Black L F, Kueppers F
Am Rev Respir Dis. 1978 Mar;117(3):421-8. doi: 10.1164/arrd.1978.117.3.421.
The clinical symptoms, measurements of pulmonary function, and interpretations of thoracic roentgenograms in 18 patients with alpha1-antitrypsin deficiency associated with the PiZ phenotype are reported. The patients had never smoked and had little or no exposure to occupational and urban air pollution. The findings were compared with those in a group of patients who also had the PiZ phenotype, but who were smokers. The results showed that the clinical course, rate of pulmonary-function deterioration, and appearance of the thoracic roentgenograms in persons who had never smoked are variable and suggested that other factors, in addition to phenotype and environmental pollutants, are determinants of the chronic obstructive pulmonary disease that develops in these patients. Many of these patients lived into their sixth and seventh decades, suggesting that those patients who avoid respiratory irritants do not necessarily have an ominous prognosis. These are important considerations in the diagnosis and treatment of patients who have this deficiency.
报告了18例与PiZ表型相关的α1抗胰蛋白酶缺乏症患者的临床症状、肺功能测量结果及胸部X线片解读。这些患者从不吸烟，很少或几乎没有接触过职业和城市空气污染。将这些结果与另一组同样具有PiZ表型但吸烟的患者进行了比较。结果表明，从不吸烟的患者的临床病程、肺功能恶化率及胸部X线片表现各不相同，提示除表型和环境污染物外，其他因素也是这些患者发生慢性阻塞性肺疾病的决定因素。许多这类患者活到了六七十岁，这表明那些避免接触呼吸道刺激物的患者不一定预后不佳。这些是诊断和治疗患有这种缺陷的患者时的重要考虑因素。
