Assuntos
Terapia de Reposição de Enzimas , Iduronidase/uso terapêutico , Mucopolissacaridose I/complicações , Complicações Pós-Operatórias/tratamento farmacológico , Insuficiência Respiratória/tratamento farmacológico , Obstrução das Vias Respiratórias/etiologia , Obstrução das Vias Respiratórias/patologia , Ensaios de Uso Compassivo , Hemorragia/complicações , Humanos , Intubação Intratraqueal/efeitos adversos , Macroglossia/etiologia , Macroglossia/patologia , Masculino , Mucopolissacaridose I/tratamento farmacológico , Mucopolissacaridose I/patologia , Nasofaringe/patologia , Pneumonia Aspirativa/etiologia , Complicações Pós-Operatórias/etiologia , Proteínas Recombinantes/uso terapêutico , Insuficiência Respiratória/etiologia , Estenose Espinal/cirurgia , Traqueostomia , Adulto JovemRESUMO
OBJECTIVE: The aims of this work were to describe the therapeutic options available for severe hyperammonemia in children when ammonium levels are so high that the child's neurologic future or even their life is compromised. In addition, a comparison of the relative efficacy of each therapeutic method is made. PATIENTS AND METHODS: We present six cases, five of which suffered from inborn errors of metabolism and a six which presented with Reye's syndrome, all of whom presented or developed hyperammonemic coma. Their initial ammonium levels fluctuated between 300 and more than 2000 micrograms per deciliter. The treatment was made with exchange transfusion (ET), ET and peritoneal dialysis (DP) together or hemodialysis (HD). RESULTS: Peritoneal dialysis was the method that obtained the greatest reduction in plasma ammonium levels. However, the quickest reduction was observed with ET and HD. There were no significant complications with any of the methods except for hemodynamic deterioration in one patient during HD. CONCLUSIONS: We believe that HD is the treatment of choice for these patients because it is able to obtain a quick and lasting clearance of plasma ammonium. However, this method is not always available and has not been used very often in small babies. In these cases, the combined use of ET and DP can be very useful.
