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.

Frongillo, F., Bianco, G., Silvestrini, N., Lirosi, M. C., Sanchez, A. M., Nure, E., Gaspari, R., Avolio, A. W., Sganga, G., Agnes, S., Acute Liver Failure in an Adult, a Rare Complication of Alagille Syndrome: Case Report and Brief Review, <<TRANSPLANTATION PROCEEDINGS>>, 2015; 47 (7): 2179-2181. [doi:10.1016/j.transproceed.2014.11.072] [http://hdl.handle.net/10807/70265]

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

Frongillo, Francesco;Bianco, Giuseppe;Silvestrini, Nicola;Lirosi, Maria Carmen;Sanchez, Alejandro Martin;Nure, Erida;Gaspari, Rita;Avolio, Alfonso Wolfango;Sganga, Gabriele;Agnes, Salvatore
2015

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.
Inglese
Frongillo, F., Bianco, G., Silvestrini, N., Lirosi, M. C., Sanchez, A. M., Nure, E., Gaspari, R., Avolio, A. W., Sganga, G., Agnes, S., Acute Liver Failure in an Adult, a Rare Complication of Alagille Syndrome: Case Report and Brief Review, <<TRANSPLANTATION PROCEEDINGS>>, 2015; 47 (7): 2179-2181. [doi:10.1016/j.transproceed.2014.11.072] [http://hdl.handle.net/10807/70265]
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Utilizza questo identificativo per citare o creare un link a questo documento: https://hdl.handle.net/10807/70265
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