Document Detail

Glutathione deficiency as a complication of methylmalonic acidemia: response to high doses of ascorbate.
MedLine Citation:
PMID:  8804337     Owner:  NLM     Status:  MEDLINE    
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.
E Treacy; L Arbour; P Chessex; G Graham; L Kasprzak; K Casey; L Bell; O Mamer; C R Scriver
Related Documents :
24023287 - Antitumor activity of elacytarabine combined with bevacizumab, cetuximab and trastuzuma...
6677877 - Pediatric dosing of acetaminophen.
22236347 - The effects of age on maintenance of intense neuromuscular block with rocuronium.
17458517 - Effect of monosodium glutamate on oxidative stress and apoptosis in rat thymus.
25382757 - Evaluation of the selective glucocorticoid receptor agonist compound a for ototoxic eff...
3661117 - Effect of clomiphene citrate on the ovary of a wild rat, bandicota bengalensis.
Publication Detail:
Type:  Case Reports; Journal Article    
Journal Detail:
Title:  The Journal of pediatrics     Volume:  129     ISSN:  0022-3476     ISO Abbreviation:  J. Pediatr.     Publication Date:  1996 Sep 
Date Detail:
Created Date:  1996-10-22     Completed Date:  1996-10-22     Revised Date:  2004-11-17    
Medline Journal Info:
Nlm Unique ID:  0375410     Medline TA:  J Pediatr     Country:  UNITED STATES    
Other Details:
Languages:  eng     Pagination:  445-8     Citation Subset:  AIM; IM    
Biochemical Genetics Unit, Montreal Children's Hospital, McGill University, Montreal, Quebec, Canada.
Export Citation:
APA/MLA Format     Download EndNote     Download BibTex
MeSH Terms
Acidosis, Lactic / etiology,  therapy
Acute Disease
Amino Acid Metabolism, Inborn Errors / complications*,  therapy
Ascorbic Acid / administration & dosage*
Glutathione / deficiency*
Methylmalonic Acid / blood*
Renal Dialysis
Reg. No./Substance:
50-81-7/Ascorbic Acid; 516-05-2/Methylmalonic Acid; 70-18-8/Glutathione

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine

Previous Document:  Biliary sludge in sickle cell disease.
Next Document:  Isovaleric acidemia: response to a leucine load after three weeks of supplementation with glycine, L...