Mahl T C, Shockcor W, Boyer J L
Liver Study Unit, Yale University School of Medicine, New Haven, CT 06510.
J Hepatol. 1994 Jun;20(6):707-13. doi: 10.1016/s0168-8278(05)80139-4.
We extended by 10 years, a follow-up study of 279 patients with primary biliary cirrhosis initially evaluated at the Yale Liver Study Unit between 1955 and 1979. Thirty-six patients (13%) were asymptomatic at the time of diagnosis. Accurate follow-up survival data were available for 247 patients (89%), ranging up to 24 years after the original diagnosis. Median predicted survival of patients in this study from the time of diagnosis is twice as long for patients who present without symptoms compared to symptomatic patients (16 vs 7.5 years, p < 0.0001). However, overall survival of those asymptomatic patients is shorter than that predicted for an age- and gender-matched control population (p < 0.0001), a difference that became apparent only after 11 years of follow up. With a median follow up of 12.1 years, 33% of the asymptomatic patients remained free of symptoms of liver disease, However, once symptoms develop, their survival is similar to those presenting with symptoms. Independent predictors of diminished survival include: elevated bilirubin, increasing age, ascites, advanced fibrosis and the degree of portal bile stasis on liver biopsy. It was not possible to predict which asymptomatic patients would remain symptom free.
我们将一项针对279例原发性胆汁性肝硬化患者的随访研究延长了10年，这些患者最初于1955年至1979年间在耶鲁肝脏研究室接受评估。36例患者（13%）在诊断时无症状。247例患者（89%）有准确的随访生存数据，自最初诊断后随访时间长达24年。本研究中，从诊断时起，无症状患者的中位预测生存期是有症状患者的两倍（16年对7.5年，p<0.0001）。然而，那些无症状患者的总体生存期短于年龄和性别匹配的对照人群的预测生存期（p<0.0001），这种差异仅在随访11年后才变得明显。中位随访12.1年，33%的无症状患者仍无肝病症状。然而，一旦出现症状，他们的生存期与有症状患者相似。生存期缩短的独立预测因素包括：胆红素升高、年龄增长、腹水、肝纤维化进展以及肝活检时门静脉胆汁淤积程度。无法预测哪些无症状患者将一直无症状。
