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Optimal liver transplant procedure in progressive familial intrahepatic cholestasis type 1 treated with biliary diversion or intestinal transplantation: Lessons learned from three cases treated with different approaches by Hajime Uchida; Seisuke Sakamoto; Ryuji Komine; Tasuku Kodama; Toshimasa Nakao; Yusuke Yanagi; Seiichi Shimizu; Syed Hasnain Abbas; Akinari Fukuda; Mureo Kasahara is a Medicine article available to read on EtoBox.

What is Optimal liver transplant procedure in progressive familial intrahepatic cholestasis type 1 treated with biliary diversion or intestinal transplantation: Lessons learned from three cases treated with different approaches about?

## Abstract ## Background Progressive familial intrahepatic cholestasis type 1 (PFIC1) is an autosomal recessive cholestatic liver disorder caused by ATP8B1 gene mutations. Although liver transplantation (LT) is indicated for progressive liver disease, postoperative complications, including severe diarrhea and graft steatohepatitis leading to graft loss, have been reported. ## Cases The first patient had jaundice, pruritus, diarrhea, and growth retardation (weight z‐score: −2.5; height z‐score: −3.7). She underwent LT with total internal biliary diversion (TIBD) to the colon at 2 years of age. Graft biopsy at the 7‐year follow‐up examination revealed microvesicular steatosis (60%). Her diarrhea improved, and her growth failure was recovering (weight z‐score: −1.0; height z‐score: −1.7). The second patient underwent sequential intestine–liver transplantation at 8 years of age due to end‐stage liver disease (ESLD) and short bowel syndrome caused by massive bowel resection for internal hernia after partial external biliary diversion (PEBD) at 21 months of age. She developed severe pancreatitis induced by steroid–bolus therapy for rejection after transplantation. She died 1.7 years aft

Who reads Optimal liver transplant procedure in progressive familial intrahepatic cholestasis type 1 treated with biliary diversion or intestinal transplantation: Lessons learned from three cases treated with different approaches?

Author

Hajime Uchida; Seisuke Sakamoto; Ryuji Komine; Tasuku Kodama; Toshimasa Nakao; Yusuke Yanagi; Seiichi Shimizu; Syed Hasnain Abbas; Akinari Fukuda; Mureo Kasahara

Publisher

Wiley

Published

2023

Language

EN

Field

Medicine (Health Sciences)