Jorge A, Findor J, Esley C, Bruch E
Department of III Internal Medicine, University of Cuyo, Buenos Aires, Argentina.
Z Gastroenterol. 1988 Jun;26(6):322-30.
Thirty patients, 18 males and 12 females, with PSC have been studied between 1972 and 1986. In nine PSC was associated with ulcerative colitis (30%), in 4 with Crohn's disease (13%) and 17 were free of any chronic bowel disease. Four of these 17 were siblings. Alkaline Phosphatase, Gamma GT, total bile acids and 7-alpha-hydroxilated bile acids were elevated in all patients. In 14 patients plasma and urinary copper as well as ceruloplasmin were studied, showing the copper in all cases and ceruloplasmin in 10 cases elevated, 4 were normal. By means of cholangiography alterations of the extrahepatic ducts, the intrahepatic ducts, and both the intra- and extrahepatic ducts were seen in 13%, 27% and 40% of the cases, respectively. Alterations of the pancreatic duct were observed by pancreatography in 21% of cases. 40% of the PSC-patients had also a Sjögren's syndrome, while 3% were associated with diabetes mellitus Type I and II, psoriasis and red lichen planus, iridocyclitis and hepatic hemangioma.
1972年至1986年间，对30例原发性硬化性胆管炎患者进行了研究，其中男性18例，女性12例。9例原发性硬化性胆管炎与溃疡性结肠炎相关（30%），4例与克罗恩病相关（13%），17例无任何慢性肠道疾病。这17例中有4例是兄弟姐妹。所有患者的碱性磷酸酶、γ-谷氨酰转肽酶、总胆汁酸和7-α-羟基胆汁酸均升高。对14例患者的血浆、尿液铜以及铜蓝蛋白进行了研究，结果显示所有患者的铜均升高，10例患者的铜蓝蛋白升高，4例正常。通过胆管造影，分别在13%、27%和40%的病例中发现了肝外胆管、肝内胆管以及肝内外胆管的改变。通过胰管造影，在21%的病例中观察到胰管改变。40%的原发性硬化性胆管炎患者还患有干燥综合征，而3%的患者与I型和II型糖尿病、银屑病和扁平苔藓、虹膜睫状体炎以及肝血管瘤相关。
