Predictors of mortality in α1-antitrypsin deficiency
Predictors of mortality in α1-antitrypsin deficiency
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DOI：
10.1136/thorax.58.12.1020
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发表时间：
2003-12-01
期刊：
影响因子：
10
通讯作者：
Stockley, RA
中科院分区：
文献类型：
--
作者：
Dawkins, PA;Dowson, LJ;Stockley, RA
背景资料：以前已经发现，在α（1）-抗胰蛋白酶缺乏的肺气肿中，通过计算机断层扫描测量肺密度是比肺功能测量更敏感的疾病进展指标。本研究的目的是探讨包括CT扫描在内的几个参数对严重α 1-抗胰蛋白酶缺乏症患者死亡率的预测潜力。方法：在5年的时间里，对256例α 1-抗胰蛋白酶缺乏症（PiZ表型）患者进行了评估，其中254例进行了肺功能检查，197例进行了胸部CT扫描。肺功能，CT扫描，健康状况（圣乔治的呼吸问卷，SGRQ），和其他临床资料的幸存者和非幸存者进行了比较，这些参数被应用到生存analysis.Results：有22例死亡，在这个病人队列，其中10人被归类为“自杀性”死亡。非存活者的基线肺功能参数（1秒用力呼气量（FEV 1）、一氧化碳转移系数（KCO））和CT评分显著低于存活者。256名患者中有170名患者有完整的数据可进入多元回归分析（考克斯比例风险模型）。在单变量分析中，上区呼气扫描与全因（p = 0.001）和呼吸死亡率（p< 0.001）相关性最好，而FEV 1（p = 0.158全因，0.015呼吸）和KCO（p = 0.002全因，0.012呼吸）与死亡率相关性较差。当CT扫描进入生存分析时，只有年龄才能提供有关全因或呼吸道死亡率的进一步独立预测信息。结论：CT扫描可预测α（1）-抗胰蛋白酶缺乏症的呼吸道和全因死亡率，并且似乎优于肺功能参数（尤其是FEV 1）的上级。
Background: Lung density measurements by computed tomography have previously been found to be a more sensitive indicator of disease progression in emphysema of alpha(1)-antitrypsin deficiency than lung function measurements. The aim of this study was to investigate the predictive potential of several parameters, including CT scanning, for mortality in patients with severe alpha(1)-antitrypsin deficiency.Methods: Over a 5 year period, 256 patients with alpha(1)-antitrypsin deficiency (PiZ phenotype) were assessed, of whom 254 underwent lung function testing and 197 had thoracic CT scans. Lung function, CT scans, health status (St George's Respiratory Questionnaire, SGRQ), and other clinical data of survivors and non-survivors were compared and these parameters were applied to survival analyses.Results: There were 22 deaths in this patient cohort, 10 of which were classified as "respiratory'' deaths. Baseline lung function parameters ( forced expiratory volume in 1 second (FEV1), carbon monoxide transfer coefficient (KCO)), and CT scores were significantly lower in the non-survivors than in the survivors. 170 of the 256 patients had complete data for entry into multiple regression analyses ( Cox proportional hazards model). In the univariate analysis, upper zone expiratory scan had the best association with all cause ( p = 0.001) and respiratory mortality (p< 0.001), whereas FEV1 ( p = 0.158 all cause, 0.015 respiratory) and KCO ( p = 0.002 all cause, 0.012 respiratory) had poorer associations with mortality. Only age gave further independent predictive information regarding all cause or respiratory mortality when the CT scan was entered into the survival analyses.Conclusions: CT scanning predicts respiratory and all cause mortality in alpha(1)-antitrypsin deficiency and appears to be superior to lung function parameters, especially FEV1.