A cellular defect associated with decreased bactericidal activity of the polymorphonuclear leukocyte has been found in a 2(1/2) yr old Negro boy with the typical clinical and pathological findings of chronic granulomatous disease. Unlike previously described patients his polymorphonuclear leukocytes were shown to undergo apparently normal degranulation and vacuole formation after phagocytosis. Metabolic studies of the leukocytes indicated a failure to increase oxygen consumption with phagocytosis or to reduce Nitroblue tetrazolium dye. These metabolic abnormalities are identical with those previously reported in patients with chronic granulomatous disease. Two additional patients with chronic granulomatous disease have also been found to have apparently adequate degranulation of polymorphonuclear leukocytes after phagocytosis. Our studies suggest that failure of degranulation may not be a necessary part of this functional leukocyte abnormality.

Child, Preschool↗

PubMed1967

Serum precipitins to milk, gluten and rice in tropical sprue.

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Animals↗

PubMed1966

Precautionary note on the use of the intestinal-biopsy capsule in infants and emaciated children.

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Biopsy↗

PubMedDate not supplied

A comparison of liver ultrastructure in salicylate intoxication and Reye's syndrome.

All childhood liver biopsy specimens from The Cincinnati Children's Hospital Research Foundation which had been prepared for light and electron microscopy were reviewed to identify biopsies from children with salicylate intoxication. Only two cases of primary salicylate intoxication were identified. The histopathology and ultrastructural pathology were compared to that in two cases of Reye's syndrome which were selected because they had been treated with salicylates and had comparable serum salicylate concentrations at the time of liver biopsy. Liver biopsy specimens from the cases of salicylate intoxication were nearly normal by light microscopy. Lipid accumulation was minimal, and the content of glycogen and succinic acid dehydrogenase activity was normal. Mitochondria and peroxisomes appeared normal. Light and electron microscopy of liver specimens from the two cases of Reye's syndrome revealed swollen hepatocytes with microvesicular fat and central nuclei. Glycogen content and succinic acid dehydrogenase activity were diminished. All hepatocyte mitochondria were enlarged, pleomorphic, and had an expanded matrix and no mitochondrial dense bodies. The histopathology and ultrastructural pathology of liver biopsy specimens in salicylate intoxication were different from those in Reye's syndrome. In children in whom the diagnosis of Reye's syndrome is obscure, liver biopsy with electron microscopic examination is necessary for definitive diagnosis.

Child↗

PubMedDate not supplied

Reye's syndrome: current concepts.

Despite greater than 23 years of study, an incomplete understanding of the etiology, epidemiology and pathogenesis of Reye's syndrome persists. Better understanding of the disease has been hampered by the lack of a good animal model on which hypotheses of its pathogenesis could be tested. Human studies indicate that a primary mitochondrial injury may lead to complex metabolic disturbances that produce the observed pathophysiology. Specific directions regarding avenues for future research should pursue two lines: a good animal model still needs to be developed in which the biochemical and morphologic alterations identified in Reye's syndrome are duplicated. This model should include an antecedent viral illness but may not require aspirin exposure as an essential ingredient. With the identification of a satisfactory model, specific questions about the roles of environmental toxins or medications may be answered. Study of noncomatose cases of Reye's syndrome should continue. The specific emphasis should be to delineate what factors (NH3, free fatty acids and dicarboxylic acids) may be implicated in the pathogenesis of the CNS disease with the hopes of devising strategies for more effective treatment of encephalopathy and its attendant morbidity and mortality.

Adolescent↗

PubMedDate not supplied

Brain ultrastructure in Reye's disease. II. Acute injury and recovery processes in three children.

Acute and recovery biopsies of three patients with Reye's Disease are described. Pleomorphic changes of neuronal mitochondria were identified in all of the acute biopsies, similar in appearance to the characteristic alterations of hepatic mitochondria. Distinctive myelin bleb formation may be directly attributable to the mitochondrial injury. The mitochondrial lesion is reversible. There is morphologic evidence for regeneration and repair of myelin; but the presence of myelin ovoids at long intervals after recovery indicates a loss of some myelinated fibers. The neuronal mitochondrial changes, pleomorphism with matrix expansion, and myelin bleb formation, reflect a specific biochemical injury be attributable to ischemic injury secondary to brain edema.

Adolescent↗