Question
In Reye’s syndrome, the histological finding is-
| A. |
Budding and branching of mitochondria |
| B. |
Swelling of endoplasmic reticulum |
| C. |
Paranuclear micro dense deposits |
| D. |
Glycogen depletion |
|
Correct Answer � D Explanation |
 |
Ans.D. Glycogen depletion
Histology of the liver on Reye’s syndrome:
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Histology of the liver shows diffuse microvesicular steatosis (fatty vacuolization) with minimal inflammatory changes.
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Glycogen is virtually absent from the hepatocytes in a biopsy specimen.
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Mitochondria are large pleomorphic and have decreased matricial density on electron microscopy.
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The kidney changes include swelling and fatty degeneration of proximal tubules.
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Astrocyte edema and loss of neurons in the brain.
Reye’s syndrome
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It is an acute self-limiting metabolic insult of diverse etiology resulting in generalized mitochondrial dysfunction due to inhibition of fatty acid 8 oxidation.
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Salicylates or viral infection can precipitate this metabolic impairment.
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The pathogenesis is thought to be damage to mitochondria caused by salicylate metabolite or some other toxin or chemical in the setting of viral infection.
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The reasons for mitochondrial dysfunction are unknown. No toxic factor has yet been conclusively identified but studies have suggested an etiological link among Reye’s syndrome.
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The activities of hepatic mitochondrial enzymes including ornithine transcarbamylase, carbamoyl phosphate synthetase, and pyruvate dehydrogenase are reduced often to less than half their normal values.
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Mitochondrial dysfunction leads to elevated short-chain fatty acids and leads directly to cerebral edema.
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It is prudent to avoid the use of aspirin as an antipyretic in pediatric patients with influenza or varicella.
Clinical manifestations:-
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Classic Reye’s syndrome exhibits a stereotypic biphasic course.
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It usually occurs in a previously healthy child.
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A prodromal febrile illness, an upper respiratory tract infection, or chickenpox is followed by an interval in which the child has seemingly recovered.
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The abrupt onset of protracted vomiting then occurs usually within 5-7 days after the onset of the viral illness.
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Delirium, combative behavior, and stupor may occur simultaneously within a few hours after the onset of vomiting.
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Neurological symptoms may rapidly progress to seizures, coma, and death; focal neurological signs are absent.
Laboratory features:-
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There is an explosive release from the liver and muscle of enzymes such as aminotransferases, creatine kinase, and lactic dehydrogenase.
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The activity of the mitochondrial enzyme serum glutamate dehydrogenase is greatly increased.
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The Serum Ammonia level is increased.
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The prothrombin time is mild to moderately increased.
