AMA阳性的原发性胆汁性肝硬化患者的临床特征

目的探讨原发性胆汁性肝硬化(primary biliary cirrhosis,PBC)的临床特征。方法回顾36例AMA阳性的PBC患者临床资料,同期住院的36例慢性乙型肝炎肝硬化(chronic hepatitis B virus-related cirrhosis,HBV-C)患者为对照组,分析两组患者的临床特点和生物化学指标。结果 PBC组以女性(83.3%)多见,平均年龄(53.5±11.5)岁,HBV-C组主要是男性(77.8%),平均年龄(56.4±12.8)岁。黄疸(66.7%)和乏力(63.9%)是PBC组的主要表现,HBV-C组以腹水(50.0%)和上消化道出血(38.9%)常见。HBV-C组的PLT和WBC水平较PBC组显著下降(P<0.01)。两组患者均有Hb和RBC水平明显降低,PBC组更明显,但两组间差异无统计学意义。ALT、AST、GGT、ALP、TBIL和DBIL水平在PBC组中均明显升高(P<0.05),而PT在HBV-C组显著延长(P=0.000)。血脂各项指标在Child-Pugh A级中,PBC组均高于HBV-C组,但差异无统计学意义;Child-Pugh B级中,TG、TC及LDL-C水平PBC组显著高于HBV-C组(P<0.05),而HDL-C水平在PBC组显著降低(P=0.006)。结论 PBC患者具有许多独特的临床特征,包括:①常合并其他自身免疫性疾病;②更容易发生黄疸和贫血;③反映胆管功能和胆汁代谢指标明显异常;④HDL-C的下降可能是反映PBC病情进展的良好指标。

Clinical features of AMA-positive primary biliary cirrhosis

Objective To investigate the clinical features of primary biliary cirrhosis(PBC). Methods A total of 36 anti-mitochondrial antibody(AMA) positive PBC patients were included in this study,and compared with 36 chronic hepatitis B virus-related cirrhosis(HBV-C) patients in terms of clinical and biochemical features. Results Patients in PBC group were mainly females(83.3%) with the mean age of 53.5±11.5,and most of HBV-C group were males(77.8%) with the mean age of 56.4±12.8.The main symptoms and physical signs of PBC group were jaundice(66.7%) and fatigue(63.9%),while ascites(50%) and upper gastrointestinal hemorrhage(38.9%) were most commonly seen in HBV-C group.HBV-C group had lower levels of platelet(PLT) and white blood cell(WBC)(P<0.01).Hemoglobin(Hb) and red blood cell(RBC) levels decreased in all patients especially those with PBC,however,there was no significant difference between two groups.Alanine aminotransferase(ALT),aspirate aminotransferase(AST),γ-glutamyltransferase(GGT),alkaline phosphatase(ALP),total bilirubin(TBIL),direct bilirubin(DBIL) markedly elevated in PBC group(P<0.05),while prothrombin time(PT) significantly prolonged in HBV-C group(P=0.000).Among Child-Pugh stage A patients,PBC group had higher levels of total cholesterol(TC),triglyceride(TG),lower density lipoprotein cholesterol(LDL-C) and higher density lipoprotein cholesterol(HDL-C) than HBV-C group,although with no significant difference.In Child-Pugh stage B PBC patients,the levels of TC,TG and LDL-C were much higher(P<0.05),while HDL-C level was lower(P=0.006). Conclusion This study shows several characteristic clinical features of PBC,including: ① is commonly associated with other autoimmune diseases;② is more likely to suffer jaundice and anemia;③ has significant abnormal biochemical tests related to bile ducts function and bilirubin metabolism;④ HDL-C level may be a valuable marker of disease progression.