Gu Er-li, Yao Guang-bi
Shanghai Jinganqu Central Hospital, Shanghai 200040, China.
Zhonghua Gan Zang Bing Za Zhi. 2009 Nov;17(11):861-6.
To summarize the clinical features, diagnosis and treatment of patients with primary biliary cirrhosis (PBC) in China.
Systematic analysis of clinical characteristics by searching the Chinese literatures.
From 1955 to 2007, 2740 PBC patients were reported in 103 papers (duplicated reports were deleted). The detailed information of 985 patients from 16 papers were collected. Female : male was 6.82:1. The age range was 42 to 56.2-year-old. The time from onset to diagnosis was 12 to 98.4 months. The most common symptoms were fatigue (72.40%), jaundice (67.41%), anorexia (68.58%) and pruritus (45.60%). 20% patients were asymptomatic at onset. The most frequent physical signs were splenomegaly (57.53%), hepatomegaly (43.56%) and ascites (18.45%). Serum alkaline phosphatase (ALP) and gamma glutamyl transpeptidase (GGT) levels were markedly elevated in most of these patients. The immunological marks of AMA and M2 were positive in 88.98% and 82.65% patients, respectively. The most common comorbidity were Sjögren syndrome (9.14%), rheumatoid arthritis (3.95%) and diabetes type II (2.54%). Of the 507 patients treated with ursodeoxycholic acid (UDCA), 345 patients got complete or partial clinical biochemical response. The common complications were gastrointestinal bleeding (41.67%) and liver failure (41.67%). Liver transplantation was the only effective way for the treatment of the end-stage liver disease.
The clinical feature of primary biliary cirrhosis in China was similar to the overseas literatures. Further research should focus on epidemic investigation, early diagnosis, long term follow up of asymptomatic patients, immunological mechanism and the efficacy of liver transplantation.
总结我国原发性胆汁性肝硬化（PBC）患者的临床特征、诊断及治疗情况。
通过检索中文文献对临床特征进行系统分析。
1955年至2007年，103篇文献报道了2740例PBC患者（剔除重复报道）。收集了16篇文献中985例患者的详细信息。男女比例为6.82∶1。年龄范围为42至56.2岁。从发病到诊断的时间为12至98.4个月。最常见的症状为乏力（72.40%）、黄疸（67.41%）、厌食（68.58%）和瘙痒（45.60%）。20%的患者起病时无症状。最常见的体征为脾肿大（57.53%）、肝肿大（43.56%）和腹水（18.45%）。这些患者多数血清碱性磷酸酶（ALP）和γ-谷氨酰转肽酶（GGT）水平显著升高。AMA和M2的免疫学标志物分别在88.9八%和82.65%的患者中呈阳性。最常见的合并症为干燥综合征（9.14%）、类风湿关节炎（3.95%）和2型糖尿病（2.54%）。在507例接受熊去氧胆酸（UDCA）治疗的患者中，345例获得了完全或部分临床生化缓解。常见并发症为消化道出血（41.67%）和肝衰竭（41.67%）。肝移植是终末期肝病唯一有效的治疗方法。
我国原发性胆汁性肝硬化的临床特征与国外文献报道相似。进一步研究应集中在流行病学调查、早期诊断、无症状患者的长期随访、免疫机制及肝移植疗效等方面。
