Biliary drainage as treatment for allograft steatosis following liver transplantation for PFIC-1 disease: A single-center experience

Leina S Alrabadi; Raffaella A Morotti; Pamela L Valentino; Manuel I Rodriguez-Davalos; Udeme D Ekong; Sukru H Emre

doi:10.1111/petr.13184

Biliary drainage as treatment for allograft steatosis following liver transplantation for PFIC-1 disease: A single-center experience

Pediatr Transplant. 2018 Jun;22(4):e13184. doi: 10.1111/petr.13184. Epub 2018 Apr 14.

Authors

Leina S Alrabadi¹, Raffaella A Morotti², Pamela L Valentino¹, Manuel I Rodriguez-Davalos³, Udeme D Ekong¹, Sukru H Emre³

Affiliations

¹ Department of Pediatrics, Section of Pediatric Gastroenterology and Hepatology, Yale University School of Medicine, New Haven, CT, USA.
² Department of Pathology, Yale University School of Medicine, New Haven, CT, USA.
³ Department of Surgery, Section of Transplantation and Immunology, Yale University School of Medicine, New Haven, CT, USA.

PMID: 29654655
DOI: 10.1111/petr.13184

Abstract

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.

Publication types

Case Reports

MeSH terms

Allografts
Cholestasis, Intrahepatic / surgery*
Drainage / methods*
Fatty Liver / etiology
Fatty Liver / therapy*
Female
Humans
Infant
Liver Transplantation*
Male
Postoperative Complications / therapy*
Transplantation, Homologous

Supplementary concepts

Cholestasis, progressive familial intrahepatic 1