【摘要】 目的探讨原发性胆汁性肝硬化(PBC)的临床特点及肝脏组织学病理特征。方法回顾性分析83例PBC患者的病例资料。结果 83例PBC患者男女比例约为7︰76。临床表现依次为乏力、纳差、黄疸、脾脏肿大、腹水、皮肤瘙痒、干燥综合征及肝脏肿大。血清碱性磷酸酶(ALP)和γ-谷氨酰转肽酶(GGT)水平分别为(330.58±255.03)U/L和(255.58±234.65)U/L。抗线粒体抗体(AMA)和抗-线粒体抗体M2亚型(AMA-M2)阳性率分别为88%和71.1%。21例PBC患者肝组织病理检查提示小胆管改变最为突出。Ⅰ、Ⅱ期和Ⅲ、Ⅳ期两组患者病程及总胆红素水平差异有统计学意义(P<0.05)。结论 PBC好发于女性,临床表现缺乏特异性,血清ALP及GGT水平明显升高,自身抗体AMA和AMA-M2阳性为主要特征,病程和总胆红素水平与肝组织学进展有关。更多还原

【Abstract】 Objective To explore the clinical and pathological features of primary biliary cirrhosis (PBC). Methods Clinical data of 83 patients with PBC has been analyzed retrospectively. Results All 83 patients, the male to female ratio was 7︰76. The manifestation were fatigue, anorexia, jaundice, splenomegaly, ascites, pruritus, Sj?gren’s syndrome and hepatomegalia in order. The levels of serum alkaline phosphatase (ALP) and serum γ glutamyl transpeptidase (GGT) were significanly elevated at level of (330.58 ± 255.03) U/L and (255.58 ± 234.65) U/L respectively. The positive rate of anti-mitochondrial antibody (AMA) and anti-mitochondrial antibody M2 subtype (AMA-M2) were 88% and 71.1%, respectively. The results of 21 cases with liver histopathological examination indicated that the main changes focus on small bile duct. There were significant difference in course of disease and total bilirubin between Ⅰ, Ⅱstage and Ⅲ, Ⅳ stage (P < 0.05). Conclusions PBC most often occur in women. Clinical manifestations lack of specificity. The levels of serum ALT and GGT were increased significantly, and the positive AMA/AMA-M2 are key feature. The course of disease and the total bilirubin are relate to histopathological progression.更多还原