Publication Date:
1980-02-08
Description:
Treatment of a patient deficient in carbamyl phosphate synthetase with benzoate or phenylacetic acid resulted in an increase in urinary nitrogen, which could be accounted for by the respective amino acid acylation product, hippurate or phenylacetylgultamine. Benzoate treatment of four hyperammonemic comatose patients led to clinical improvement and a return of plasma ammonium levels toward normal.〈br /〉〈span class="detail_caption"〉Notes: 〈/span〉Brusilow, S -- Tinker, J -- Batshaw, M L -- New York, N.Y. -- Science. 1980 Feb 8;207(4431):659-61.〈br /〉〈span class="detail_caption"〉Record origin:〈/span〉 〈a href="http://www.ncbi.nlm.nih.gov/pubmed/6243418" target="_blank"〉PubMed〈/a〉
Keywords:
Adolescent
;
Amino Acid Metabolism, Inborn Errors/*drug therapy
;
Amino Acids/blood
;
Ammonia/blood
;
Benzoates/*therapeutic use
;
Carbamoyl-Phosphate Synthase (Ammonia)/*deficiency
;
Child
;
Female
;
Glutamine/metabolism
;
Glycine/metabolism
;
Hippurates/urine
;
Humans
;
Infant
;
Male
;
Nitrogen/blood
;
*Ornithine Carbamoyltransferase Deficiency Disease
;
Phenylacetates/*therapeutic use
;
Phosphotransferases/*deficiency
Print ISSN:
0036-8075
Electronic ISSN:
1095-9203
Topics:
Biology
,
Chemistry and Pharmacology
,
Computer Science
,
Medicine
,
Natural Sciences in General
,
Physics
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