Nasobiliary drainage prior to surgical biliary diversion in progressive familial intrahepatic cholestasis type II
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ORIGINAL ARTICLE
Nasobiliary drainage prior to surgical biliary diversion in progressive familial intrahepatic cholestasis type II Giulia Jannone 1 & Xavier Stephenne 1 & Isabelle Scheers 1 & Françoise Smets 1 & Catherine de Magnée 2 & Raymond Reding 2 & Etienne M. Sokal 1 Received: 27 December 2019 / Revised: 18 March 2020 / Accepted: 27 March 2020 # Springer-Verlag GmbH Germany, part of Springer Nature 2020
Abstract 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. Conclusion: Our limited data suggest that NBD is a safe and effective way to predict pruritus response before performing permanent biliary diversion surgery in PFIC patients. What is Known: • Surgical biliary diversion techniques have been developed to relieve intractable pruritus in progressive familial intrahepatic cholestasis (PFIC). • No clinical or genetic features can currently predict pruritus response to surgery. What is New: • Our data suggest that nasobiliary drainage could be a safe and effective tool to predict pruritus response to biliary diversion and avoid unnecessary surgery in PFIC patients.
Keywords Biliary diversion . Nasobiliary drainage . Familial cholestasis . Pruritus . Bile acids
Communicated by Peter de Winter * Giulia Jannone [email protected]
Raymond Reding [email protected]
Xavier Stephenne [email protected] Isabelle Scheers [email protected]
Etienne M. Sokal [email protected] 1
Department of Pediatrics, Pediatric Gastroenterology and Hepatology Unit, Cliniques Universitaires Saint-Luc, Avenue Hippocrate 10, Brussels 1200, Belgium
2
Department of Surgery, Pediatric Surgery and Transplantation Unit, Cliniques Universitaires Saint-Luc, Brussels, Belgium
Françoise Smets [email protected] Catherine de Magnée [email protected]
Eur J Pediatr
Abbreviations ERCP Endoscopic retrograde cholangiopancreatography MARS Molecular adsorbents recirculating system NBD Nasobiliary drainage PEBD Partial external biliary diversion PFIC Progressive familial intrahepatic cholestasis PIBD Partial internal biliary diversion TBA Serum total bile acids
Materials and methods
Introduction
Endoscopic nasob
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