Total biliary diversion as a treatment option for patients with progressive familial intrahepatic cholestasis and Alagille syndrome

Journal of Pediatric Surgery
Wendy L van der WoerdRoderick H Houwen

Abstract

Progressive familial intrahepatic cholestasis (PFIC) with low gamma-glutamyl transpeptidase (GGT) and Alagille syndrome are associated with persistent cholestasis and severe pruritus. Various types of biliary diversion have been used to reduce this pruritus and prevent liver dysfunction. We report our experience concerning the efficacy and safety of total biliary diversion (TBD) as an additional treatment option. TBD was performed in four PFIC patients and one patient with Alagille syndrome, and was accomplished by anastomosing a jejunal segment to the choledochal duct terminating as an end stoma, or by disconnecting the choledochal duct after previous cholecystojejunocutaneostomy. TBD resulted in a marked improvement of symptoms and biochemical parameters in all PFIC patients. Despite relief of pruritus, cholestasis persisted in the Alagille patient. During 5-15years of follow-up, no clinical signs of fat malabsorption such as diarrhea or weight loss were encountered. However, to maintain adequate levels of fat-soluble vitamins, especially of vitamin K, substantial supplementation was necessary. Total biliary diversion can be a useful surgical treatment option for patients with low-GGT PFIC and possibly also Alagille syndrome,...Continue Reading

References

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Apr 14, 2009·Journal of Pediatric Surgery·Adam Rahn DavisThomas B Newman
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Citations

Oct 27, 2016·Pediatric Transplantation·James E Squires
May 3, 2016·F1000Research·Martin Wagner, Michael Trauner
Feb 23, 2017·World Journal of Gastroenterology : WJG·Wendy L van der WoerdStan Fj van de Graaf
Jan 4, 2017·Journal of Pediatric Gastroenterology and Nutrition·James E SquiresRobert H Squires

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