Advances in management of polycystic liver disease.
Expert Rev Gastroenterol Hepatol
; 2(4): 563-76, 2008 Aug.
Article
em En
| MEDLINE
| ID: mdl-19072404
ABSTRACT
The focus of this review is polycystic liver disease, a genetic disorder characterized by multiple macroscopic liver cysts that initially bud from biliary epithelium but subsequently lack communication with the biliary tree. There are two main clinical presentations polycystic liver associated with autosomal dominant polycystic kidney disease and isolated polycystic liver disease. Both of these forms of polycystic liver disease exhibit an autosomal dominant pattern of inheritance. Clinical manifestations of polycystic liver disease are related to either mass effect of the volume of hepatic cysts or to complications arising within the cysts. Polycystic liver disease rarely progresses to hepatic failure or clinical complications of portal hypertension. Management is directed at counseling patients and families, treating complications and reducing cyst load by surgical techniques cyst fenestration, hepatic resection or, rarely, hepatic transplantation. Recent research suggests that blockade of cyst secretion or inhibition of epithelial cells might be useful in halting progression of disease--these observations are discussed in this review.
Texto completo:
1
Coleções:
01-internacional
Base de dados:
MEDLINE
Assunto principal:
Cistos
/
Hepatopatias
Limite:
Humans
Idioma:
En
Revista:
Expert Rev Gastroenterol Hepatol
Assunto da revista:
GASTROENTEROLOGIA
Ano de publicação:
2008
Tipo de documento:
Article
País de afiliação:
Estados Unidos