Recovery of graft steatosis and protein-losing enteropathy after biliary diversion in a PFIC 1 liver transplanted child

Emanuele Nicastro, Xavier Stephenne, Françoise Smets, Fabio Fusaro, Catherine De Magnée, Raymond Reding, Etienne M. Sokal

Research output: Contribution to journalArticle

Abstract

PFIC 1 is a genetic disorder characterized by hepatic and gastrointestinal disease, often requiring LT during childhood. Extrahepatic symptoms, such as diarrhea and malabsorption, do not improve or may be aggravated after LT, as graft steatosis or steatohepatitis as consequences of the interaction between transplanted liver and native bowel. We describe a patient with PFIC 1 who presented with cholestasis in infancy, who developed intractable pruritus and liver fibrosis. The child underwent living donor LT at 3.6 yr of age, and he early developed severe refractory diarrhea, secondary malabsorption with protein-losing enteropathy, and an early fatty liver disease trough graft steatohepatitis. As the response to cholestyramine was unsatisfactory, we decided to perform an EBD by using the jejunal loop used for the cholangiojejunostomy. Diarrhea resolved rapidly after surgery. He remained well after six months following biliary diversion, with normal stool output and no protein loss. We documented a dramatic improvement of graft steatosis at histology as well as normalization of liver function test. EBD can be considered a valuable treatment option to avoid organ disfunction and loss in PFIC 1 transplanted patients who develop graft steatohepatitis.

Original languageEnglish
JournalPediatric Transplantation
Volume16
Issue number5
DOIs
Publication statusPublished - Aug 2012

Keywords

  • diarrhea
  • external biliary diversion
  • graft steatohepatitis
  • liver transplantation
  • progressive familial intrahepatic cholestasis type 1
  • protein-losing enteropathy

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Transplantation

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