141例原發(fā)性膽汁性膽管炎臨床特征分析
發(fā)布時間：2018-07-23 14:10
【摘要】：目的:觀察分析本院診斷為原發(fā)性膽汁性膽管炎(primary biliary cholangitis,PBC)的141例患者的臨床資料(一般資料、臨床表現(xiàn)、血液生化、自身抗體、影像學(xué)檢查等)及其特點,探索抗Ro-52抗體與PBC之間的聯(lián)系,對PBC患者食管/胃底靜脈曲張相關(guān)指標(biāo)做診斷價值評估,以進(jìn)一步提高對該病的認(rèn)識。方法:對我院2011年06月份~2016年12月份收治的具有完整病例資料的141例診斷為原發(fā)性膽汁性膽管炎患者的一般資料、臨床癥狀及體征、臨床并發(fā)癥及合并癥、血清生化指標(biāo)、自身抗體、內(nèi)鏡檢查、影像學(xué)檢查及Fibro Scan檢查資料進(jìn)行回顧性分析。同一病人多次住院僅取第一次住院資料。結(jié)果:1.本研究男性26例(18.4%),女性115例(81.6%),男女比例為:1:4.42,所有病例平均確診年齡(57.8±10.8)歲,病程240(60-700)天,住院天數(shù)11(8-15)天。2.本研究資料PBC患者常見的臨床表現(xiàn):黃疸、肝脾腫大、乏力、食欲減退、上腹部不適、腹水、皮膚瘙癢、眼干口干、下肢水腫、肝掌蜘蛛痣等。3.主要并發(fā)癥有:電解質(zhì)紊亂(37例)、消化道出血(20例)、感染(14例)、肝性腦病(2例);主要的合并癥有:干燥綜合征(Sjogren's syndrome 11例)、糖尿病(6例)、橋本氏甲狀腺炎(5例)、類風(fēng)濕關(guān)節(jié)炎(1例)、系統(tǒng)性紅斑狼瘡(1例)、成人STILL病(1例)、系統(tǒng)性硬化(1例)。4.PBC患者血清生化檢查以ALP及GGT升高為主[261.6(155.5-489.1)U/L,179.1(81.0-430.5)U/L],并且于疾病早期階段升高顯著,到晚期升高不明顯;AST、ALT輕度升高[69.0(43.0-114.0)U/L,54.0(32.0-93.5)U/L],血清總膽紅素為30.5(19.2-64.6)umol/L,為正常上限,總膽汁酸TBA 20.1(8.5-45.8)umol/L,高于正常上限2倍,免疫球蛋白Ig M升高,為3.1(2.0-4.2)G/L。5.自身抗體檢查,AMA陽性有91例(64.5%),AMA-M2陽性120例(85.1%),125例(88.7%)患者ANA陽性,31例(22.0%)抗著絲點B抗體陽性,66例(46.8%)抗Ro-52抗體陽性,22例(15.6%)SSA抗體陽性,15例(10.6%)SSB抗體陽性。6.研究對象中行內(nèi)鏡檢查者總共65例,有35例(53.9%)有食管靜脈曲張,9例(13.9%)有胃底靜脈曲張,8例(12.3%)提示有潰瘍和(或)糜爛,8例(12.3%)有門脈高壓性胃病,29例(44.6%)提示慢性胃炎。8例(12.3%)同時有食管靜脈曲張和胃底靜脈曲張。7.共有118例患者行腹部超聲檢查,超聲提示98例(83.1%)肝臟彌漫性病變,38例(32.2%)腹水,81例(68.6%)存在肝脾腫大,35例(29.7%)提示門脈/脾靜脈增寬或流速減低,5例提示(4.2%)膽汁淤積,4例(3.4%)提示門脈血栓形成;6例(5.1%)超聲檢查正常。有41例患者行上腹部CT增強(qiáng)掃描或平掃,結(jié)果提示有25例(61.0%)肝硬化,有14例(34.2%)提示腹水,有29例(70.7%)存在肝脾大,有7例(17.1%)提示門脈高壓,有19例(46.3%)提示食管靜脈曲張,有16例(39.0%)提示胃底靜脈曲張。有8例(19.5%)同時有食管胃鏡靜脈曲張及胃底靜脈曲張。8.本研究共有26例患者行Fibro Scan檢查,結(jié)果提示9例(34.6%)患者處于肝硬化階段。對141例患者進(jìn)行Mayo評分,評分均值為5.7±1.5。Mayo危險評分與Fibro Scan分期之間存在顯著的正相關(guān)關(guān)系(相關(guān)系數(shù)0.948,P值0.000)。9.男性病程150(30-361)較女性組病程300(60-1000)短,有統(tǒng)計學(xué)差異(P=0.023)。AMA在女性組(68.7%)檢出率高于男性組(46.2%),差異具有統(tǒng)計學(xué)差異。余臨床表現(xiàn)、生化檢查及影像學(xué)檢查兩組間無明顯差異。10.Ro-52陽性組較陰性組更容易出現(xiàn)眼干口干、腹水,有統(tǒng)計學(xué)差異(P分別為0.014和0.012);陽性組合并干燥綜合征的較陰性組多,存在統(tǒng)計學(xué)上存在差異(P=0.002);陽性組血清Ig G較陰性組高(P值0.023),但CHE較陰性組低(P=0.008)。11.對食管靜脈曲張的診斷價值評價指標(biāo)中,當(dāng)Mayo評分等于4.8、血小板計數(shù)為130×109/L、血清白蛋白38.6g/L時所對應(yīng)約登指數(shù)最大,三者對食管靜脈曲張有一定的診斷價值。總膽紅素和凝血酶原時間的靜脈曲張的診斷價值有限。結(jié)論:原發(fā)性膽汁性膽管炎主要累及中年女性,女性患者病程更長,主要表現(xiàn)為黃疸、肝脾大、乏力、食欲減退、上腹不適、腹水、皮膚瘙癢、眼干口干,癥狀缺乏特異性,早期部分患者無明顯癥狀,到疾病后期并發(fā)肝硬化并發(fā)癥,也伴隨自身免疫疾病,如干燥綜合征。非侵入性檢查中最具有診斷價值的是血清AMA/AMA-M2抗體,Ro-52陽性組更容易合并干燥綜合征,患者血清中檢出此抗體則更容易出現(xiàn)腹水,Ro-52陰性的患者可能肝臟合成功能較低。當(dāng)Mayo評分大于4.8、血小板計數(shù)小于130×109/L、血清白蛋白小于38.6g/L的患者需密切定期隨訪胃鏡檢查。目前治療以UCDA為主,需要找到更加有效安全的方法早期診斷早期治療。
[Abstract]:Objective: To observe and analyze the clinical data of 141 patients diagnosed as primary biliary cholangitis (PBC) in our hospital (general data, clinical manifestation, blood biochemistry, autoantibody, imaging examination, etc.) and their characteristics, to explore the relationship between anti Ro-52 antibody and PBC, and to refer to the related indications of esophageal / gastric varices in PBC patients. Evaluation of the diagnostic value to further improve the understanding of the disease. Methods: 141 patients with complete cases of cholangitis diagnosed in our hospital in 2011, ~2016, ~2016 December, were diagnosed as general data, clinical symptoms and signs, clinical complications and complications, serum biochemical indexes, autoantibodies, and endoscopy. Review, imaging examination and Fibro Scan examination data. The same patient was hospitalized for the first time in hospitalization. Results: 1. men were 26 (18.4%) and 115 women (81.6%). The male and female ratio was 1:4.42, the average age of all cases was (57.8 + 10.8) years, the course of disease was 240 (60-700) days, and the hospital days were 11 (8-15) days.2. Ben. The common clinical manifestations of PBC patients: jaundice, hepatomegaly, asthenia, anorexia, upper abdominal discomfort, ascites, skin itching, dry mouth, edema of the lower extremities, and spider nevus of the liver, including electrolyte disorder (37 cases), gastrointestinal bleeding (20 cases), infection (14 cases), hepatic encephalopathy (2 cases); main complication: drying synthesis Syndrome (Sjogren's syndrome 11), diabetes (6 cases), Hashimoto's thyroiditis (5 cases), rheumatoid arthritis (1 cases), systemic lupus erythematosus (1 cases), adult STILL disease (1 cases), systemic sclerosis (1 cases) of.4.PBC patients with ALP and GGT elevation as the main [261.6 (155.5-489.1) U/L, 179.1 (81.0-430.5) U/L], and in the early stage of disease. Gao Xian was not significantly higher at the late stage; AST, ALT slightly elevated [69.0 (43.0-114.0) U/L, 54 (32.0-93.5) U/L], serum total bilirubin 30.5 (19.2-64.6) umol/L, the normal upper limit, the total bile acid TBA 20.1 (8.5-45.8) umol/L, 2 times higher than the normal upper limit, 3.1 (6) autoantibody examination, 91 cases (6) positive. 4.5%) AMA-M2 positive 120 cases (85.1%), 125 (88.7%) patients with ANA positive, 31 (22%) anti filal B antibody positive, 66 (46.8%) anti Ro-52 antibody positive, 22 cases (15.6%) SSA antibody positive, 15 (10.6%) SSB positive.6. research subjects in total 65 cases, there are esophageal varices in patients with esophageal varices and gastric fundus veins 8 cases (12.3%) showed ulcers and (or) erosion, 8 cases (12.3%) had portal hypertensive gastropathy, 29 cases (44.6%) showed chronic gastritis.8 (12.3%) with esophageal varices and fundus varicose.7. in 118 patients with abdominal ultrasound, 98 (83.1%) liver diffuse lesions, 38 (32.2%) ascites, 81 cases (68.6%)). In hepatomegaly, 35 cases (29.7%) showed portal / splenic vein widening or decreased flow rate, 5 cases (4.2%) cholestasis, 4 cases (3.4%) indicating portal vein thrombosis; 6 cases (5.1%) ultrasound examination normal. There were 41 patients with upper abdominal CT scan or plain scan. The results suggested that 25 cases (61%) cirrhosis, 14 cases (34.2%) suggest ascites, and there are 29 cases. In the liver and spleen, 7 cases (17.1%) showed portal hypertension, 19 cases (46.3%) were suggestive of esophageal varices, 16 cases (39%) were suggestive of varicose gastric fundus vein. There were 8 cases (19.5%) with esophageal gastroscope varicose and fundus varicose.8. in 26 patients with Fibro Scan examination. The results suggested that 9 patients (34.6%) were in the stage of cirrhosis. 14 (14) patients were in the period of cirrhosis. 1 patients with Mayo score, the mean value of 5.7 + 1.5.Mayo risk score and Fibro Scan staging have significant positive correlation (correlation coefficient 0.948, P value 0).9. male course 150 (30-361) compared with female group course 300 (60-1000) short, statistical difference (P= 0.023).AMA in female group (68.7%) detection rate is higher than male group (46.2%), difference There were statistical differences. There was no significant difference between the two groups in the clinical and biochemical examination. The.10.Ro-52 positive group was more prone to dry mouth dry mouth, ascites, with statistical difference (P respectively 0.014 and 0.012); positive combination and Sjogren syndrome were more than negative groups, there were statistical differences (P=0.002); positive group; positive group The serum Ig G was higher than that of the negative group (P value 0.023), but CHE was lower (P=0.008).11. for the diagnostic value of esophageal varices, when the Mayo score was equal to 4.8, the platelet count was 130 x 109/L, the serum albumin 38.6g/L was the largest, and the three had a certain diagnostic value for the esophageal varices. Total bilirubin and coagulation were of certain value. The diagnostic value of venous varicose in the time of blood enzyme is limited. Conclusion: primary biliary cholangitis mainly involves middle-aged women, and female patients have longer course of disease. The main manifestations are jaundice, liver and spleen, asthenia, anorexia, abdominal discomfort, ascites, skin itching, dry mouth and dry mouth, symptoms lack specificity, early part of the patients have no obvious symptoms, to disease in early part of the patients to disease. Later complications of liver cirrhosis accompanied by autoimmune diseases such as Sjogren's syndrome. The most diagnostic value of the non invasive examination was the serum AMA/AMA-M2 antibody. The Ro-52 positive group was more likely to merge with Sjogren syndrome. The antibody in the patient's sera was more prone to abdominal water, and the Ro-52 negative patients may have lower liver function. When the Mayo score is greater than 4.8, the platelet count is less than 130 x 109/L, the patients with serum albumin less than 38.6g/L need to follow up the gastroscopy regularly. At present, the treatment is based on UCDA, and it is necessary to find a more effective and safe method for early diagnosis of early treatment.
【學(xué)位授予單位】：蘭州大學(xué)
【學(xué)位級別】：碩士
【學(xué)位授予年份】：2017
【分類號】：R575.2
[Abstract]:Objective: To observe and analyze the clinical data of 141 patients diagnosed as primary biliary cholangitis (PBC) in our hospital (general data, clinical manifestation, blood biochemistry, autoantibody, imaging examination, etc.) and their characteristics, to explore the relationship between anti Ro-52 antibody and PBC, and to refer to the related indications of esophageal / gastric varices in PBC patients. Evaluation of the diagnostic value to further improve the understanding of the disease. Methods: 141 patients with complete cases of cholangitis diagnosed in our hospital in 2011, ~2016, ~2016 December, were diagnosed as general data, clinical symptoms and signs, clinical complications and complications, serum biochemical indexes, autoantibodies, and endoscopy. Review, imaging examination and Fibro Scan examination data. The same patient was hospitalized for the first time in hospitalization. Results: 1. men were 26 (18.4%) and 115 women (81.6%). The male and female ratio was 1:4.42, the average age of all cases was (57.8 + 10.8) years, the course of disease was 240 (60-700) days, and the hospital days were 11 (8-15) days.2. Ben. The common clinical manifestations of PBC patients: jaundice, hepatomegaly, asthenia, anorexia, upper abdominal discomfort, ascites, skin itching, dry mouth, edema of the lower extremities, and spider nevus of the liver, including electrolyte disorder (37 cases), gastrointestinal bleeding (20 cases), infection (14 cases), hepatic encephalopathy (2 cases); main complication: drying synthesis Syndrome (Sjogren's syndrome 11), diabetes (6 cases), Hashimoto's thyroiditis (5 cases), rheumatoid arthritis (1 cases), systemic lupus erythematosus (1 cases), adult STILL disease (1 cases), systemic sclerosis (1 cases) of.4.PBC patients with ALP and GGT elevation as the main [261.6 (155.5-489.1) U/L, 179.1 (81.0-430.5) U/L], and in the early stage of disease. Gao Xian was not significantly higher at the late stage; AST, ALT slightly elevated [69.0 (43.0-114.0) U/L, 54 (32.0-93.5) U/L], serum total bilirubin 30.5 (19.2-64.6) umol/L, the normal upper limit, the total bile acid TBA 20.1 (8.5-45.8) umol/L, 2 times higher than the normal upper limit, 3.1 (6) autoantibody examination, 91 cases (6) positive. 4.5%) AMA-M2 positive 120 cases (85.1%), 125 (88.7%) patients with ANA positive, 31 (22%) anti filal B antibody positive, 66 (46.8%) anti Ro-52 antibody positive, 22 cases (15.6%) SSA antibody positive, 15 (10.6%) SSB positive.6. research subjects in total 65 cases, there are esophageal varices in patients with esophageal varices and gastric fundus veins 8 cases (12.3%) showed ulcers and (or) erosion, 8 cases (12.3%) had portal hypertensive gastropathy, 29 cases (44.6%) showed chronic gastritis.8 (12.3%) with esophageal varices and fundus varicose.7. in 118 patients with abdominal ultrasound, 98 (83.1%) liver diffuse lesions, 38 (32.2%) ascites, 81 cases (68.6%)). In hepatomegaly, 35 cases (29.7%) showed portal / splenic vein widening or decreased flow rate, 5 cases (4.2%) cholestasis, 4 cases (3.4%) indicating portal vein thrombosis; 6 cases (5.1%) ultrasound examination normal. There were 41 patients with upper abdominal CT scan or plain scan. The results suggested that 25 cases (61%) cirrhosis, 14 cases (34.2%) suggest ascites, and there are 29 cases. In the liver and spleen, 7 cases (17.1%) showed portal hypertension, 19 cases (46.3%) were suggestive of esophageal varices, 16 cases (39%) were suggestive of varicose gastric fundus vein. There were 8 cases (19.5%) with esophageal gastroscope varicose and fundus varicose.8. in 26 patients with Fibro Scan examination. The results suggested that 9 patients (34.6%) were in the stage of cirrhosis. 14 (14) patients were in the period of cirrhosis. 1 patients with Mayo score, the mean value of 5.7 + 1.5.Mayo risk score and Fibro Scan staging have significant positive correlation (correlation coefficient 0.948, P value 0).9. male course 150 (30-361) compared with female group course 300 (60-1000) short, statistical difference (P= 0.023).AMA in female group (68.7%) detection rate is higher than male group (46.2%), difference There were statistical differences. There was no significant difference between the two groups in the clinical and biochemical examination. The.10.Ro-52 positive group was more prone to dry mouth dry mouth, ascites, with statistical difference (P respectively 0.014 and 0.012); positive combination and Sjogren syndrome were more than negative groups, there were statistical differences (P=0.002); positive group; positive group The serum Ig G was higher than that of the negative group (P value 0.023), but CHE was lower (P=0.008).11. for the diagnostic value of esophageal varices, when the Mayo score was equal to 4.8, the platelet count was 130 x 109/L, the serum albumin 38.6g/L was the largest, and the three had a certain diagnostic value for the esophageal varices. Total bilirubin and coagulation were of certain value. The diagnostic value of venous varicose in the time of blood enzyme is limited. Conclusion: primary biliary cholangitis mainly involves middle-aged women, and female patients have longer course of disease. The main manifestations are jaundice, liver and spleen, asthenia, anorexia, abdominal discomfort, ascites, skin itching, dry mouth and dry mouth, symptoms lack specificity, early part of the patients have no obvious symptoms, to disease in early part of the patients to disease. Later complications of liver cirrhosis accompanied by autoimmune diseases such as Sjogren's syndrome. The most diagnostic value of the non invasive examination was the serum AMA/AMA-M2 antibody. The Ro-52 positive group was more likely to merge with Sjogren syndrome. The antibody in the patient's sera was more prone to abdominal water, and the Ro-52 negative patients may have lower liver function. When the Mayo score is greater than 4.8, the platelet count is less than 130 x 109/L, the patients with serum albumin less than 38.6g/L need to follow up the gastroscopy regularly. At present, the treatment is based on UCDA, and it is necessary to find a more effective and safe method for early diagnosis of early treatment.
【學(xué)位授予單位】：蘭州大學(xué)
【學(xué)位級別】：碩士
【學(xué)位授予年份】：2017
【分類號】：R575.2
【參考文獻(xiàn)】
相關(guān)期刊論文 前10條
1 鄧紅梅;胡世玲;陳維賢;黃英;;抗核孔膜蛋白和抗核體蛋白抗體對原發(fā)性膽汁性肝硬化診斷價值的meta分析[J];中華肝臟病雜志;2016年01期
2 陳成偉;成軍;竇曉光;段維佳;段鐘平;范建高;傅青春;高春芳;侯金森;胡和平;胡錫琪;黃建榮;韓濤;韓英;賈繼東;陸倫根;劉玉蘭;馬雄;茅益民;南月敏;�？∑�;邱德凱;任紅;尚佳;唐紅;王貴強(qiáng);王吉耀;王建設(shè);王磊;王宇明;魏來;許建明;徐銘益;謝青;謝渭芬;徐小元;楊長青;楊云生;尤紅;曾民德;張文宏;張躍新;周新民;莊輝;鄒曉平;;原發(fā)性膽汁性肝硬化(又名原發(fā)性膽汁性膽管炎)診斷和治療共識(2015)[J];臨床肝膽病雜志;2015年12期
3 于樂成;侯金林;;《2014年澳大利亞肝病學(xué)會專家共識:瞬時彈性成像在慢性病毒性肝炎中的應(yīng)用建議》簡介[J];臨床肝膽病雜志;2015年04期
4 Xue-Xiu Zhang;Li-Feng Wang;Lei Jin;Yuan-Yuan Li;Shu-Li Hao;Yan-Chao Shi;Qing-Lei Zeng;Zhi-Wei Li;Zheng Zhang;George KK Lau;Fu-Sheng Wang;;Primary biliary cirrhosis-associated hepatocellular carcinoma in Chinese patients:Incidence and risk factors[J];World Journal of Gastroenterology;2015年12期
5 杜瑩;方蕾;;中藥湯劑/中成藥聯(lián)合熊去氧膽酸治療原發(fā)性膽汁性肝硬化療效的Meta分析[J];臨床肝膽病雜志;2015年01期
6 Satoshi Yamagiwa;Hiroteru Kamimura;Masaaki Takamura;Yutaka Aoyagi;;Autoantibodies in primary biliary cirrhosis:Recent progress in research on the pathogenetic and clinical significance[J];World Journal of Gastroenterology;2014年10期
7 劉紅虹;福軍亮;徐軍;羅生強(qiáng);劉振文;王福生;;123例原發(fā)性膽汁性肝硬化臨床表現(xiàn)與自身抗體譜[J];北京大學(xué)學(xué)報(醫(yī)學(xué)版);2013年02期
8 ;B cell depletion in treating primary biliary cirrhosis:Pros and cons[J];World Journal of Gastroenterology;2012年30期
9 王培之;華文浩;劉穎;;抗著絲點抗體與抗核抗體在原發(fā)性膽汁性肝硬化的臨床意義[J];北京醫(yī)學(xué);2012年06期
10 史旭華;張奉春;張p，
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