title: 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.
authors.affiliation: Département de Pédiatrie, Cliniques Universitaires Saint Luc, Université Catholique de Louvain, Brussels, Belgium.
authors.forename: Emanuele
authors.initials: E
authors.lastname: Nicastro
authors.forename: Xavier
authors.initials: X
authors.lastname: Stephenne
authors.forename: Françoise
authors.initials: F
authors.lastname: Smets
authors.forename: Fabio
authors.initials: F
authors.lastname: Fusaro
authors.forename: Catherine
authors.initials: C
authors.lastname: de Magnée
authors.forename: Raymond
authors.initials: R
authors.lastname: Reding
authors.forename: Etienne M
authors.initials: EM
authors.lastname: Sokal
mesh_terms: D001662:Biliary Tract Surgical Procedures / Q000379:methods*; D002675:Child, Preschool; D002780:Cholestasis, Intrahepatic / Q000601:surgery*; D005234:Fatty Liver / Q000175:diagnosis / Q000209:etiology / Q000601:surgery*; D006801:Humans; D007582:Jejunostomy / Q000379:methods*; D016031:Liver Transplantation; D008297:Male; D011183:Postoperative Complications / Q000175:diagnosis / Q000601:surgery*; D011504:Protein-Losing Enteropathies / Q000175:diagnosis / Q000209:etiology / Q000601:surgery*