Fasting is accompanied by a decrease in the availability ofglucose for energy use in peripheral tissues and, consequently,an increased reliance of these tissues on the availability ofketone bodies and fatty acids for energy. The availability ofketone bodies depends almost exclusively on hepatic ketogenesis.Failure of ketogenesis may occur in patients with any defectof the enzymes associated with the mitochondrial oxidation offatty acids.1 These defects are typically manifested by hypoglycemia,which results from the inadequate supply of alternative substrate(ketones). Other clinical features are more variable and mayinclude myopathy, cardiomyopathy, hepatocellular damage, andneuropathies. . . . [Full Text of this Article]
Case Report
Methods
Provocative Tests
Analyses of Enzymes and Metabolites
Liver Biopsy
HMG-CoA Synthase Activity
Results
Discussion
Source Information
From the Murdoch Institute, Parkville, Victoria, Australia (G.N.T., E.T.); the Division of Endocrinology and Diabetes, Children's Hospital of Philadelphia, Philadelphia (B.Y.L.H., C.A.S.); and the Department of Biochemistry, Royal Children's Hospital, Parkville, Victoria, Australia (J.J.P.).
Address reprint requests to Dr. Thompson at 96 Kensington Rd., Toorak Gardens, S.A. 5065, Australia.
References
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