Author/Editor		Dajčman, Davorin
Title		Primarni sklerozantni holangitis
Translated title		Primary sclerosing cholangitis
Type		članek
Source		Med Razgl
Vol. and No.		Letnik 38, št. 2
Publication year		1999
Volume		str. 239-52
Language		slo
Abstract		The aetiology and pathogenesis of the inflammatory and fibrotic bile duct lesions characteristic of primary sclerosing cholangitis is unknown, but several lines evidence support connection that genetic and immunologic factors are involved. Disease is characterised by ongoing inflammation, destruction and fibrosis of intrahepatic and extrahepatic bile duct. Irreversible damage to the bile ducts leads cholestasis, cirrhosis and liver failure with premature death unless liver transplantation is performed. It is frequently associated with inflammatory bowel disease, usually ulcerative colbis. There is no satisfactory medical therapy for patients with primary sclerosing cholangitis. Lacking a specific remedy the goal of treatment is controlling inflammation and infection and to prevent scaring. Liver transplantation is treatment of choice in advanced liver disease.
Summary		Izvleček. Etiologija in patogeneza vnetnega dogajanja in fibroze žolčnih vodov pri bolnikih s primarnim sklerozantnim holangitisom še ni pojasnjena. Obstajajo številni dokazi o tesni zvezi z genetskimi in imunskimi dejavniki. Za bolezen je značilno vnetje, propad in fibroza znotraj jetrnih in zunaj jetrnih žolčnih vodov. Trajna okvara žolčnih vodov vodi do holestaze, ciroze in odpovedi jeter, ki se brez presaditve jeter konča smrtno. Pogosto sovpada s kroničnim vnetnim črevesnim obolenjem, najpogosteje z ulceroznim kolitisom. Še zmeraj ne poznamo zadovoljivega zdravijenja z zdravili, zato ostaja bistvo zdravljenja v obvladovanju vnetja, preprečevanju okužb in zaviranju brazgotinjenja. Izborno zdravljenje v napredovalih bolezenskih obdobjih je presaditev jeter.
Descriptors		CHOLANGITIS, SCLEROSING