title: Total biliary diversion as a treatment option for patients with progressive familial intrahepatic cholestasis and Alagille syndrome.
abstract: BACKGROUND
  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.
  
  
  METHODS
  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.
  
  
  RESULTS
  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.
  
  
  CONCLUSIONS
  Total biliary diversion can be a useful surgical treatment option for patients with low-GGT PFIC and possibly also Alagille syndrome, when partial biliary diversion is insufficient. It can be performed without inducing clinical signs of fat malabsorption although individualized supplementation of fat-soluble vitamins with careful monitoring is warranted.
authors: Department of Pediatric Gastroenterology, Wilhelmina Children's Hospital, University Medical Centre Utrecht, Utrecht, The Netherlands. Electronic address: wwoerd@umcutrecht.nl.
authors: Wendy L
authors: WL
authors: van der Woerd
authors: Department of Pediatric Gastroenterology, Wilhelmina Children's Hospital, University Medical Centre Utrecht, Utrecht, The Netherlands.
authors: Freddy T
authors: FT
authors: Kokke
authors: Department of Pediatric Surgery, Wilhelmina Children's Hospital, University Medical Centre Utrecht, Utrecht, The Netherlands.
authors: David C
authors: DC
authors: van der Zee
authors: Department of Pediatric Gastroenterology, Wilhelmina Children's Hospital, University Medical Centre Utrecht, Utrecht, The Netherlands.
authors: Roderick H
authors: RH
authors: Houwen
mesh_terms: D000293:Adolescent; D016738:Alagille Syndrome / Q000601:surgery*; D000714:Anastomosis, Surgical / Q000379:methods; D002648:Child; D002675:Child, Preschool; D002779:Cholestasis / Q000150:complications / Q000601:surgery; D002780:Cholestasis, Intrahepatic / Q000201:enzymology / Q000601:surgery*; D005260:Female; D006801:Humans; D007223:Infant; D007583:Jejunum / Q000601:surgery*; D008099:Liver / Q000601:surgery*; D008297:Male; D011537:Pruritus / Q000209:etiology / Q000601:surgery; D054047:Surgical Stomas; D005723:gamma-Glutamyltransferase / Q000172:deficiency
chemical_list: D005723:gamma-Glutamyltransferase
keywords: Alagille syndrome; Biliary diversion; Fat-soluble vitamins; Malabsorption; Progressive familial intrahepatic cholestasis