Nasobiliary drainage prior to surgical biliary diversion in progressive familial intrahepatic cholestasis type II

被引：0
作者：
Giulia Jannone
Xavier Stephenne
Isabelle Scheers
Françoise Smets
Catherine de Magnée
Raymond Reding
Etienne M. Sokal
机构：
[1] Cliniques Universitaires Saint-Luc,Department of Pediatrics, Pediatric Gastroenterology and Hepatology Unit
[2] Cliniques Universitaires Saint-Luc,Department of Surgery, Pediatric Surgery and Transplantation Unit
来源：
European Journal of Pediatrics | 2020年 / 179卷
关键词：
Biliary diversion; Nasobiliary drainage; Familial cholestasis; Pruritus; Bile acids;
D O I：
暂无
中图分类号：
学科分类号：
摘要：
Progressive familial intrahepatic cholestasis (PFIC) can cause intense pruritus that is refractory to medical therapy. Surgical biliary diversion techniques, including partial internal biliary diversion (PIBD), have been developed over the years to relieve pruritus without requiring liver transplantation. No clinical or genetic features can currently predict postoperative pruritus response. We present three PFIC type 2 (PIFC 2) patients who underwent transient endoscopic nasobiliary drainage (NBD) prior to PIBD surgery. Two patients repeatedly responded to NBD and presented with complete pruritus resolution after subsequent PIBD. NBD failed technically in the third patient, and PIBD was partially successful. Mild post-endoscopic biological pancreatitis occurred in 2/6 NBD procedures and resolved spontaneously. The only adverse effect observed within 7 years post-PIBD was very mild transient osmotic diarrhea.
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页码：1547 / 1552
页数：5
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