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Titolo:
HEPATOBILIARY MANIFESTATIONS OF CYSTIC-FIBROSIS
Autore:
COLOMBO C; BATTEZZATI PM;
Indirizzi:
UNIV SASSARI,DEPT PEDIAT,VIALE SAN PIETRO 12 I-07100 SASSARI ITALY
Titolo Testata:
European journal of gastroenterology & hepatology
fascicolo: 8, volume: 8, anno: 1996,
pagine: 748 - 754
SICI:
0954-691X(1996)8:8<748:HMOC>2.0.ZU;2-8
Fonte:
ISI
Lingua:
ENG
Soggetto:
URSODEOXYCHOLIC ACID THERAPY; DUCT EPITHELIAL-CELLS; CHRONIC LIVER-DISEASE; BILIARY PROBLEMS; CIRRHOSIS; TRANSPLANTATION; CHOLESTASIS; GALLSTONES; STENOSIS; CHILDREN;
Keywords:
CYSTIC FIBROSIS; LIVER DISEASE; HEPATOBILIARY MANIFESTATIONS; CFTR; URSODEOXYCHOLIC ACID;
Tipo documento:
Review
Natura:
Periodico
Settore Disciplinare:
Science Citation Index Expanded
Citazioni:
48
Recensione:
Indirizzi per estratti:
Citazione:
C. Colombo e P.M. Battezzati, "HEPATOBILIARY MANIFESTATIONS OF CYSTIC-FIBROSIS", European journal of gastroenterology & hepatology, 8(8), 1996, pp. 748-754

Abstract

Cystic fibrosis is the most common, potentially lethal genetic defectin the Caucasian population. During recent years it has been increasingly associated with a number of hepatic and biliary abnormalities, ofwhich chronic cholestatic liver disease is by far the most relevant. Plugging of intrahepatic bile ducts with inspissated secretions is thought to play a major role in the pathogenesis. Attempts have been madeto provide uniform criteria to identify patients with early, possiblyreversible, hepatic lesions, as well as to assess severity of liver disease. It has been estimated that bout 13% of cystic fibrosis patients present serum liver enzyme abnormalities, but prevalence of liver involvement is likely to be higher. Due to decreasing mortality from extrahepatic causes in cystic fibrosis and to the widespread use of laboratory tests and ultrasound examination, patients with minor degree of liver involvement will be increasingly represented in future. Oral bile acid therapy is promising, but its long-term benefits in terms of survival and prevention of major complications of liver cirrhosis remainto be established. Liver transplantation is the only potentially curative treatment for patients with advanced stage liver disease and mildpulmonary involvement.

ASDD Area Sistemi Dipartimentali e Documentali, Università di Bologna, Catalogo delle riviste ed altri periodici
Documento generato il 02/12/20 alle ore 13:50:12