44例原发性胆汁性胆管炎临床特征分析  

Clinical analysis of 44 patients with primary biliary cholangitis

作　　者：赵凤丛[1] 张永萍[1] ZHAO Fengcong;ZHANG Yongping(Liver Diseases Center,People's Hospital of Xinjiang Uygur Autonomous Region,Urumqi,Xinjiang,830000,China)

机构地区：[1]新疆维吾尔自治区人民医院肝病中心

出　　处：《新疆医学》2019年第2期169-171,182,共4页Xinjiang Medical Journal

摘　　要：目的对44例新疆维吾尔族原发性胆汁性胆管炎(PBC)患者的临床特征进行回顾性分析,探讨维吾尔族中PBC患者的临床特点。方法选取2013年1月-2017年1月新疆维吾尔自治区人民医院收治的具有完整病例资料的维吾尔族PBC患者44例,收集所有患者的一般资料、临床表现、血清生化指标、免疫学指标、并发症、治疗等,并进行统计学分析。结果44例维吾尔族PBC患者中男女比例为6:38,发病平均年龄为(45.57±10.47)岁,确诊平均年龄为(46.68±10.23)岁。最常见的临床症状依为乏力(64%,28/44)、纳差(48%,21/44)、皮肤瘙痒(39%,17/44)。最主要的体征包括黄疸(41%,18/44)、脾大(39%,17/44)和肝掌(23%,10/44)。生化指标以血清碱性磷酸酶(ALP)和γ-谷氨酰转肽酶(γ-GGT)升高为主,GGT(362.25±324.82)U/L、ALP(421.97±275.46)U/L,AST及ALT仅轻到中度升高,分别为(105.18±72.81)U/L和(110.03±90.51)U/L。免疫指标检测结果显示43例患者(98%)抗核抗体(ANA)阳性,43例患者(98%)抗线粒体抗体(AMA)阳性,42例患者(95%)抗线粒体抗体M2亚型(AMA-M2)阳性,36例患者(82%)M2-3E阳性,11例患者(25%)抗核体蛋白抗体(SP100)阳性,15例患者(34%)抗核孔膜蛋白抗体(GP210)阳性。IgM升高患者36例(82%),平均(3.74±1.44)g/L,IgG升高患者20例(45%),平均(16.38±4.62)g/L。12例患者(27%)有合发症或并发症,最主要的疾病为甲状腺疾病。治疗上均采用熊去氧胆酸(UDCA)为主的综合治疗。结论维吾尔族PBC患者主要累计中老年女性,多数于发病后1年左右确诊,主要临床表现为乏力、纳差、皮肤瘙痒,以ALP和GGT升高为主,AMA和AMA-M2阳性对于诊断具有重要意义,多伴发甲状腺疾病,治疗上以UDCA为主。Objective To study the clinical features of Uygur patients with primary biliary cholangitis(PBC). Method 44 Uygur patients with primary biliary cirrhosis in our hospital from January 2013 to January 2017 were collected.The general data, clinical manifestations, serum biochemical indexes, immunological indexes, complications, treatment and so on of all patients were collected and analyzed statistically. Results Thirty-eight patients were female(86%), and the mean age was 46.68 ±10.23 years when PBC was definitely diagnosed. The most frequent symptoms complained were fatigue(64%, 28/44), anorexia(48%, 21/44)and pruritus(39%,17/44). The major signs included jaundice(41%, 18/44), splenomegaly(39%, 17/44)and liver palms(23%, 10/44). Serum alkaline phosphatase(ALP)and γ glutamyl transpeptidase(γ-GGT)levels were signifcantly increased in most patients(421.97±275.46 U/L and 362.25±324.82 U/L, respectively), while alanine aminotransferase(ALT)and aspartate aminotransferase(AST)levels were slightly to mildly elevated(110.03 ±90.51 U/L and 105.18 ±72.81 U/L, respectively). Positive antinuclear antibody(ANA) was found in forty-three Uygur patients(98%). The positive rate of anti-mitochondrial antibody(AMA), AMA-M2, M2-3 E, nuclear envelope protein antibody(GP210)and nuclear multiple point antibody(SP100)were 98%, 95%,82%, 34% and 25% respectively. In these cases, there were 36 cases with elevated level of immunoglobin M(IgM, 3.74±1.44 g/L), and twenty patients(45%)had increased immunoglobin G(IgG, 16.38 ±4.62 g/L). The rate of Uygur patients with other diseases was 27%. The main disease was thyroid disease, including hypothyroidism and nodular goiter. Besides, All the patients were treated by Ursodeoxycholic acid. Conclusion PBC occurs mainly in middle and old aged Uygur women. The main clinical manifestations include fatigue, anorexia and pruritus. The elevated levels of ALP,γ-GGT and positive AMA/AMA-M2 are critical to diagnosis of PBC in Uygur patients.

关 键 词：原发性胆汁性胆管炎 维吾尔族 临床特征 抗线粒体抗体 

分 类 号：R446.9[医药卫生—诊断学]