A child suffered from a viral illness for which he took aspirin. 3 days later the child presented to the emergency with altered sensorium and icterus. What is the underlying biochemical defect -: (A) Beta oxidation of fatty acids, (B) Glucose-6-phosphatase deficiency, (C) Pyruvate dehydrogenase deficiency, (D) Urea cycle defect
Answer is A. Reye syndrome is characterized by acute non-inflammatory encephalopathy and fatty degenerative liver failure. Reye syndrome typically occurs after a viral illness and is associated with the use of aspirin during the illness. The pathogenesis of Reye syndrome: Appears to involve mitochondrial injury resulting in dysfunction that inhibits oxidative phosphorylation and fatty-acid beta-oxidation