title: Acute Liver Failure in an Adult, a Rare Complication of Alagille Syndrome: Case Report and Brief Review.
abstract: Alagille syndrome (AS) is an autosomal-dominant, multisystem disorder affecting the liver, heart, eyes, skeleton, and face. The manifestations are predominantly pediatric. Diagnosis is based on findings of a paucity of bile ducts on liver biopsy combined with ≥3 of 5 major clinical criteria. Orthotopic liver transplantation (OLT) is the only option for treating patients who developed liver failure, portal hypertension, severe itching, and xanthomatosis. It is difficult to establish clear criteria for OLT; indications are controversial because of the wide variety of clinical symptoms and the multisystem involvement. Generally, AS-associated liver disease is never an acute illness. We report the case of a 28-year-old woman with AS who underwent urgent OLT for acute liver failure. At 24 months posttransplant, the patient is in good clinical condition and with normal hepatic and renal function.
authors.affiliation: Department of Surgery, Transplantation Service, Catholic University of the Sacred Heart, Rome, Italy.
authors.forename: F
authors.initials: F
authors.lastname: Frongillo
authors.affiliation: Department of Surgery, Transplantation Service, Catholic University of the Sacred Heart, Rome, Italy. Electronic address: bianco.g@live.com.
authors.forename: G
authors.initials: G
authors.lastname: Bianco
authors.affiliation: Department of Surgery, Transplantation Service, Catholic University of the Sacred Heart, Rome, Italy.
authors.forename: N
authors.initials: N
authors.lastname: Silvestrini
authors.affiliation: Department of Surgery, Transplantation Service, Catholic University of the Sacred Heart, Rome, Italy.
authors.forename: M C
authors.initials: MC
authors.lastname: Lirosi
authors.affiliation: Department of Surgery, Transplantation Service, Catholic University of the Sacred Heart, Rome, Italy.
authors.forename: A M
authors.initials: AM
authors.lastname: Sanchez
authors.affiliation: Department of Surgery, Transplantation Service, Catholic University of the Sacred Heart, Rome, Italy.
authors.forename: E
authors.initials: E
authors.lastname: Nure
authors.affiliation: Department of Anaestesiology and Intensive Care, Catholic University of the Sacred Heart, Rome, Italy.
authors.forename: R
authors.initials: R
authors.lastname: Gaspari
authors.affiliation: Department of Surgery, Transplantation Service, Catholic University of the Sacred Heart, Rome, Italy.
authors.forename: A W
authors.initials: AW
authors.lastname: Avolio
authors.affiliation: Department of Surgery, Transplantation Service, Catholic University of the Sacred Heart, Rome, Italy.
authors.forename: G
authors.initials: G
authors.lastname: Sganga
authors.affiliation: Department of Surgery, Transplantation Service, Catholic University of the Sacred Heart, Rome, Italy.
authors.forename: S
authors.initials: S
authors.lastname: Agnes
mesh_terms: D000328:Adult; D016738:Alagille Syndrome / Q000150:complications*; D005260:Female; D006801:Humans; D017114:Liver Failure, Acute / Q000209:etiology* / Q000601:surgery*; D016031:Liver Transplantation / Q000379:methods*; D016896:Treatment Outcome