Rasmussen H H, Fallingborg J F, Mortensen P B, Freund L G, Tage-Jensen U, Rasmussen S N
Aalborg Sygehus, medicinsk gastroenterologisk afdeling.
Ugeskr Laeger. 1991 May 20;153(21):1487-91.
Primary sclerosing cholangitis (PSC) is an uncommon disorder of unknown etiology, characterized by chronic inflammation and fibrosis of the intra- and extrahepatic bile ducts. PSC is commonly associated with chronic inflammatory bowel disease, especially ulcerative colitis, and often in younger men with an extensive colitis. The diagnosis is made by endoscopic retrograde cholangiography. The characteristic findings are multiple strictures and dilatations of the intra- and extrahepatic bile ducts. Patients with PSC usually have a cholestatic biochemical profile. The liver biopsy findings are often non-specific. Different survivals in these patients have been described. However, asymptomatic patients seems to have a more favorable prognosis. The only curative treatment is liver transplantation.
原发性硬化性胆管炎(PSC)是一种病因不明的罕见疾病,其特征为肝内和肝外胆管的慢性炎症和纤维化。PSC通常与慢性炎症性肠病相关,尤其是溃疡性结肠炎,且多见于患有广泛性结肠炎的年轻男性。诊断通过内镜逆行胆管造影进行。特征性表现为肝内和肝外胆管的多发狭窄和扩张。PSC患者通常具有胆汁淤积的生化特征。肝活检结果往往不具有特异性。已描述了这些患者的不同生存期。然而,无症状患者似乎预后更佳。唯一的治愈性治疗方法是肝移植。