Abstracts
Isovaleric Acidemia
Isovaleric acidemia
Glycine in addition to L-carnitine may increase isovaleryl conjugate excretion during metabolic stress. The effects of glycine and carnitine therapy on isovaleryl conjugate excretion in isovaleric acidemia were investigated. The effects of supplementation with glycine alone, L-carnitine alone, and glycine and L-carnitine combined were studied in an 8-year-old patient. Glycine treatment doubled isovalerylglycine(IVG) excretion, while isovalerylcarnitine(IVC) excretion remained minimal. L-carnitine therapy decreased IVG excretion and increased IVC excretion. During glycine and L-carnitine combination therapy, excretion of both isovalerylglycine and isovalerylcarnitine were increased. Leucine challenge increased excretion of both IVG and IVC by 3.5 - 4-fold, which was the highest total conjugate excretion. The combination of glycine and L-carnitine maximally increases isovaleryl conjugate excretion during metabolic stress.
Fries, MH; Rinaldo, P; Schmidt, Sommerfeld E; Jurecki, E; Packman, S: Isovaleric acidemia: response to a leucine load after three weeks of supplementation with glycine, L-carnitine, and combined glycine-carnitine therapy, J-Pediatr. 1996 Sep; 129(3): 449-52
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