Document Detail


Quinolinic acid in children with congenital hyperammonemia.
MedLine Citation:
PMID:  7694541     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
Levels of the excitotoxin quinolinic acid (QUIN) were measured in the cerebrospinal fluid of infants and children with congenital hyperammonemia. Twofold to tenfold elevations of QUIN were found in 4 neonates in hyperammonemic coma (QUIN range, 250-990 nM; control mean, 110 +/- 90 nM; p < 0.005). Similar elevations of neopterin were found (range, 24-75 nM; control mean, 9.0 +/- 4.9 nM; p < 0.005). In addition, significant elevations of QUIN were found in 14 older children with congenital hyperammonemia (mean, 50 +/- 20 vs 17 +/- 6 nM; p < 0.05). Neopterin levels were not elevated in these children. The QUIN may originate from an increase in tryptophan transport across the blood-brain barrier or from induction of indolamine-2,3-dioxygenase activity. These findings support a role for QUIN in the neuropathology of congenital hyperammonemia. They also suggest the potential utility of N-methyl-D-aspartate receptor-blocking agents or inhibitors of QUIN synthesis in the treatment of hyperammonemic coma.
Authors:
M L Batshaw; M B Robinson; K Hyland; S Djali; M P Heyes
Publication Detail:
Type:  Journal Article; Research Support, Non-U.S. Gov't; Research Support, U.S. Gov't, P.H.S.    
Journal Detail:
Title:  Annals of neurology     Volume:  34     ISSN:  0364-5134     ISO Abbreviation:  Ann. Neurol.     Publication Date:  1993 Nov 
Date Detail:
Created Date:  1993-12-06     Completed Date:  1993-12-06     Revised Date:  2007-11-14    
Medline Journal Info:
Nlm Unique ID:  7707449     Medline TA:  Ann Neurol     Country:  UNITED STATES    
Other Details:
Languages:  eng     Pagination:  676-81     Citation Subset:  IM    
Affiliation:
Children's Seashore House, Philadelphia, PA 19104.
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MeSH Terms
Descriptor/Qualifier:
Ammonia / blood*
Biogenic Amines / cerebrospinal fluid
Biopterin / analogs & derivatives,  cerebrospinal fluid
Child, Preschool
Coma / blood,  cerebrospinal fluid*
Humans
Hydroxyindoleacetic Acid / cerebrospinal fluid
Infant
Infant, Newborn
Metabolism, Inborn Errors / cerebrospinal fluid*
Neopterin
Quinolinic Acid / cerebrospinal fluid*
Grant Support
ID/Acronym/Agency:
P-30-HD26979/HD/NICHD NIH HHS; R01-NS 28033/NS/NINDS NIH HHS
Chemical
Reg. No./Substance:
0/Biogenic Amines; 22150-76-1/Biopterin; 54-16-0/Hydroxyindoleacetic Acid; 670-65-5/Neopterin; 7664-41-7/Ammonia; 89-00-9/Quinolinic Acid

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


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