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目的研究原发性胆汁性肝硬化(PBC)患者的临床表现与实验室特征。方法收集2002年11月1日至2015年3月31日在暨南大学附属第一医院诊断为PBC的住院病例,回顾性分析其临床表现与实验室特征。结果共纳入PBC患者36例,男女性别比为1∶3,中位年龄为53.5岁;以肝大(88.9%)、黄疸(69.4%)、乏力(66.7%)、腹胀(52.8%)为主要临床表现,老年患者腹胀、上消化道出血的发生率均高于青中年患者(P<0.05)。血清谷氨酰转肽酶(GGT)升高最为显著,其次为碱性磷酸酶(ALP),中位数值分别为正常值的7.1和2.4倍,同时伴有明显的胆汁淤积性黄疸,总胆红素(TBIL)、结合胆红素(DBIL)、非结合胆红素(IBIL)、总胆汁酸(TBA)的中位数值分别为正常值的4.8、6.2、3和10倍。患者血清抗线粒体抗体(AMA)、抗核抗体(ANA)、抗核胞膜糖蛋白210抗体(抗gp210)+抗可溶性酸性核蛋白抗体(抗Sp100)的阳性率分别为55.6%、58.3%和41.7%,IgM升高率为65.0%。结论PBC以中年女性多见,肝大、黄疸、乏力、腹胀为主要临床表现,老年患者病情较重;血清GGT、ALP等升高明显;AMA阳性率较既往研究结果低,联合抗gp210、抗Sp100检测有助于该病的诊断。
Objective To study the clinical manifestations and laboratory characteristics of patients with primary biliary cirrhosis (PBC). Methods The hospitalized cases diagnosed as PBC in the First Affiliated Hospital of Jinan University from November 1, 2002 to March 31, 2015 were collected. The clinical manifestations and laboratory characteristics were retrospectively analyzed. Results A total of 36 patients with PBC were enrolled. The sex ratio was 1: 3, and the median age was 53.5 years old. The main diseases were hepatomegaly (88.9%), jaundice (69.4%), fatigue (66.7%) and abdominal distension Clinical manifestations, elderly patients with abdominal distension, upper gastrointestinal bleeding were higher than the incidence of middle-aged patients (P <0.05). Serum glutamyl transpeptidase (GGT) increased the most significant, followed by alkaline phosphatase (ALP), the median values were 7.1 and 2.4 times the normal, accompanied by significant cholestatic jaundice, total gall The median values of TBIL, DBIL, IBIL and TBA were 4.8, 6.2, 3 and 10 times of normal, respectively. The positive rates of serum anti-mitochondrial antibody (AMA), antinuclear antibody (ANA), anti-nuclear glycoprotein 210 antibody (anti-gp210) and anti-soluble acid ribosomal antibody (anti-Sp100) were 55.6%, 58.3% 41.7%, IgM increased 65.0%. Conclusions PBC is more common in middle-aged women, liver, jaundice, fatigue, abdominal distension as the main clinical manifestations, elderly patients with severe disease; serum GGT, ALP increased significantly; AMA positive rate than previous studies, combined with anti-gp210, Anti-Sp100 test can help diagnose the disease.