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目的探讨原发性胆汁性肝硬化(PBC)患者的临床表现、实验室检查及肝穿刺病理的特征性。方法12例PBC患者静脉抽血,采用全自动生化分析仪检测肝功能指标;采用酶联免疫吸附试验检测自身抗体;B超引导下肝穿刺组织检查病理组织学特征。结果12例患者血清肝功能改变以γ-谷酰基转移酶(GGT)和碱性磷酸酶(ALP)升高明显[分别为(245±190)和(294±153)U/L];免疫功能检测抗线粒体抗体)AMA)和(或)AMA-M2亚型阳性9例(占75%),抗平滑肌抗体(ASMA)4例(占33%),抗核抗体(ANA)1例(8%);肝穿刺病理改变:Ⅰ期患者8例;Ⅱ期患者2例;Ⅲ～Ⅳ期患者2例。结论PBC患者的AMA和(或)AMA-M2阳性率较高,肝组织病理的病变特征主要是小胆管的破坏、胆管周炎性细胞浸润及汇管区纤维化,肝细胞改变缺乏特异性。
Objective To investigate the clinical manifestations, laboratory tests and pathological features of liver biopsy in patients with primary biliary cirrhosis (PBC). Methods Twelve patients with PBC were subjected to venous blood sampling. The liver function was detected by automatic biochemical analyzer. The autoantibodies were detected by enzyme - linked immunosorbent assay. The histopathological features of liver biopsy were examined by B - ultrasonography. Results The changes of serum liver function in 12 patients were significantly higher than that of GGT and ALP [(245 ± 190) vs (294 ± 153) U / L], respectively. Immune function 9 cases (75%) were positive for anti-mitochondrial antibody (AMA) and / or AMA-M2 subtype, 4 cases (33%) were anti-smooth muscle antibodies ); Liver biopsy pathological changes: 8 cases of stage Ⅰ; 2 cases of stage Ⅱ; Ⅲ ~ Ⅳ patients in 2 cases. Conclusions The positive rate of AMA and / or AMA-M2 in PBC patients is high. The pathological features of liver pathology are mainly the destruction of small bile ducts, inflammatory cell infiltration in biliary ducts, fibrosis in portal area and lack of specificity of hepatocyte changes.