Catalogo Articoli (Spogli Riviste)

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Titolo:
Chronic cholestatic diseases
Autore:
Poupon, R; Chazouilleres, O; Poupon, RE;
Indirizzi:
Hop St Antoine, Serv Hepatogastroenterol, F-75571 Paris 12, France Hop St Antoine Paris France 12 togastroenterol, F-75571 Paris 12, France Fac Med Necker, INSERM, U370, Paris, France Fac Med Necker Paris FranceFac Med Necker, INSERM, U370, Paris, France
Titolo Testata:
JOURNAL OF HEPATOLOGY
, volume: 32, anno: 2000, supplemento:, 1
pagine: 129 - 140
SICI:
0168-8278(2000)32:<129:CCD>2.0.ZU;2-Q
Fonte:
ISI
Lingua:
ENG
Soggetto:
PRIMARY BILIARY-CIRRHOSIS; PRIMARY SCLEROSING CHOLANGITIS; FAMILIAL INTRAHEPATIC CHOLESTASIS; URSODEOXYCHOLIC ACID THERAPY; PYRUVATE-DEHYDROGENASE COMPLEX-E2; ANTIMITOCHONDRIAL ANTIBODY STATUS; GIANT-CELL HEPATITIS; BILE-ACIDS; AUTOIMMUNE HEPATITIS; LIVER-DISEASE;
Keywords:
bile acids; cholestatis; overlap syndrome; primary biliary cirrhosis; primary sclerosing cholangitis; progressive familial intrahepatic cholestasis; ursodeoxycholic acid;
Tipo documento:
Review
Natura:
Periodico
Settore Disciplinare:
Clinical Medicine
Life Sciences
Citazioni:
120
Recensione:
Indirizzi per estratti:
Indirizzo: Poupon, R Hop St Antoine, Serv Hepatogastroenterol, 184 Rue Fauborg St Antoine, F-75571 Paris 12, France Hop St Antoine 184 Rue Fauborg St Antoine Paris France 12 rance
Citazione:
R. Poupon et al., "Chronic cholestatic diseases", J HEPATOL, 32, 2000, pp. 129-140

Abstract

Chronic cholestatic diseases, whether occurring in infancy, childhood or adulthood, are characterized by defective bile acid transport from the liverto the intestine, which is caused by primary damage to the biliary epithelium in most cases. In this article, approaches to diagnosis and management of the main specific disorders are provided and some of the recent developments in this field are discussed. Major advances in the understanding of the cellular and molecular physiology of bile secretion have led to identification of genetic defects responsible for the different types of progressivefamilial intrahepatic cholestasis (PFIC). The potential role of the genes involved in PFIC in some adult cholestatic disorders remains to be determined. The majority of adult patients with chronic cholestasis have primary biliary cirrhosis (PBC) or primary sclerosing cholangitis (PSC). Recently, variant forms of PBC have been described. The term autoimmune cholangitis is used to describe patients having chronic non-suppurative cholangitis with negative antimitochondrial antibodies (AMA) but positive antinuclear and/or antismooth muscle antibodies. Autoimmune cholangitis and AMA-positive PBC are quite similar in terms of clinical presentation, survival and response to ursodeoxycholic acid (UDCA) therapy. In contrast, autoimmune cholangitis must be distinguished from PBC-autoimmune hepatitis (AIH) overlap syndrome in which biochemical and histological characteristics of both PBC and AIH coexist. Combination of UDCA and corticosteroids is required in most patients with overlap syndrome to obtain a complete clinical and biochemical response. Long-term UDCA treatment improves survival without liver transplantation in PBC patients. Among the putative mechanisms of the beneficial effectsof UDCA, description of anti-apoptotic properties and effect on endotoxin disposal in biliary cells have provided new insights. In patients with incomplete response to UDCA, combination of UDCA with antiinflammatory or immunosuppressive drugs is under evaluation. Variant forms of PSC have also beendescribed, including PSC-AIH overlap syndrome, especially in children or young adults, and small-duct PSC, which is characterized by normal cholangiogram in patients having chronic cholestasis, histologic features compatiblewith PSC and inflammatory bowel disease. Development of cholangiocarcinoma(CC) is a major feature of PSC, occurring in 10-15% of patients. Early diagnosis of CC is a difficult challenge, although positron emission tomography seems a promising tool. Unlike PBC, effective medical therapy is not yet available in PSC, reflecting the lack of knowledge about the exact pathogenesis of the disease. Currently, liver transplantation is the only effectivetherapy for patients with advanced disease, although recurrence of PSC in the graft may occur.

ASDD Area Sistemi Dipartimentali e Documentali, Università di Bologna, Catalogo delle riviste ed altri periodici
Documento generato il 30/11/20 alle ore 06:58:14