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.

Posttransplant metabolic syndrome in children and adolescents after liver transplantation: A systematic review

“…Posttransplant NAFLD in pediatric liver transplant recipients has been documented only in case series and reports69‐77 (Table 5). Four of the 19 children reported in these studies underwent transplantation for cirrhosis associated with nonalcoholic steatohepatitis (NASH) 69‐71.…”

“…Twelve children with posttransplant NAFLD had progressive intrahepatic familial cholestasis type 1 (PFIC1); none were overweight. Instead, 11 had chronic diarrhea and malnutrition thought to be secondary to restored bile flow from the liver (with persistently abnormal intestinal reabsorption) and pancreatic insufficiency 76. NASH was resolved for 1 patient after external biliary diversion 76…”

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

“…Surgical techniques have included external biliary diversion versus internal biliary diversion in both the pre‐LT and post‐LT setting. As previously mentioned, Nicastro et al reported successful outcome with external biliary diversion using a jejunal loop in a post‐LT patient with improvement in diarrhea within 1 week, resolution of protein‐losing enteropathy, and dramatic reversal of steatosis at 6 months. Usui et al reported a patient who underwent an external biliary diversion with a jejunal loop at the time of re–liver transplantation.…”

Pathology of Pediatric Gastrointestinal and Liver Disease

“…This interesting phenomenon may have to do with faulty gut regulation of uptake of bile acids and other substances (Oude Elferink et al 2002 ;Chen et al 2004 ). Both diarrhea and hepatic steatosis after LTX may respond to biliary diversion (Nicastro et al 2012 ), that is, to directing bile from the allograft liver away from the native small bowel. Both diarrhea and hepatic steatosis after LTX may respond to biliary diversion (Nicastro et al 2012 ), that is, to directing bile from the allograft liver away from the native small bowel.…”