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Recovery of graft steatosis and protein‐losing enteropathy after biliary diversion in a PFIC 1 liver transplanted child
Author(s) -
Nicastro Emanuele,
Stephenne Xavier,
Smets Françoise,
Fusaro Fabio,
de Magnée Catherine,
Reding Raymond,
Sokal Etienne M.
Publication year - 2012
Publication title -
pediatric transplantation
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.457
H-Index - 69
eISSN - 1399-3046
pISSN - 1397-3142
DOI - 10.1111/j.1399-3046.2011.01514.x
Subject(s) - medicine , steatosis , enteropathy , malabsorption , gastroenterology , protein losing enteropathy , steatohepatitis , progressive familial intrahepatic cholestasis , congenital hepatic fibrosis , liver transplantation , fatty liver , short bowel syndrome , diarrhea , cholestasis , transplantation , cirrhosis , disease , portal hypertension , parenteral nutrition
Nicastro E, Stephenne X, Smets F, Fusaro F, de Magnée C, Reding R, Sokal EM. Recovery of graft steatosis and protein‐losing enteropathy after biliary diversion in a PFIC 1 liver transplanted child. 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.