Abstract
The biochemical abnormalities of 16 disorders associated with pyruvate metabolism are discussed. The pathopneumonic symptoms of human hereditary pyruvate decarboxylase complex and pyruvate carboxylase enzyme deficiencies are presented, including periventricular hemorrhage, subacute necrotizing encephalomyelopathy, ataxia, spinocerebellar degeneration, infantile lactic acidosis, motion retardation, seizures, hypoglycemia and visual failure. Normal pyruvate metabolism is reviewed.