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

Author:

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

Publisher

Elsevier BV

Subject

Pediatrics, Perinatology, and Child Health

Reference12 articles.

1. Mammalian alpha-ketoaciddehydrogenase complexes. VI. Nature of the multiple forms of pig heart lipoamide-dehydrogenase;Sakurai;J Biol Chem,1970

2. Deficiency of dihydrolipoyldehydrogenase (a component of the pyruvate and α-ketoglutarate dehydrogenase complexes): A cause of congenital lactic acidosis in infancy;Robinson;Pediatr Res,1977

3. The defect in branched chain amino acids metabolism in a patient with congenital lactic acidosis;Taylor;Pediatr Res,1978

4. Lactic acidosis due to lipoamide dehydrogenase deficiency: Improvement after oral lipoic acid;Matalon;Am J Hum Genet,1981

5. Diagnosis of organic acidemias by gas chromatography-mass spectroscopy;Goodman;Lab Res Methods Biol Med,1982

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