RESUMO
Progressive familial intrahepatic cholestasis is a heterogeneous group of autosomal recessive disorders, and liver transplant is the only curative treatment. A biliary diversion operation for disruption of enterohepatic circulation in patients with progressive familial intrahepatic cholestasis type 1 without cirrhosis is another option. We present a pediatric patient with progressive familial intrahepatic cholestasis type 1 who underwent liver transplant due to end-stage liver disease. After transplant, diarrhea and growth retardation complications resolved after partial external biliary diversion surgery.
Assuntos
Colestase Intra-Hepática , Transplante de Fígado , Criança , Colestase Intra-Hepática/diagnóstico , Colestase Intra-Hepática/etiologia , Colestase Intra-Hepática/cirurgia , Diarreia/diagnóstico , Diarreia/etiologia , Diarreia/cirurgia , Humanos , Transplante de Fígado/efeitos adversos , Resultado do TratamentoRESUMO
Portal hypertensive biliopathy may occur in patients with noncirrhotic hepatic fibrosis. Portal hypertensive biliopathy treatment should be focused on management of portal hypertension and relief of biliary obstruction. In patients with noncirrhotic portal fibrosis and symptomatic portal hypertensive biliopathy, portal decompression surgery by proximal splenorenal shunt is one successful treatment option.