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作者复习50例原发性硬化性胆管炎(primarysclerosing cholangeitis,PSC)患者的病史。PSC 诊断主要依据 X 线和/或病理学标准。临床特征 82%患者<50岁;70%为男性;就诊时90%患者有症状,依次为黄疸68%,搔痒62%,腹痛50%,发热28%,体重减轻28%。半数病人有体征:黄疽44%,肝大38%,脾大29%,余为蜘蛛痣,腹水和杵状指。PSC 伴发炎症性肠道疾患者54%,以慢性溃疡性结肠炎为主占46%;少见的伴发病还有甲状腺炎、甲状腺功能减退和胰腺炎、慢性活动性肝炎。实验室检查发现碱性磷酸酶全部上升,85%超过正常上限的3倍,谷草转氨酶也常上升,通常不到正常上限的4倍,2/3有高胆红素血症,1/4血清蛋白质异常,血液学检查无特异性。X 线检查47例,其中38例
The authors reviewed the history of 50 patients with primary sclerosing cholangeitis (PSC). PSC diagnosis is mainly based on X-ray and / or pathological criteria. Clinical Features 82% of patients <50 years of age; 70% of men; 90% of patients had symptoms at visit, followed by 68% jaundice, itching 62%, abdominal pain 50%, fever 28% and weight loss 28%. Half of the patients had signs: jaundice 44%, liver 38%, splenomegaly 29%, more than spider nevus, ascites and clubbing. 54% of patients with PSC accompanied by inflammatory bowel disease and 46% of patients with chronic ulcerative colitis. Thyroiditis, hypothyroidism, pancreatitis and chronic active hepatitis are the rare complication. Laboratory tests showed that all of the alkaline phosphatase was elevated, 85% above the upper limit of normal, aspartate aminotransferase, and often up to 4 times the upper limit of normal, 2/3 of hyperbilirubinemia, and 1 of 4 serum Protein abnormalities, no specific hematological examination. X-ray examination in 47 cases, of which 38 cases