Document Detail

Hyperammonemia related to carnitine metabolism with particular emphasis on ornithine transcarbamylase deficiency.
MedLine Citation:
PMID:  3440448     Owner:  NLM     Status:  MEDLINE    
Carnitine status was evaluated in 8 patients with partial ornithine transcarbamylase (OTC) deficiency and 19 patients with secondary carnitine deficiency, who were used as positive references. Laboratory findings indicated that all patients with OTC deficiency had secondary carnitine deficiency especially in hyperammonemic attack. After L-carnitine administration in 2 patients with OTC deficiency, the number of attacks was significantly reduced in both cases.
I Matsuda; Y Ohtani; K Ohyanagi; S Yamamoto
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Publication Detail:
Type:  Journal Article    
Journal Detail:
Title:  Enzyme     Volume:  38     ISSN:  0013-9432     ISO Abbreviation:  Enzyme     Publication Date:  1987  
Date Detail:
Created Date:  1988-04-18     Completed Date:  1988-04-18     Revised Date:  2004-11-17    
Medline Journal Info:
Nlm Unique ID:  1262265     Medline TA:  Enzyme     Country:  SWITZERLAND    
Other Details:
Languages:  eng     Pagination:  251-5     Citation Subset:  IM    
Department of Pediatrics, Kumamoto University Medical School, Japan.
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MeSH Terms
Amino Acid Metabolism, Inborn Errors / blood,  metabolism*,  therapy
Ammonia / blood*
Benzoates / therapeutic use
Benzoic Acid
Carnitine / metabolism*,  therapeutic use
Dietary Proteins / administration & dosage
Reye Syndrome / metabolism,  physiopathology
Reg. No./Substance:
0/Benzoates; 0/Dietary Proteins; 541-15-1/Carnitine; 65-85-0/Benzoic Acid; 7664-41-7/Ammonia

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