Assuntos
Erros Inatos do Metabolismo dos Aminoácidos/complicações , Anorexia/terapia , Terapia Comportamental , Transtornos do Comportamento Infantil/terapia , Transtornos da Alimentação e da Ingestão de Alimentos/terapia , Adulto , Erros Inatos do Metabolismo dos Aminoácidos/líquido cefalorraquidiano , Anorexia/etiologia , Ácido Argininossuccínico/urina , Criança , Transtornos do Comportamento Infantil/etiologia , Pré-Escolar , Ingestão de Energia , Comportamento Alimentar , Feminino , Ácido Homovanílico/líquido cefalorraquidiano , Humanos , Ácido Hidroxi-Indolacético/líquido cefalorraquidiano , Lactente , Masculino , Ácido Metilmalônico/sangue , Doença da Deficiência de Ornitina Carbomoiltransferase , Propionatos/sangueRESUMO
We studied serotonin metabolism in a metabolically stable 7-year-old girl with argininosuccinic aciduria who had severe anorexia. The CSF concentration of 5-hydroxyindoleacetic acid (HIAA), the metabolite of serotonin, was markedly elevated at 79 ng/ml (normal 33 +/- 11 ng/ml). Altered serotonin metabolism was also reflected in the sleep EEG, which showed decreased REM sleep. Reducing her intake of tryptophan, the precursor of serotonin, from 35 mg/kg/day to 7 mg/kg/day resulted in a decrease in CSF concentration of HIAA to 20 ng/ml and the onset of spontaneous eating for the first time in 4 1/2 years. REM sleep increased from 3% to 9.5% of total sleep time. Two days after increasing tryptophan intake to 25 mg/kg/day, spontaneous feeding stopped, associated with a rise in CSF HIAA to 45 ng/ml. Caloric/carbohydrate intake was found to affect CSF HIAA levels and food intake in an additive manner with tryptophan intake. These observations suggest that altered serotonin metabolism affected feeding behavior in this child, and that this effect could be modified by changing tryptophan or carbohydrate intake.