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首页> 外文期刊>Thorax: The Journal of the British Thoracic Society >Clinical features and prognosis of life time non-smokers with severe alpha 1-antitrypsin deficiency (published erratum appears in Thorax 1998 Dec;53(12):1096)
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Clinical features and prognosis of life time non-smokers with severe alpha 1-antitrypsin deficiency (published erratum appears in Thorax 1998 Dec;53(12):1096)

机译：严重α1-抗胰蛋白酶缺乏症的终身不吸烟者的临床特征和预后（发表于勘误表的文章刊登于Thorax 1998 Dec; 53（12）：1096）

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BACKGROUND: The hereditary disorder alpha 1-antitrypsin deficiency is characterised by development of severe emphysema at an early age with smoking being the most significant additional risk factor. The purpose of the present paper was to analyse potential risk factors other than smoking for emphysema and to estimate the prognosis of life time non-smokers. METHODS: Patients were identified through the files of the Danish alpha 1-antitrypsin deficiency register which contains information on more than 700 persons with the condition. Many of the patients, the non-index cases, were identified from family studies. RESULTS: There were 75 life time non-smokers with PiZ (27 index cases and 48 non-index cases) aged 20 years or more at entry. Twenty one subjects died during the follow up period. The Standardised Mortality Ratio (SMR) was 3.0 (95% confidence intervals (CI) 1.9 to 4.6). There was no significant difference in SMR between males and females. The SMR was 8.8 (95% CI 5.0 to 14) for the index cases and 0.96 (95% CI 0.3 to 2.3) for the non-index cases based on five deaths. The overall mean % predicted forced expiratory volume in one second (FEV1) at entry was 83% with a significant difference between index cases (54%) and non-index cases (100%) (p < 0.001). The difference in the ratio of FEV1 to forced vital capacity (FVC) was also highly significant with values of 0.57 and 0.79 for index and non-index cases, respectively (p < 0.001). In the non-index group only three had an FEV1% predicted of less than 70%. CONCLUSIONS: Occupational exposure to airway irritants did not have any significant influence on the development of emphysema. Only a few life time non-smokers develop severe emphysema; most never develop pulmonary symptoms and thus remain undetected unless family members of index cases are screened.
机译：背景：遗传性疾病α1-抗胰蛋白酶缺乏症的特征是在幼年时出现严重的肺气肿，吸烟是最重要的附加危险因素。本文的目的是分析肺气肿以外吸烟的潜在危险因素，并评估非吸烟者终生的预后。方法：通过丹麦α1-抗胰蛋白酶缺乏症登记册的文件对患者进行了鉴定，该文件包含有关700多名患有此病的人的信息。从家庭研究中鉴定出许多患者，即非索引病例。结果：入院时有75名终生不吸烟者，其PiZ年龄为20岁或以上，分别为27例和48例。在随访期间有21名受试者死亡。标准化死亡率（SMR）为3.0（95％置信区间（CI）为1.9至4.6）。男女之间的SMR没有显着差异。基于五例死亡，指数病例的SMR为8.8（95％CI为5.0至14），非指数病例的SMR为0.96（95％CI为0.3至2.3）。入院时平均一秒内预测的平均强制呼气量（FEV1）的平均百分比为83％，在指数病例（54％）和非指数病例（100％）之间存在显着差异（p <0.001）。 FEV1与强制肺活量（FVC）之比的差异也非常显着，指数和非指数病例分别为0.57和0.79（p <0.001）。在非指数组中，只有三个人的FEV1％预测值低于70％。结论：职业暴露于气道刺激物对肺气肿的发生没有显着影响。一生中只有少数不吸烟者会出现严重的肺气肿。大多数人永远不会出现肺部症状，因此除非筛查索引病例的家庭成员，否则不会被发现。

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