Document Detail


Use of human somatotrophin in the treatment of a patient with methylmalonic aciduria.
MedLine Citation:
PMID:  8529687     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
Growth hormone (GH) insufficiency was demonstrated in a patient with methylmalonic aciduria. GH administration at 14-21 U/m2 per week accelerated linear growth, stimulated lipolysis, and produced clinical improvement and reduced urinary methylmalonate excretion. The clinical and metabolic benefits were lost as the dose of GH was increased to 28 U/m2 per week. CONCLUSION. The use of GH in other patients with methylmalonic aciduria (and other disorders of intermediary metabolism demonstrating similar clinical and metabolic features) warrants further study.
Authors:
M D Bain; S S Nussey; M Jones; R A Chalmers
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Publication Detail:
Type:  Case Reports; Journal Article    
Journal Detail:
Title:  European journal of pediatrics     Volume:  154     ISSN:  0340-6199     ISO Abbreviation:  Eur. J. Pediatr.     Publication Date:  1995 Oct 
Date Detail:
Created Date:  1996-01-29     Completed Date:  1996-01-29     Revised Date:  2004-11-17    
Medline Journal Info:
Nlm Unique ID:  7603873     Medline TA:  Eur J Pediatr     Country:  GERMANY    
Other Details:
Languages:  eng     Pagination:  850-2     Citation Subset:  IM    
Affiliation:
Department of Child Health, St George's Hospital Medical School, London, United Kingdom.
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MeSH Terms
Descriptor/Qualifier:
Amino Acid Metabolism, Inborn Errors / enzymology,  therapy*
Body Height / drug effects
Child
Female
Follow-Up Studies
Growth Hormone / deficiency,  therapeutic use*
Humans
Lipid Mobilization / drug effects
Methylmalonic Acid / urine*
Methylmalonyl-CoA Mutase / deficiency
Recombinant Proteins / therapeutic use
Chemical
Reg. No./Substance:
0/Recombinant Proteins; 516-05-2/Methylmalonic Acid; 9002-72-6/Growth Hormone; EC 5.4.99.2/Methylmalonyl-CoA Mutase

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


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