Osler-Weber-Rendu and liver transplant
KMJ-Kuwait Medical Journal. 2006; 38 (3): 186-190
em Inglês
| IMEMR
| ID: emr-78837
ABSTRACT
Osler-Weber-Rendu [OWR] or Hereditary Hemorrhagic Telangiectasia [HHT] is a rare autosomal dominant disease characterized by angiodysplastic lesions that may affect many organs. Hepatic involvement in HHT is observed in 8-31% of cases and can lead to arteriovenous shunts within the liver causing high output cardiac failure. Liver transplantation may provide cure for patient with severe hepatic involvement, cholangitis and / or perturbed hemodynamics causing high output cardiac failure. The aim of this article is to review reported cases in the literature, the indication for liver transplantation and the outcomes. A MEDLINE search was performed [1970-present] for all case reports and case series of HHT requiring liver transplantation. Atotal of 22 cases were reported in the literature. 95.4% were females. Indications for transplantation included decompensated liver disease, congestive heart failure and biliary sepsis. Nine patients [41%] required transplantation for more than one indication. The overall survival was 90.9% ranging from one month to 7.5 years of follow up period. Liver transplant is a viable option for patients suffering from HHT with complications related to liver involvement
Buscar no Google
Índice:
IMEMR (Mediterrâneo Oriental)
Assunto principal:
Telangiectasia Hemorrágica Hereditária
/
Transplante de Fígado
Limite:
Feminino
/
Humanos
/
Masculino
Idioma:
Inglês
Revista:
Kuwait Med. J.
Ano de publicação:
2006
Similares
MEDLINE
...
LILACS
LIS