原发性硬化性胆管炎的诊断与治疗

目的探讨原发性硬化性胆管炎的误诊原因和诊疗方法。方法自1998年6月至2004年12月问我院收治原发性硬化性胆管炎21例,回顾性分析21例病人的误诊原因、诊断要点、糖皮质激素的治疗结果及其预后。结果患者都有从初诊医院-传染病医院-综合医院的诊治史。21例患者都应用糖皮质激素治疗,激素的用量、时间因人而异,采用个体化治疗方案。有16例患者获长期缓解,有5例需要长期服用激素治疗,有3例病程进展快发生“胆汁性肝硬化门静脉高压症”,死亡1例。尚无肝移植手术的病例。结论原发性硬化性胆管炎病因不明,本病表现复杂多样、误诊率高、治疗效果差。激素可以长期缓解部分病例,有复发病例;部分病例需长期口服激素治疗。激素可以缓解症状、延缓病程发展进程;部分患者病程进展速度快, 激素仅能使胆红素和转氨酶降低,不能阻断病程的进展,胆汁性肝硬化门静脉高压症是其必然的结果,肝移植术是唯一的有效的治疗方法。

DIAGNOSIS AND TREATMENT OF PRIMARY SCLEROSING CHOLANGITIS

Objective To investigate the reasons of misdialgnosis and treatment strategy of primary sclerosing cholangitis. Methods Between June 1998 and December 2004, 21 patients with primary sclerosing cholangitis received therapy in Yuhuangding hospital. A retrospective study was conducted to review the causes of misdiagnosis, key points of diagnosis, curative effects of cortisone and prognosis. Result All cases have the history from preliminary hospital to contagious hospital, and to general hospital. 21 cases were treated with cortisone individually upon from the hormone dose to time duration. 16 cases achieved long-term relief. 5 cases need take hormone for a long time, 3 of them progressed rapidly to portal vein hypertension of biliary cirrhosis, and one case died. To date, no liver transplantation was performed. Conclusion primary sclerosing cholangitis is characterized with no destined Pathogenesis, complicated clinical findings, high misdialgnosis, and poor response to treatment. Part of the patients can get relief in long term by using hormone, but some of them relapse; Some patients need take hormone perennially. Hormone can release its symptoms, delay the progression; Some patients developed rapidly, hormone can only decrease the level of bilirubin and aminotransferase, cannot block its progression, portal vein hypertension syndrome of biliary cirrosis is the necessarily result, and liver transplantion is the effective and last way.