title: [Liver coma with fatal course in 2 children under long-term antiepileptic therapy].
abstract: This is a report of two non related children who died after antiepileptic therapy for years in hepatic coma. In both children no other cause for hepatic failure could be found than treatment with anticonvulsive drugs. In both cases family history was interesting. Unexplained deaths in early infancy in the family of the girl, a carbamyl phosphate synthetase deficiency in the family of the boy, whose grandfather could have been heterozygote for this disturbance. It is supposed that heterozygotes for inborn errors of ammonia metabolism tolerate antiepileptic therapy worse showing increased hepatodiagnostic parameters, even necrosis of hepar and hepatic coma. In future examinations of the concerned families are suggested if disturbances of liver function are observed.
mesh_terms: D000328:Adult; D000592:Amino Acid Metabolism, Inborn Errors / Q000235:genetics; D000927:Anticonvulsants / Q000008:administration & dosage / Q000009:adverse effects*; D002222:Carbamoyl-Phosphate Synthase (Ammonia) / Q000172:deficiency; D002648:Child; D002675:Child, Preschool; D005260:Female; D006501:Hepatic Encephalopathy / Q000139:chemically induced* / Q000175:diagnosis / Q000235:genetics; D006801:Humans; D007231:Infant, Newborn; D008099:Liver / Q000473:pathology; D008111:Liver Function Tests; D008297:Male; D010375:Pedigree; D013398:Sudden Infant Death
chemical_list: D000927:Anticonvulsants; D002222:Carbamoyl-Phosphate Synthase (Ammonia)
vernacular_title: Leberkoma mit letalem Ausgang bei zwei Kindern unter antiepileptischer Dauertherapie.