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The present study was designed to draw conclusions concerning the characteristics of a referral population of 124 patients with \u03b11AT deficiency and symptomatic emphysema. Typically, the \u03b11AT level was 30 mg\/dl, and the \u03b11AT phenotype was almost always PiZZ. The individuals in this population were most often male, caucasian, and ex-smokers, and they had become dyspneic between 25 and 40 yr of age. Most routine blood tests were normal. The chest radiographs and ventilation-perfusion studies typically showed abnormalities with a lower zone distribution, and about one third of the study population had evidence suggestive of pulmonary hypertension. Lung function tests were typical for emphysema; the FEV1 and Dl \u00a0CO were the parameters most dramatically reduced, and the annual rate of decline of those parameters was greater than that of the general population. The cumulative probability of survival of this population indicated a significantly shortened lifespan with a mean survival of 16% at 60 yr of age compared with 85% for normal persons.<\/jats:p>","DOI":"10.1164\/ajrccm\/138.2.327","type":"journal-article","created":{"date-parts":[[2013,4,4]],"date-time":"2013-04-04T16:58:21Z","timestamp":1365094701000},"page":"327-336","source":"Crossref","is-referenced-by-count":235,"title":["Clinical Features and History of the Destructive Lung Disease Associated with Alpha-1-Antitrypsin Deficiency of Adults with Pulmonary Symptoms"],"prefix":"10.1093","volume":"138","author":[{"given":"Mark L.","family":"Brantly","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lester D.","family":"Paul","sequence":"additional","affiliation":[{"name":"From the Pulmonary Branch, National Heart, Lung and Blood Institute; the Diagnostic Radiology Department, Clinical Center, the Population Studies Section, Environmental Epidemiology Branch, National Cancer Institute; and the Division of Epidemiology and Clinical Applications, National Heart, Lung and Blood Institute, National Institutes of Health, Bethesda, Maryland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Barbara H.","family":"Miller","sequence":"additional","affiliation":[{"name":"From the Pulmonary Branch, National Heart, Lung and Blood Institute; the Diagnostic Radiology Department, Clinical Center, the Population Studies Section, Environmental Epidemiology Branch, National Cancer Institute; and the Division of Epidemiology and Clinical Applications, National Heart, Lung and Blood Institute, National Institutes of Health, Bethesda, Maryland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Roni T.","family":"Falk","sequence":"additional","affiliation":[{"name":"From the Pulmonary Branch, National Heart, Lung and Blood Institute; the Diagnostic Radiology Department, Clinical Center, the Population Studies Section, Environmental Epidemiology Branch, National Cancer Institute; and the Division of Epidemiology and Clinical Applications, National Heart, Lung and Blood Institute, National Institutes of Health, Bethesda, Maryland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Margaret","family":"Wu","sequence":"additional","affiliation":[{"name":"From the Pulmonary Branch, National Heart, Lung and Blood Institute; the Diagnostic Radiology Department, Clinical Center, the Population Studies Section, Environmental Epidemiology Branch, National Cancer Institute; and the Division of Epidemiology and Clinical Applications, National Heart, Lung and Blood Institute, National Institutes of Health, Bethesda, Maryland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ronald G.","family":"Crystal","sequence":"additional","affiliation":[{"name":"From the Pulmonary Branch, National Heart, Lung and Blood Institute; the Diagnostic Radiology Department, Clinical Center, the Population Studies Section, Environmental Epidemiology Branch, National Cancer Institute; 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