Development of macrovesicular steatosis post-LT in patients with PFIC-1 is increasingly being observed, with the etiology not fully understood. We highlight successful and effective EBD for reversal of allograft steatosis in 2 patients with PFIC-1 disease and discuss our experience with internal biliary diversion in this patient population.
Keywords: ATP8B1; cholestasis; diarrhea; disease recurrence; graft dysfunction; jaundice.
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