Clinical features of primary biliary cirrhosis: a clinical analysis of 70 cases
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摘要: 目的探讨原发性胆汁性肝硬化(PBC)患者的临床特点。方法回顾性分析2008年1月至2013年3月诊断为PBC的住院患者70例,记录患者的首诊症状、肝功能情况、AMA亚型抗体(M2、M4、M9)以及3例病理结果,并对ALT、AST、ALP、GGT增高的程度进行秩和检验,采用Wilcoxon法。结果 84.3%为女性患者,平均年龄(59.2±8.7)岁。主要的临床表现为乏力25.7%,皮肤瘙痒24.3%,右胁不适18.6%,腹胀18.6%。生化学异常以GGT[(324.5±250.4)U/L]、ALP[(381.1±259.0)U/L]增高最明显。GGT增高5倍以上的患者较多,与ALT、AST、ALP相比差异有统计学意义(u值分别为-5.861、-4.036、-4.445,P均为0.000),AST、ALP增高2倍以上的患者亦较ALT多(u值分别为-4.405、-3.625,P均为0.000)。87.1%的患者AM2A阳性;AM4A阳性占31.4%,AM9A阳性占11.4%。结论 PBC是女性多见的以GGT、ALP增高为明显的慢性肝功能损害,AMA抗体亚型(M2、M4、M9)的阳性是诊断该...Abstract: Objective To investigate the clinical features of patients with primary biliary cirrhosis ( PBC) . Methods A retrospective analysis was performed on the clinical data of 70 inpatients diagnosed with PBC from January 2008 to March 2013. The initial symptoms, liver function, and antimitochondrial antibody ( AMA) subtypes ( M2, M4, and M9) of patients, as well as the pathological results of 3 cases, were recorded. The increases in alanine aminotransferase ( ALT) , aspartate aminotransferase ( AST) , alanine phosphatase ( ALP) , and gamma-glutamyl transpeptidase ( GGT) were analyzed by Wilcoxon rank sum test. Results Of all patients, 84. 3% were females; the mean age was 59. 2 ± 8. 7 years. The main clinical manifestations included weakness ( 25. 7%) , itching ( 24. 3%) , discomfort in the upper right abdominal quadrant ( 18. 6%) , and abdominal distension ( 18. 6%) . Abnormal liver function was mainly characterized by significant increases in GGT ( 324. 5 ± 250. 4 U / L) and ALP ( 381. 1 ± 259. 0 U / L) . The number of patients with more than 5-fold increase in GGT was significantly higher than the numbers of patients with more than 5-fold increases in ALT, AST, and ALP ( u =-5. 861, P = 0. 000;u =-4. 036, P = 0. 000; u =-4. 445, P = 0. 000) . The numbers of patients with more than 2-fold increases in AST and ALP were significantly higher than the number of patients with more than 2-fold increase in ALT ( u =-4. 405, P = 0. 000; u =-3. 625, P = 0. 000) .Among all patients, 87. 1% were positive for antimitochondrial M2 antibody ( AM2A) , 31. 4% were positive for AM4A, and 11. 4% were positive for AM9A. Conclusion PBC, more common in females, is the chronic liver damage characterized by significant increases in GGT and ALP. Detection of AMA subtypes ( M2, M4and M9) is valuable for confirming the diagnosis of PBC.
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Key words:
- liver cirrhosis, biliary /
- autoimmune diseases
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[1]HEATHCOTE EJ.Management of primary biliary cirrhosis.The American association for the study of lire diseases practice guidelines[J].Hepatology, 2000, 31 (4) :1005-1013. [2]MA AL, DU SY, XU Q, et al.Clinicopathological features of primary biliary cirrhosis with or without jaundice:a comparative study[J].Beijing Med J, 2007, 29 (9) :562-563. (in Chinese) 马安林, 杜时雨, 徐潜, 等.原发性胆汁性肝硬化有无黄疸的临床病理比较[J].北京医学, 2007, 29 (9) :562-563. [3]SHI TY, ZHANG FC.Role of autoimmunity in primary biliary cirrhosis[J].World J Gastroenterol, 2012, 18 (48) :7141-7148. [4]FAN X, JIA JD.Diagnosis and treatment of primary biliary cirrhosis in Pacific Asia region[J].J Clin Hepatol, 2010, 26 (4) :365-367. (in Chinese) 范雪, 贾继东.亚洲地区原发性胆汁性肝硬化的诊疗现状[J].临床肝胆病杂志, 2010, 26 (4) :365-367. [5]MAKYO M.Natural history of primary biliary cirrhosis[J].Clin Liver Dis, 2008, 12 (2) :277-288. [6]MIVAKAWA H, TANAKA A, KIKUCHI K, et al.Detection of antimitochondrial autoantibodies in immunofluorescent AMA-negative patients with primary biliary cirrhosis using recombinant autoantigens[J].Hepatology, 2001, 34 (2) :243-248. [7]HUANG GJ, ZHU WD, LU ZP.Investigation of diagnosis and treatment of early primary biliary cirrhosis[J].J Clin Hepatol, 2012, 15 (4) :356-357. (in Chinese) 黄国进, 祝卫东, 陆志平.早期原发性胆汁性肝硬化诊治探讨[J].实用肝脏病杂志, 2012, 15 (4) :356-357. [8]YAO DK.Clinical features of primary biliary cirrhosis:An analysis of 462 cases[J].J Clin Hepatol, 2013, 16 (2) :105-107. (in Chinese) 姚定康.462例原发性胆汁性肝硬化临床特点分析[J].实用肝脏病杂志, 2013, 16 (2) :105-107. [9]LIU HH, FU JL, FU BY, et al.The pathogenesis and risk factors related to prognosis of primary biliary cirrhosis[J].J Clin Hepatol, 2012, 28 (2) :153-155. (in Chinese) 刘红虹, 福军亮, 付宝云, 等.原发性胆汁性肝硬化的发病机制及影响预后的危险因素研究进展[J].临床肝胆病杂志, 2012, 28 (2) :153-155. [10]ZHANG LN, SHI TY, SHI XH, et al.Early biochemical response to ursodeoxycholic acid and long-term prognosis of primarybiliary cirrhosis:Results of a 14-year cohort study[J].Hepatology, 2013, 58 (1) :264-272. [11]SHI TY, ZHANG LN, CHEN H, et al.Risk factors for hepatic decompensation in patients with primary biliary cirrhosis[J].World J Gastroenterol, 2013, 19 (7) :1111-1118. [12]CHAN CW, CARPENTER JR, RIGAMONTI C, et al.Survival following the development of ascites and/or peripheral oedema in primary biliary cirrhosis:a staged prognostic model[J].Scand J Gastroenterol, 2005, 40 (9) :1081-1089. [13]GENDA T, ICHIDA T, SAKISAKA S, et al.Waiting list mortality of patients with primary biliary cirrhosis in the Japanese transplant allocation system[J].J Gastroenterol, 2013, Mar 12.[Epub ahead of print]
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