摘要
目的 分析原发性胆汁性肝硬化（PBC）患者不同阶段临床及肝组织病理学特征,为该病的早期识别及有效治疗提供循证医学依据. 方法 收集经病理学确诊的临床资料完整的130例PBC病例,依病理学分为早期组和晚期组,对其一般资料、临床表现、实验室检查及病理学改变等进行回顾性分析.两组间计量资料比较采用t检验,计数资料比较采用χ2检验. 结果 PBC患者平均年龄（43.5±7.1）岁,以中年女性多见,占89％;最常见症状是乏力,其次是黄疸、皮肤瘙痒,晚期出现腹胀;11.5％患者可合并自身免疫性疾病;转氨酶随疾病的进展有降低趋势,两组间比较,差异无统计学意义（P＞ 0.05）;多数病例血清胆红素升高,以直接胆红素升高为主,血清总胆红素、直接胆红素水平随病程进展呈增高趋势,两组间差异有统计学意义（P＜ 0.01）;患者血清碱性磷酸酶、γ-谷氨酰转移酶水平升高,随着病情进展,前者升高,后者降低,但二者在早期组及晚期组间差异均无统计学意义（P＞ 0.05）.抗线粒体抗体的阳性率为85％.PBC的组织病理学改变为汇管区及其周围病变重,小叶内病变较轻.早期主要表现为小叶间胆管的损伤及小胆管的增生,周围可见单个核细胞聚集性浸润,形成淋巴滤泡样结构.晚期表现为汇管区纤维性扩大、纤维间隔及假小叶形成、肝硬化. 结论 PBC好发于中年女性,起病隐匿,早期、晚期有其相对临床及病理学特点,血清学检测无特征性改变者,需依据肝穿刺检查确诊,以避免漏诊、误诊.
Objective To investigate the clinicopathological features of different stages of primary biliary cirrhosis （PBC）,and to provide a basis of evidence-based medicine for early identification and effective treatment of this disease.Methods A total of 130 patients with pathologically confirmed PBC and complete clinical data were enrolled and divided into early-stage group and late-stage group based on pathological results.A retrospective analysis was performed for patients＇ general information,clinical manifestations,laboratory examinations,and pathological changes.Results The PBC patients had a mean age of 43.5±7.1 years,with middle-aged female patients accounting for 89％.The most common symptom was fatigue,followed by jaundice,pruritus,and abdominal distension in the late stage.Of all patients,11.5％ were complicated by autoimmune disease.The level of aminotransferases tended to decrease with the progression of PBC and showed no significant differences between the two groups （P ＞ 0.05）.Most patients showed an increase in serum bilirubin,mainly direct bilirubin;serum total bilirubin and direct bilirubin tended to increase with disease progression and showed significant differences between the two groups （P ＜ 0.01）.The patients showed increases in the serum levels of alkaline phosphatase （ALP） and γ-glutamyl transpeptidase （GGT）,but with the disease progression,the serum level of ALP increased and that of GGT decreased;the serum levels of ALP and GGT showed no significant differences between the early-and late-stage groups （P ＞ 0.05）.The positive rate of antimitochondrial antibody was 85％.The histopathological changes of PBC included severe lesions in the portal area and surrounding areas and slight lobular lesions.In the early stage,there were injuries of the interlobar bile ducts,proliferation of small bile ducts,aggregation and invasion of mononuclear cells in surrounding tissues,and the formation of lymphoid follicle-like structure;in the late stage,there were fibrotic expans
出处
《中华肝脏病杂志》
CAS
CSCD
北大核心
2016年第6期412-416,共5页
Chinese Journal of Hepatology
关键词
肝硬化
胆汁性
病理学
临床
Liver cirrhosis, biliary
Pathology
Clinical
