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Glutathione deficiency as a complication of methylmalonic acidemia: response to high doses of ascorbate.

Abstract
A 7-year-old boy with deficient activity of methylmalonyl coenzyme A mutase (mut-methylmalonic acidemia) was seen in severe metabolic crisis. After hemodialysis and clearance of toxic metabolites, severe lactic acidosis persisted with multiorgan failure. Glutathione deficiency was noted and high-dose ascorbate therapy (120 mg/kg) commenced. Glutathione deficiency may contribute to the lactic acidosis observed during decompensation in patients with methylmalonic acidemia.
AuthorsE Treacy, L Arbour, P Chessex, G Graham, L Kasprzak, K Casey, L Bell, O Mamer, C R Scriver
JournalThe Journal of pediatrics (J Pediatr) Vol. 129 Issue 3 Pg. 445-8 (Sep 1996) ISSN: 0022-3476 [Print] United States
PMID8804337 (Publication Type: Case Reports, Journal Article)
Chemical References
  • Methylmalonic Acid
  • Glutathione
  • Ascorbic Acid
Topics
  • Acidosis, Lactic (etiology, therapy)
  • Acute Disease
  • Amino Acid Metabolism, Inborn Errors (complications, therapy)
  • Ascorbic Acid (administration & dosage)
  • Child
  • Glutathione (deficiency)
  • Humans
  • Male
  • Methylmalonic Acid (blood)
  • Renal Dialysis

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