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Nonketotic hypoglycemia

An early indicator of systemic carnitine deficiency

Departments of Pediatries, Biochemistry, and Pathology, Vanderbilt University Medical Center, Nashville, TN.

In systemic carnitine deficiency, an early phase of nonketotic hypoglycemia and liver dysfunction may precede a late phase of encephalopathy and myopathy. We studied two children with recurrent episodes of nonketotic hypoglycemia who were found to have systemic carnitine deficiency without myopathy or encephalopathy. Early institution of appropriate therapy prevented the onset of late debilitating complications in one patient, whereas therapeutic noncompliance in the other patient led to an episode of encephalopathy and myopathy. Metabolic studies indicated that decreased availability of alanine may be a factor in the genesis of hypoglycemia; concurrent depletion of cerebral glucose, ketones, and glycogen may be the cause of the encephalopathy.

Address correspondence and reprint requests to Dr. Slonim, Department of Pediatries, Vanderbilt University, Medical Center North, Nashville, TN 31232.

Supported by Clinical Research Grant from National Foundation-March of Dimes No. 6-239, BRSG RK-05424. and a grant from Vanderbilt University Research Council.

Accepted for publication June 4, 1982.