{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,3,26]],"date-time":"2026-03-26T23:17:20Z","timestamp":1774567040081,"version":"3.50.1"},"reference-count":16,"publisher":"BMJ","issue":"4","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Thorax"],"published-print":{"date-parts":[[1995,4,1]]},"DOI":"10.1136\/thx.50.4.424","type":"journal-article","created":{"date-parts":[[2008,12,13]],"date-time":"2008-12-13T03:22:59Z","timestamp":1229138579000},"page":"424-425","source":"Crossref","is-referenced-by-count":17,"title":["Bronchiectasis and homozygous (P1ZZ) alpha 1-antitrypsin deficiency in a young man."],"prefix":"10.1136","volume":"50","author":[{"given":"W","family":"Rodriguez-Cintron","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"K","family":"Guntupalli","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"A E","family":"Fraire","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"239","reference":[{"key":"ref_1","article-title":"Ericksson","year":"1965","unstructured":"Ericksson (1965)6"},{"key":"ref_2","author":"Longstreth","year":"1975","unstructured":"Longstreth (1975)"},{"key":"ref_3","first-page":"1","author":"case, Current","year":"1994","unstructured":"Current case (1994) 1"},{"key":"ref_4","article-title":"Although no clear risk for the development of bronchiectasis in homozygous ol-antitrypsin deficient individuals has ever been established, a few reports have helped to confirm an association between this deficiency and bronchiectasis and pulmonary infections.' It has been suggested that the absence of this protease inhibitor accentuates the damage due to the unopposed protease activity which occurs in bronchitis and its accompanying neutrophilic infiltration of the bronchial wall.34 Repeated bronchopulmonary infection with severe PI, ZZ have been reported.5 It has been postulated that in the axl-antitrypsin-deficient individual repeated episodes of ordinary bronchitis, of whatever cause, may lead to the development of bronchiectasis.4 This suggestion seems to be supported by the study by Ericksson6 in which most of the 35 cases who underwent necropsy had bronchitis, although only two of the 35 had bronchiectasis","unstructured":"Although no clear risk for the development of bronchiectasis in homozygous ol-antitrypsin deficient individuals has ever been established, a few reports have helped to confirm an association between this deficiency and bronchiectasis and pulmonary infections.' It has been suggested that the absence of this protease inhibitor accentuates the damage due to the unopposed protease activity which occurs in bronchitis and its accompanying neutrophilic infiltration of the bronchial wall.34 Repeated bronchopulmonary infection with severe PI,ZZ have been reported.5 It has been postulated that in the axl-antitrypsin-deficient individual repeated episodes of ordinary bronchitis, of whatever cause, may lead to the development of bronchiectasis.4 This suggestion seems to be supported by the study by Ericksson6 in which most of the 35 cases who underwent necropsy had bronchitis, although only two of the 35 had bronchiectasis."},{"key":"ref_5","article-title":"Studies from Sweden indicate that the frequency of the association of oc,-antitrypsin deficiency and bronchiectasis may be as high as 10%.i8 However, the true frequency of bronchiectasis in ocl-antitrypsin-deficient individuals remains to be determined. Conversely, the frequency of ac,-antitrypsin deficiency in series","volume-title":"Although ocl-antitrypsin is the major anti-elastase of the normal lower respiratory tract, other anti-elastases also exist.910 Because ofthe possible protection by anti-elastases other than ocl-antitrypsin that are not measured, the association between bronchiectasis and antielastase deficiency may not be well established","year":"unknown.4","unstructured":"Studies from Sweden indicate that the frequency of the association of oc,-antitrypsin deficiency and bronchiectasis may be as high as 10%.i8 However, the true frequency of bronchiectasis in ocl-antitrypsin-deficient individuals remains to be determined. Conversely, the frequency of ac,-antitrypsin deficiency in series of bronchiectasis (from all causes) is also unknown.4 Although ocl-antitrypsin is the major anti-elastase of the normal lower respiratory tract, other anti-elastases also exist.910 Because ofthe possible protection by anti-elastases other than ocl-antitrypsin that are not measured, the association between bronchiectasis and antielastase deficiency may not be well established."},{"key":"ref_6","doi-asserted-by":"crossref","first-page":"233","DOI":"10.1378\/chest.67.2.233","article-title":"Bronchiectasis and homozygous alpha-l-antitrypsin deficiency","volume":"67","author":"Longstreth, G.; Weitzman, S.A.; Browning, R.J.; Lieberman, J.","year":"1975","unstructured":"Longstreth G, Weitzman SA, Browning RJ, Lieberman J. Bronchiectasis and homozygous alpha-l-antitrypsin deficiency. Chest 1975;67:233-5.","journal-title":"Chest"},{"key":"ref_7","doi-asserted-by":"crossref","first-page":"535","DOI":"10.1378\/chest.71.4.535","article-title":"AlphaI-antitrypsin deficiency with diffuse bronchiectasis and cirrhosis of the liver","volume":"71","author":"Scott, J.; Anderson, C.L.; Shankar, P.S.; Staurides, A.","year":"1977","unstructured":"Scott J, Anderson CL, Shankar PS, Staurides A. AlphaI-antitrypsin deficiency with diffuse bronchiectasis and cirrhosis of the liver. Chest 1977;71:535-8.","journal-title":"Chest"},{"issue":"30","key":"ref_8","first-page":"1","article-title":"Alpha-l-antitrypsin deficiency presenting as bronchiectasis","volume":"79","author":"Jones, D.K.; Gooden, D.; Cavanaugh, P.","year":"1985","unstructured":"Jones DK, Gooden D, Cavanaugh P. Alpha-l-antitrypsin deficiency presenting as bronchiectasis. Br J Dis Chest 1985;79:30 1-4.","journal-title":"Br J Dis Chest"},{"key":"ref_9","doi-asserted-by":"crossref","first-page":"643","DOI":"10.1016\/S0272-5231(21)00589-X","article-title":"Chronic bronchitis: the antiproteinase\/proteinase balance and the effect of infections and corticosteroids","volume":"9","author":"Stockley, R.A.","year":"1988","unstructured":"Stockley RA. Chronic bronchitis: the antiproteinase\/proteinase balance and the effect of infections and corticosteroids. Clin Chest Med 1988;9:643-56.","journal-title":"Clin Chest Med"},{"key":"ref_10","doi-asserted-by":"crossref","first-page":"206","DOI":"10.7326\/0003-4819-111-3-206","article-title":"Anti-neutrophil-elastase defenses of the lower respiratory tract in osl-antitrypsin deficiency directly augmented with an aerosol of ox,-antitrypsin","volume":"111","author":"Hubbard, R.C.; Brantly, M.L.; Sellers, S.E.; Mitchell, M.E.; Crystal, R.G.","year":"1989","unstructured":"Hubbard RC, Brantly ML, Sellers SE, Mitchell ME, Crystal RG. Anti-neutrophil-elastase defenses of the lower respiratory tract in osl-antitrypsin deficiency directly augmented with an aerosol of ox,-antitrypsin. Ann Intern Med 1989;111:206-12.","journal-title":"Ann Intern Med"},{"key":"ref_11","first-page":"1","article-title":"Studies in alpha-l-antitrypsin deficiency","volume":"177","author":"Ericksson, S.","year":"1965","unstructured":"Ericksson S. Studies in alpha-l-antitrypsin deficiency. Acta Med Scand 1965;177:1-85.","journal-title":"Acta Med Scand"},{"key":"ref_12","first-page":"69","article-title":"Pathological findings in alpha-lantitrypsin deficiency","volume-title":"emphysema and proteolysis. New York and","author":"Orell, SR, Mazodier; P.","year":"1971","unstructured":"Orell SR, Mazodier P. Pathological findings in alpha-lantitrypsin deficiency. In: Mittmon C, ed. Pulmonary emphysema and proteolysis. New York and London: Academic Press, 1971:69-89."},{"key":"ref_13","doi-asserted-by":"crossref","first-page":"345","DOI":"10.1111\/j.0954-6820.1978.tb08452.x","article-title":"Natural history and life expectancy in severe alpha-l-antitrypsin deficiency","volume":"204","author":"Larsson, C.","year":"1978","unstructured":"Larsson C. Natural history and life expectancy in severe alpha-l-antitrypsin deficiency. Acta Med Scand 1978;204: 345-51.","journal-title":"Acta Med Scand"},{"key":"ref_14","doi-asserted-by":"crossref","first-page":"889","DOI":"10.1172\/JCI110344","article-title":"Antielastases of the human alveolar structures","volume":"68","author":"Gadek, J.E.; Fells, G.A.; Zimmerman, R.L.; Rennard, S.I.; Crystal, R.G.","year":"1981","unstructured":"Gadek JE, Fells GA, Zimmerman RL, Rennard SI, Crystal RG. Antielastases of the human alveolar structures. J Clin Invest 1981;68:889-98.","journal-title":"J Clin Invest"},{"key":"ref_15","doi-asserted-by":"crossref","first-page":"14","DOI":"10.1016\/0007-0971(83)90002-5","article-title":"Alpha-l-antitrypsin deficiency: the clinical and physiological features of pulmonary emphysema in subjects homozygous for Pi type","volume":"77","author":"Tobin, M.J.; Cook, P.J.L.; Hutchison, D.C.S.","year":"1983","unstructured":"Tobin MJ, Cook PJL, Hutchison DCS. Alpha-l-antitrypsin deficiency: the clinical and physiological features of pulmonary emphysema in subjects homozygous for Pi type Z. BrJ Dis Chest 1983;77:14-27.","journal-title":"Z. BrJ Dis Chest"},{"key":"ref_16","first-page":"441","article-title":"Soskolne CL, Zwi","volume":"111","author":"Kagan, E.","year":"1975","unstructured":"Kagan E, Soskolne CL, Zwi 5, Hurwitz 5, Maier GMG, Ipp T, et al. Immunologic studies in patients with recurrent bronchopulmonary infections. Am Rev Respir Dis 1975; 111:441-51.","journal-title":"Am Rev Respir Dis; Immunologic studies in patients with recurrent bronchopulmonary infections"}],"container-title":["Thorax"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/syndication.highwire.org\/content\/doi\/10.1136\/thx.50.4.424","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2021,9,27]],"date-time":"2021-09-27T19:16:51Z","timestamp":1632770211000},"score":1,"resource":{"primary":{"URL":"https:\/\/thorax.bmj.com\/lookup\/doi\/10.1136\/thx.50.4.424"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[1995,4,1]]},"references-count":16,"journal-issue":{"issue":"4","published-print":{"date-parts":[[1995,4,1]]}},"URL":"https:\/\/doi.org\/10.1136\/thx.50.4.424","relation":{},"ISSN":["0040-6376"],"issn-type":[{"value":"0040-6376","type":"print"}],"subject":[],"published":{"date-parts":[[1995,4,1]]}}}