GLUTATHIONE DEFICIENCY AS A COMPLICATION OF METHYLMALONIC ACIDEMIA - RESPONSE TO HIGH-DOSES OF ASCORBATE

Authors
TREACY E ARBOUR L CHESSEX P GRAHAM G KASPRZAK L CASEY K BELL L MAMER O SCRIVER CR
Citation
E. Treacy et al., GLUTATHIONE DEFICIENCY AS A COMPLICATION OF METHYLMALONIC ACIDEMIA - RESPONSE TO HIGH-DOSES OF ASCORBATE, The Journal of pediatrics, 129(3), 1996, pp. 445-448
Citations number
14
Categorie Soggetti
Pediatrics
Journal title
The Journal of pediatrics → ACNP
ISSN journal
00223476
Volume
129
Issue
3
Year of publication
1996
Pages
445 - 448
Database
ISI
SICI code
0022-3476(1996)129:3<445:GDAACO>2.0.ZU;2-E
Abstract
A 7-year-old boy with deficient activity of methylmalonyl coenzyme A m utase (mut(-) methylmalonic acidemia) was seen in severe metabolic cri sis. After hemodialysis and clearance of toxic metabolites, severe lac tic acidosis persisted with multiorgan failure. Glutathione deficiency was noted and high-dose ascorbate therapy (120 mg/kg) commenced. Glut athione deficiency may contribute to the lactic acidosis observed duri ng decompensation in patients with methylmalonic acidemia.