Dawkins P A, Dawkins C L, Wood A M, Nightingale P G, Stockley J A, Stockley R A
Lung Investigation Unit, UniversityHospital Birmingham and University of Birmingham, Birmingham, UK.
Eur Respir J. 2009 Jun;33(6):1338-44. doi: 10.1183/09031936.00061208. Epub 2009 Jan 22.
The aim of the present study was to identify alpha(1)-antitrypsin (alpha(1)-AT)-deficient patients who had rapidly progressive disease. PiZ patients (n = 101) underwent annual lung function measurements over a 3-yr period, and the results were related to factors that may influence decline. The mean annual decline in forced expiratory volume in 1 s (FEV(1)) was 49.9 mL. The greatest FEV(1) decline occurred in the moderate severity group (FEV(1) 50-80% of the predicted value), with a mean annual decline of 90.1 mL, compared with 8.1 mL in the very severe group (FEV(1) <30% pred). However, annual decline in transfer coefficient of the lung for carbon monoxide (K(CO)) was greatest in the severe and very severe groups. When the whole group was divided into tertiles of FEV(1) decline, the fast tertile compared with the slow tertile had more patients with bronchodilator reversibility (BDR) (73 versus 41%; p = 0.010), more males (79 versus 56%; p = 0.048) and lower body mass index (BMI) (24.0 versus 26.1; p = 0.042). Logistic regression analyses confirmed that FEV(1) decline was independently associated with BMI, BDR, exacerbation rate and high physical component 36-item short-form health survey scores. In PiZ alpha(1)-AT-deficient patients, FEV(1) decline was greatest in moderate disease, unlike K(CO) decline, which was greatest in severe disease. The FEV(1) decline showed associations with BDR, BMI, sex and exacerbation rate.
本研究的目的是识别患有快速进展性疾病的α1抗胰蛋白酶（α1-AT）缺乏患者。101名PiZ型患者在3年期间每年进行肺功能测量，结果与可能影响肺功能下降的因素相关。第1秒用力呼气量（FEV1）的年平均下降量为49.9 mL。FEV1下降最大的发生在中度严重程度组（FEV1为预测值的50%-80%），年平均下降量为90.1 mL，而在极重度组（FEV1<预测值的30%）为8.1 mL。然而，一氧化碳肺转移系数（KCO）的年下降量在重度和极重度组中最大。当将整个组按FEV1下降的三分位数划分时，与下降较慢的三分位数相比，下降较快的三分位数中有更多的患者具有支气管扩张剂可逆性（BDR）（73%对41%；p = 0.010）、更多男性（79%对56%；p = 0.048）和更低的体重指数（BMI）（24.0对26.1；p = 0.042）。逻辑回归分析证实，FEV1下降与BMI、BDR、急性加重率和健康调查简表36项身体成分高分独立相关。在PiZ型α1-AT缺乏患者中，与KCO下降不同，FEV1下降在中度疾病中最大，而KCO下降在重度疾病中最大。FEV1下降与BDR、BMI、性别和急性加重率相关。
