Buist A S, Adams B E, Azzam A H, Sexton G J
Am Rev Respir Dis. 1980 Dec;122(6):817-22. doi: 10.1164/arrd.1980.122.6.817.
In this paper we report the initial cross-sectional data from a prospective study of pulmonary function in children with moderately severe and severe alpha 1-antitrypsin deficiency. Using a case-control design, our cases were 19 children 3 to 7 years of age with alpha 1-antitrypsin deficiency, Pi phenotype ZZ or SZ. Control subjects were selected from healthy children participating in a study to establish reference values for functional residual capacity and maximal expiratory flow at functional residual capacity, using a 1:1 match for sex, height, age, and weight. We found no significant difference between the cases and their matched control subjects with respect to functional residual capacity and maximal expiratory flow at functional residual capacity. We conclude that through 7 years of age there is no gross impairment in overall pulmonary function in children with moderately severe and severe alpha 1-antitrypsin deficiency, Pi phenotypes ZZ and SZ.
在本文中，我们报告了一项对患有中度严重和严重α1-抗胰蛋白酶缺乏症儿童的肺功能进行前瞻性研究的初始横断面数据。采用病例对照设计，我们的病例为19名3至7岁患有α1-抗胰蛋白酶缺乏症、Pi表型为ZZ或SZ的儿童。对照受试者选自参与一项研究以建立功能残气量和功能残气量时最大呼气流量参考值的健康儿童，按照性别、身高、年龄和体重1:1匹配。我们发现病例组与其匹配的对照组在功能残气量和功能残气量时最大呼气流量方面无显著差异。我们得出结论，对于患有中度严重和严重α1-抗胰蛋白酶缺乏症、Pi表型为ZZ和SZ的儿童，至7岁时其整体肺功能无明显损害。
