回肠内转流术治疗1型进行性家族性肝内胆汁淤积症相关的肝移植术后移植物炎症和脂肪变性
Internal Ileal Diversion as Treatment for Progressive Familial Intrahepatic Cholestasis Type 1-Associated Graft Inflammation and Steatosis after Liver Transplantation.
作者信息
Kavallar Anna M, Messner Franka, Scheidl Stefan, Oberhuber Rupert, Schneeberger Stefan, Aldrian Denise, Berchtold Valeria, Sanal Murat, Entenmann Andreas, Straub Simon, Gasser Anna, Janecke Andreas R, Müller Thomas, Vogel Georg F
机构信息
Department of Paediatrics I, Medical University of Innsbruck, 6020 Innsbruck, Austria.
Department of Visceral, Transplant and Thoracic Surgery, Medical University of Innsbruck, 6020 Innsbruck, Austria.
出版信息
Children (Basel). 2022 Dec 14;9(12):1964. doi: 10.3390/children9121964.
BACKGROUND
Progressive Familial Intrahepatic cholestasis type I (PFIC1) is a rare congenital hepatopathy causing cholestasis with progressive liver disease. Surgical interruption of the enterohepatic circulation, e.g., surgical biliary diversion (SBD) can slow down development of liver cirrhosis. Eventually, end stage liver disease necessitates liver transplantation (LT). PFIC1 patients might develop diarrhea, graft steatosis and inflammation after LT. SBD after LT was shown to be effective in the alleviation of liver steatosis and graft injury.
CASE REPORT
Three PFIC1 patients received LT at the ages of two, two and a half and five years. Shortly after LT diarrhea and graft steatosis was recognized, SBD to the terminal ileum was opted to prevent risk for ascending cholangitis. After SBD, inflammation and steatosis was found to be reduced to resolved, as seen by liver biochemistry and ultrasounds. Diarrhea was reported unchanged.
CONCLUSION
We present three PFIC1 cases for whom SBD to the terminal ileum successfully helped to resolve graft inflammation and steatosis.
背景
I型进行性家族性肝内胆汁淤积症（PFIC1）是一种罕见的先天性肝病，可导致胆汁淤积并伴有进行性肝病。肠肝循环的手术阻断，例如外科胆道改道（SBD），可以减缓肝硬化的发展。最终，终末期肝病需要进行肝移植（LT）。PFIC1患者在肝移植后可能会出现腹泻、移植肝脂肪变性和炎症。肝移植后进行SBD已被证明对减轻肝脂肪变性和移植肝损伤有效。
病例报告
三名PFIC1患者分别在2岁、2岁半和5岁时接受了肝移植。肝移植后不久，发现了腹泻和移植肝脂肪变性，于是选择对回肠末端进行SBD以预防上行性胆管炎的风险。进行SBD后，从肝脏生化检查和超声检查结果来看，炎症和脂肪变性减轻至消退。据报告腹泻情况未变。
结论
我们报告了三例PFIC1病例，对其回肠末端进行SBD成功地帮助解决了移植肝炎症和脂肪变性问题。
Zotero 插件