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Pulmonary infections in children.

Organs and Tissues

Procedures

Possible or Expected Findings

Spleen

Other organs

Brain and spinal cord

Bones and bone marrow

Record weight. Photograph cut surface. Submit samples of fresh material for biochemical study, and snap-freeze specimens for histochemical analysis. Prepare unstained smears for phase-contrast microscopy. Request PAS and Masson's trichrome stains (p. 173). Prepare material for electron microscopy (p. 132). Submit tissue samples of liver, pancreas, kidneys, gastrointestinal tract, intrathoracic and intraabdominal lymph nodes, thymus, tonsils, thyroid, and adrenal glands. For processing, see above under "Spleen." For removal and specimen preparation, see pp. 65 and 67, respectively. See also above under "Spleen." Submit specimens of involved bones, as indicated on skeletal roentgenograms; include femur in all instances. Photograph saw section of femur. For prosthetic repair and for decalcification, see p. 97; for specimen preparation, see p. 95. For preparation of bone marrow sections and smears, see p. 96.

Splenomegaly caused by accumulation of glucocerebroside-containing Gaucher cells. Increased acid phosphatase in Gaucher cells.

Hepatomegaly; manifestations of portal hypertension; lymphadenopathy. Infiltration of organs (listed in middle column) by Gaucher cells; hemosiderosis.

Acute nerve cell degeneration. Accumulation of glucocerebrosides and—in children with acute neuronopathic disease—gangliosides. See above under "External examination and skin."

Reference

1. Cox TM, Schofield JP. Gaucher's disease: clinical features and natural history. Baillieres Clin Haematol 1997;10:657-689.

Disease, Glycogen Storage

Synonyms: Andersen's disease or brancher deficiency (glycogenosis, type IV); Cori's or Forbes' disease (glycogenosis, type III); cyclic AMP dependent kinase (type X); glycogen synthetase deficiency (type O); Hers' disease (glycogenosis, type VI); McArdle's disease (glycogenosis type V); phospho-rylase B kinase deficiency (types IXa, b, and c); Pompe's disease (glycogenosis, type II); Tarui disease (glycogenosis type VII); von Gierke's disease (glycogenosis, type Ia); X-linked glycogenosis (type VIII).

NOTE: If the diagnosis had not been confirmed prior to death, samples of liver, skeletal muscle, blood, and fascia (for fibro-blast 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 (1) 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 Ic.

Organs and Tissues

Procedures

Possible or Expected Findings

External examination and skin

Blood

Record body weight and length. Submit tissue samples of skin lesions. Record size of tongue and submit specimens for histologic study (may be easier to do after removal with neck organs). For specimen preparation, see below under "Heart "

Submit sample for uric acid and ketone determination. If blood is to be used for tissue culture, follow procedures described in Chapter 10 (see also "Fascia lata" below).

Growth retardation.

Xanthomas in von Gierke's disease.

Macroglossia.

Hyperuricemia in gout.* Ketoacidosis may be associated with sudden death. Hypoglycemia* and hyperlipidemia occur in von Gierke's disease.

Organs and Tissues

Procedures

Possible or Expected Findings

Fascia lata

Liver

Heart, blood vessels, lungs, skeletal muscles, esophagus, intestine, pancreas, spleen, kidneys, adrenal glands, urinary bladder, lymph nodes, bone marrow.

Eyes

Brain and spinal cord

Joints

Specimens should be collected using aseptic technique for tissue culture for biochemical studies (see Chapter 10). For recommended fixatives and special stains, see below. Frozen sections protected with celloidin and then stained with PAS allows an accurate determination of the glycogen content.

Prepare samples for electron microscopic study (p. 132), particularly in glycogenosis types II and IV.

Photograph enlarged or discolored organs and obtain samples for histologic study. Recommended fixatives for glycogen include alcohol, Bouin's (p. 129) or Carnoy's fixative and formalin alcohol (p. 130). Glycogen may still be dissolved during exposure to watery staining solutions. Request van Gieson's stain, PAS stain with and without diastase digestion, and Best's stain for glycogen (p. 172). Request Sudan-stained frozen sections of myocardium, liver, and skeletal muscles. For use of frozen sections for study of glycogen, see above under "Liver." Embed tissue samples for electron microscopic study. For removal and specimen preparation, see p. 85. Use Zenker's solution for fixation (p. 131). Other fixatives and procedures are listed above.

For removal and specimen preparation, see pp. 65 and 67, respectively. Submit specimens of sympathetic nerve ganglia for histologic study.

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

For enzyme deficiencies, see above under "Note."

Enlarged hepatocytes with glycogen storage in types I, III, and IV. Fatty changes most common in types 0, I and III. Periportal fibrosis in types III and IV and, rarely, cirrhosis in type IV. Adenomas and, rarely, hepatocellular carcinomas may be found in type Ia. No abnormalities in types V and VII. See also above under "Note." Uric acid nephropathy and glomerulo-sclerosis in type Ia. Distribution of glycogen storage and other abnormalities varies with subtype of disease. Glycogen depositis may be found in myocardium (cardiomegaly), small and large arteries, skeletal muscle (for instance, of diaphragm, neck, trunk, and extremities), bronchial mucosa, and all other organs listed in left-hand column. See also above under "Note."

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.

Organs and Tissues

Procedures

Possible or Expected Findings

External examination and skin; oral cavity

Record and photograph skin lesions and prepare histologic sections of normal and abnormal skin.

Small biopsies of labial salivary glands and buccal mucosa may be useful to evaluate chronic GVHD (1).

Generalized erythroderma and jaundice. Microscopic examination shows irregular epidermal-dermal junctions with basal cell vacuolation, spongiosis, and eosinophilic bodies associated with infiltrates of aggressor lymphocytes.

Buccal mucositis; lichenoid lesions in chronic GVHD (1).

Organs and Tissues

Procedures

Possible or Expected Findings

Heart Lungs

Liver

Esophagus

Small and large intestine

Other organs

Eyes

Bone marrow

Record volume of pericardial fluid.

Perfuse one lung with formalin (p. 47). Submit samples from other lung for microbiologic study (p. 103).

Record weight. Submit samples for histologic study.

Prepare photographs of mucosa and sample for histologic study.

For in situ fixation of small intestinal mucosa, see p. 54. Submit samples for histologic study.

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

For removal and specimen preparation, see p. 85.

For preparation of sections and smears, see p. 96.

Pericardial effusion (in rare cases with features of polyserositis in chronic GVHD) (2). Diffuse alveolar damage; lymphocytic bronchitis/bronchiolitis obliterans; organizing pneumonia (3). Bronchiectases in rare instances (4).

Hepatomegaly. Portal and periportal hepatitis with destruction of interlobular ducts; oncocytic metaplasia of bile duct epithelium (5); endotheliitis; cholestasis. Infectious esophagitis or chronic GVHD with vesicobullous lesions or, in late stages, strictures.

Enteritis with cellular debris in crypts, atypical epithelial lining of crypts, and inflammatory infiltrates. Inflammatory infiltrates. Hemorrhagic necroses in lymph nodes and spleen. Immune-mediated myelopathy (6). Keratoconjunctivitis. Optic neuropathy (6).

Evidence of proliferating graft cells.

References

Nakamura S, Hiroki A, Shinohara M, Gondo H, Ohyama Y, Mouri T, et al. Oral involvement in chronic graft versus host disease after allogenic bone marrow transplantation. Oral Surg Oral Med Oral Pathol 1996;82:556-563.

Toren A, Nagler A. 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. Bone Marrow Transplant 1997;20:805-807. Yousem AS. The histological spectrum of pulmonary graft-versus-host disease in bone marrow transplant recipients. Hum Pathol 1995; 26:668-675.

Morehead RS. Bronchiectasis in bone marow transplantation. Thorax 1997;52:392-393.

Bligh J, Morton J, Durrant S, Walker N. Oncocytic metaplasia of bile duct epithelium in hepatic GVHD. Bone Marrow Transplant 1995; 16:317-319.

Openshaw H, Slatkin NE, Parker PM, Forman SJ. Immune-mediated myelopathy after allogeneic marrow transplantation. Bone Marrow Transplant 1995;15:633-636.

Disease, Graves' (See "Hyperthyroidism.")

Disease, Günther's (See "Porphyria, congenital erythropoietic.")

Disease, Heavy-Chain

Synonyms and Related Terms: Gamma heavy-chain disease (Franklin's disease); alpha heavy-chain disease (Seligmann's disease); ^ heavy-chain disease.

NOTE: Alpha heavy-chain disease is related to Mediterranean lymphoma and ^ heavy-chain disease occurs in rare patients with chronic lymphocytic leukemia.* Infections generally are the cause of death in gamma heavy-chain disease. Evidence of malabsorption* may be observed in alpha heavy-chain disease.

Organs and Tissues

Procedures

Possible or Expected Findings

External examination and oral cavity Blood

Lungs

Urine

Record body weight and length.

Submit samples for microbiologic study and for protein electrophoresis (p. 102).

Submit one lobe for microbiologic study. (p. 103).

Submit sample for protein electrophoresis.

Profound wasting in alpha-chain disease. For oral changes, see under "Neck organs." Septicemia. See also above under "Note." Anomalous serum M component in gamma heavy-chain disease.

Pneumonia in gamma heavy-chain disease. Rarely lymphoplasmacytoid infiltrates in alpha heavy-chain disease. Anomalous serum M component in gamma heavy-chain disease.

Organs and Tissues

Procedures

Possible or Expected Findings

Lymph nodes

Small bowel and mesentery

Neck organs

Bone marrow Bones

Prepare Wright stains of touch preparations (p. 173). Fix tissue in Zenker's or Helly's solution (p. 131).

For in situ fixation and preparation for study under dissecting microscope, see p. 54. For microscopic study, submit samples of all segments of gastrointestinal tract and portions of mesentery with lymph nodes. Remove together with pharynx.

For preparation of sections and smears, see p. 96.

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

Mesenteric and para-aortic lympha-denopathy with infiltrates of lymphocytes and plasma cells.

Infiltrates of lymphocytes and plasma cells in lamina propria of small bowel in alpha heavy-chain disease. See also under "Syndrome, malabsorption."

Palatal edema (Waldeyer ring lympha-denopathy) in gamma heavy-chain disease.

Infiltrates of lymphocytes and plasma cells; eosinophilia.

Osteoporosis* in alpha heavy-chain disease.

Disease, Hippel-Lindau (See "Disease, von Hippel-Lindau.")

Disease, Hirschsprung's (See "Megacolon, congenital.") Disease, Hodgkin's (See "Lymphoma.") Disease, Hookworm (See "Ancylostomiasis.") Disease, Huntington's (See "Chorea, hereditary.") Disease, Hydatid (See "Echinococcosis.")

Disease, Inflammatory Bowel

Synonyms and Related Terms: Chronic ulcerative colitis; Crohn's disease;* idiopathic proctocolitis.

Possible Associated Conditions: Alphaj-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.

Organs and Tissues

Procedures

Possible or Expected Findings

External examination, skin, and oral cavity

Synovial fluid

Blood Heart

Lungs

Record nature and extent of skin lesions, photograph, and submit specimens of accessible lesions for histologic study.

Record appearance of hands and feet. Prepare roentgenograms of fistulas after injection of contrast medium (p. 138). Prepare skeletal roentgenograms. If arthritis is suspected, submit sample of synovial fluid for microbiologic study, cell counts, and smears (p. 96). Submit sample for microbiologic study (p. 102).

If pericarditis or endocarditis are expected, follow procedures described under these headings (p. 103).

Dissect pulmonary arteries; sample all lobes for histologic study. Request Verhoeff-van Gieson stain (p. 173).

Aphthous stomatitis; pyoderma gangrenosum; erythema nodosum and multiforme; papular or pustular dermatitis; ulcerating erythematous plaques; neuro-dermatitis; herpes zoster; anal fissures. Clubbing of fingers and toes. Emaciation. Perianal abscesses and fistulas. See below under "Bones and joints." Arthritis.*

Septicemia.

Endocarditis;* pericarditis.* Thromboemboli; pulmonary vasculitis.

Organs and Tissues

Procedures

Possible or Expected Findings

Abdominal cavity with retroperitoneum and pelvic organs

Small and large intestine

Bile ducts Liver

Stomach and duodenum

Pancreas Kidneys, ureters, and urinary bladder

Veins and arteries Eyes

Brain and cerebral venous sinuses Bones and joints

If peritonitis is present, submit exudate for aerobic and anaerobic bacteriologic study (p. 102). Aspirate contents of abscesses and record their size and location. Record volume of exudate and prepare smears. For in situ fixation, see p. 54. If fistulas are present, dissect affected areas in situ. Opened colon and affected portions of small bowel should be pinned on corkboard and fixed with formalin. Submit samples of all types of lesions for histologic study.

For cholangiography, see p. 56. Dissect extra-

hepatic bile ducts in situ (see also under

"Tumor of the bile ducts").

Record weight; submit samples for histologic study.

Submit samples for histologic study. Submit samples for histologic and bacteriologic study. If glomerulitis is suspected, follow procedures described under "Glomerulonephritis."

Describe size and contents of urinary bladder, ureters, and renal pelves.

For removal of femoral vessels, see p. 34.

For removal and specimen preparation, see p. 85. If there is evidence of Sjogren's syndrome,* remove lacrimal glands (p. 87). For removal and specimen preparation, see pp. 65 and 67, respectively. For removal, prosthetic repair, and specimen preparation, see p. 95.

Peritonitis.* Perianal, presacral, and ischiorectal abscesses; fistulas and anal fissures. Dilatation of colon ("toxic megacolon"). Fournier's gangrene (necrotizing fasciitis of the genitalia) in Crohn's disease. Segmental (skip areas), transmural and granulomatous inflammation in Crohn's disease. Toxic megacolon more common in chronic ulcerative colitis. Retroperitoneal and rectovaginal fistulas; mucosal ulcers and pseudopolyps; multicentric lymphoma; dysplasia; carcinoma(s); hemorrhage. Rectal stricture. "Backwash ileitis." Colonic cytomegalovirus inclusions, associated with toxic dilatations. See also ref. (1). Sclerosing cholangitis;* adenocarcinoma of bile ducts.

Biliary cirrhosis.* Cholangiocarcinoma.

Ulcerative gastritis and duodenitis in Crohn's disease.

Pancreatitis.

Glomerulonephritis;* pyelonephritis;* tubular degeneration; nephrocalcinosis. Renovascular disease (2).

Cystopyelitis; urolithiasis; nephrolithiasis (oxalate stones).

Thrombophlebitis; arteritis (2) and arterial thromboses.

Blepharitis; conjunctivitis; corneal ulcers; iritis; keratitis; neuroretinitis; retrobulbar neuritis; uveitis.

Cerebral venous sinus thrombosis* (3).

Osteoporosis;* ankylosing spondylitis;* arthritis of peripheral joints; periarthritis; hypertrophic osteoarthropathy;* tendinitis (particularly of ankle and Achilles tendons).

References

1. Podolsky D. Inflammatory bowel disease (first of two parts). N Engl J Med 1991;325:928-937 (part 1) and 1008-1016 (part 2).

2. Sakhuja V, Gupta KL, Bhasin DK, Malik N, Chugh KS. Takayasu's arteritis associated with idiopathic ulcerative colitis. Gut 1990;31:831-833.

3. Johns D. Cerebrovascular complications of inflammatory bowel disease. Am J Gastroenterol 1991;86:367-370.

Disease, Iron Storage (See "Hemochromatosis.")

Disease, Ischemic Heart

Related Terms: Atherosclerotic heart disease.

NOTE: The most common anatomic finding at autopsy in subjects older than 30 yr is coronary atherosclerosis. Unusual underlying 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);

Disease, Ischemic Heart (continued)

coronary vasculitis (for instance, in polyarteritis nodosa* or acute hypersensitivity arteritis); hyperthyroidism,* gastrointestinal hemorrhage;* hypothyroidism,* idiopathic arterial calcification of infancy; intramural coronary amyloidosis; pheochromocy-toma, polycythemia vera;* pseudoxanthoma elasticum,* radiation-induced coronary stenosis; severe pulmonary hypertension (with right ventricular ischemia); sickle cell disease;* and others. If bypass surgery had been performed, see "Surgery, coronary bypass."

Organs and Tissues

Procedures

Possible or Expected Findings

External examination

Blood Heart

Aorta

Other organs

Prepare chest roentgenogram. If underlying metabolic disease is suspected, submit sample for biochemical study. For coronary arteriography, see p. 118. For specimen preparation and grading of coronary arteries, see p. 21. Request Verhoeff-van Gieson stain (p. 172).

For dissection technique of the heart and for histologic sampling, see pp. 22 and 30. For detection of early myocardial infarction, see p. 32.

For study of valvular cardiac lesions, see p. 32. Record actual and expected heart weight, ventricular wall thicknesses, and valvular annular circumferences. Record appearance, extent, and location of infarcts, mural thrombus, and aneurysms.

Cyanosis; edema of legs; venous congestion. Gangrene of toes. Diabetic ulcers. Cardiomegaly; pleural effusions.* See above under "Note."

Coronary atherosclerosis; coronary thrombosis or embolism; congenital malformation(s) of coronary arteries; accidental operative coronary ligation; coronary arteritis (see above under "Note.") Myocardial infarction, old or acute. Mural thrombus. Ventricular aneurysm, true or false. Ventricular rupture (free wall, septum, or papillary muscles). Aortic insufficiency;* aortic stenosis.*

Acute aortic dissection.* Manifestations of congestive heart failure* and of possible underlying conditions (see above under "Note"), such as diabetes mellitus.*

Disease, Jakob-Creutzfeldt (See "Disease, Creutzfeldt-Jakob.")

Disease, Kawasaki (See "Syndrome, mucocutaneous lymph node.")

Disease, Krabbe's (See "Leukodystrophy, globoid cell.")

Disease, Legionnaires'

Synonyms and Related Terms: Legionella pneumophila infection; Pontiac fever.

NOTE: (1) Collect lung specimens, serum, and other tissues that appear to be infected. These should be inoculated on a nonselective medium, such as BCYE agar supplemented with a-ketoglutaric acid. A good selective agar is BCYE supplemented with antibiotics. (2) Request aerobic and anaerobic cultures for exclusion of other bacterial diseases. (3) Request Gram, Kinyoun, and Grocott's methenamine silver stains for exclusion of other bacterial or fungal diseases (p. 172). The Dieterle silver impregnation procedure is recommended for demonstration of the organism in paraffin-embedded sections (1-3). (4) No special precautions are indicated. (5) Serologic immunoflu-orescent studies are available from the Centers for Disease Control and Prevention, Atlanta, GA (p. 135). (6) This is a reportable disease.

Organs and Tissues Procedures Possible or Expected Findings

Blood, pleural fluid Submit sample for culture (p. 102).

Lungs Culture any areas of consolidation (p. 102). Multifocal fibrinopurulent pneumonia with

Perfuse one lung with formalin (see p. 47). sparing of the bronchi and bronchioles.

Slice in the parasagittal plane. Submit affected Exudate is rich in phagocytes, fibrin, and areas for histological study. karyorrhectic debris.

References

1. Edelstein PH. Legionnaires' disease. Clin Infect Dis 1993;16(6):741-747.

2. Stout JE, Yu VL. Legionellosis. NEJM 1997;337(10):682-687.

3. Bhopal R. Source of infection for sporadic Legionnaires' disease: a review. J Infect 1995;30:9-12.

Disease, Lyme

Synonym: Lyme arthritis

NOTE: This infection is caused by the spirochete, Borrelia burgdorferi, which is transmitted from rodents to human by the hard deer ticks, Ixodes dammini, I. ricinus, and others.

Organs and Tissues

Procedures

Possible and Expected Findings

External examination and skin

Cerebrospinal fluid

Blood

Joints

Heart Liver

Brain and spinal cord

Photograph skin lesions. Record presence of enlarged lymph nodes. Submit sections of affected skin for histologic study. For removal, see p. 104. Submit for IgG study and prepare smear.

Obtain blood for chemical and serologic analysis.

Aspirate fluid from joint effusions. Submit synovium of affected joints for histologic study.

Submit sections for histologic study (p. 30).

Submit sections for histologic study. For removal and specimen preparation, see pp. 65 and 67, respectively. Include meninges in histologic sections.

Erythema chronicum migrans; skin vesicles; annular skin lesions; lymphadenopathy; conjunctivitis.

Antispirochete IgG; lymphocytes and plasma cells (1).

Elevated liver enzymes; elevated IgM early in illness; normal or elevated C3 and C4; rheumatoid factor usually absent. Neutrophils in synovial fluid; synovitis resembling early rheumatoid arthritis with a distinctive arteritis with onionskin-like lesions; later in the disease, cartilage destruction.

Myocarditis; spirochetes may be demonstrable.

Dense portal infiltrates.

Mononuclear meningitis and meningoradiculitis.

Reference

1. Sindern E, Malin JP. Phenotypic analysis of cerebrospinal fluid cells over the course of Lyme meningoradiculitis. Acta Cytol 1995;39:73-75. Disease, Lymphatic

NOTE: In all diseases of the thoracic duct and its major tributaries and also in cases of lymphedema or other peripheral lymphovascular diseases, postmortem lymphangiography (p. 34) may be an effective method of study.

Disease, Maple Syrup Urine

Synonyms and Related Terms: Branched-chain hyperaminoacidemia (various types); classic maple syrup urine disease; hyperleucinemia; hypervalinemia. See also aminoaciduria.*

NOTE: Collect all obtainable urine and freeze at -20°C; this should be done as soon as possible.

Organs and Tissues

Procedures

Possible or Expected Findings

Blood and urine

Fascia lata (or skin)

Other organs Brain and spinal cord

Submit samples for biochemical study.

Submit sample for karyotype and biochemical analysis (p. 109).

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

Increased amino acids and urinary organic acids. The urine may have a "maple syrup" odor.

Enzyme deficiency can be demonstrated in cultured fibroblasts, leukocytes, or amniocytes.

Bronchopneumonia. Steatosis or increased glycogen deposition in liver. Spongiosis; gliosis; edema; defective myelinization.

Disease, Marble Bone (See "Osteopetrosis.")

Disease, Marchiafava-Bignami

NOTE: Patients with this disease suffer from chronic alcoholism. Malnutrition, nutritional amblyopia,* and Wernicke-Korsakoff syndrome* may be present.

Organs and Tissues Procedures Possible or Expected Findings

Brain and spinal cord For removal and specimen preparation, Symmetric and zonal demyelination in see pp. 65 and 67, respectively. Request corpus callosum, anterior commissure, optic

Luxol fast blue stain for myelin (p. 172). chiasm, optic tracts, and white matter of frontal lobes.

Disease, Mast Cell (See "Mastocytosis, systemic.")

Disease, Medullary Cystic Renal (See "Cyst(s), renal.")

Disease, Meningococcal

Synonyms: Meningococcemia; Neisseria meningitidis infection; Waterhouse-Friderichsen syndrome (fulminant meningo-coccemia).

NOTE: (1) Submit 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) Usually, serologic studies are not available. However, isolates should be segregated by seroagglutination into serogroups, i.e., A,B,C,D,X,Y,Z. (6) This is a reportable disease.

Possible Associated Conditions: Disseminated intravas-cular coagulation* is a common component of the disease.

Organs and Tissues

Procedures

Possible or Expected Findings

External examination and skin

Cerebrospinal fluid

Blood

Heart and pericardial fluid

Lungs Spleen

Adrenal glands Genital organs Brain and spinal cord

Middle and inner ears

Record extent of skin lesions and prepare photographs; submit tissue samples of skin for histologic study.

Prepare skeletal roentgenograms if bone lesions are expected.

Submit sample for aerobic bacterial culture (p. 104).

Submit sample for microbiologic study

(p. 102) and determination of serum cortisol concentration.

Submit samples of pericardium, pericardial fluid, and myocardium for aerobic bacterial cultures and Gram stain (p. 172). For procedures in infective endocarditis, see p. 103. Submit consolidated areas for culture (p. 103). Record weight and submit tissue specimens for histologic study.

Photograph; record weights; request Gram stain for histologic sections (p. 172).

Submit tissue samples for histologic study.

Infectious material should be obtained as described on p. 104. For removal and specimen preparation of brain and spinal cord, see pp. 65 and 67, respectively. Request Bodian stain (p. 74).

For removal and specimen preparation, see p. 72.

Cutaneous or subcutaneous hemorrhages (purpura fulminans, with or without skin loss and deep muscle damage (1)); herpes labialis; rarely, jaundice.

Osteomyelitis and osteonecrosis (see below) (2).

Meningococcal septicemia. Low serum cortisol level.

Pericarditis.* Infective endocarditis.

Primary or secondary pneumonia; pleuritis. Splenitis.

Acute hemorrhage and necrosis.

Urethritis; orchitis; epididymitis; endometritis.

Scant exudate with numerous bacteria in the hyperacute form; In the acute form, abundant pus surrounds the entire brain, vertex, and base and may extend to the ventricular system. In the chronic form, communicating hydrocephalus and cortical infarction are common complications. Otitis media.*

Organs and Tissues Procedures Possible or Expected Findings

Nasopharynx For exposure, see p. 71. Prepare smears and Posterior nasopharyngeal meningococcal submit tissue samples for histologic study. infection.

Eyes For removal and specimen preparation, Conjunctivitis; panophthalmitis.

Bones, joints, Remove synovial fluid and submit sample for Purulent arthritis; necrosis and hemorrhage and soft tissues bacteriologic study (p. 96). For removal of of synovia. Osteonecrosis (rare in adults [2])

bones and joints, prosthetic repair, and specimen and osteomyelitis; rhabdomyolysis (3). preparation, see pp. 95-97, respectively. Prepare histologic sections of synovia and skeletal muscle.

References

1. Huang S, Clarke JA. Severe skin loss after meningococcal septicemia: complications in treatment. Acta Paediatr 1997;86:1263-1266.

2. Campbell WN, Joshi M, Sileo D. Osteonecrosis following meningo-coccemia and disseminated intravascular coagulation in an adult: case report and review. Clin Infect Dis 1997;24:452-455.

3. Van Deuren M, Neeleman C, Assmann KJ, Wetzels JF, van der Meer JW. Rhabdomyolysis during the subacute stage of meningococcal sepsis. Clin Infect Dis 1998;26:214-215.

Disease, Motor Neuron

Synonyms and Related Terms: Infantile spinal muscular atrophy; progressive spinal muscular atrophy, Werdnig-Hoffman disease.

Organs and Tissues

Procedures

Possible or Expected Findings

External examination

Brain and spinal cord

Skeletal muscles

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

For sampling and specimen preparation, see p. 80.

Congenital fixation of multiple joints of extremities.

Degeneration and loss of motor neurons from anterior horn and brainstem motor nuclei (particularly hypoglossal and facial) and thalamus (posteroventral nucleus). Neurogenic atrophy.

Disease, Multicystic Renal (See "Cyst(s), renal.") Disease, Niemann-Pick

Synonyms and Related Terms: Sphingomyelinase deficiency; sphingomyelin lipidosis; Niemann-Pick disease, types A, B, C, or D.

NOTE: For further details on specimen preparation, see ref. (1).

Organs and Tissues

Procedures

Possible or Expected Findings

External examination and fascia lata

Skin and conjunctiva

Heart Lungs

Liver and spleen

If diagnosis must be confirmed, prepare fibro-blast culture for assay of sphingomyelinase (p. 109).

Prepare samples for electron microscopic study (see p. 132).

In infants, snap-freeze portion of fresh lung and perfuse one lung with formalin (p. 47). Submit consolidated areas for microbiologic study (p. 103).

Record sizes and weights; snap-freeze tissue for biochemical sphingomyelin determination. Special stains of frozen sections for phospho-lipids and cholesterol are positive but not diagnostic.

Growth retardation.

Membrane-bound lamellar cytoplasmic inclusions.

Endocardial fibroelastosis. Vacuolated histiocytes (foam cells) containing sphingomyelin, cholesterol, and ganglioside within alveoli and interstitium. Acute or organizing bronchopneumonia. Hepatosplenomegaly; foam cell transforma-mation of Kupffer cells and hepatocytes; cholestasis; intra-acinar fibrosis and, rarely, cirrhosis. Hepatocellular giant cells may be present. Abundant foam cells in spleen.

Organs and Tissues

Procedures

Possible or Expected Findings

Liver and spleen

Submit sample for electron microscopic study

Laminated inclusions in the cytoplasm of

(continued)

(p. 132).

affected cells.

Other organs

Your Heart and Nutrition

Your Heart and Nutrition

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