Lipoamide dehydrogenase deficiency with primary lactic acidosis: Favorable response to treatment with oral lipoic acid

Reuben Matalon, David A. Stumpf, Kimberlee Michals, Robert D. Hart, Janice K. Parks, Stephen I. Goodman

Research output: Contribution to journalArticle

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An 8-month-old boy with severe lactic acidosis was found to have lipoamide dehydrogenase deficiency. Treatment with thiamine, biotin, bicarbonate, protein restriction, and ketogenic diet failed to alleviate the lactic acidosis. Oral administration of lipoic acid 25 to 50 mg/kg produced dramatic improvement in lactic and pyruvic acidemia, which has continued for 2 years and which has been accompanied by clinical improvement.

Original languageEnglish (US)
Pages (from-to)65-69
Number of pages5
JournalThe Journal of Pediatrics
Issue number1
StatePublished - Jan 1984


ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health

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