Acute Liver Failure in an Adult, a Rare Complication of Alagille Syndrome: Case Report and Brief Review

Rita Gaspari, Alfonso Wolfango Avolio, Gabriele Sganga, Salvatore Agnes, Francesco Frongillo, Giuseppe Bianco, Nicola Silvestrini, Maria Carmen Lirosi, Alejandro Martin Sanchez, Erida Nure

Risultato della ricerca: Contributo in rivistaArticolo in rivista

3 Citazioni (Scopus)

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.
Lingua originaleEnglish
pagine (da-a)2179-2181
Numero di pagine3
RivistaTransplantation Proceedings
Volume47
DOI
Stato di pubblicazionePubblicato - 2015

Keywords

  • alagille syndrom
  • liver transplantation

Fingerprint

Entra nei temi di ricerca di 'Acute Liver Failure in an Adult, a Rare Complication of Alagille Syndrome: Case Report and Brief Review'. Insieme formano una fingerprint unica.

Cita questo