ADAMTS13 activity may predict the cumulative survival of patients with liver cirrhosis in comparison with the Child-Turcotte-Pugh score and the Model for End-Stage Liver Disease score
ADAMTS13 activity may predict the cumulative survival of patients with liver cirrhosis in comparison with the Child-Turcotte-Pugh score and the Model for End-Stage Liver Disease score
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DOI：
10.1111/j.1872-034x.2011.00950.x
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发表时间：
2012-05-01
影响因子：
4.2
通讯作者：
Fukui, Hiroshi
中科院分区：
文献类型：
--
作者：
Takaya, Hiroaki;Uemura, Masahito;Fukui, Hiroshi
目的：血浆ADAMTS 13活性降低（ADAMTS 13：AC）导致异常大的血管性血友病因子多聚体蓄积和血小板血栓形成。方法：检测108例肝硬化患者血浆ADAMTS 13：AC及其相关参数，结果：ADAMTS 13：AC随肝病严重程度的增加而降低（平均值：对照组100%，Child A型79%，Child B型63%，Child C型31%）。ADAMTS 13：AC在肝肾综合征、顽固性腹水和肝性脑病患者中明显降低。ADAMTS 13：AC重度至中度缺乏者（50%）的累积生存时间最短（中位数：4.5个月）。相反，根据Child-Turcotte-Pugh（CTP）评分，Child C型糖尿病患者的生存率最差，但Child A型和B型糖尿病患者的生存概率无差异。根据终末期肝病模型（MELD）评分，第四个四分位数的患者生存率最差，但前三个四分位数的患者之间无差异。考克斯比例风险回归分析显示ADAMTS 13：AC和血清白蛋白是影响生存的独立因素。这种活性可能是一个有用的预后指标，它等于或上级CTP评分和MELD评分，不仅预测短期预后，而且还预测患者的长期生存。
Aim: 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.Methods: Plasma ADAMTS13: AC and its related parameters were examined in 108 cirrhotic patients.Results: 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 (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.Conclusions: 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.