Larsson C
Acta Med Scand. 1978;204(5):345-51. doi: 10.1111/j.0954-6820.1978.tb08452.x.
Clinical data from 246 adult Swedish individuals with severe alpha1-antitrypsin deficiency, Pi Z, diagnosed in 1963--77, were analyzed. Primary emphysema was present in 109 cases. Of 75 Pi Z patients with other types of chronic obstructive pulmonary disease (COPD), all but 7 showed signs of emphysema. Median age at onset of dyspnoea in Pi Z smokers was 40 years, compared to 53 in non-smokers (p less than 0.001). Of the Pi Z individuals over the age of 50, 19% had a diagnosis of liver cirrhosis and 15% signs of glomerular renal damage. Of 91 deceased patients, 56 died from COPD and 12 from liver disease. A greatly reduced survival was demonstrated in Pi Z individuals, regardless of sex. Smoking Pi Z individuals had a significantly lower life expectancy than Pi Z non-smokers (p less than 0.01).
对1963年至1977年间诊断出患有严重α1-抗胰蛋白酶缺乏症（Pi Z型）的246名成年瑞典人的临床数据进行了分析。109例患者存在原发性肺气肿。在75例患有其他类型慢性阻塞性肺疾病（COPD）的Pi Z患者中，除7例之外，其余患者均有肺气肿迹象。Pi Z型吸烟者出现呼吸困难的中位年龄为40岁，非吸烟者为53岁（p<0.001）。在50岁以上的Pi Z个体中，19%被诊断为肝硬化，15%有肾小球肾损伤迹象。在91例死亡患者中，56例死于COPD，12例死于肝病。无论性别如何，Pi Z个体的生存率均大幅降低。吸烟的Pi Z个体的预期寿命明显低于不吸烟的Pi Z个体（p<0.01）。
