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肝移植后进行 1 型进行性家族性肝内胆汁淤积症患者的严重腹泻的部分外胆道分流术。

Partial External Biliary Diversion for Severe Diarrhea After Liver Transplant in Patients with Progressive Familial Intrahepatic Cholestasis Type 1.

机构信息

From the Department of Pediatric Gastroenterology, Gazi University, Ankara, Turkey.

出版信息

Exp Clin Transplant. 2022 May;20(Suppl 3):81-84. doi: 10.6002/ect.PediatricSymp2022.O27.

Abstract

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.

摘要

进行性家族性肝内胆汁淤积症是一组异质性的常染色体隐性遗传病,肝移植是唯一的治愈性治疗方法。对于无肝硬化的 1 型进行性家族性肝内胆汁淤积症患者,破坏肠肝循环的胆肠分流术也是一种选择。我们报告了 1 例因终末期肝病而行肝移植的 1 型进行性家族性肝内胆汁淤积症患儿。移植后,部分外引流术后腹泻和生长迟缓并发症得到解决。

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