title: [Partial external biliary diversion: a therapeutic alternative for Alagille syndrome. First case in Argentina].
abstract: BACKGROUND
  Alagille 's syndrome is the main syndromic chronic intrahepatic cholestasis characterized by hypoplasia of the intrahepatic bile ducts. It is a multisystem disorder of autosomal dominant inheritance with involvement of multiple organs. Usually it becomes apparent in the neonatal period, presenting as subclinical cases or severe degrees of the disease with the consequent development of liver cirrhosis and subsequent liver failure associated with multiple abnormalities: defects in the vertebral arches, typical facies, pulmonary stenosis, mental retardation and hypogonadism.
  
  
  OBJECTIVE
  To present the first case of partial external biliary diversion in Argentina, showing the surgical technique and the improvement in the quality of life, as an alternative to be considered in patients with Alagille's syndrome before the development of cirrhosis.
  
  
  RESULTS
  It has been shown that partial external biliary diversion can stop the process of liver fibrogenesis, halting the progression of the disease and avoiding the need for transplantation in some types of intrahepatic cholestasis when cirrhosis has not been established.
  
  
  CONCLUSIONS
  This surgical technique can improve the quality of life and morbidity associated with hypercholesterolemia in patients with Alagille's syndrome, delaying and maybe avoiding the need for liver transplantation.
authors.affiliation: Instituto de Trasplante Multiorgánico, Hospital Universitario, Fundación Favaloro, Ciudad Autónoma de Buenos Aires, Argentina. crumbo@ffavaloro.org
authors.forename: Carolina
authors.initials: C
authors.lastname: Rumbo
authors.forename: Marcelo
authors.initials: M
authors.lastname: Sandi
authors.forename: Juan M
authors.initials: JM
authors.lastname: Padín
authors.forename: Pablo
authors.initials: P
authors.lastname: Farinelli
authors.forename: Diego
authors.initials: D
authors.lastname: Ramisch
authors.forename: Pablo
authors.initials: P
authors.lastname: Barros-Schelotto
authors.forename: Ana
authors.initials: A
authors.lastname: Cabanne
authors.forename: Dolores
authors.initials: D
authors.lastname: García
authors.forename: Julio
authors.initials: J
authors.lastname: Trentadue
authors.forename: Gabriel E
authors.initials: GE
authors.lastname: Gondolesi
mesh_terms: D016738:Alagille Syndrome / Q000150:complications / Q000601:surgery*; D001118:Argentina; D001662:Biliary Tract Surgical Procedures / Q000379:methods*; D002675:Child, Preschool; D002780:Cholestasis, Intrahepatic / Q000209:etiology / Q000601:surgery*; D005260:Female; D006801:Humans; D014973:Xanthomatosis / Q000601:surgery*