RESUMO
El síndrome de Alagille es una patología poco frecuente. Afecta a uno de cada 100 000 recién nacidos vivos. Se caracteriza por una hipoplasia de vías biliares que se asocia a otras malformaciones. Se presenta el caso de un niño chino de cuatro años al que se le diagnosticó esta patología tras ser adoptado a los 11 meses de edad (AU)
Alagille syndrome is an uncommon pathology. It is found in 1/100,000 live births. It is characterized by biliary duct hypoplasia associated with other malformations. We report the case of a four-year-old Chinese child who was diagnosed with this pathology after being adopted at 11 months of age (AU)
Assuntos
Humanos , Masculino , Lactente , Síndrome de Alagille/dietoterapia , Síndrome de Alagille/patologia , Doenças dos Ductos Biliares/complicações , Colestase/complicações , Atenção Primária à Saúde , Hipertelorismo/complicações , Cardiopatias/complicações , Cardiopatias , Tomografia Computadorizada de Emissão/métodos , Tomografia Computadorizada de EmissãoAssuntos
Síndrome de Alagille/diagnóstico , Síndrome de Alagille/complicações , Síndrome de Alagille/dietoterapia , Ductos Biliares/patologia , Criança , Opacidade da Córnea/etiologia , Dieta com Restrição de Gorduras , Hepatomegalia/etiologia , Humanos , Índia , Icterícia/etiologia , Masculino , Prurido/etiologia , Estenose da Valva Pulmonar/etiologiaRESUMO
Two children were weaned from long-term tube feeding after liver transplant because of Alagille syndrome. The children were successfully weaned, one in seven days and the other in 13 days, using our standard and highly specialized intensive treatment protocol. Normal feeding behavior and stabilization of body weight were established. Children fed by long-term enteral tubes can be weaned from enteral feeding even after a long period of treatment. The return to age-appropriate self-feeding should be introduced as early as possible. Our weaning program time is brief and effective and can be recommended generally to improve quality of life and withhold unintended side-effects of enteral nutrition.
