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

doi:10.1016/S0022-3476(84)80591-0

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

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Abstract

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 language	English (US)
Pages (from-to)	65-69
Number of pages	5
Journal	The Journal of Pediatrics
Volume	104
Issue number	1
DOIs	https://doi.org/10.1016/S0022-3476(84)80591-0
State	Published - Jan 1984
Externally published	Yes

ASJC Scopus subject areas

Pediatrics, Perinatology, and Child Health

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10.1016/S0022-3476(84)80591-0

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abstract = "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.",

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T1 - Lipoamide dehydrogenase deficiency with primary lactic acidosis

T2 - Favorable response to treatment with oral lipoic acid

AU - Matalon, Reuben

AU - Stumpf, David A.

AU - Michals, Kimberlee

AU - Hart, Robert D.

AU - Parks, Janice K.

AU - Goodman, Stephen I.

PY - 1984/1

Y1 - 1984/1

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AB - 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.

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

Abstract

ASJC Scopus subject areas

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