title: Quinolinic acid in children with congenital hyperammonemia.
abstract: 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.affiliation: Children's Seashore House, Philadelphia, PA 19104.
authors.forename: M L
authors.initials: ML
authors.lastname: Batshaw
authors.forename: M B
authors.initials: MB
authors.lastname: Robinson
authors.forename: K
authors.initials: K
authors.lastname: Hyland
authors.forename: S
authors.initials: S
authors.lastname: Djali
authors.forename: M P
authors.initials: MP
authors.lastname: Heyes
mesh_terms: D000641:Ammonia / Q000097:blood*; D001679:Biogenic Amines / Q000134:cerebrospinal fluid; D001708:Biopterins / Q000031:analogs & derivatives / Q000134:cerebrospinal fluid; D002675:Child, Preschool; D003128:Coma / Q000097:blood / Q000134:cerebrospinal fluid*; D006801:Humans; D006897:Hydroxyindoleacetic Acid / Q000134:cerebrospinal fluid; D007223:Infant; D007231:Infant, Newborn; D008661:Metabolism, Inborn Errors / Q000134:cerebrospinal fluid*; D019798:Neopterin; D017378:Quinolinic Acid / Q000134:cerebrospinal fluid*
chemical_list: D001679:Biogenic Amines; D001708:Biopterins; D006897:Hydroxyindoleacetic Acid; D019798:Neopterin; D000641:Ammonia; D017378:Quinolinic Acid