Info

right-hand column.

kidney failure.*

1. Kurumaya H, Kono N, Nakanuma Y, Tomoda F, Takazahura E. Hepatic granulomata in long-term hemodialysis patients with hyperalbuminemia. Arch Pathol Lab Med 1989;113:1132-1134.

Reference

1. Kurumaya H, Kono N, Nakanuma Y, Tomoda F, Takazahura E. Hepatic granulomata in long-term hemodialysis patients with hyperalbuminemia. Arch Pathol Lab Med 1989;113:1132-1134.

Diathesis, Bleeding (See "Coagulation, disseminated intravascular," "Disease, Christmas," "Disease, von Willebrand's," and "Hemophilia."

Digitalis (See "Poisoning, digitalis.")

Diphtheria

Synonyms: Corynebacterium diphtheriae infection; diphtheric fever.

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. (2) Request aerobic bacterial cultures. (3) Request Gram stain (p. 172). (4) Special precautions are indicated (p. 146). (5) Serologic studies are not helpful, but the organism may be typed for epidemiologic purposes. Toxin assays are also available. (6) This is a reportable disease.

Organs and Tissues

Procedures

Possible or Expected Findings

Head and neck

Heart Kidneys

Brain and peripheral nerves

Nasal cavities, sinuses, and middle ears

Remove neck organs with oropharynx, tongue, tonsils, soft palate, and uvula. Record degree of laryngeal obstruction. Photograph larynx and pharynx before and after opening. Submit sample of pharyngeal pseudomembranes for culture; prepare smears of membranes. Photograph. Record weight and submit samples for histologic study (see p. 30). Submit samples for histologic study.

For removal and specimen preparation, see pp. 66 and 70, respectively. Request Luxol fast blue stain (p. 172). For exposure of epipharynx, nasal cavities, sinuses, and middle ears, see pp. 71-73. Prepare smears and swab cultures of these spaces. Photograph, prepare histologic sections, and request Gram stain (p. 172).

Diphtheric pharyngitis.

Gram-positive pleomorphic bacilli.

Diphtheric myocarditis.

Nonsuppurative interstitial nephritis. Renal tubular necrosis.* Myelin degeneration and destruction of myelin sheaths.

Diphtheritic pseudomembranes.

Disease,... (See subsequent entries and under "Sickness,..." and "Syndrome,...")

Disease, Addison's (See "Insufficiency, adrenal.")

Disease, Albers-Schönberg (See "Osteopetrosis.")

Disease, Alcoholic Liver

Related Terms: Alcoholic cirrhosis; alcoholic fatty liver; alcoholic hepatitis.

NOTE: Several conditions such as obesity-related steatohepatitis may be histologically indistinguishable from alcoholic liver disease (1). Thus, the diagnosis should not be based on liver histology alone.

Organs and Tissues

Procedures

Possible or Expected Findings

External examination

Serosal cavities Blood and urine

Heart Lungs

Esophagus Liver

Record presence or absence of features listed in right-hand column.

Record volume of effusions.

Submit samples for alcohol determination and other toxicologic studies.

Prepare frozen sections for fat stains.

For demonstration of varices, see p. 53. Record weight and sample for histologic study.

Jaundice; clubbing of fingers; Dupuytren's contractures; decreased body hair and gynecomastia in men. Ascites; pleural effusions.* Alcoholic cardiomyopathy* (2).

Record weight. Alcoholic cardiomyopathy* (2). Fat embolism* (if severe, systemic circulation may be involved—for instance, kidneys and brain). Esophageal varices.

Micro- or macronodular alcoholic cirrhosis; alcoholic hepatitis (steatohepatitis); alcoholic fatty liver. Hepatocellular carcinoma. Typical groundglass changes in some patients who were treated with disulfiram or cyanamide (3).

Organs and Tissues

Procedures

Possible or Expected Findings

Portal vein system

Spleen

Pancreas

Brain, peripheral nerves, skeletal muscles, and other organs

Testes

Record weight.

For removal of muscles, peripheral nerves, and brain, see pp. 65, 67, and 85, respectively.

For removal of lacrimal glands, see p. 87. Remove parotid tissue from scalp incision (p. 65) with biopsy needle. Record weights.

See "Hypertension, portal." Congestive splenomegaly. Alcoholic pancreatitis.* Myopathy; neuropathy;* see also under "Alcoholism and alcohol intoxication" and "Syndrome, Wernicke-Korsakoff." Parotid and lacrimal gland enlargement with increased glandular secretions.

Testicular atrophy.

References

Yokoyama A, Sato S, Maruyama K, Nakano M, Takahashi H, Okuyama K, et al. Cyanamide-associated alcoholic liver disease: a sequential histologic evaluation. Alcohol Clin Exp Res 1995;19:1307-1311.

1. Kanel GC. Hepatic lesions resembling alcoholic liver disease. Pathology 1994;3:77-104.

2. Estruch R, Fernandez-Sola J, Sacanella E, Pare C, Rubin E, Urbano-Marquez A. Relationship between cardiomyopathy and liver disease in chronic alcoholism. Hepatology 1995;22:532-538.

Disease, alpha-Chain (See "Disease, heavy-chain.") Disease, Alzheimer's

Synonyms and Related Terms: Alzheimer's dementia; presbyophrenic dementia; presenile dementia syndrome. NOTE: For pathogenesis and criteria for staging, see refs. (1-3).

Organs and Tissues

Procedures

Possible or Expected Findings

Brain and spinal cord

For removal and specimen preparation, see pp. 65 and 67, respectively. Record brain weight. Histologic sections should include frontal, temporal, occipital, cingulate, enthorinal, and amygdala, hippocampus, deep nuclei and thalamus, substantia nigra, and occipital cortex and hippocampus. For silver impregnation of paraffin sections, request Bielchowsky silver stain (p. 172). Immunostain for PA4 and tau protein are available for plaques and tangles. Some tissue samples should be kept frozen for biochemical studies.

Cortical atrophy, particularly of frontal and temporal lobes, with dilatation of ventricles. Neuronal loss and reactive astrocytosis; characteristic senile plaques (argentophilic neuritic plaques) and Alzheimer's neurofibrillary tangles. In some cases, cerebral meningeal and cortical blood vessels show amyloid angiopathy.

References

Esiri MM, Hyman BT, Beyreuther K, Masters CL. Aging and dementia in Greenfield's Neuropathology, vol. 2. Graham BI, Lantos PL, eds. Arnold, London, 1997, pp. 153-233.

The National Institute on Aging, and Reagan Institute Working Group on Diagnostic Criteria for the Neuropathological Assessment of Alzheimer's Disease. Consensus recommendations for the postmortem diagnosis of Alzheimer's disease. Neurobiol Aging 1997;Jul-Aug;18(4 Suppl): S1-2.

The Ronald and Nancy Reagan Research Institute of the Alzheimer's Association and the National Institute on Aging Working Group. Consensus report of the Working Group on: Molecular and Biochemical Markers of Alzheimer's Disease. Neurobiol Aging 1998;Mar-Apr;19 (2):109-116. (Published erratum appears in Neurobiol Aging 1998; May-Jun;19(3):285.)

Disease, Atherosclerotic Heart (See "Disease, ischemic heart.")

Disease, Bornholm (See "Pleurodynia, epidemic.")

Disease, Bourneville's (See "Sclerosis, tuberous.")

Disease, Buerger's

Synonyms: Thromboangitis obliterans; Winiwater-Buerger syndrome.

Organs and Tissues

Procedures

Possible or Expected Findings

External examination

Extremities

Abdominal and visceral vasculature

Brain

Record presence or absence of abnormalities listed in right-hand column.

If permitted, submit samples from dorsal artery of the foot, and tibial, anterior fibular, popliteal, and femoral arteries. Include specimens of accompanying veins (p. 34). Section arteries and veins crosswise at different levels. Request Verhoeff-van Gieson stain (p. 173). Section veins that have gross evidence of thrombosis* or thrombophlebitis.*

Dissect abdominal aorta with iliac, mesenteric, and renal arteries. Dissect coronary arteries. Submit samples for histologic study, including Verhoeff-van Gieson stain (p. 173). For removal and specimen preparation, see p. 65. For cerebral arteriography, see p. 80.

Ischemic ulcers of digits; gangrene; amputations; elevated skin lesions accompanying thrombophlebitis.* Arterial lesions are often segmental. Digital arteries are involved more often than are ulnar and radial arteries. Thrombophlebitis* is part of the disease.

Thrombi in small and medium-sized vessels contain mixed inflammatory cells, giant cells, and sterile microabscesses. Later stages of the process are characterized by hypercellular intraluminal granulation tissues without medial scarring.

Thromboses in mesenteric, renal, and coronary arteries are rare. Aortoiliac disease is also rare. Manifestations of the Budd-Chiari syndrome* may be present. Cerebral artery involvement may be present and may be associated with cortical ischemic lesions.

Disease, Caisson (See "Sickness, decompression.")

Disease, Canavan's (See "Degeneration, spongy, of white matter.")

Disease, Caroli's

Synonyms and Related Terms: Caroli's syndrome; fibro-polycystic liver disease; idiopathic dilatation of intrahepatic bile ducts.

NOTE: The term "Caroli's syndrome" often is used for cases that also show histologic features of congenital hepatic 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.

Organs and Tissues

Procedures

Possible or Expected Findings

Blood

Liver and extrahepatic bile ducts

Kidneys Other organs

Submit samples for aerobic and anaerobic bacterial cultures (p. 102). If there are superficial abscesses or easily accessible cysts, sterilize capsule of liver and aspirate contents for aerobic and anaerobic cultures. Remove small and large bowel, and open duodenum in situ. Aspirate bile from gallbladder or dilated ducts for bacterial culture. For cholangiography, see p. 56. Open extra-hepatic bile ducts in situ and record width. Slice liver in frontal or horizontal plane and submit samples for histologic study. If abnormalities are present, prepare photographs prior to histologic sampling.

Septicemia.

Dilatation of the hepatic and common bile ducts (may not involve entire liver [1]); choledochal-type cyst;* hepatolithiasis; cholelithiasis;* choledocholithiasis; rupture of bile duct (3); suppurative cholangitis;* hepatic abscesses. Adenocarcinoma of bile ducts.

See above under "Possible Associated Conditions."

Manifestations of portal hypertension.*

References

1. Dagli U, Atalay F, Sasmaz N, Bostanoglu S, Temucin G, Sahin B. Caroli's disease: 1977-1995 experiences. Eur J Gastroenterol Hepatol 1998;10: 109-112.

2. Mousson C, Rabec M, Cercueil JP, Virot JS, Hillon P, Rifle G. Caroli's disease and autosomal dominant polycystic kidney disease: a rare association? Nephrol Dialysis Transplant 1997;12:1481-1483.

3. Chalasani N, Nguyen CC, Gitlin N. Spontaneous rupture of a bile duct and its endoscopic management in a patient with Caroli's syndrome. Am J Gastroenterol 1997;92:1062-1063.

Disease, Cat Scratch

Possible Associated Conditions: AIDS and other immunodeficient conditions.

Organs and Tissues

Procedures

Possible or Expected Findings

External examination and skin Heart

Liver

Other organs

Skeletal system Brain and spinal cord

If endocarditis is suspected, follow procedures described under that heading (p. 103). Sample for histologic study.

Photograph lesions that might have been caused by the infection.

Sample material for microbiologic and histologic study; prepare Gram stains (p. 172).

If osteomyelitis is suspected, follow procedures described under that heading. For removal and specimen preparation, see pp. 65 and 67, respectively.

Cat-scratch mark and lymphadenopathy. Endocarditis (1).

Granulomatous hepatitis; bacillary peliosis hepatis (2) (see also below under "Other organs").

Infection caused by Bartonella hensleae or Afipia felis. In patients with AIDS, bacillary (epithelioid) angiomatosis and bacillary peliosis hepatis are associated with B. hensleae (1) infection. Osteomyelitis.*

Encephalitis; meningitis; transverse myelitis.

References

1. Holmes AH, Greenough TC, Balady GJ, Regnery RL, Anderson BE, O'Keane JC, et al. Bartonella henselae endocarditis in an immunocompetent adult. Clin Inf Dis 1995;21:1004-1007.

2. Chomel BB. Cat-scratch disease and bacillary angiomatosis. Rev Scientifique Technique 1996;15:1061-1073.

Disease, Celiac (See "Sprue, celiac.")

Disease, Cerebrovascular (See "Attack, transient cerebral ischemic" and "Infarction, cerebral.")

Disease, Chagas'

Synonyms and Related Terms: American trypanosomiasis; Chagas' syndrome; Trypanosoma cruzi infection.

NOTE: (1) Collect all tissues that appear to be infected. (2) Request cultures for trypanosomiasis. (3) Request Giemsa stain (p. 172). (4) Special precautions are indicated (p. 146). (5) Serologic studies are available from the Centers for Disease Control and Prevention, Atlanta, GA (p. 135). (6) Usually, this is not a reportable disease.

Possible Associated Conditions: AIDS (1) and other conditions associated with immunosuppression.

Organs and Tissues

Procedures

Possible or Expected Findings

External examination and skin

Body cavities Blood

Heart

Record and photograph the findings listed in right-hand column. Prepare sections of skin lesions.

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 (p. 102).

Record weight. In chronic Chagas' disease, perfuse intact heart with formalin (p. 28) and slice fixed heart in a frontal plane so as to create anterior and posterior halves. Prepare photographs. Histologic samples should include conduction system (p. 26).

In acute disease, unilateral bipalpebral edema, chemosis, and swelling of preauricular lymph nodes (Romana's sign); skin nodules showing histiocytic and granulomatous inflammation; regional lymphadenitis, primarily in uncovered regions (chagoma), and subcutaneous edema. Hypopigmentation.

Effusions in congestive cardiac failure.* In acute Chagas' disease, trypanosomes in blood.

In acute cases, positive hemagglutination and precipitin tests; in chronic cases, positive complement-fixation tests. In chronic Chagas' disease, cardiac hypertrophy and dilatation; fibrous epicarditis, myocardial cell hypertrophy; apical aneurysm; endomyocardial fibrosis, and atrial and apical ventricular mural thrombi. Valves and coronary arteries are normal.

Organs and Tissues

Procedures

Possible or Expected Findings

Heart

(continued)

Lungs

Esophagus and gastrointestinal tract

Liver and biliary system

Spleen

Kidneys, ureters, and urinary bladder

Placenta

Brain and spinal cord

Skeletal muscles, peripheral nerves, and other tissues

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

Perfuse one lung with formalin (p. 47).

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 pp. 65 and 67, respectively.

For sampling of skeletal muscles, see p. 80. For sampling of peripheral nerves, see p. 79.

There may be parasitic pseudocysts or granulomas, fibrosis, myocytolysis, and degeneration and fibrous replacement of ganglion cells. In acute Chagas' disease, heart shows acute or subacute myocarditis* with dilatation. Intracellular parasites (i.e., pseudocysts with amastigote forms); necrosis of ganglion cells in atrial walls. In chronic Chagas' disease, emboli with infarctions, bronchiectasis,* fibrosis, hemo-siderosis, and, rarely, acute hemorrhage. Megaesophagus is frequent, with or without carcinoma. Stomach, duodenum, colon (2), and appendix (rarely) may be enlarged; diminution in number of ganglion cells in Auerbach plexus.

In acute Chagas' disease, hepatomegaly may be present. Rarely, in chronic cases, the gallbladder and bile ducts may be enlarged. Infarctions. In acute Chagas' disease, splenomegaly.

Renal infarctions. Rarely, in chronic Chagas' disease, the ureters and urinary bladder may be enlarged.

Pale, enlarged placenta; chronic villitis; increased perivillous fibrin; amastigotes in Hofbauer cells, amniotic epithelium and syncytiotrophoblasts.* Cerebral infarctions.* Meningoencephalitis (particularly in reactivated forms in immunodeficient patients [3]) with or without involvement of spinal cord; cerebral atrophy with pressure atrophy of frontal gyri. Histologically, ruptured pseudocysts with spread of amastigote forms. There is a predilection for muscle and nerve tissue, but all organs and tissues can be involved.

References

Sartori AM, Shikanai-Yasuda MA, Amato Neto V, Lopes MH. Follow-up of 18 patients with human immunodeficiency virus infection and chronic Chagas' disease, with reactivation of Chagas' disease causing cardiac disease in three patients. Clin Inf Dis 1998;26: 177-179.

Oliveira EC, Lette MS, Ostermayer AL, Almeida AC, Moreira H. Chagasic megacolon associated with colon cancer. Am J Trop Med Hyg 1997;56:596-598.

Chimelli L, Scaravilli F. Trypanosomiasis. Brain Pathol 1997;7:599-611.

Disease, Cholesteryl Ester Storage

Related Terms: Lysosomal acid lipase deficiency; Wolman's disease.*

Organs and Tissues

Procedures

Possible or Expected Findings

Fascia lata

Blood

Specimens should be collected using aseptic technique for tissue culture for biochemical studies (see Chapter 10).

The lysosomal acid lipase deficiency can be demonstrated in cultured fibroblasts.

Hyperbetalipoproteinemia; hypercholesterolemia.

Organs and Tissues

Procedures

Possible or Expected Findings

Liver and spleen

Other organs and tissues

Accumulation of cholesteryl esters may be demonstrated by thin-layer chromatography of lipid extracts of liver tissue. Lipid is PAS and aldehyde-fuchsin positive.

Hepatosplenomegaly. Hepatic fibrosis or cirrhosis with fatty changes in hepatocytes, cholangiocytes, portal macrophages, and Kupffer cells; deposition of cholesteryl crystals and triglycerides in Kupffer cells (1). Atherosclerosis and its manifestations may be more severe than expected for the age of the patient (2).

References

1. Di Bisceglie AM, Ishak KG, Rabin L, Hoeg JM. Cholesteryl ester 2. Tylki-Szymanska A, Rujner J, Lugowska A, Sawnor-Korsznska D, storage disease: Hepatopathology and effects of therapy with love- Wozniewicz B, Czarnowska E. Clinical, biochemical and histologi-

statin. Hepatology 1990;11:764-772. cal analysis of seven patients with cholesterol ester storage disease.

Acta Paediatr Japan 1997;39:643-646.

Disease, Christmas

Synonyms: Christmas factor deficiency; Factor IX deficiency.

NOTE: Follow procedures described under "Hemophilia." The expected findings are the same as for hemophilia.

Disease, Chronic Granulomatous

Synonyms and Related Terms: Autosomal recessive chronic granulomatous disease; chronic granulomatous disease of childhood; X-linked chronic granulomatous disease.

NOTE: The condition occurs not only in children but also in adults. Infections with catalase-positive microorganisms such as S. aureus, Pseudomonas sp. or Aspergillus sp., predominate. The disease is part of a family of inherited disorders of phagocyte function (neutrophil dysfunction syndrome); other disorders in this family include the Chediak-Higashi syndrome,* myeloper-oxidase deficiency, and other rare disorders.

Organs and Tissues

Procedures

Possible or Expected Findings

External examination, skin, and oral cavity

Abdominal cavity Chest cavity

Blood

Lymph nodes

Heart

Record extend and character of skin lesions, particularly those around body orifices. Photograph skin lesions and prepare sections.

Prepare chest and skeletal roentgenograms.

Submit sample of exudate for microbiologic study (see below under "Lymph nodes"). Submit sample of exudate for microbiologic study (see below under "Lymph nodes"). Record volume of contents. Submit sample for bacterial and fungal cultures (p. 102).

Submit samples of inguinal, axillary, mediastinal, mesenteric, and other grossly involved lymph nodes for microbiologic (p. 102) and histologic study. Request Gram and Grocott methenamine silver stains for fungi and Sudan black-stained frozen sections for lipid (p. 172). If pericarditis or endocarditis are suspected, follow procedures described under these headings (p. 103).

Seborrheic dermatitis, mainly around eyes (with conjunctivitis), around mouth (with stomatitis), and around nose and anus. Aphthous ulcers; gingivitis. Bacterial or fungal perianal and perineal abscesses and fistulas; wound infections. Skin granulomas with pigmented macrophages (1).

Pulmonary infiltrates. Osteomyelitis,* particularly of hands and feet.

Subphrenic empyema.*

Pleural effusions;* empyema.

Septicemia (staphylococci, gram-negative organisms, or fungi, such as Aspergillus and Candida).

Lymphadenitis with abscesses and lipid-filled macrophages; granulomas with central necrosis. For suspected organisms, see above under "Blood."

Pericarditis and, rarely, endocarditis* (2).

Organs and Tissues

Procedures

Possible or Expected Findings

Lungs

Esophagus and gastrointestinal tract

Liver and spleen

Other organs

Brain, spinal cord, and eyes Bones and bone marrow

Submit one lobe for microbiologic study

Prepare photographs of abnormal lesions.

Submit samples of normal and abnormal appearing areas for histologic study.

Record weights; photograph. Submit samples for microbiologic and histologic study

(see above under "Lymph nodes").

Submit samples of abnormal appearing areas for histologic study.

For removal and specimen preparation, see pp. 65, 67, and 85.

For removal, prosthetic repair, and specimen preparation of bones, see p. 95. For microbiologic sampling, see p. 102. For preparation of sections and smears of bone marrow, see p. 96.

Bacterial and fungal bronchopneumonia and abscesses; hilar lymphadenitis. Involvement by granulomatous disease may occur from mouth to anus. Colon lesions may resemble chronic ulcerative colitis (3). Hepatosplenomegaly with bacterial and fungal abscesses and granulomas.

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.

References

1. Dohil M, Prendiville JS, Crawford RI, Speert DP. Cutaneous manifestations of chronic granulomatous disease. A report of four cases and review of the literature. J Am Acad Dermatol 1997;36:899-907.

2. Casson DH, Riordan FA, Ladusens EJ. Aspergillus endocarditis in chronic granulomatous disease. Acta Pediatr 1996;85:758-759.

3. Sloan JM, Cameron CH, Maxwell RJ, McCluskey DR, Collins JS. Colitis complicating chronic granulomatous disease. A clinicopatho-logical case report. Gut 1996;38:619-622.

4. Adachi M, Hayashi A, Ohkoshi N, Nagata H, Mizusawa H, Shoji S, et al. Hypertrophic cranial pachymeningitis with spinal epidural granulomatous lesion. Intern Med 1995;34:806-810.

5. Valluri S, Chu FC, Smith ME. Ocular pathologic findings of chronic granulomatous disease of childhood. Am J Ophthalmol 1995;120: 120-123.

Disease, Chronic Obstructive Pulmonary (See "Bronchitis, chronic" and "Emphysema.")

Disease, Collagen

Synonym: Connective tissue disease. NOTE: See under specific name, such as "Arthritis, rheumatoid," "Dermatomyositis," "Lupus erythematosus, systemic," "Polyarteritis nodosa," "Sclerosis, systemic," and "Syndrome, Sjogren's."

Disease, Congenital Heart

(See under specific name of malformation.)

Disease, Creutzfeldt-Jakob

Synonyms and Related Terms: Creutzfeldt-Jakob disease (CJD), "new variant"; iatrogenic Creutzfeldt-Jakob disease; familial Creutzfeldt-Jakob disease; fatal familial insomnia; Gerstmann-Straussler-Scheinker syndrome; Kuru; Prion disease; sporadic spongiforme encephalopathy; subacute spongiforme encephalopathy; transmissible spongiforme encephalopathy; variant Creutzfeldt-Jakob disease.

NOTE:

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 (1-4):

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 (see p. 67) 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. If no 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 a 2 N NaOH solution for 1 h (alternatively, 1 N NaOH 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% NaOCl (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-to-tissue ratio of at least 20:1 for at least 48 h and then soaked in concentrated formic acid (95-100%) for 1 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 p. 135.

Organs and Tissues

Procedures

Organs and Tissues

Cerebrospinal fluid Brain

Submit sample (p. 104) for neuron-specific enolase (NSE).

For removal and specimen preparation, see p. 65 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 of PrPres protein on paraffin-embedded tissue is possible.

Increased concentrations of NSE (5).

Spongiforme changes, astrocytosis, neuronal loss, and amyloid plaque formation are the typical findings.

References

Ironside JW. Review: Creutzfeldt-Jakob disease. Brain Pathol 1996;6: 379-388.

Gajdusek DC, Gibbs CJ Jr. Survival of Creutzfeldt-Jakob disease virus in formol-fixed brain tissue. N Engl J Med 1976;294:553. Brown P. Guidelines for high risk autopsy cases: special precautions for Creutzfeldt-Jakob disease. In: (Hutchins GM, ed.) Autopsy Performance and Reporting. College of American Pathologists, North-field, IL, 1990, pp. 68-74.

Budka H, Aguzzi A, Brown P, Brucher JM, Bugiani O, Collinge J, et al. Tissue handling in suspected Creutzfeldt-Jakob disease and other human spongiforme encephalopathies (prion diseases). Brain Pathol 1995;5:319-322.

Zerr I, Bodemer M, Racker S, Grosche S, Poser S, Kretzschmar HA, Weber T. Cerebrospinal fluid concentration of neuron-specific enolase in diagnosis of Creutzfeldt-Jakob disease. Lancet 1995;345: 1609-1610.

Disease, Crohn's

Synonyms and Related Terms: Inflammatory bowel disease;* regional enteritis.

NOTE: If the distinction between Crohn's disease and chronic ulcerative colitis cannot be made clearly, see under "Disease, inflammatory bowel."

Possible Associated Conditions: Amyloidosis;* ankylosing spondylitis;* polyarthritis; Sjogren's syndrome.*

Organs and Tissues Procedures Possible or Expected Findings

External examination Record character and extent of skin lesions. Orbital edema and lid edema; ulcerative oral and skin Submit samples for histologic study. lesions; cutaneous fistulas after laparotomies;

clubbing of fingers; perianal fistulas; vulval abscesses; cutaneous polyarteritis nodosa; erythema multiforme; erythema nodosum; pyoderma gangrenosum. Granulomatous inflammatory changes in mucosal/skin lesions (1).

Prepare skeletal roentgenograms. See below under "Bones and joints."

Vitreous If dehydration or other electrolyte disturbances Dehydration;* electrolyte disorders.*

are expected, request determination of sodium, chloride, potassium, and urea nitrogen concentrations (pp. 85 and 115).

Organs and Tissues

Procedures

Possible or Expected Findings

Blood

Heart Lung

Esophagus Gastrointestinal tract

Mesentery Liver

Gallbladder Retroperitoneal tissues with pancreas Kidneys with ureters

Internal genital organs Eyes

Skeletal muscles Bones and joints

Brain and spinal cord

Submit sample for culture and for determination of immunoglobulin concentrations (p. 102). See "Stenosis, acquired valvular aortic." Submit at least one sample from each lobe for histologic study.

Leave specimen attached to stomach; submit tissue samples for histologic study. For in situ fixation and preparation for study under the dissecting microscope, see p. 54. In some instances, adhesions may be so severe that the intestines must be removed and sliced en bloc. Dissect fistulas in situ, or inject for roentgenographic study.

Submit samples of stomach and of all portions of intestinal tract for histologic study.

Submit lymph nodes for histologic study.

Record weight. For postmortem cholangiography, see p. 56. Submit multiple samples for histologic study.

Record nature of concrements.

Submit abscess contents for microbiologic study

Submit stones for chemical analysis. Photograph kidneys with renal pelves and ureters. Sample for histologic study. Submit purulent material for microbiologic study. For removal and specimen preparation, see p. 85.

For sampling and specimen preparation, see p. 80.

For removal, prosthetic repair, and specimen preparation, see p. 95.

For removal and specimen preparation, see pp. 65 and 67, respectively.

Septicemia; selective IgA deficiency. Aortic stenosis.*

Noncaseating granulomas in rare instances (2).

Esophagus may be affected by the disease.

All segments of the gastrointestinal tract (appendix included) may be affected. Complications include adenocarcinoma, lymphoma,* or other tumors (rare), pneumatosis coli, fistulas (enterovaginal, perirectal, and others), and perirectal abscess. Acute toxic dilatation of the colon may be present.

Mucosal abnormalities also may be present in grossly normal portions of colon and rectum.

Granulomatous lymphadenitis. Mesenteric fibromatosis (3).

Primary sclerosing cholangitis,* with or without cholangiocarcinoma* (4); biliary cirrhosis;* fatty changes; granulomas. Cholelithiasis.*

Psoas abscess; para-aortic lymphadenopathy. Granulomatous pancreatitis (5). Nephrolithiasis* (uric acid and calcium stones); hydronephrosis.* Hydroureters; periureteral fibrosis and ureteral obstruction. Pyosalpinx.

Conjunctivitis; marginal corneal ulcers; keratitis; scleritis; episcleritis; retinitis; neuroretinitis; optic neuritis. Myositis; in rare instances, dermatomyo-sitis* (6).

Aseptic necrosis of bone; ossifying periostitis; granulomatous bone disease; ankylosing spondylitis;* polyarthritis; nonspecific or granulomatous synovitis. Manifestations of disseminated intravascular coagulation.*

References

1. Kafity A, Pellegrini A, Fromkes J. Metastatic Crohn's disease: a rare cutaneous manifestation. J Clin Gastroenterol 1993;17:300-303.

2. Calder CJ, Lacy D, Raafat F, Weller PH, Booth IW. Crohn's disease with pulmonary involvement in a 3 year old boy. Gut 1993;34:1636-1638.

3. DiGiacomo JC, Lasenby AJ, Salloum LJ. Mesenteric fibromatosis associated with Crohn's disease. Am J Gastroenterol 1994;89:1103-1105.

4. Choi PM, Nugent FW, Zelig MP, Munson JL, Schoetz DJ Jr. Cholangiocarcinoma and Crohn's disease. Dig Dis Sci 1994;39:667-670.

5. Gschwantler M, Kogelbauer G, Klose W, Bibus B, Tscholakoff D, Weiss W. The pancreas as a site of granulomatous inflammation in Crohn's disease. Gastroenterology 1995;108:1246-1249.

6. Leibowitz G, Eliakim R, Amir G, Rachmilewitz D. Dermatomyositis associated with Crohn's disease 1994;18:48-52.

Disease, Cushing's (See "Syndrome, Cushing's.")

Disease, Cytomegalic Inclusion (See "Infection, cytomegalovirus.")

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 pulmoOrgans and Tissues Procedures

External examination and skin

Blood Heart

Lungs

Urine Kidneys

Other organs

Brain and spinal cord

Eyes

1. Elleder M, Bradovä V, Smid F, Budesinsky M, Harzer K, Kuster-mann-Kuhn B, et al. Cardiocyte storage and hypertrophy as a sole manifestation of Fabry's disease. Report on a case simulating hypertrophic non-obstructive cardiomyopathy. Virchows Arch [Pathol Anat] 1990;417:449-455.

2. Von Scheidt W, Eng CM, Fitzmaurice TF, Erdmann E, Hubner G, Olsen EG, et al. An atypical variant of Fabry's disease with manifestations confined to the myocardium. N Engl J Med 1991;324:395-399.

nary," "Lipoproteinosis, pulmonary alveolar," "Microlithiasis, pulmonary alveolar," "Pneumonia, lipoid," and "Syndrome, Goodpasture's."

Disease, Eosinophilic Endomyocardial

(See "Cardiomyopathy, restrictive [eosinophilic type].")

Disease, Fabry's

Synonyms: Alpha-galactosidase deficiency; Anderson-Fabry disease; angiokeratoma corporis diffusum; glycosphingolipid lipidosis.

Possible or Expected Findings

Telangiectatic lesions. Glycolipid storage (PAS-positive, Sudan black-positive, metachromatic, and double refractile with toluidine blue) in arrectores pilorum muscles, vascular endothelium, and sweat glands. Leukocyte alpha-galactosidase deficiency. Glycolipid storage with nonobstructive hypertrophic cardiomyopathy* (this may be the only manifestation [1,2]). Myocardial infarction.

Narrowing of airways by glycosphingolipid in patients with clinical features of obstructive lung disease (3). "Mulberry cells" in sediment. Glycosphingolipids in glomeruli and distal convoluted tubules. If applicable, see also under "Failure, kidney." Glycosphingolipid storage in liver, spleen, small and large bowel, lymph nodes, and bone marrow.

Elongated tortuous and ectatic vertebral and basilar arteries (4), sometimes with thrombosis (5). Glycosphingolipid storage. Cerebral infarction(s)* or hemorrhages.

Glycosphingolipid storage in cornea; lens opacities; dilated vessels in conjunctiva and lens; thrombi in blood vessels (5).

3. Brown LK, Miller A, Bhuptani A, Sloane MF, Zimmerman MI, Schilero G, et al. Pulmonary involvement in Fabry disease. Am J Respir Crit Care Med 1997;155:1004-1110.

4. Mitsias P, Levine SR. Cerebrovascular complications of Fabry's disease. Ann Neurol 1996;40:8-17.

5. Utsumi K, Yamamoto N, Kase R, Takata T, Okumiya T, Saito H, et al. High incidence of thrombosis in Fabry's disease. Intern Med 1997;36:327-329.

Prepare skin sections from multiple sites. Request Sudan black, PAS, and toluidine blue O stains (p. 173).

For recommended special stains, see above under "External examination and skin."

For recommended special stains, see above under "External examination and skin."

Examine sediment.

For recommended special stains, see above under "External examination and skin."

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

For removal and specimen preparation, see pp. 65 and 67. For cerebral angiography and dissection of vertebral arteries, see pp. 80 and 82. For recommended special stains, see above under "External examination and skin." For removal and specimen preparation, see p. 85. For recommended special stains, see above under "External examination and skin."

References

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, however, may overlap or occur together and hence need a collective designation. The conditions include autosomal-recessive (infantile) and autosomal dominant (adult) polycystic disease of the liver; Caroli's disease or syndrome;* choledochal cyst,* congenital hepatic fibrosis,* multiple biliary microhamartomas, and related disorders. For autopsy procedures, see also under more specific designations.

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