From the Department of Pediatric Gastroenterology, Gazi University, Ankara, Turkey.
Exp Clin Transplant. 2022 May;20(Suppl 3):81-84. doi: 10.6002/ect.PediatricSymp2022.O27.
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.
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。