Third Department of Internal Medicine Departments of Blood Transfusion Medicine Community Health and Epidemiology, Nara Medical University, Kashihara, Nara, Japan.
Hepatol Res. 2012 May;42(5):459-72. doi: 10.1111/j.1872-034X.2011.00950.x. Epub 2012 Jan 31.
Decreased plasma ADAMTS13 activity (ADAMTS13:AC) results in accumulation of unusually large von Willebrand factor multimers and platelet thrombi formation. Our aim was to evaluate whether ADAMTS13:AC is a prognostic marker in patients with liver cirrhosis.
Plasma ADAMTS13:AC and its related parameters were examined in 108 cirrhotic patients.
ADAMTS13:AC decreased as the severity of liver disease increased (means: controls 100%, Child A-cirrhotics 79%, Child B-cirrhotics 63%, and Child C-cirrhotics 31%). ADAMTS13:AC markedly decreased in the cirrhotics with hepatorenal syndrome, refractory ascites and hepatic encephalopathy. The cumulative survival time was the shortest (median: 4.5 months) in the cirrhotics with severe to moderate ADAMTS13:AC deficiency (<3-25%), followed by those with mild ADAMTS13:AC deficiency (25-50%), and was the longest in those with normal activity (>50%). In contrast, based on the Child-Turcotte-Pugh (CTP) score, Child C-cirrhotics had the worst survival, but the survival probabilities did not differ between Child A and B cirrhotics. Based on the Model for End-Stage Liver Disease (MELD) score, the survival was the worst for the cirrhotics in the fourth quartile, but it was not different among cirrhotics in the first three quartiles. Cox proportional-hazards regression analysis showed that ADAMTS13:AC and serum albumin were independent factors affecting the survival.
ADAMTS13:AC concomitantly decreases as the functional liver capacity decreases. This activity may be a useful prognostic marker that is equal or superior to the CTP score and the MELD score to predict not only the short-term prognosis but also the long-term survival of the cirrhotic patients.
血浆 ADAMTS13 活性（ADAMTS13:AC）降低导致异常大的 von Willebrand 因子多聚体积聚和血小板血栓形成。我们的目的是评估 ADAMTS13:AC 是否是肝硬化患者的预后标志物。
检查了 108 例肝硬化患者的血浆 ADAMTS13:AC 及其相关参数。
随着肝病严重程度的增加，ADAMTS13:AC 降低（平均值：对照组 100%，Child A 肝硬化患者 79%，Child B 肝硬化患者 63%，Child C 肝硬化患者 31%）。肝性肾综合征、难治性腹水和肝性脑病的肝硬化患者 ADAMTS13:AC 明显降低。严重至中度 ADAMTS13:AC 缺乏（<3-25%）的肝硬化患者累积生存时间最短（中位数：4.5 个月），轻度 ADAMTS13:AC 缺乏（25-50%）的患者次之，正常活性（>50%）的患者最长。相比之下，根据 Child-Turcotte-Pugh（CTP）评分，Child C 肝硬化患者的生存率最差，但 Child A 和 B 肝硬化患者的生存率没有差异。根据终末期肝病模型（MELD）评分，第四四分位数的肝硬化患者生存率最差，但前三四分位数的肝硬化患者生存率没有差异。Cox 比例风险回归分析表明，ADAMTS13:AC 和血清白蛋白是影响生存率的独立因素。
随着功能性肝容量的降低，ADAMTS13:AC 同时降低。这种活性可能是一种有用的预后标志物，与 CTP 评分和 MELD 评分一样，甚至优于它们，可预测肝硬化患者的短期和长期预后。
