Outcomes of pediatric liver transplantation for progressive familial intrahepatic cholestasis.

Anu K Vasudevan,Naresh Shanmugam,Ashwin Rammohan,Joseph J Valamparampil,Kshetra Rinaldhy,Jagadeesh Menon,Ravikumar Thambithurai,Saravanapandian Namasivayam,Ilankumaran Kaliamoorthy,Mohamed Rela

Pediatric transplantation（2023）

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Abstract

Ours is the largest to-date series of LT for PFIC illustrating their short- and long-term outcomes. While the results for the whole cohort were excellent, those after LT for PFIC1 was relatively poorer as reflected by catch-up growth, graft steatosis, and post-LT diarrhea, which can be optimized by the addition of TIBD during LT.

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Key words

outcomes, progressive familial intrahepatic cholestasis, quality of life, Total internal biliary diversion

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