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目的 研究原发性胆汁性肝硬化 (PBC)的临床病理特点、伴发疾病及治疗反应 ,以提高对PBC的认识。方法 分析 37例PBC的临床资料及其中 13例肝穿刺病理资料 :抗线粒体抗体(AMA)采用间接免疫荧光法检测 ,M2 亚型采用ELISA法检测。结果 ① 37例患者 ,发病年龄 (5 0±10 )岁。②症状 :乏力 2 0例 (5 4 1% ) ,瘙痒 10例 (2 7 0 % ) ,腹痛 8例 (2 1 6 % ) ,肝性脑病 1例(2 7% ) ;体征 :肝大 2 6例 (70 3% ) ,脾大 2 0例 (5 4 1% ) ,黄疸 16例 (43 2 % ) ,腹水 4例 (10 8% ) ,门脉高压症 7例 (18 9% )。③实验室检查 :血清碱性磷酸酶水平升高 37例 (10 0 0 % ) ,(45 0± 32 2 )U/L ;总胆红素升高 31例 (83 8% ) ,(12 6± 14) μmol/L ;丙氨酸转氨酶升高 32例 (86 5 % ) ,(71± 17)U/L ;IgM升高 32例 (86 5 % ) ;ANA阳性 10例 (2 7 0 % ) ;抗SSA和 /或抗SSB抗体阳性 5例(13 5 % )。④合并干燥综合征 (SS) :符合哥本哈根标准 17例 (45 9% ) ,符合董怡标准 10例(2 7 0 % )。⑤肝穿刺病理 :取材满意 9例中 ,胆管炎 9例 ,肝细胞点状及碎片状坏死 1例 ,纤维化 8例。⑥治疗反应 :单纯皮质激素和 /或免疫抑制剂治疗者 2 6例 ,有效 10例 ,无效 16例 ,有效组IgG升高及合并SS例数均显著高于无效组 (P分别 <0 0 1?
Objective To study the clinical and pathological features of primary biliary cirrhosis (PBC), concomitant diseases and treatment response to improve the understanding of PBC. Methods Clinical data of 37 cases of PBC and pathological data of 13 cases of liver biopsy were analyzed. Anti-mitochondrial antibody (AMA) was detected by indirect immunofluorescence and M2 subtype was detected by ELISA. Results ① In 37 patients, the age of onset was (50 ± 10) years. ② Symptoms: 20 cases of fatigue (541%), pruritus in 10 cases (270%), abdominal pain in 8 cases (21.6%) and hepatic encephalopathy in 1 case (27%). 6 cases (70.3%), 20 cases of splenomegaly (541%), 16 cases of jaundice (43.2%), 4 cases of ascites (108%) and 7 cases of portal hypertension (18.9%). (3) Laboratory tests showed that serum alkaline phosphatase level was increased in 37 cases (100%), (45 0 ± 32 2) U / L, elevated total bilirubin in 31 cases (83.8%), ± 14) μmol / L, alanine aminotransferase increased 32 cases (86 5%), (71 ± 17) U / L; IgM increased in 32 cases (86 5%); ANA positive in 10 cases (270% ); Anti-SSA and / or anti-SSB antibody positive in 5 cases (13 5%). ④ combined with Sjogren’s syndrome (SS): in line with the Copenhagen standard 17 cases (45 9%), in line with Dong Yi standard 10 cases (270%). ⑤ liver biopsy pathology: satisfactory material 9 cases, 9 cases of cholangitis, hepatocyte punctate and debris necrosis in 1 case, fibrosis in 8 cases. ⑥ Therapeutic response: 26 cases of corticosteroid and / or immunosuppressive therapy were treated, 10 cases were effective and 16 cases were ineffective. The number of elevated IgG and the number of combined SS in effective group were significantly higher than those in ineffective group (P <0 01 ?