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Primarna biliarna ciroza in primarni sklerozantni holangitis = Primary biliary cirrhosis and primary sclerosing cholangitis
Pernat Drobež, Cvetka ; Skalicky, Marjan
Primarna biliarna ciroza je kronična avtoimuna holestatska jetrna bolezen. Glavni znaki bolezni so utrujenost, srbečica in znaki drugih avtoimunih bolezni. Diagnoza temelji na treh kriterijih: ...povišane vrednosti holestatskih encimov, prisotnost antimitohondrijskih protiteles v serumu in histološke spremembe. Če bolezni ne zdravimo, napreduje v jetrno cirozo. Zdravimo jo z ursodeoxiholno kislino. Pri bolnikih z izraženo cirozo in jetrno odpovedjo je končna oblika zdravljenja presaditev jeter. Primarni sklerozantni holangitis je kronična, imunsko pogojena holestatska bolezen jeter, pogosto pridružena kronični vnetni črevesni bolezni. Mnogo bolnikov je ob postavitvi diagnoze brez težav ali opisujejo le blago izraženo utrujenost in srbečico. Večinoma bolezen napreduje v cirozo z jetrno odpovedjo. Pri 8 do 30% bolnikov se razvije holangiokarcinom. Diagnoza temelji na tipičnem holangiogramu in histoloških spremembah. V zdravljenju so učinkoviti visoki odmerki ursodeoksiholne kisline. Pri bolnikih z izraženo cirozo in jetrno odpovedjo je končna oblika zdravljenja presaditev jeter.
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