NonKetotic hypoglycemia: An early indicator of systemic carnitine deficiency

Alfred E. Slonim, Peggy R. Borum, Robert E. Mrak, Jennifer Najjar, Douglas Richardson, Michael P. Diamond

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Abstract

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.

Original languageEnglish (US)
Pages (from-to)29-33
Number of pages5
JournalNeurology
Volume33
Issue number1
Publication statusPublished - Jan 1983
Externally publishedYes

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ASJC Scopus subject areas

  • Clinical Neurology

Cite this

Slonim, A. E., Borum, P. R., Mrak, R. E., Najjar, J., Richardson, D., & Diamond, M. P. (1983). NonKetotic hypoglycemia: An early indicator of systemic carnitine deficiency. Neurology, 33(1), 29-33.