermatomyositis.* Peripheral neuropathy. See above under "External examination and skin." Bones (with red marrow) are common sites of metastases. Record presence or absence of testes.

Procedures depend on expected findings or grossly identified abnormalities as listed in right-hand column.

Thmor of the Soft Tissues The possible sites and characteristics of soft tissue tumors vary so much that no universally applicable autopsy techniques can be presented. In all instances, the size, weight, and location of the tumor(s) must be recorded and tissue must be sampled for histologic study. If the tumor had not been classified prior to death, samples should be snap-frozen for immunohistochemical study. Other samples should be prepared for electron microscopic study. Evidence of paraneoplastic syndromes (see below) may require additional procedures.

Possible Associated Conditions: Only a few paraneoplastic syndromes or systemic complications can be presented here. For the type of soft tissue tumor that was associated with each condition, see title of reference. Kasabach-Merritt syndrome (1) (thrombocytopenia, microangiopathic hemolytic anemia, and acute or chronic coagulopathy associated with a rapidly enlarging hemangioma); liver function abnormalities (2); neurofibromatosis (3); osteomalacia (4).

Heart Esophagus, stomach, and duodenum

Other organs and tissues T

In most instances, detennination of vitreous sugar concentration and of blood group is not indicated.

Inspect valves and prepare photographs and sections of vegetations.

Leave esophagus and part of duodenum attached to stomach. Submit samples of tumor and of grossly uninvolved stomach for histologic study.

Request PAS stain and Warthin-Starry stain for identification of H. pylori.

Portions of endocrine gastric tumor should be snap-frozen for immunohistochemical study and possible hormone assay.

Record location of tumor metastases.

Other organs Procedures depend on expected findings or grossly identified abnormalities as listed in right-hand column. For dissection of the thoracic duct (for search of tumor cell clusters), see Chapter 3.

Tumor of the Uterus (with Cervix) Possible Associated Conditions: Acquired immunodeficiency syndrome* (AIDS) (1) If peritonitis is present, submit exudate for bacteriologic study. Record volume of exudate and location of perforation. See "Disease, ischemic heart."

Other procedures depend on grossly identified abnormalities as listed in right-hand column. Procedures depend on expected findings or grossly identified abnormalities as listed in right-hand column. Perfuse at least one lung with formalin. For celiac arteriography, see Chapter 2. Open stomach and duodenum in situ and record site of perforation or penetration. Record measured or estimated volume of blood in gastrointestinal tract. Rinse ulcer with saline to locate eroded vessel(s). Pin stomach and duodenum on corckboard (serosa toward board) and fix specimen in formalin before sectioning. Prepare histologic sections of ulcer(s) and of remainder of stomach. Request Warthin-Starry stain for H. pylori.

Procedures depend on expected findings or grossly identified abnormalities as listed in right-hand column.

Free air in abdomen suggests perforation of ulcer. Peritonitis. * Perforation of ulcer.

Coronary atherosclerosis and manifestations of coronary insufficiency are commonly associated with peptic ulcer disease.

In rare instances, pericardial fistula may be present from ulcer in hiatus hernia. Peptic ulcers may be associated with emphysema,* tuberculosis,* and other chronic pulmonary diseases. Vasculitis (See "Aortitis," "Arteritis, .�.," "Phlebitis," and "Purpura,���") Ventricle, Double Inlet Left (1) Synonyms: Single functional ventricle; univentricular heart; univentricular atrioventricular connection; Holmes heart. NOTE: The basic anomaly is the connection of both atrioventricular valves to the left ventricle, often with transposed great arteries and a restrictive ventricular septal defect. There are four major types, based on the ventriculoarterial connection: (1) with congenitally corrected transposition (60%); (2) with complete transposition (30%); (3) with normally related great arteries (Holmes heart; 5%); and (4) double outlet, persistent truncal artery, or pulmonary atresia (5%). For general dissection techniques, see Chapter 3.

Possible Associated Conditions: Bicuspid pulmonary valve; bilateral mirror-image mitral valves (without tricuspid morphology); subvalvular aortic stenosis,* often with hypoplasia, coarctation, or interruption of the aortic arch; subvalvular pulmonary stenosis;* dual AV nodes and progressive heart block in patients with congenitally corrected transposition. Ventricle, Double Outlet Right Synonym: Origin of both great arteries from right ventricle; Taussig-Bing anomaly.

NOTE: The basic anomaly is the origin of the aorta and pulmonary artery primarily from the right ventricle, usually with a ventricular septal defect, and often with subpulmonary stenosis. For general dissection techniques, see Chapter 3.

Possible Associated Conditions: Complete atrioventricular septal defect (often with asplenia syndrome); muscular discontinuity between aortic and mitral valves; right ventricular infundibular stenosis; ventricular septal defect* that may be subaortic, subpulmonary, doubly committed, or remote. Examine histologically with H&E and oil red a stains.

Hepatomegaly and splenomegaly, jaundice. Deficiency of acid lipase (1).

Foam cells in the lamina propria.

Hepatomegaly, severe steatosis with cholesterol clefts in hepatocytes and Kupffer cells. Fibrosis.

Splenomegaly with lipid-laden foam cells.

Symmetric enlargement with dystrophic calcifications, giving the adrenals a gritty texture. Vacuolated cells of the inner zona fasciculata and entire zona reticularis with cholesterol clefts. Foam cells may be found in the lungs, bone marrow, lymph nodes, vessel walls, as well as Schwann and ganglion cells.

New insights into lipoprotein assembly and vitamin E metabolism from a rare genetic disease

Angioid streaks associated with abetalipoproteinemia

Familial hypobetalipoproteinemia: genetics and metabolism

Aviation pathology

Scuba diving accidents: decompression sickness, air embolism

Aetiology and occurrence of diving injuries. A review of diving safety

Scuba decompression illness and diving fatalities in an overseas military community

Diving-related emergencies

Bodies found in water

Achalasia and squamous cell carcinoma of the esophagus: analysis of 241 patients

Esophageal achalasia and adenocarcinoma in Barrett's esophagus: a report of two cases and a review of the literature

A technique for necropsy evaluation of stenosis of the foramen magnum and rostral spinal canal in osteochondrodysplasia

Mutation analysis of the MEN I gene in multiple endocrine neoplasia type I, familial acromegaly and familial isolated hyperparathyroidism. I

Increased incidence of neoplasia in females with acromegaly

Benign and malignant tumors in patients with acromegaly

Pathology of acromegaly

Ethanol in sequestered hematomas

Stages of acute alcoholic influence/intoxication

Electrolyte imbalance in alcoholic liver disease

Collection and storage of specimens for alcohol analysis

Analysis for alcohol in postmortem specimens

Disposition of alcohol in man

Medicolegal Aspects of Alcohol. Garriott JC

Blood, urine and other tissue specimens for alcohol analysis

Relationship between postmortem blood and vitreous humor ethanol levels

Larson CPo Alcohol: fact and fallacy

Gradwohl's Legal Medicine

Left ventricular hypertrophy is more prominent in patients with primary aldosteronism than in patients with other types of secondary hypertension

Intracranial aneurysm and hemorrhagic stroke in glucocorticoid-remediable aldosteronism

Association of adrenal medullae and cortical nodular hyperplasia: a report of two cases with clinical and morpho-functional consideration

Amyloidosis: recognition, confirmation, prognosis, and therapy

Electron microscopy of primary and secondary cutaneous amyloidosis and systemic amyloidosis

Ocular amyloidosis

Current status review: cerebral amyloid

Spinal cord compression by amyloid deposits

The burden of "sticky" amyloid: typing challenges

Quantification of cerebral amyloid angiopathy and parenchymal amyloid plaques with Congo Red histochemical stain

Kidney involvement in Takayasu arteritis

Pathogenesis of rheumatoid arthritis

Splenic involvement in rheumatic diseases

Aspergillus sinusitis in patients with AIDS: report of three cases and review

Invasive aspergillosis in systemic lupus erythematosus

Aspiration (See "Obstruction, acute airway

Aspergillus endocarditis, myocarditis and pericarditis complicating necrotizing fasciitis. Case report and subject review

Aspergillus-related lung disease

Biliary atresia and the polysplenia syndrome: its impact on final outcome

Biliary atresia

Nephromegaly and elevated hepatocyte growth factor in children with biliary atresia

Coccidiomycosis pneumonia in a nonendemic area associated with inftiximab

Codeine (See "Dependence, drug[s], all types or type unspecified

All Types or Type Unspecified (See "Enterocolitis, Other Types or Type Undetermined

Chronic Ulcerative (See "Disease, inflammatory boweI

Cytologic diagnosis of Cryp -tococcus neofonnans in HIV-positive patients

Cryptococcosis as an opportunistic infection in immunodeficiency secondary to paracoccidioidomycosis

Hypereosinophilia in disseminated cryptococcal disease

Cryptococcosis-a review of 13 autopsy cases from a 54-year period in a large hospital

Cryptosporidiosis in persons with HIV infection

Broncho-pulmonary cryptosporidiosis in four HIV-infected patients

Cryptosporidiosis and inflammatory bowel disease

Sclerosing cholangitis associated with chronic cryptosporidiosis in a child with a congenital immunodeficiency disorder

Cyanide (See "Poisoning, cyanide

Cyst(s), Choledochal

Choledochal cyst-dinical features and classification

Cyst(s), Liver (See "Disease, fibropolycystic, of the liver and biliary tract

Pulmonary Related Terms: Congenital cystic adenomatoid malformation; congenital pulmonary lymphangiectasis; intralobular bronchopulmonary sequestration. References Infection or calcification of cysts; pyelonephritis;* perinephric abscess. Obstructive uropathy

In recessive polycycstic renal disease, congenital hepatic fibrosis is present and cystic bile ducts may be present in dominant cases (5). See above under "Possible Associated Conditions

The pathology ofhuman renal cystic disease

Cystic kidney: Genetics, pathologic anatomy, clinical picture, and prenatal diagnosis

Hepatic fibrosis associated with hereditary cystinosis: a novel form of noncirrhotic portal hypertension

Ophthalmic manifestations and histopathology of infantile nephropathic cyctinosis: report of a case and review of the literature

Pathological investigations of deaths following surgery, anaesthesia and medical procedures

Clinical outcome versus post-mortem finding in thoracic surgery: a lO-year experience

Order from American Registry of Pathology Sales Office

Intra-alveolar pulmonary siderophages in sudden infant death: a marker for previous imposed suffocation

A contribution to a possible differentiation between SIDS and asphyxiation

Sudden infantdeath syndrome (SIDS)-standardized investigations and classification: recommendations

Investigation of the sudden deth of infants: a multicenter analysis

Alpha l-antitrypsin deficiency-associated panniculitis: resolution with intravenous alpha I-antitrypsin administration and liver transplantation

Clinical manifestations of alpha l-antitrypsin deficiency

Risk of hepatobiliary disease in adults with severe alpha I-antitrypsin deficiency (PiZZ): is chronic viral hepatitis B or C an additional risk factor for cirrhosis and hepatocellular carcinoma?

Alpha I-antitrypsin deficiency and inflammatory bowel disease

Severe alphal-antitrypsin deficiency (PiZ homozygosity) with membranoproliferative glomerulonephritis and nephrotic syndrome, reversi-bleafterorthotopic livertransplantation

Klippel-Feil syndrome associated with malfonned larynx. Case report

Klippel-Feil syndrome in association with a craniocervical dennoid cyst presenting as aseptic meningitis in an adult: case report

Klippel-Feil syndrome accompanied by an aneurysm of the non-coronary sinus of Valsalva

The hereditary ataxias

Clinical and genetic characterizations of 16q-linked autosomal dominant spinocerebellar ataxia (AD-SCA) and frequency analysis of AD-SCA in the Japanese population

Cocain-related medical problems. Consecutive series of 233 cases

Direct cocaine cardiotoxicity demonstrated by endomyocardial biopsy

The pathology and etiology of cocaineinduced heart disease

Ischemic colitis related to cocaine abuse

Hepatic dysfunction accompanying acute cocaine intoxication

High incidence of malignancies in patients with dennatomyositis and polymyositis: an lI-year analysis

Partial lipodystrophy associated with juvenile dennatomyositis: report of two cases

Chronic pneumomediastinum and subcutaneous emphysema: association with dennatomyositis

Spontaneous esophageal rupture in adult dennatomyositis

Polyneuropathy in juvenile dermatomyositis

Combined glucose and lactate values in vitreous humor for postmortem diagnosis of diabetes mellitus

Diabetes is related to cerebral infarction but not to AD pathology in older persons

Hepatic lesions resembling alcoholic liver disease

Relationship between cardiomyopathy and liver disease in chronic alcoholism

Cyanamide-associated alcoholic liver disease: a sequential histologic evaluation

Caroli's disease: 1977-1995 experiences

Caroli 's disease and autosomal dominant polycystic kidney disease: a rare asso-ciation?

Spontaneous rupture of a bile duct and its endoscopic management in a patient with Caroli's syndrome

Bartonella henselae endocarditis in an immunocompetent adult

Cat-scratch disease and bacillary angiomatosis

Attack, transient cerebral ischemic" and "Infarction, cerebral!')

Cholesteryl ester storage disease: Hepatopathology and effects of therapy with lovestatin

Clinical, biochemical and histological analysis of seven patients with cholesterol ester storage disease

Cutaneous manifestations of chronic granulomatous disease. A report of four cases and review of the literature

Aspergillus endocarditis in chronic granulomatous disease

Colitis complicating chronic granulomatous disease. A clinicopathological case report

Hypertrophic cranial pachymeningitis with spinal epidural granulomatous lesion

Ocular pathologic findings of chronic granulomatous disease of childhood

Review: Creutzfeldt-Jakob disease

Survival of Creutzfeldt-Jakob disease virus in formol-fixed brain tissue

Guidelines for high risk autopsy cases: special precautions for Creutzfeldt-Jakob disease

Tissue handling in suspected Creutzfeldt-Jakob disease and other human spongiforme encephalopathies (prion diseases)

Cerebrospinal fluid concentration of neuron-specific enolase in diagnosis of Creutzfeldt-Jakob disease

Autopsy case of sporadic Creutzfeldt-Jakob disease presenting with signs suggestive of brainstem and spinal cord involvement

Metastatic Crohn's disease: a rare cutaneous manifestation

Crohn's disease with pulmonary involvement in a 3 year old boy

Mesenteric fibromatosis asso-ciated with Crohn's disease

Cholangiocarcinoma and Crohn's disease

The pancreas as a site of granulomatous inflammation in Crohn's disease

Dermatomyositis associated with Crohn's disease 1994

Cardiocyte storage and hypertrophy as a sole manifestation of Fabry's disease. Report on a case simulating hypertrophic non-obstructive cardiomyopathy

An atypical variant of Fabry's disease with manifestations confined to the myocardium

Pulmonary involvement in Fabry disease

Cerebrovascular complications ofFabry's disease

High incidence of thrombosis in Fabry's disease

Oral involvement in chronic graft versus host disease after allogenic bone marrow transplantation

Massive pericardial effusion complicating the course of chronic graft-versus-host disease (cGVHD) in a child with acute lymphoblastic leukemia following allogeneic bone marrow transplantation

The histological spectrum of pulmonary graft-versushost disease in bone marrow transplant recipients

Bronchiectasis in bone marow transplantation

Oncocytic metaplasia of bile duct epithelium in hepatic GVHD

Immune-mediated myelopathy after allogeneic marrow transplantation

Gunther's (See ''Porphyria, congenital erythropoietic

Heavy-Chain Synonyms and Related Terms: Gamma heavy-chain

Inflammatory bowel disease (first of two parts)

Takayasu's arteritis associated with idiopathic ulcerative colitis

Cerebrovascular complications ofinflammatory bowel disease

Pathology of inflammatory bowel disease

Legionnaires' disease

Source of infection for sporadic Legionnaires' disease: a review

Legionnaire's disease associated with macular rash; two cases

Severe skin loss after meningococcal septicemia: complications in treatment

Osteonecrosis following meningococcemia and disseminated intravascular coagulation in an adult: case report and review

Rhabdomyolysis during the subacute stage of meningococcal sepsis

Primary meningococcal arthritis in a child: case report and literature review

Chondrosarcoma as a complicating factor in Paget's disease of bone

Lymphoma arising in Paget's disease

Osteoarthritis and Paget's disease

Sickle cell disease

Sickle cell disease and sudden death: a retrospective/prospective study of 21 autopsy cases and literature review

Vascular lesions of the liver in sickle cell disease. A clinicopathologic study in 26 living patients

Whipple's disease and "Tropheryma whippelii

Periodic acid-Schiff-negative granulomatous lymphadenopathy in patient with Whipple's disease. Localization of the Whipple bacillus to noncaseating granulomas by electron microscopy

Serial imaging studies of cerebral Whipple's disease: from onset to end

The Wilson's disease gene is a putative copper transporting P-type ATPase similar to Menke's gene

The liver biopsy diagnosis of Wilson's disease. Methods in pathology

Hepatolenticular degeneration (Wilson's disease)

Venous air embolism in homicidal blunt impact head trauma: Case reports

A practical device for demonstrating air embolism

Amniotic Fluid 3. Kulka W. Laboratory methods and technical notes: a practical device for demonstrating air embolism

Proof of fatal air embolism

Venous air embolism from head and neck wounds

Hantavirus pulmonary syndrome: a clinical description of 17 patients with a newly recognized disease

Liver involvement in hemorrhagic fever with renal syndrome

Hantavirus infection induces a typical myocarditis that may be responsible for myocardial depression and shock in hanta virus pulmonary syndrome

Lassa fever in the United States. Investigation ofa case and new guidelines for management

Lassa fever: review of virology, immunopathogenesis, and algorithms for control and therapy

Early diagnosis of Lassa fever by reverse transcription-PCR

Rheumatic pneumonia: reappearance of a previously recognized complication of rheumatic fever

Acute rheumatic fever and poststreptococcal acute glomerulonephritis caused by T serotype 12 Streptococcus

Scleritis, uveitis, and glaucoma in a patient with rheumatic fever

Comparison of clinical features and pathologic findings in fatal cases of typhoid fever during the initial and later stages of the disease

Typhoid fever complicated by acute renal failure and hepatitis: case reports and review

Hemophagocytosis and granulomas in the bone marrow of a child with Down syndrome

Diseases involving intrahepatic bile ducts

Idiopathic eosinophilic esophagitis with stricture formation in a patient with longstanding eosinophilic gastroenteritis

Eosinophilic gastroenteritis presenting with colitis and cholangitis

Eosinophilic gastroenteritis presenting with acute pancreatitis

Idiopathic midline destructive disease: does it 3. MendenhallWMetaI.Lethalmidlinegranoloma-nasalnaturalkillerff-celi exist?

Nasal cocain abuse mimicking midline granuloma

Pulmonary lymphomatoid granulomatosis in acquired immunodeficiency syndrome: lesions with Epstein-Barr virus infection

Recurrent bilateral spontaneous pneumothoraces associated with pulmonary angiocentric immunoproliferative lesion

Lymphomatoid granulomatosis: pathogenesis, pathology and clinical implications

Wegener's granulomatosis: histological documentation of common and uncommon manifestations in 216 patients

Necrotizing granulomatosis of the breast

Wegener's granulomatosis with gangrene of toes

Pathology of pulmonary granulomatous vasculitis. Sarcoidosis

Gunshot (See "Injury, firearm

Splenic involvement in rheumatic diseases

Wegener's granulomatosis, acute laryngotracheal airway obstruction and death in a 17-year-old female: case report and review of the literature

Antiendothelial antibodies in patients with Wegener's granulomatosis: prevalence and correlation with disease activity and manifestations. I RheumatoI2007

Transfusion-transmitted disease

References Flattening, broadening, and possible fusion of villi. Phlegmonous inflammation and edema, mainly in ileocecal region

Encephalitis.* Leukopenia; thrombocytopenia; aplastic anemia (4) (pancytopenia*)

Synovitis (arthritis*)

Hepatitis G virus infection in hepatitis C virus-positive patients co-infected or not with hepatitis B virus and/or human immunodeficiency virus

Hepatitis G and C coinfection in children

Tumor of the liver

Diaphragmatic Related Terms: Congenital diaphragmatic hernia

Fulminant hepatitis in patients undergoing liver transplantation: evidence for anon-A, non-B, non-C

Marrow transplantation for hepatitis-associated aplastic anemia: a follow up oflong-term survivors

Langerhans cell histiocytosis research. Past, present, and future

An update on c1onality, cytokines, and viral etiology in Langerhans cell histiocytosis

Langerhans cell histiocytosis in adults

Pulmonary Langerhans cell granulomatosis

Central nervous system disease in Langerhans cell histiocytosis

Immunohistochemical analysis oflangerin in langerhans cell histocytosis and pulmonary inflammatory and infectious diseases

Pathological approach to the diagnosis of hydrocephalus

Thoracic lipomeningocele associated with diastematomyelia, tethered spinal cord, and hydrocephalus. Case report

Infectious causes ofhydrops fetalis

Human parvovirus B19 infection associated with hydrops fetalis

Cardiac abnormalities associated with hydrops fetal is

GMI-gangliosidosis presenting as nonimmune hydrops fetalis: a case report

A case of recurrent infantile polycystic kidney associated with hydrops fetalis

The pathologist and the hydropic placenta, fetus, or infant

Chronic diarrhea associated with thymoma and hypogammaglobulinemia (Good's syndrome)

Systemic amyloidosis in a patient with hypogammaglobulinemia. I

HCV infection in patients with primary defects of immunoglobulin production

Encepahomyelitis in primary hypogammaglobulinemia

Retinitis pigmentosaand hypogammaglobulinemia

Intestinal obstruction in the newborn with congenital syphilis

Meconium ileus and its equivalent as a risk factor for the development of cirrhosis: an autopsy study in cystic fibrosis

Biliary atresia and meconium ileus associated with Nieman-Pick disease

Postmortem cranial MRI and autopsy correlation in suspected child abuse

Cytomegalovirus-associated pseudotumor simulating gastric malignancy in acquired immuno-deficiency syndrome: a case report with review of literature

Bone marrow fibrin ring granulomas and cytomegalovirus infection

Cutaneous reactions following herpes zoster infections: report of three cases and a review of the literature

Herpes zoster and internal malignancy

Posteriorhorn varicella-zoster virus myelitis

Death or injury caused by electrocution

Myocardial hemorrhagic necrosis in delayed death from electrocution

Safety in bullet recovery procedures: a study of the Black Talon bullet

Lightning injuries: Prognostic signs of death

Lightning injuries

Adrenal insufficiency, recurrent bacteremia, and disseminated abscesses caused by Nocardia asteroides in a patient with acquired immunodficiency syndrome

ARDS and adrenal insufficiencyassociated with the antiphospholipid antibody syndrome

Adrenal insufficiency from bilateral adrenal hemorrhage after total knee replacement surgery

Non-Hodgkin's lymphoma presenting with adrenal insufficiency and hypothyroidism: an autopsy case report

A case ofprimary unilateral adrenal Burkitt-like large cell lymphoma presenting as adrenal insufficiency

Effect of growth hormone on fatty liver in panhypopituitarism

Increased fracture frequency in adult patients with hypopituitarism and GH deficiency

Pituitary abscess

The Liver: An Atlas and Text of Ultrastructural Pathology

Intubation (See "Injury, intubation

Iodine (See "Poisoning, iodine

Attack, transient cerebral ischemic" and "Infarction, cerebral

Heart (See "Disease, ischemic heart

Isomorism (See "Syndrome, polysplenia and asplenia

A study on performance of two serological assays for diagnosis of leprosy

Detection of Mycobacterium leprae infection by PCR

Pathology of eye in leprosy

References Intervillous abscesses containing grampositive, non-acid-fast bacilli

Bacteria are predominantly intracellular. Enterocolitis. Listeria monocytogenes can be cultured from meconium. Generalized lymphadenitis. Disseminated abscesses and/or granulomas, particularly after transplacental infection

Listeria monocytogenes empyema in an HIV infected patient

Fatal Listeria meningitis, endocarditis and pericarditis in a patient with haemochromatosis

Liver abscesses due to Listeria monocytogenes

Yust 1. Brainstem abscess and meningitis due to Listeria monocytogenes in an adult with juvenile chronic arthritis

Pulmonary hypertension associated with systemic lupus erythematosus: predominantly thrombotic arteriopathy accompanied by plexiform lesions

The liver in systemic lupus erythematosus: pathologic analysis of 52 cases and review of Japanese autopsy registry data

Chronic pancreatitis: a complication of systemic lupus erythematosus

Disseminated perivenous necrotizing encephalomyelitis in systemic lupus erythematosus: report of an autopsy case

Skeletal muscle lymphocytic vasculitis in systemic lupus erythematosus: relation to disease activity

Clinically occult avascular necrosis of the hip in systemic lupus erythematosus

Storage histiocytes and hemophagocytosis: a common finding in the bone marrow of patients with active systemic lupus erythematosus

Extensive medium vessel vasculitis with SLE: an unsual association

Cutaneous manifestations of sexually transmitted

ing cause of proctitis in men who have sex with men

Lymphogranuloma venereum as a cause

Cutaneous malakoplakia in a patient with acquired immunodeficiency syndrome (AIDS)

Extensive malakoplakia ofthe nasopharynx: management of a rare disease

Malakoplakia and colorectal adenocarcinoma

Coinfection is common in measles associated pneumonia

Giant cell pneumonia: light microscopy, immunohistochemical, and ultrastructural study of an autopsy case

Subacute sclerosing panencephalitis manifesting as viral retinitis: clinical and histopathologic findings

Intestinal neuronal dysplasia

Thoracic lipomeningocele associated with diastematomyelia, tethered spinal cord, and hydrocephalus. Case report

Disease, meningococcal

Encephalitis, all types or type unspecified" and "Meningitis

Mercury (See "Poisoning, mercury

With Myelofibrosis Synonym: Idiopathic myelofibrosis

Acute cardiac tamponade associated with pericardial extramedullary hematopoiesis in agnogenic myeloid metaplasia

Methyl Alcohol) (See "Poisoning, methanol (methyl alcohol)

Thrombotic Thrombocytopenic (See "Purpura, thrombotic thrombocytopenic

Epstein-Barr virus in inflammatory diseases of the liver and liver allografts: an in situ hybridization study

Fulminant hepatitis due to Epstein-Barr virus infection

The mucopolysaccharidoses: a clinical review and guide to management

Biochemical diagnosis of mucopolysaccharidoses: experience of 297 diagnoses in a 15-year period (1997-1991)

Mucormycosis Synonym: Phycomycosis; zygomycosis. NOTE: Diseases that may be complicated by mucormycosis include bums,* diabetes mellitus,* leukemia,* lymphoma,* and tuberculosis

Cardiac involvement in mucopolysaccharidosis: effects of allogeneic bone marrow transplantation

Mitral and aortic regurgitation in 84 patients with mucopolysaccharidosis

Toxic epidermal necrolysis possibly linked to hyperacute graft-versus-host disease after allogeneic bone marrow transplantation

Pulmonary complications in toxic epidermal necro-Iysis: a prospective clinical study

References Possible or Expected Findings Pyelonephritis;* nephrocalcinosis; granulomas; tumor infiltrates; manifestations of the conditions listed below under "Other organs

Renal stones in patients with rheumatoid arthritis

Nephrolithiasis as a presenting feature ofchronic sarcoidosis: a prospective study

Nephrolithiasis and risk of hypertension

Posterior lens opacities; retinal hamartomas. Peripheral neuropathy with focal schwannomatous changes or onion-bulb-like Schwann cell or perineural cell proliferation

Neurofibromatosis type I. In: Pathology and Genetics of Tumours of the Nervous System

Neurofibromatosis type 2. In: Pathology and Genetics ofTumours ofthe Nervous System

Neurological complications involving the central nervous system in neurofibromatosis type I

Primary cutaneous Nocardia asteroides infection after heart transplantation

Native valve endocarditis due to Nocardia-like organisms

Hepatobiliary complications of total parenteral nutrition

Total parenteral nutrition: a histopathologic analysis of the liver changes in 20 children

Obesity and breast cancer risk

Nonalcoholic steatohepatitis

Obesity cardiomyopathy: pathogenesis and pathophysiology

Bennett CPo Pachydennoperiostitis in childhood

Unilateral hypertrophic osteoarthropathy associated with aortofemoral graft infection

Gastroesophageal reflux and esophagitis-associated hypertrophic osteoarthropathy

Hypertrophicosteoarthropathy caused by lipoid pneumonia

Achondroplasia" and Dyschondroplasia, OIlier's

Osteogenesis Imperfecta Synonyms and Related Terms: Lobstein's syndrome

Osteogenesis imperfecta congenita; 01 type I, II, and III; osteogenesis imperfecta cystica

Hypercalcemia in osteogenesis imperfecta treated with pamidronate

Gastrointestinal problems in patients who have type-III osteogenesis imperfecta

Osteomalacia Related Terms: Osteoporosis;* renal osteodystrophy; rickets. NOTE: Many possible causes of osteomalacia may not be apparent at autopsy, e.g., sodium fluoride or diphosphonate toxicity or use of anticonvulsant drugs

Osteomalacia associated with adult Fanconi syndrome: clinical and diagnostic features

Epstein-Barr virus in B-celllymphomas associated with chronic suppurative inflammation

Osteomyelitis and osteonecrosis in inflammatory bowel disease

References Hyperlipidemia; hyperuricemia. Fracture or traumatic dislocation of hip

See above under "Note

Osteomyelitis and osteonecrosis in inflammatory bowel disease

Osteonecrosis and human immunodeficiency virus infection

Report of fifteen cases. Therapeutic recommendations

Malignant infantile osteopetrosis: otolaryngological complications and management

Renal tubular acidosis and osteopetrosis with carbonic anhydrase II deficiency: pathogenesis of impaired acidification

Spondylolysis in children who have osteopetrosis

Osteopetrosis with Arnold chiari malformation type 1 and brain stem compression

Osteoporosis and atherosclerosis in chronic renal failure

Endocrine disorders and osteoporosis

Otosclerosis: a measles virus associated inflammatory disease

Correlations between pathologic changes in the stapes and conductive hearing loss in otosclerosis

The aetiology of otosclerosis: a review of the literature

Etiologic links between chronic pancreatitis and pancreatic cancer

Hyperparathyroidism and pancreatitis during pregnancy

Pancytopenia Related Terms: Agranulocytosis;* aplastic anemia; Fanconi's anemia.* NOTE: Some causes of pancytopenia such as adverse drug reactions (e.g., due to antimetabolites, sulfa drugs

Thrombotic thrombocytopenic purpura/hemolytic uremic syndrome secondary to pancreatitis

Retinopathy as asys-temic complication of acute pancreatitis

References Mesenteric panniculitis

Necrotizing pancreatitis. * Vasculitis with thrombi; adrenocortical infarctions. Vasculitis with thrombi and infarctions. Relapsing polychondritis (4). See also above under "Possible Associated Conditions

Subcutaneous panniculitic T-cell lymphoma is a tumor of cytotoxic T lymphocytes

AbecassisM. Alpha I-antitrypsindefi-ciencyassociated panniculitis: resolution with intravenous alpha l-anti-trypsin administration and liver transplantation

Blind loop syndrome: an unusual cause of panniculitis. J Am Acad Paracoccidioidomycosis Synonyms: Paracoccidioides brasiliensis infection

Cutaneous panniculitis and relapsing polychondritis: two cases

Normal subcutaneous fat, necrosis of adipocytes and classification of the panniculitides

Paraneoplastic pemphigus

A case of pemphigus vulgaris with esophageal involvement

Rheumatoid constrictive pericarditis (clinical conference)

Benign Paroxysmal (See "Fever, familial Mediterranean

Pneumatosis intestinalis in pediatric acquired immunodeficiency syndrome

Pneumatosis cystoides intestinalis: an unusual complication of systemic amyloidosis

Pneumatosis intestinalis in children with primary combined immunodeficiency

Pneumatosis cystoides intestinalis with abdominal free air in a 2-year-old girl after allogeneic bone marrow transplantation

Pneumatosis intestinalis: a review

Pneumatosis coli: a proposed pathogenesis based on study of 25 cases and review of the literature

Collagenous inorganic dust pneumoconiosis, diffuse type: Aluminosis; asbestosis; chronic pulmonary berylliosis; talcosis; Collagenous inorganic dust pneumoconiosis, nodular type: Silicosis; Noncollagenous inorganic dust pneumoconiosis (includes mixed-dust fibrosis): Baritosis; China clay pneumoconiosis; chromite pneumoconiosis; coal worker's pneumoconiosis; fuller's earth pneumoconiosis; hematite or magnetite miner's lung; siderosis or welder's lung; stannosis; Organic dust pneumoconiosis: Byssinosis. NOTE: In addition to the aforementioned classic forms of pneumoconiosis, many other airborne substances have been impli-cated in recent years, for example, cerium

Uncommon causes ofoccupational interstitial lung diseases

Rare earth (cerium oxide) pneumoconiosis: analytical scanning electron microscopy and literature review

Diagnostic criteria for chronic beryl1ium disease (CBD) based on the UK registry 1945-1991

Pneumocystis Carinii (See "Infection, Pneumocystis carinii

This condition is diagnosed by inspection, palpation, and roentgenography. Tension pneumomediastinum may

Autopsy evaluation of asbestos exposure: retrospective study of 135 cases with quantitation of ferruginous bodies in digested lung tissue

Atypical mycobacteriosis as a complication of talc pneumoconiosis

Silicosis, mixed dust pneumoconiosis, and lung cancer

Complications associated with the use ofthe Esophageal-Tracheal Combitube

Pneumomediastinum: an unusual complication of asthma in a young man

Spontaneous pneumomediastinum in a patient with bronchiolitis obliterans after bone marrow References I. Katzenstein A-L, Myers J. Idiopathic pulmonary fibrosis: clinical relevance of pathologic classification

Update on lymphoid interstitial pneumonitis

Lymphocytic interstitial pneumonitis and nonspecific interstitial pneumonitis

Histologic diagnosis of extrinsic allergic alveolitis

Role of electron microscopy in interstitial lung disease

Detennination of arsenic in hair using neutron activation

Arsenic intoxication associated with tubulointerstitial nephritis

Atrioventricular block after administration of atropine in patients follow-ing cardiac transplantation

Death following intentional methyl bromide poisoning: toxicological data and literature review

Pulmonary edema, alveolar wall damage, and interstitial pneumonia after acute inhalation. Severe pulmonary fibrosis may develop in chronic cases. Gastroenteritis after nonlethal food poisoning. Degeneration of proximal tubules and proteinuria in acute poisoning

Degenerative changes of liver and myocardium

Problems in the analysis of cadmium in autopsied tissues

Elevated urinary cadmium concentrations in a patient with acute cadmium pneumonitis

Cadmium-associated renal disease

The effects of storage conditions on the stability of carbon monoxide in postmortem blood

Acute renal failure, Poisoning, Carbon Tetrachloride Synonym: Tetrachloromethane poisoning. NOTE: Toxicologic sampling of body fluids and organs should be done routinely in all cases. In many instances, however, death occurs I wk to 10 d after exposure, and by this time no carbon tetrachloride is demonstrable. Death may be sudden compartment syndrome, and systemic capillary leak syndrome complicating carbon monoxide poisoning

Southern JE Acute hydrocephalus following carbon monoxide poisoning

Prevention of occupational cyanide exposure in autopsy prosectors

Gradwohl's Legal Medicine

References Possible or Expected Findings Digoxin values in digitalis toxicity are greater than 2 ng/mL (1)

Digoxin concentration may be higher or lower than concentration in serum, depending on how long before death drug was taken (I)

Digoxin concentrations in postmortem specimens after overdose and therapeutic use

A fluorimetric determination ofmyocardial digoxin at autopsy, with identification of digitalis leaf, digitoxin and gitonin

Ethylene glycol poisoning: toxicokinetic and analytical factors affecting laboratory diagnosis

Ethylene glycol poisoning: case report of a record-high level and a review

Lead concentrations in human plasma, urine and whole blood

Trace metals (lead and cadmium screening)

An EMG case report oflead neuropathy 19 years after a gunshot injury

Aminoaciduria and glycosuria following severe childhood lead poisoning

Risk of nervous system cancer among workers exposed to lead

Demonstration of mercury in the human brain and other organs 17 years after metallic mercury exposure

Spontaneous exfoliation of teeth following severe elemental mercury poisoning: case report and histological investigation for mechanism. Oral Surg Oral Med Oral PathoI1997

Acute chemical pancreatitis associated with nonfatal strychnine poisoning

Homicide by strychnine poisoning

Coronary arteritis, with or without aneurysms and infarctions, primarily in childhood. Myocardial hypertrophy secondary to hypertension. * Minimal or no involvement by polyarteritis nodosa; considerable involvement in other types ofnecrotizing vasculitis (see "Arteritis, all types or type unspecified")

with or without formation of aneurysms and infarctions, may occur in all organs. The liver may show bile duct injury and rarely, nodular regenerative hyperplasia (8)

More frequently involved in giant cell elastic arteritis.* Polyarteritis of small muscular arteries, including vasa nervorum. Arthritis* may rarely be present with swollen joints. Infarctions;* subarachnoid hemorrhage; arteritis of cerebral arteries. Papillitis; retinal hemorrhages

Polyarteritis nodosa-like vasculitis in human immunodeficiency virus infection

The coexistence of familial Mediterranian fever and polyarteritis nodosa: report of a case

Microscopic polyarteritis during polymyalgia rheumatica remission

Development of polyarteritis nodosa in the course of inactive systemic lupus erythematosus

Bone marrow pathology in relapsing polychonditis: high frequency of myelodysplastic syndrome

Relapsing polychondritis associated with subclinical Sjo-gren's syndrome and phlegmon of the neck

Giant cell arteritis and polymyalgia rheumatica

Posterior scleritis and polymyalgia rheumatica

Polymyalgia rheumatica in patients with ankylosing spondylitis: a report of 5 cases

Inflammatory changes of hip synovial structures in polymyalgia rheumatica

Polymyositis (See "Dermatomyositis

Polyneuritis (See "Syndrome, Guillain-Barre

Familial, and Related Syndromes Related Terms: Cowden's disease (multiple hamartoma syndrome); familial colonic polyposis

Gardner's syndrome; non-polyposis syndrome (hereditary nonpolyposis colorectal cancer syndrome)

Sampling for histologic study depends on expected findings or grossly identified abnormalities as listed in right-hand column. (See also below under "Soft tissues

syndrome; mucocutaneous pigmentations (buccal, perioral, priorbital, distal extremities) in Peutz-Jeghers syndrome;* papules in face and oral mucosa in Cowden's disease; tumors of skin and subcutis (see below under "Soft tissues

Gardner's syndrome. Osteomas or exostoses (mandible, calvara

Desmoid in familial adenomatous polyposis

Malignant potential in intestinal juvenile polyposis syndromes

Ampullary carcinoma in familial adenomatous polyposis

Adenoma of the common bile duct in Gardner's syndrome may cause relapsing acute pancreatitis

Orbital osteoma in Gardner's syndrome

MyelopathylMyelitis," and "Syndrome, Guillain-Barre.") References Possible or Expected Findings Hypertrichosis; erythrodontia; scarring and mutilation of hands and face. Hemolytic anemia. Erythrocytes contain large amounts of uroporphyrin I; nonnoblasts and reticulocytes exhibit intense red fluorescence

Uroporphyrin I and coproporphyrin I in high concentrations. Splenomegaly (may have been treated by splenectomy)

Correction of congenital erythropoietic porphyria by bone marrow transplantation

Successful cord blood stem cell transplantation for congenital erythropoietic porphyria (Gunther's disease)

Congenital erythropoietic porphyria associated with nephrotic syndrome

Atypical red cell inclusions in congenital erythropoietic porphyria

Possible Associated Conditions: Adverse drug reaction; chronic alcoholism;* chronic hepatitis C (1); human immunodeficiency virus infection

Porphyria cutanea tarda and human immunodeficiency virus: two cases associated with hepatitis C

Porphyria cutanea tarda and antibodies to hepatitis C virus

An unhappy triad: hemochromatosis, porphyria cutanea tarda and hepatocel1ularcarcinoma-a case report

Variegate porphyria

Possible Associated Conditions: Ebstein's malformation of tricuspid valve. References Possible or Expected Findings Chronic eczematous skin lesions or superficial scarring; nail changes. Perivascular PAS-positive hyaline (2)

Brown protoporphyrin deposits with red to yellow birefringence with a maltese cross configuration in hepatocytes, Kupffer cells, and bile canaliculi

Erythropoietic protoporphyria

Cytofluorometry as a diagnosis of protoporphyria

Recessive inheritance of erythropoietic protoporphyria with liver failure

Pseudogout Synonym: Calcium pyrophosphate dihydrate (CPPD) deposition disease

Puncture grossly affected joints and submit sample of synovial fluid for crystal analysis under compensated polarized light (2)

References Possible or Expected Findings Punctate calcium deposits in kneejoints and, less commonly, in joints of hips, ankles, shoulders, or wrists or in symphysis ossium pubis and intervertebral disks. Arthritis* with synovitis. Crystals of calcium pyrophosphate dihydrate in periarticular tissue (1) and synovial fluid. Cartilage contains calcium salts of pyrophosphate, hydroxyapatite, and orthophosphate. Alkaptonuria;* gout

Calcium pyrophosphatedihydratedeposition disease presenting as tumoral calcinosis (periarticularpseudogout)

Calcifications in dermis; calcifications of blood vessels. Diaphragmatic hernia.* Hypertensive cardiomegaly. Characteristic plaques in pericardium and endocardium, with or without mitral valve involvement. Coronary atherosclerosis may have been a causeofangina(2).Coronarythrombosisand myocardial infarction may be present also. Accelerated atherosclerosis; calcification of peripheral vessels. Gastrointestinal hemorrhage;* peptic ulcer

Degeneration of Bruch's membrane with retinal hemorrhages; sclerosis of choroid vessels

Calcification of elastic fibers in pseudoxanthoma elasticum

New report of severe coronary artery disease in an eighteen-year-old girl with pseudoxanthoma e1asticum. Case report and review of the literature

HIVrelated thrombotic thrombocytopenic purpura: report of 2 cases and a review of the literature

Beham-Pyelonephritis Synonyms and Related Terms: Acute ascending pyelone

Angiotropic large cell lymphoma presenting as thrombotic micro-angiopathy (thrombotic thrombocytopenic purpura)

Splenic pathology in thrombotic thrombocytopenic purpura

Emphysematous pyelonephritis in diabetic patients

Nephrobronchialfistulaandlungabscessresulting from nephrolithiasis and pyelonephritis

A case ofan enterorenal Pyothorax (See "Empyema, pleuraI.") fistula and pyelonephritis with airin renal pelvis

Systemic amyloidosis secondary to pyonephrosis. Resolution after nephrectomy

Medicolegal Investigation of Death

The laboratory's role in detecting sexual assault

Toluidine blue in the detection at autopsy of perineal and anal lacerations in victimes of sexual abuse

Sarcoidosis presenting as heart disease

Sarcoidosis and its ocular manifestations

Rheumatic features of sarcoidosis

Cutaneous sarcoidosis: differential diagnosis

Schistosoma mansoni and S. haematobium infections in Egypt. I. Evaluation of techniques for recovery of worms and eggs at necropsy. Am I

A quantitative post-mortem study of schistosomiasis mansoni in man

Immunodiagnosis of schistosomiasis. Screen with FAST-ELISA and confinn with imrnunoblot

Hepatic connective tissue changes in hepatosplenic schistosomiasis

Schistosomiasis in women: manifestations in the upper reproductive tract

Sclerosis, systemic

Amyotrophic Lateral (See "Disease, motor neuron

Diffuse (See "Sclerosis, Schilder's cerebraI

Multiple Synonyms and Related Terms: Acute and subacute variants: Acute multiple sclerosis (Marburg type), acute necrotizing myelopathy; concentric sclerosis (Ba16 type); concentric lacunar leukoencephalopathy; encephalitis periaxialis diffusa (Schilder's type; see also next entry

Silicone ganuloma in acral skin in a patient with silicone-gel implants and systemic sclerosis

Organizing diffuse alveolar damage associated with progressive systemic sclerosis

Bayle 0, Fiessinger IN. Brain involvement in scleroderma: two autopsy cases

Skeletal muscle pathology in systemic sclerosis

Mechanisms of scleroderma-induced lung disease

Thberous sclerosis complex and subependymal giant cell astrocytoma

Bilateral temporal arachnoid cysts associated with tuberous sclerosis

Poisoning, alkaloid

Accident, diving [skin or scuba

Scurvy (See "Deficiency, vitamin C

Shigellosis (See "Dysentery, bacillary

Shock Related Terms: Anaphylactic shock; bacteremic shock; many others. NOTE: See also under name of suspected underlying condition, such as "Bums

Intestinal histological and ultrastructura1 inflammatorychanges in spondyloarthropathy and rheumatoid arthritis

The sacroiliac joint in the spondyloarthropathies

Granulomatous ileitis in a patient with ankylosing spondylitis

Sporotrichosis Synonym: Sporothrix (Sporotrichum) schenckii infection. NOTE: (1)

McKiernan 1. Partial lipodystrophy in coeliac disease

Coeliac disease and lymphoma: current status

Review: nonalcoholic steatohepatitis

Alcoholic and non-alcoholic Wernicke's encephalopathy. Be alert to the preventable and treatable disease

Non-alcoholic fatty liver disease in the metabolic syndrome

The placenta in stillbirth

Estimating the time of death in stillborn fetuses: I. Histologic examination of fetal organs: an autopsy study of 150 stillborns

Estimating the time of death in stillborn fetuses: II. Histologic evaluation ofthe placenta; a study of71 stillborns

Estimating the time ofdeath in stillborn fetuses: III. External fetal examination; a study of86 stillborns

Fat distribution in the adrenal cortex as an indication of the mode of intrauterine death

Stimulant(s) (See "Dependence, drug(s), all types or type undetermined

Cutaneous manifestations of the acquired immunodeficiency syndrome

AIDS: cardiac findings from 115 autopsies

The liver in HIV infection

Neuropathology of the spinal cord in the acquired immunodeficiency syndrome

Postmortem enzyme immunoassay for human immunodeficiency virus

BK virus-associated renal failure among HIV patients

-2100 or write to American Registry ofPathology Sales Office

Pathology of acquired immunodeficiency syndrome (AIDS) in children

Thymus involution in the acquired immunodeficiency syndrome

Micrencephaly in children congenitally infected with human immunodeficiency virus-a gross-anatomical morphometric study

A rapid method for detecting the predominant Ashkenazi Jewish mutation in the Bloom's syndrome gene

Bloom syndrome: multiple retinopathies in a chromosome breakage disorder

Severe eczema in a patient with Di-George's syndrome

DiGeorge's syndrome orthe III -IV pharyngeal pouch syndrome: pathology and a theory of pathogenesis

Basal ganglia calcification and psychosis in 22q 11.2 deletion syndrome

Down's Synonyms and Related Terms: Mongolism

Possible Associated Conditions: Acute lymphocytic, my

References Epicanthal folds; cleft lip/palate; high arched palate; furrowed tongue; rhagades. Depressed nasal bridge

small, broad or fiat nose; slanted palpebral fissures; fiat occiput; brachycephaly; palmar "simian crease"; short fifth middle finger

Ventricular septal defect;* complete atrioventricular septal defect* (1). Duodenal stenosis and atresia; imperforate anus. Microcephaly; poorly developed secondary gyri

hypoplastic brain stem, medulla and cerebellum; polymicrogyria; neurofibrillary tangles; meningomyelocoele. Acute lymphocytic leukemia; acute myelocytic leukemia; acute megakaryocytic leukemia

Cardiovascular disease in Down syndrome

Possible or Expected Findings Hyperelasticity of skin, bruises or scars, hemorrhages, and hyperpigmentation. Lipomatous pseudotumors that may be calcified or ossified. Normal or abnormal amounts and fragmentation of elastic tissue. Abnormalities of collagen. Dislocation of hip, shoulder, patella, radius, or clavicle. Loose-end clavicles; spondylolisthesis

Mitral valve prolapse; Aortic insufficiency.* Aortic dissection, aneurysm, ectasia, occlusion (1). Various congenital anomalies. Congenital anomalies of gastrointestinal and genitourinary tracts. Bleeding from various organ sites

Vascular Ehlers-Danlos syndrome: imaging findings

NOTE: This syndrome belongs to a large family (with more photograph abnormal features as listed in right-hand column. Record appearance of external genitalia. Prepare skeletal roentgenograms. Procure long bones and vertebral bodies Short-limb dwarfism* with narrowing of the rib cage; polydactyly; dysplasia of fingernails; thin and sparse hair; premature eruption of teeth; defective dentition; eye abnormalities; upper lip bound down by multiple frenula. Cryptorchidism; epispadias; hypospadias. Chondrodysplasia with acromelic micromelia (shortening of the distal segment of the limb); fusion of capitate and hamate bones of wrist; defects of lateral aspect of proximal tibia

See lesions listed above under "External examination

Empty Sella Synonyms and Related Terms: Primary empty sella syndrome; secondary empty sella syndrome (e.g., after surgical removal or spontaneous infarction of pituitary adenoma)

Chorea, philic pneumonia: Histopathologic features in nine cases. Am Rev Resp hereditary

Ascariasis and hookworm

Chronic eosinophilic pneupophosphatemic vitamin D-resistant rickets; galactosemia;transport defect; tyrosinemia. * Excludes Fan-1988

weight and external measurements; record and photograph abnormalities as listed in right-hand column. Prepare skeletal roentgenograms. Radiographs of long bones. Submit for tissue culture for possible enzyme analysis. Possible or Expected Findings Redlblond hair, fair skin (diminished pigmentation); dehydration;* stigmata of hypothyroidism;* delay of sexual maturation. Rickets; osteomalacia.* Positive rheumatoid factor. Various types of pneumonia

After splenectomy, presence of accessory spleen may account for treatment failure. Manifestations of portal hypertension.* Lymphadenopathy of mediastinal and paraaortic lymph nodes. Anemia;* neutropenia

Sinusoidal lymphocytosis of the liver in Felty's syndrome with a review of the liver involvement in Felty's syndrome

Splenic involvement in rheumatic diseases

Felty's and pseudo-Felty's syndrome

Nodular regenerative hyperplasia of the liver in rheu-matic diseases: report of seven cases and review of the literature

Fetal alcohol syndrome: craniofacial and central nervous system manifestations

Goodpasture's syndrome

Anti-glomerular basement membrane glomerulonephritis: a morphologic study of 80 cases

Gronblad-Strandberg (See "Pseudoxanthoma elasticum

Acute inflammatory polyradiculoneuropathy; Guillain-Barre-Strohl syndrome

Hemolytic uremic syndrome/thrombotic thrombocytopenic purpura: pathophysiology and treatment

Cyclosporin-induced hemolytic uremic syndrome: factors that obscure its diagnosis

Enterohemorrhagic Escherichia coli 0157:H7: an emerging pathogen

Hemolytic uremic syndrome as a presenting form of HIV infection

Hemolytic uremic syndrome in prostatic carcinoma

Diabetes secondary to genetic disorders. Baillieres

Suprasellar tumors of maldevelopmental origin in Klinefelter's syndrome. A report of two cases

Klippel-FeU Synonym: Congenital fusion of cervical vertebrae

Primary yolk sac tumor of the prostate in a patient with Klinefelter's syndrome

Extragonodal germ cell tumors are often associated with Klinefelter syndrome

Organs and Tissues Procedures Possible or Expected Findings External examination Prepare roentgenograms of chest, neck, and head

Skull, spine, brain, and spinal cord

Myasthenia gravis

Disorders with dysraphia (see below), Fusion of cervical vertebrae. Congenital elevation of the scapula (Sprengel's deformity)

Chiari malformation;* basilar impression;* meningomyelocele; platybasia;* spinal cord compression; weight and length; record and photograph abnormalities as listed in right-hand column. Record weight and sample for histologic study. If renal transplantation (3) had been carried out, see also under that heading. Follow procedures described under "glomerulonephritis

Retinal dystrophy (1) and other retinal changes

The cardinal manifestations of Bardet-Biedl syndrome, a form of Laurence-Moon-Biedl syndrome

Laurence-Moon-Biedl syndrome accompanied by congenital hepatic fibrosis

Pediatric renal transplantation in Laurence-Moon-Biedl syn-drome

Possible or Expected Findings Typical skeletal proportions. Arachnodactyly; pectus excavatum

genu recurvatum; dislocation of joints

Diaphragmatic hernia. * Infective endocarditis. * Atrial septal defect. * Myxomatous transformation of mitral ring. Mitral valve prolapse. Aortic and pulmonary dilatation and valvular insufficiency.* Aortic dissection* with dissection of adjacent vessels. Ascending aortic aneurysm

Multiple cysts (see "Cyst(s), pulmonary")

Aortopulmonary fistula: an uncommon complication in dystrophic aortic aneurysm

Peripartum acute myocardial infarction in Marfan's syndrome

Aneurysm of the cervical internal carotid artery associated with Marfan's syndrome--ease report

Noonan's syndrome in association with acute leukemia

Lymphatic dysplasia in a neonate with Noonan's syndrome

Noonan syndrome: a clinical description emphasizing the cardiac findings

Cerebral arteriovenous malformation in Noonan's syndrome

Clinical variability in a Noonan syndrome family with a new PTPNII gene mutation

Peutz-Jeghers syndrome

Bilateral large-cell calcifying Sertoli cell tumor of the testes in Peutz-Jeghers syndrome: a case report

Ovarian tumors associated with the Peutz-Jeghers syndrome

Robin sequence and a deficiency of the left forearm in a girl with a deletion of chromosome 4g33-gter

Congenital heart disease in the Pierre Robin syndrome

Glossoptosis-apnea syndrome

Chiari I malformation, caudal regression syndrome, and Pierre Robin syndrome: previously unreported combination

Risk factors for squamous cell carcinoma of the esophagus

Heterotaxy syndrome; Ivemark's syndrome

Possible Associated Conditions: With asplenia: Right isomerism (bilateral mirror-image right-sided symmetry) of heart, lungs, and abdominal viscera; common atrium; total anomalous pulmonary venous connection; absent coronary sinus; complete atrioventricular septal defect;* subpulmonary stenosis;* pulmonary valve atresia;* midline symmetric liver; malrotation of bowel; absent spleen. With polysplenia: Left isomerism (bilateral mirror-image left-sided symmetry) ofheart and lungs, with variable sidedness ofabdominal viscera

Primary immunodeficiencies

Reiter's syndrome-like patternin AIDS-associated psoriasiformdermatitis

Reference Pneumothorax;* pneumomediastinum;* pneumoperitoneum. Septicemia. Right ventricular hypertrophy and/or dilatation. Intubation trauma and mural edema and hemorrhage in trachea; hyaline membrane disease

Intubation trauma. Hemorrhages or other evidence of birth trauma; germinal matrix hemorrhages in premature infants; infarctions of subcortical white matter in term infants

Coalson 11. Pathology of bronchopulmonary dysplasia

Pathology of arrested acinar development in postsurfactant bronchopulmonary dysplasia

Inborn errors of metabolism and Reye's syndrome: differential diagnosis

Prevalence and non-specificity of microvesicular fatty changes

Liver histopathology in clinical Reye syndrome

Ultrastructural study of intranuclear inclusions in the exo-crine pancreas in Reye's syndrome

Neuropathologic findings in Reye syndrome

Sanfilippo's (See "Mucopolysaccharidosis

Scheie's (See "Mucopolysaccharidosis

Segawa's (See "Syndrome, dystonia

Severe Acute Respiratory (SARS) Note: This highly contagious illness, caused by a coronavirus (SARS-CoV)details, see

Biosafety level 3 laboratory for autopsies of patients with severe acute respiratory syndrome: principles, practices, and prospects

Pathology and pathogenesis of severe acute respiratory syndrome

Pulmonary involvement in primary Sjogren's syndrome

Polymyositis and Sjogren's syndrome associated with bronchiolitis obliterans organizing pneumonia

The gastrointestinal manifestations of Sjogren's syndrome

Clinicopathological factors relating malignant lymphoma with Sjogren's syndrome

Acute transverse myelopathy and cutaneous vasculopathy in primary Sjogren's syndrome

Primary localized cutaneous amyloidosis with unusual clinical features in a patient with Sjogren's syndrome

Disease, Parkinson's

Erythema multiforme

Man Related Terms: Armadillo disease; continuous muscle fiber activity; neuromyotonia

Autoimmune central nervous system paraneoplastic disorders: mechanisms, diagnosis, and therapeutic options

Sudden Infant Death (SIDS) (See "Death, Sudden unexpected, of Infant.") Syndrome, Superior Vena Cava Related Term: Superior vena cava obstruction

Continue dissection of veins into neck and axillas. Record and photograph site of thrombosis or of compression by surrounding pathologic conditions. Submit samples for histologic study. Benign or malignant tumors; fibrosing mediastinitis;* postradiation fibrosis; infectious disease (tuberculosis,* histoplasmosis*); thoracic aortic aneurysm;* chronic constrictive pericarditis;* chest trauma; arteriovenous fistula between ascending aorta and superior vena cava

Toxic Shock Synonyms and Related Terms: Staphylococcal scarlet fever. NOTE: (1) Collect all tissues

This is a reportable disease. Premature aging; increased number of pigmented nevi; infantile sex organs; webbed neck; broad chest with wide spacing of nipples. Short fourth metacarpal; abnormal epiphyseal fusions; osteochondrosis-like changes of spine

Bicuspid aortic valve;* coarctation of aorta. * Rarely other anomalies (1)

Decreased/absent follicles. Intestinal telangiectases. Biliary atresia. * Horseshoe kidney; double ureters. Streak gonads without germ cells or follicles

Hashimoto's thyroiditis. * Manifestations of diabetes mellitus,* hypertension,* or thyrotoxicosis. Neuroblastoma and related tumors (2). Keratoconus

Acute aortic dissection, aortic insufficiency, and a single coronary artery in a patient with Turner's syndrome

Neuroblastoma and related tumors in Turner's syndrome

Turner's syndrome associated with bilateral retinal detachments

Weil's (See "Leptospirosis

Related Terms: Alcoholic Wernicke's encephalopathy; Korsakoff's psychosis; nonalcoholic Wernicke's encephalopathy (1,2); and specimen preparation, see Chapter 4. For selection of histologic samples, see right-hand column

Melissas 1. Wernicke's encephalopathy after vertical banded gastroplasty for morbid obesity

Syndrome, respiratory distress, of infant.") Syndrome, Wiskott-Aldrich (See "Syndrome, primary immunodeficiency

Malformation, Ebstein's" and "Preexcitation, ventricular

Alcoholic and non-alcoholic Wernicke's encephalopathy. Be alert to the preventable and treatable disease

cerebro-hepato-renal syndrome; infantile Refsum's disease. * NOTE: This congenital familial cholestatic syndrome results from impaired assembly of peroxisomes and has its main manifestations in the brain, liver, and kidneys

Postmortem findings and prenatal diagnosis ofZellwegersyndrome. Case report

Alcoholism and alcohol intoxication" and "Disease, alcoholic liver

Possible Associated Condition: Multiple endocrine neoplasia

Primary cardiac gastrinoma causing Zollinger-Ellison syndrome

Localization, Syphilis, Acquired Synonym: Treponema pallidum infection. NOTE: Congenital syphilis is presented below under a separate heading

and surgical management of gastrinoma

Fundic argyrophil carcinoid tumor in a patient with sporadic-type Zollinger-Ellison syndrome

Nonimmune hydrops fetalis due to congenital syphilis associated with negative intrapartum mater-nal serology screening

Syringomyelia Synonyms and Related Term: Hydromyelia; idiopathic syringomyelia; secondary syringomyelia; syringobulbia. Possible Associated Conditions: With idiopathic syringomyelia-Arnold Chiari malformation, type I;* basilar impression;* 2. Oppenheimer EH, Dahms BB. Congenital syphilis in the fetus and neonate

Congenital syphilis: detection of Treponema pallidum in stillborns

With secondary syringomyelia-Intramedullary gliomas (ependymoma, pilocytic astrocytoma) and vascular tumors; spinal arachnoiditis and pachymeningitislisted in right-hand column. Prepare roentgenograms of spine and joints. For removal and specimen preparation, see Chapter 4. For histologic sampling, see right-hand column. Hypertrophy of body parts. Muscle atrophy of upper extremities and hands

Cervical spinal cord is swollen and tense, with cavitation (syrinx) containing clear fluid. Wall of cavity consists of degenerated glial and neural elements, with marked gliosis. Spinal cord parenchyma is markedly compressed. See also above under

Hydrops fetalis caused by alpha-thalassemia: an emerging health care problem

Limb defects in homozygous alpha-thalassemia: report of three cases

Poisoning, thallium

Thirst (See "Dehydration

Thromboangiitis Obliterans (See "Disease, Buerger's

Purpura, thrombotic thrombocytopenic" and "Syndrome, hemolytic uremic

The pathological manifestations of pulmonary toxoplasmosis in the acquired immunodeficiency syndrome

A spectrum in the pathology of toxoplasmosis in patients with acquired immu Transfusion (See "Reaction to transfusion

Bone Marrow NOTE: If the patient had symptoms of acute or chronic graft-versus-host disease (GVHD), see "Disease, graftversus-host

Morphology and diagnostics of human toxoplasmosis

Fatal myocardial coinfection by Toxoplasma gondii and Parvovirus B 19 in an HIV patient

If there is evidence of infection, submit material for microbiologic studies. If there is evidence of recurrent leukemia or lymphoma, sample as described under those headings. If the patient had symptoms of thrombotic thrombocytopenic purpura (TIP) or hemolytic uremic syndrome (HUS), see these headings. Record average size. Fix specimens in B-Plus®. Make touch preparations. Request Giemsa or Wright stain. For preparation of sections and smears (imprints), see Chapter 2. Manifestations of graft-versus-host disease (e.g., scleroderma-like changes).* Septicemia. Interstitial pneumonia* and cytomegalovirus infection* or human herpesvirus 6 infection (1). Bacterial, fungal

Bacterial or fungal infections in GVHD.* Other manifestations of GVHD. * Recurrent leukemia,* lymphoma* (including posttransplant, Epstein-Barr virus associated lymphoproliferative disorder). Recurrent solid tumor, e.g., carcinoma of breast, lung (small cell carcinoma), ovary

Lymphomatous or leukemic infiltrates

Myeloid cells may be absent in marrow graft rejection or nonimmunologic marrow graft failure. References Hematomas; hemorrhagic necroses; infarcts; bacterial or fungal infections

leukoencephal-opathy; vascular siderocalcinosis; neuro-axonal spheroids (6)

Human herpesvirus 6 infection and associated pathogenesis following bone marrow transplantation

Pulmonary veno-occlusive disease in an adult following bone marrow transplantation. Case report and review of the literature

Acute renal failure following bone mar-row transplantation

Transplantation-associated thrombotic thrombocytopenic purpura and hemolytic uremic syndrome

Thrombotic microangiopathies associated with drugs and bone marrow transplantation

Record status of all vascular anstomoses. If there are hemorrhages, record volume and site. Record weight, ventricular thickness, and valve circumferences. Take multiple sections from all areas of myocardium. Cut coronary arteries in cross sections; request Verhoeff-van Gieson stain. Procedures depend on expected findings or grossly identified abnormalities as listed in right-hand column. References Cushingoid features (see "Syndrome, Cushing's") after steroid therapy. Suture dehiscence. Hemothorax or hemomediastinum in recent cases. Left ventricular hypertrophy, from cyclosporine-related hypertension. Acute transplant rejection (for grading, see ref. 1). Chronic transplant vasculopathy with coronary artery stenosis (2)

Opportunistic infection (1). Pneumonia

A working formulation for the standardization of nomenclature in the diagnosis of heart and lung rejection: Heart and Lung Rejection Study Group

Autopsy findings in cardiac transplant patients: a lO-year experience

Fulminant toxoplasmosis following heart transplantation

Banff schema for grading liver allograft rejection: an international consensus document

Record status of all vascular anstomoses. If there are hemorrhages, record volume and site. Record lung weights separately. If lung infection is suspected, submit material for microbiologic Possible or Expected Findings Cushingoid features (see "Syndrome, Cushing's") after steroid therapy. Suture dehiscence. Hemothorax or hemomediastinum in recent cases. Opportunistic infection (in patients with a single lung transplant, infections may involve References the nontransplant lung, as well). Chronic bronchitis* and bronchiectasis.* Acute rejection (rare); chronic airway rejection (obliterative bronchiolitis); chronic vascular rejection. (For grading of rejection, see ref 1.) Post-transplant lymphoprolijerative disorder

A working formulation for the standardization of nomenclature in the diagnosis of heart and lung rejection: Heart and Lung Rejection Study Group

Recurrent Salmonella enteritidis and hepatic tuberculosis

TUlaremia Synonyms and Related Terms: Francisella tularensis infection; Pasteurella tularensis infection

Cerebral abscesses complicating tularemia meningitis

Endocrine (See "Neoplasia, multiple endocrine

Any Type NOTE: Ifthe tumor had been treated by surgery, irradiation, chemotherapy, or other means (the most common situation

Possible Associated Conditions: With adrenocortical adenoma-Conn's syndrome

With adrenocortical carci-noma-Cushing's syndrome;* hypoglycemia;* virilization. With pheochromocytoma-cerebellarhemangioblastoma

ery-throcytosis; hypercalcemia; hypertension,* multiple enarteries Record body weight and abnormal features. Photograph skin changes. Record heart weight and thickness of ventricles. Procure multiple sections of myocardium

Possible Associated Conditions.") Focal myocarditis. * Hypertensive cardiovascular disease (See "Hypertension

Myocardial infarction without severe coronary artery disease (with pheochromocytoma)

Asystematicreviewoftheassociationbetween Barrett's esophagus and colon neoplasm

Malakoplakia and colorectal adenocarcinoma

An unusual case of colonic mixed adenoendocrine carcinoma: collision vs composite tumor. A case report and review of the literature

Submit lymph nodes for histologic study. Remove neck organs with tongue together with esophagus. Open pharynx and esophagus in posterior midline. If fistulas and abscesses are suspected, dissect esophagus but leave attached to mediastinum, stomach and diaphragm. Record width of lumen of esophagus at various levels. Photograph and record size and location of tumor; sample tumor and uninvolved esophagus for histologic study. Request PAS stain of uninvolved esophagus (sample from all levels). Procedures depend on expected findings or grossly identified abnormalities as listed in right-hand column. References Cachexia. Eczematoid dermatitis with adult renal cell carcinoma. Cushing's syndrome,* galactorrhea or feminization or masculinization in some cases of renal cell carcinoma. Evidence of hyperalcemia. Pulmonary tumor embolism after invasion of renal vein and inferior vena cava. Acquired renal cystic disease

prolactin, renin, and prostaglandins in some renal cell carcinomas. See above under "Possible Associated Conditions

Wilms tumor associated with polycythemia: case report and review of the literature

Renal medullary carcinoma: a potential sickle cell nephropathy of children and adolescents

Acquired cystic kidney disease

Byler's disease, carcinoid syndrome,*cirrhosis* of any type (mostly nonbiliary), congenital hepatic fibrosis, * Cushing's syndrome,* erythrocytosis, genetic hemochromatosis, * glycogen storage disease (type 1),* hepatitis B or C virus infection, hereditary tyrosinemia (type I),* hypercalcemia, hypercholesterolemia, hypoglycemia,* neurofibromatosis,* Osler-Rendu-Weber disease* (hereditary hemorrhagic telangiectasia), polycythemia,* porphyria (acute intermittent and porphyria cutanea tarda), * pseudohyperparathyroidism,* and thorium (thorotrast) deposition. With bile duct carcinoma (cholangiocarcinoma}-Clonorchis sinensis or Opisthorchis viverrini infection, fibropolycystic liver and biliary tract disease,* hepatolithiasis, hypercalcemia, pri-mary sclerosing cholangitis,* and thorium (thorotrast) deposition. With hepatoblastoma-Alpha-fetoproteinemia, cardiac and renal malformations, cleft palate, diaphragmatic hernia,* Down's syndrome,* familial colonic polyposis,* hemihypertrophy, nephroblastoma. With angiosarcoma-Thorium (thorotrast) deposition. See also below under "Possible or Expected Findings

Describe and photograph cut surfaces. Sample tumor and nonneoplastic liver for histologic study. If thorium deposition is suspected, prepare roentgenograms of liver slices and submit samples for energy-dispersive x-ray microanalysis of paraffin sections. Procedures depend on expected findings or grossly identified abnormalities as listed above and in right-hand column. Cachexia. Precocious puberty. Feminization and gynecomastia in rare cases of HCC. Spider angiomas; clubbing of fingers. See above under "Possible Associated Conditions

Thorotrast storage may cause cirrhosis, HCC, bile duct carcinoma, or angiosarcoma. See above under "Possible Associated Conditions

Tumor of the Lung or Bronchus Possible Associated Conditions: Abnormal concentrations of hormons or other metabolites in blood and tumor tissue (adrenocorticotropic, antidiuretic, growth hormone, parathyroid-like substances, or 5-hydroxyindolacetic acid); acanthosis nigricans

s syndrome;*dermal hyperpigmentation; feminization; hyperglycemia; hypercalcemia; hypoglycemia;* hypokalemia; hyponatremia; precocious puberty. For syndromes affecting the brain, peripheral nerves or muscles, see below under "Possible or Expected Findings

Organs and Tissues Procedures Possible or Expected Findings External examination and skin Record body weight. Record abnormal features as listed in right-hand column; prepare photographs. Prepare histologic sections of normal and grossly abnormal skin

Clubbing of fingers; spider angiomas. For other rare tumor-related changes, see above under "Possible Associated Conditions

Thmor of the Pancreas Possible Associated Conditions: With carcinoma of the exocrine pancreas--Cushing's syndrome;* diabetes mellitus* (rare); hypercalcemia; hyperglycemia

With islet cell tumor-Abnormal concentrations of hormons in blood and tumor tissue (see below under "pancreas"); features. Dermal hyperpigmentation. For other rare tumor-related changes, see above under "Possible Associated Conditions

Biliary obstruction caused by tumor in head of pancreas. Islet cell tumor with adrenocorticotropic hormone, gastrin, glucagon, insulin, or other peptide hormones. See above under "Possible Associated Conditions

Paraneoplastic pemphigus associated with pancreatic carcinoma

Pancreatic cystadenocarcinoma in Peutz-Jeghers syndrome

Testes may have been surgically removed to achieve androgen deprivation. Urinary obstruction and hydronephrosis. * Osteoblastic metastases

Hemolytic uremic syndrome in prostatic carcinoma

Thmor of the Small Intestine Possible Associated Conditions: Acquired immunode

Oncogenic osteomalacia associated with prostatic cancer

Gardner's syndrome)

Submit sample of non-neoplastic small bowel for histologic study. Procedures depend on expected findings or grossly identified abnormalities as listed above. Cachexia. Mucocutaneous pigmentations associated with Peutz-Jeghers syndrome. * Carcinoid tumor. Lymphoma (see also above under "Possible Associated Conditions"). Familial polyposis or related syndrome. * Celiac sprue. * Crohn's disease. * See above under "Possible Associated Conditions

Kasabach Merritt syndrome in infants

Paraneoplastic hepatopathy associated with soft tissue sarcoma

Neurofibro-Thmor of the Spinal Cord Possible Associated Conditions: With angioma-cerebellar hemangioblastoma; segmental cutaneous vascular nevi; with matosis in children with soft tissue sarcoma

tissues Record abnormal features; photograph and submit nevi for histologic study. For removal and specimen preparation, see Chapter 4. See also below under "Vertebral column

Bone erosion and calcification of spinal canal. Vertebral hemangiomas may be associated with arteriovenous malformation of spinal cord. Manifestations of von Hippel-Lindau disease. * Thmor of the Stomach Possible Associated Conditions: Hypoglycemia;* megaloblastic anemia;* skin changes (as listed under "Possible or Expected Findings)weight. Record abnormal features; photograph and submit samples of normal and abnormal skin for histologic study. Possible or Expected Findings Cachexia; lower leg lymphoedema (3)

Metastases common in liver, retroperitoneal and supraclavicular lymph nodes (Virchow's node), ovaries (Krukenberg tumor), and peritoneal cuI de sac (Blumer's shelf')

Carcinoma of the stomach with hyperkeratosis palmaris and plantaris and acanthosis of the esophagus

Gastric lymphoma ofmucosa-associated lymphoid tissue and Helicobacter pylori

Gastric signet-ring cell carcinoma: unilateral lower extremity lymphoedema as the presenting feature

Thmor of the Testis Possible Associated Conditions: Demyelinating neuropathy

Record location, size, and weight of both testes and of testicular tumor. Submit samples of tumor and of ininvolved testis and epididymis for histologic study. Snap-freeze tumor tissue for hormone assay. Possible or Expected Findings Cachexia. Cryptorchism; hypospadia. Gynecomastia. Increased concentrations of alphafetoprotein and human chorionic gonadotropin

Testicular microlithiasis. Secondary testicular tumors (metastases to the testes) are rare, except in association with leukemia* in children. References Pulmonari embolism.* Paraneoplastic diseases as listed above under "Possible Associated Conditions

Chronic inflammatory demyelinating polyneuropathy (ClOP) associated with seminoma

Dermatomyositis associated with intratubular germ cell tumor and metastatic germ cell cancer

Testicular cancer in association with developmental renal anomalies and hypospadias

Submit samples of lymph nodes, spleen, Peyer's plaques, and bone marrow for histologic study. Other procedures depend on expected findings or grossly identified abnormalities as listed in right-hand column. References Cachexia. Dermal hyperpigmentation. Pemphigus foliaceus (rare). Hypogammaglobulinemia and other immunoglobulin abnormalities. Usually, thymoma presents as an infiltrating, anterior mediastinal mass that rarely metastasizes. Carcinoid tumor, malignant lymphoma* (Hodgkin's disease), and metastases from carcinomaofthe breast* and other tumors also may occur in this location

herpes simplex*) and fungal infections (e.g., candidiasis*) due to thymoma-related immunodeficiency (3). Manifestations of paraneoplastic diseases and conditions as listed above under "Possible Associated Conditions

Thrombotic thrombocytopenic purpura accompanied by transient pure red cell aplasia and thymoma

Glomerulonephritis associated with myasthenia gravis

Thymoma and cellular immune deficiency in an adolescent

Possible Associated Conditions: With papillary carcinoma-Familial adenomatous polyposis

Thyroid carcinoma associated with familial adenomatous polyposis

An association between acromegaly and thyroid carcinoma

Thyroid carcinoma causing fatal laryngeal obstruction

Prostate cancer metastasis to thyroid gland

Schistosomiasis and the risk of bladder cancer in

Human papilloma virus infection (2) or herpesvirus infection (3) with invasive cervical carcinoma. Erythropoietin may be found in some tumors. Thrombosis. * Myopathy* may be associated with carcinoma of the cervix. Cerebellar cortical degeneration* may be associated with carcinoma of the uterus. Manifestations of uremia (see "Failure, kidney")

Invasive cervical cancer in human immunodeficiency virus-infected and uninfected hospital patients

Association of risk factors for cervical cancer and human papilloma viruses in invasive cervical cancer

Association of herpesvirus infection with the development of genital cancer

Tyrosinemia type II: a challenge for ophthalmologists and dermatologists

Hereditary tyrosinemia type I (chronic form): Pathologic findings in the liver

Richner-Hanhart syndrome (tyrosinemia II): early diagnosis of an incomplete presentation with unusual findings

Acute pancreatitis-associated acute gastrointestinal mucosal lesions: incidence, characteristics, and clinical significance

Uncinariasis (See "Ancylostomiasis

Uremia (See "Failure, kidney

From: Handbook of Autopsy Practice

Urticaria Pigmentosa of Childhood (See ''Mastocytosis, systemic

Acute aortic dissection;* aneurysma(s) of cerebral arteries; aortic insufficiency;* calcific aortic stenosis,* coarctation of the aorta;* infective endocarditis;* Shone's syndrome; Turner's syndrome. * Organs and Tissues Procedures Possible or Expected Findings Heart If infective endocarditis is suspected, see Chapter 7. Open heart in cross-sections (see Chapter 3). Photograph aortic valve and test valvular competence

NOTE: Reye's syndrome* is a possible postviral complication of varicella that must be distinguished from varicella encephalitis. Varicella may also cause exacerbation of tuberculosis.* (I) Collect all tissues that appear infected

Bicuspid aortic valves are associated with aortic dilatation out of proportion to coexistent valvular lesions

Varicella-zoster virus infection in children with underlying immunodeficiency virus infection

Group A beta-hemolytic streptococcal epiglottitis as a complication of varicella infection

Optic neuritis: a rare complication of primary varicella infection

HSV-l-induced acute retinal necrosis syndrome presenting with severe inflammatory orbitopathy, proptosis, and optic nerve involvement

Varicella with acute motor axonal neuropathy

Descending necrotizing mediastinitis in a child with chicken pox

Double outlet right ventricle with intact ventricular septum

Respiratory Syncytial (See "Pneumonia, all types or type unspecified

Vitamin A (See ''Deficiency, vitamin A" and "Hypervitaminosis A

Vitamin Bt (Thiamine) (See "Syndrome, Wernicke-KorsakotT

Vitamin Bt2 (See "Anemia, megaloblastic

Deficiency

Deficiency, vitamin D" and "Hypervitaminosis D

Waldenstrom's macroglobulinemia (See ''Macroglobulinemia, Waldenstrom's.)

Waterhouse-Friderichsen syndrome (See "Disease, meningococcal

Wegener's granulomatosis (See "Granulomatosis, Wegener's

Werdnig-HotTman disease (See "Disease, motor neuron

Acid esterase activity in cultured skin fibroblasts and amniotic fluid cells using 4-methylumbelliferyl palmitate

which may be secondarily infected by bacteria. Dark field microscopy will identify the organism

Expected Findings Mucosal ulcers, resembling those of typhoid fever, primarily of distal ileum and colon. Villous shortening, crypt hyperplasia with mucosal microabscesses. Microabscesses in regional mesenteric lymph nodes

Zellweger's syndrome (See "Syndrome

Mucormycosis" and procedures under "Diabetes mellitus

Abscesses and granulomas may occur in all organs and tissues. Granulomatous lesions in central nervous system (4) and eyes (5). Fungal osteomyelitis* that may be multifocal, including sites such as metacarpals and metatarsals. 

External examination Other procedures depend on expected findings or grossly identified abnormalities as listed in right-hand column.

Osteosclerosis of vertebrae, ribs, clavicles, pelvic bones, scapulae, skull, and metaphyseal ends of femur and humerus. Osteomyelofibrosis or osteoreticulosis; rarely, panhyperplasia of bone marrow; increase of megakaryocytes.Changes associated with chronic granulocytic leukemia* or with polycythemia vera* may imitate idiopathic myelofibrosis. Widespread extramedullary hematopoiesis (1) with splenomegaly. Infectious diseases, including tuberculosis;* gouty arthritis; ascites and other manifestations of portal hypertension;* or hepatic vein thrombosis (Budd-Chiari syndrome*). Cardiac tamponade (1) .

Synonyms and Related Terms: Hepatic porphyria; protoporphyrinogen oxidase deficiency (1) .NOTE: The manifestations of the disease closely resemble those in porphyria cutanea tarda and hereditary coproporphyria. Measurements of porphyrins and porphyrin precursors are the only clearly distinguishing features. For autopsy procedures, see under "Porphyria cutanea tarda."

Fibrin and platelet thrombi, with or without microaneurysms and purpura in kidneys, adrenal glands, pancreas, heart, and brain; lesions may also be present in liver; spleen (3), lymph nodes, muscle, bone marrow, and synovium. Fibrin thrombi can be demonstrated with labeled antihuman fibrin antibodies. Lesions in precapillary arterioles. 

Hypertrophic osteoarthropathy* (with pleural mesothelioma).Pleural effusions. * Asbestosis may be complicated by pleural mesothelioma.

Other organs and tissue Sample bladder tumor and uninvolved urinary bladder for histologic study.Cachexia. Hydronephrosis* (obstructive uropathy) and hydroureter, usually caused by distal ureteral obstruction. Recurrent nephrolithiasis* or pyelitis may have been present and is a risk factor for urinary bladder carcinoma.Chronic urocystitis; infestation with Schistosoma haematobium (1) (uncommon in North America). Manifestations of uremia (see "Failure, kidney").