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45例原发性胆汁性肝硬化的临床特征
作者姓名:Zhang F  Jia J  Wang B  Qian L  Yin S  Wang Y  Cui Y  You H  Ma H  Wang H  Zhang C
作者单位:1. 首都医科大学附属北京友谊医院肝病中心,100050
2. 首都医科大学附属北京友谊医院消化内科,100050
3. 首都医科大学附属北京友谊医院病理科,100050
摘    要:目的 分析原发性胆汁性肝硬化患者的临床特征 ,以指导对该病的诊断。方法 对 4 5例原发性胆汁性肝硬化患者的一般资料、病程、临床表现、生物化学、免疫学及病理学等改变进行分析。结果 本组患者中女性 4 2例 ,确诊时的平均年龄为 (5 0 8± 8 1)岁 ,初诊至确诊的时间平均为 2年 (2个月~ 12年 )。临床症状以乏力最为多见 (6 6 7% ,30 / 4 5 ) ,其次为黄疸 (5 5 6 % ,2 5 / 4 5 )和皮肤瘙痒 (40 % ,18/ 4 5 ) ,9例患者 (2 0 % )无症状 ,9例患者 (2 0 % )合并其他自身免疫性疾病 干燥综合征和(或 )类风湿关节炎 ]。所有患者血清碱性磷酸酶及γ 谷氨酰转肽酶水平明显升高 分别为 (498 5±36 9 8)IU/L和 (5 95 2± 5 18 4 )IU/L],而血清ALT、AST水平仅轻中度升高 分别为 (83 9± 5 8 8)IU/L和(10 0 8± 4 8 8)IU/L],5 5 6 % (2 5 / 4 5 )的患者血清总胆红素水平≥ 34 2 μmol/L ,88 9% (40 / 4 5 )患者血清IgM升高 ,95 6 %患者 (43/ 4 5 )线粒体抗体和 (或 )线粒体抗体M2亚型阳性。结论 在中国 ,原发性胆汁性肝硬化可能并非少见 ,主要累及中年女性 ,最常见的症状为乏力 ,部分早期患者可无症状 ,血清碱性磷酸酶及γ 谷氨酰转肽酶水平升高及抗线粒体抗体和 (或 )线粒体抗体M2亚型阳性有助于诊断

关 键 词:原发性胆汁性肝硬化  抗线粒体抗体  临床特征
修稿时间:2001年8月21日

Clinical characteristics of primary biliary cirrhosis: a report of 45 cases
Zhang F,Jia J,Wang B,Qian L,Yin S,Wang Y,Cui Y,You H,Ma H,Wang H,Zhang C.Clinical characteristics of primary biliary cirrhosis: a report of 45 cases[J].Chinese Journal of Internal Medicine,2002,41(3):163-167.
Authors:Zhang Fukui  Jia Jidong  Wang Baoen  Qian Linxue  Yin Shanshan  Wang Yu  Cui Yan  You Hong  Ma Hong  Wang Huiji  Zhang Changhuai
Institution:Liver Research Center, Beijing Friendship Hospital, Capital University of Medical Sciences, Beijing 100050, China.
Abstract:OBJECTIVE: To study the clinical features of primary biliary cirrhosis (PBC) in order to facilitate cognition of the disease. METHODS: General status, clinical manifestations and laboratory findings of 45 patients with PBC were reviewed. RESULTS: Among the 45 patients, 42 were females and the mean age at diagnosis was (50.8 +/- 8.1) years. The mean time interval between the first visit to physicians to the time of correct diagnosis was about 2 years. The most frequent symptoms were fatigue (66.7%, 30/45), then jaundice (55.6%, 25/45) and pruritus (40%, 18/45). Nine patients (20%) were asymptomatic. Nine patients (20%) were associated with other auto-immune diseases (Sjogren's syndrome and/or rheumatoid arthritis). Serum alkaline phosphatase (ALP) and glutamyl transpeptidase (gamma-GT) levels were markedly elevated in all the patients (498.5 +/- 369.8) IU/L and (595.2 +/- 518.4) IU/L, respectively], whereas ALT and AST levels were mildly to moderately elevated (83.9 +/- 58.8) IU/L and (100.8 +/- 48.8) IU/L, respectively]. 25 patients (55.6%) had a total bilirubin level >/= 34.2 micromol/L. 40 patients (88.9%) had elevated serum IgM and 43 patients (95.6%) were anti-mitochondrial antibody (AMA)/AMA-M2 positive. CONCLUSION: In China, PBC is probably not so rare as it was thought before. It is mostly found in middle-aged women. The most frequent symptom is fatigue and some patients are asymptomatic at early stage. Elevated serum ALP and gamma-GT levels together with positive AMA/AMA-M2 can help to diagnose PBC. Liver biopsy is useful to confirm the diagnosis and differentiate histopathological stages.
Keywords:Liver cirrhosis  biliary  Anti  mitochondrial antibody
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