{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,30]],"date-time":"2026-09-30T10:41:49Z","timestamp":1790764909000,"version":"4.1.0"},"reference-count":118,"publisher":"Elsevier BV","issue":"3","license":[{"start":{"date-parts":[[2016,9,1]],"date-time":"2016-09-01T00:00:00Z","timestamp":1472688000000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/tdm\/userlicense\/1.0\/"},{"start":{"date-parts":[[2016,9,1]],"date-time":"2016-09-01T00:00:00Z","timestamp":1472688000000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/legal\/tdmrep-license"}],"content-domain":{"domain":["clinicalkey.com","clinicalkey.com.au","clinicalkey.es","clinicalkey.fr","clinicalkey.jp","theclinics.com","elsevier.com","sciencedirect.com"],"crossmark-restriction":true},"short-container-title":["Clinics in Chest Medicine"],"published-print":{"date-parts":[[2016,9]]},"DOI":"10.1016\/j.ccm.2016.04.011","type":"journal-article","created":{"date-parts":[[2016,6,26]],"date-time":"2016-06-26T09:58:43Z","timestamp":1466935123000},"page":"487-504","update-policy":"https:\/\/doi.org\/10.1016\/elsevier_cm_policy","source":"Crossref","is-referenced-by-count":28,"title":["\u03b11-Antitrypsin Deficiency"],"prefix":"10.1016","volume":"37","author":[{"given":"Umur","family":"Hatipo\u011flu","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"James K.","family":"Stoller","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"78","reference":[{"issue":"15","key":"10.1016\/j.ccm.2016.04.011_bib1","doi-asserted-by":"crossref","first-page":"132","DOI":"10.1080\/00365516309051324","article-title":"The electrophoretic alpha-1 globulin pattern of serum in alpha-1 antitrypsin deficiency","author":"Laurell","year":"1963","journal-title":"Scand J Clin Lab Invest"},{"issue":"6","key":"10.1016\/j.ccm.2016.04.011_bib2","first-page":"934","article-title":"Cirrhosis associated with alpha-1 antitrypsin deficiency: a previously unrecognized inherited disorder","volume":"73","author":"Sharp","year":"1969","journal-title":"J\u00a0Lab Clin Med"},{"issue":"25","key":"10.1016\/j.ccm.2016.04.011_bib3","doi-asserted-by":"crossref","first-page":"1264","DOI":"10.1056\/NEJM197212212872502","article-title":"Liver disease in adults with alpha-1 antitrypsin deficiency","volume":"287","author":"Berg","year":"1972","journal-title":"N\u00a0Engl J Med"},{"issue":"1","key":"10.1016\/j.ccm.2016.04.011_bib4","doi-asserted-by":"crossref","first-page":"45","DOI":"10.1056\/NEJMra010772","article-title":"Alpha-1 antitrypsin deficiency\u2013a model for conformational diseases","volume":"346","author":"Carrell","year":"2002","journal-title":"N\u00a0Engl J Med"},{"issue":"7","key":"10.1016\/j.ccm.2016.04.011_bib5","doi-asserted-by":"crossref","first-page":"818","DOI":"10.1164\/rccm.168.7.818","article-title":"American Thoracic Society\/European respiratory Society Statement: Standards for the diagnosis and management of individuals with alpha-1 antitrypsin deficiency","volume":"168","author":"American Thoracic Society","year":"2003","journal-title":"Am J Respir Crit Care Med"},{"issue":"12","key":"10.1016\/j.ccm.2016.04.011_bib6","doi-asserted-by":"crossref","first-page":"694","DOI":"10.1056\/NEJM198309223091203","article-title":"Mutation of antitrypsin to antithrombin. Alpha-1 antitrypsin Pittsburgh (358 met leads to arg), a fatal bleeding disorder","volume":"309","author":"Owen","year":"1983","journal-title":"N\u00a0Engl J Med"},{"issue":"24","key":"10.1016\/j.ccm.2016.04.011_bib7","doi-asserted-by":"crossref","first-page":"1316","DOI":"10.1056\/NEJM197606102942404","article-title":"Liver disease in alpha-1 antitrypsin deficiency detected by screening of 200,000 infants","volume":"294","author":"Sveger","year":"1976","journal-title":"N\u00a0Engl J Med"},{"issue":"6","key":"10.1016\/j.ccm.2016.04.011_bib8","doi-asserted-by":"crossref","first-page":"1006","DOI":"10.1016\/S0022-3476(78)80388-6","article-title":"Neonatal screening for alpha-1 antitrypsin deficiency","volume":"92","author":"O'Brien","year":"1978","journal-title":"J\u00a0Pediatr"},{"key":"10.1016\/j.ccm.2016.04.011_bib9","unstructured":"United States Census Bureau. US and world population clock. Available at: http:\/\/www.census.gov\/popclock. Accessed August 26, 2015."},{"issue":"5","key":"10.1016\/j.ccm.2016.04.011_bib10","doi-asserted-by":"crossref","first-page":"1494","DOI":"10.1164\/ajrccm\/140.5.1494","article-title":"Guidelines for the approach to the patient with severe hereditary alpha-1-antitrypsin deficiency. American Thoracic Society","volume":"140","year":"1989","journal-title":"Am Rev Respir Dis"},{"issue":"5","key":"10.1016\/j.ccm.2016.04.011_bib11","doi-asserted-by":"crossref","first-page":"1818","DOI":"10.1378\/chest.122.5.1818","article-title":"Worldwide racial and ethnic distribution of alpha-1 antitrypsin deficiency: summary of an analysis of published genetic epidemiologic surveys","volume":"122","author":"de Serres","year":"2002","journal-title":"Chest"},{"issue":"5","key":"10.1016\/j.ccm.2016.04.011_bib12","doi-asserted-by":"crossref","first-page":"277","DOI":"10.1177\/1753465812457113","article-title":"Prevalence of alpha-1 antitrypsin deficiency alleles PI\u2217S and PI\u2217Z worldwide and effective screening for each of the five phenotypic classes PI\u2217MS, PI\u2217MZ, PI\u2217SS, PI\u2217SZ, and PI\u2217ZZ: a comprehensive review","volume":"6","author":"de Serres","year":"2012","journal-title":"Ther Adv Respir Dis"},{"issue":"19","key":"10.1016\/j.ccm.2016.04.011_bib13","doi-asserted-by":"crossref","first-page":"12627","DOI":"10.1016\/S0021-9258(18)98945-3","article-title":"Siiyama (serine 53 (TCC) to phenylalanine 53 (TTC)). A new alpha-1 antitrypsin-deficient variant with mutation on a predicted conserved residue of the serpin backbone","volume":"266","author":"Seyama","year":"1991","journal-title":"J\u00a0Biol Chem"},{"issue":"2","key":"10.1016\/j.ccm.2016.04.011_bib14","doi-asserted-by":"crossref","first-page":"63","DOI":"10.1177\/1753465810365158","article-title":"Ethnic differences in alpha1 antitrypsin deficiency in the United States of America","volume":"4","author":"de Serres","year":"2010","journal-title":"Ther Adv Respir Dis"},{"issue":"9","key":"10.1016\/j.ccm.2016.04.011_bib15","doi-asserted-by":"crossref","first-page":"1400","DOI":"10.1016\/j.rmed.2013.04.023","article-title":"Alpha-1antitrypsin deficiency - diagnostic testing and disease awareness in Germany and Italy","volume":"107","author":"Greulich","year":"2013","journal-title":"Respir Med"},{"issue":"1","key":"10.1016\/j.ccm.2016.04.011_bib16","doi-asserted-by":"crossref","first-page":"14","DOI":"10.1016\/0007-0971(83)90002-5","article-title":"Alpha-1 antitrypsin deficiency: the clinical and physiological features of pulmonary emphysema in subjects homozygous for Pi type Z. A survey by the British Thoracic Association","volume":"77","author":"Tobin","year":"1983","journal-title":"Br J Dis Chest"},{"issue":"4","key":"10.1016\/j.ccm.2016.04.011_bib17","doi-asserted-by":"crossref","first-page":"961","DOI":"10.1164\/ajrccm\/140.4.961","article-title":"Alpha-1 antitrypsin deficiency. high prevalence in the St. Louis area determined by direct population screening","volume":"140","author":"Silverman","year":"1989","journal-title":"Am Rev Respir Dis"},{"issue":"Suppl 1","key":"10.1016\/j.ccm.2016.04.011_bib18","doi-asserted-by":"crossref","first-page":"36","DOI":"10.3109\/15412555.2015.1021909","article-title":"The clinical profile of subjects included in the Swedish national register on individuals with severe alpha-1 antitrypsin deficiency","volume":"12","author":"Piitulainen","year":"2015","journal-title":"COPD"},{"issue":"Suppl 1","key":"10.1016\/j.ccm.2016.04.011_bib19","doi-asserted-by":"crossref","first-page":"15","DOI":"10.3109\/15412555.2015.1021908","article-title":"Alpha-1 antitrypsin deficiency in Canada: regional disparities in diagnosis and management","volume":"12","author":"Bradi","year":"2015","journal-title":"COPD"},{"issue":"6","key":"10.1016\/j.ccm.2016.04.011_bib20","doi-asserted-by":"crossref","first-page":"461","DOI":"10.3949\/ccjm.61.6.461","article-title":"Physical and social impact of alpha-1 antitrypsin deficiency: results of a survey","volume":"61","author":"Stoller","year":"1994","journal-title":"Cleve Clin J Med"},{"issue":"4","key":"10.1016\/j.ccm.2016.04.011_bib21","doi-asserted-by":"crossref","first-page":"1989","DOI":"10.1378\/chest.128.4.1989","article-title":"Delay in diagnosis of alpha-1 antitrypsin deficiency: a continuing problem","volume":"128","author":"Stoller","year":"2005","journal-title":"Chest"},{"issue":"2 Pt 1","key":"10.1016\/j.ccm.2016.04.011_bib22","doi-asserted-by":"crossref","first-page":"553","DOI":"10.1164\/ajrccm.162.2.9907117","article-title":"Occupational exposure risks in individuals with PI\u2217Z alpha-1 antitrypsin deficiency","volume":"162","author":"Mayer","year":"2000","journal-title":"Am J Respir Crit Care Med"},{"issue":"Suppl 1","key":"10.1016\/j.ccm.2016.04.011_bib23","doi-asserted-by":"crossref","first-page":"26","DOI":"10.3109\/15412555.2013.763782","article-title":"The challenge of detecting alpha-1 antitrypsin deficiency","volume":"10","author":"Stoller","year":"2013","journal-title":"COPD"},{"issue":"8","key":"10.1016\/j.ccm.2016.04.011_bib24","doi-asserted-by":"crossref","first-page":"669","DOI":"10.1136\/thoraxjnl-2011-201321","article-title":"Serum levels and genotype distribution of alpha-1 antitrypsin in the general population","volume":"67","author":"Ferrarotti","year":"2012","journal-title":"Thorax"},{"issue":"4","key":"10.1016\/j.ccm.2016.04.011_bib25","doi-asserted-by":"crossref","first-page":"1000","DOI":"10.1378\/chest.12-0564","article-title":"Alpha-1 antitrypsin phenotypes and associated serum protein concentrations in a large clinical population","volume":"143","author":"Bornhorst","year":"2013","journal-title":"Chest"},{"issue":"5","key":"10.1016\/j.ccm.2016.04.011_bib26","doi-asserted-by":"crossref","first-page":"1158","DOI":"10.1172\/JCI110360","article-title":"Replacement therapy of alpha-1 antitrypsin deficiency. Reversal of protease-antiprotease imbalance within the alveolar structures of PiZ subjects","volume":"68","author":"Gadek","year":"1981","journal-title":"J\u00a0Clin Invest"},{"issue":"12","key":"10.1016\/j.ccm.2016.04.011_bib27","doi-asserted-by":"crossref","first-page":"736","DOI":"10.1056\/NEJM198603203141202","article-title":"Risk of cirrhosis and primary liver cancer in alpha-1 antitrypsin deficiency","volume":"314","author":"Eriksson","year":"1986","journal-title":"N\u00a0Engl J Med"},{"issue":"5","key":"10.1016\/j.ccm.2016.04.011_bib28","doi-asserted-by":"crossref","first-page":"461","DOI":"10.1111\/j.0954-6820.1987.tb01281.x","article-title":"Alpha-1 antitrypsin deficiency and liver cirrhosis in adults. An analysis of 35 Swedish autopsied cases","volume":"221","author":"Eriksson","year":"1987","journal-title":"Acta Med Scand"},{"issue":"11","key":"10.1016\/j.ccm.2016.04.011_bib29","doi-asserted-by":"crossref","first-page":"1585","DOI":"10.1172\/JCI0216782","article-title":"Alpha-1 antitrypsin polymerization and the serpinopathies: pathobiology and prospects for therapy","volume":"110","author":"Lomas","year":"2002","journal-title":"J\u00a0Clin Invest"},{"issue":"43","key":"10.1016\/j.ccm.2016.04.011_bib30","doi-asserted-by":"crossref","first-page":"33663","DOI":"10.1074\/jbc.M004054200","article-title":"Pathogenic alpha-1 antitrypsin polymers are formed by reactive loop-beta-sheet A linkage","volume":"275","author":"Sivasothy","year":"2000","journal-title":"J\u00a0Biol Chem"},{"issue":"8","key":"10.1016\/j.ccm.2016.04.011_bib31","doi-asserted-by":"crossref","first-page":"909","DOI":"10.1164\/rccm.201308-1458OC","article-title":"Z\u00a0alpha-1 antitrypsin confers a proinflammatory phenotype that contributes to chronic obstructive pulmonary disease","volume":"189","author":"Alam","year":"2014","journal-title":"Am J Respir Crit Care Med"},{"issue":"2","key":"10.1016\/j.ccm.2016.04.011_bib32","doi-asserted-by":"crossref","first-page":"394","DOI":"10.1378\/chest.111.2.394","article-title":"Baseline characteristics of enrollees in the national heart, lung and blood institute registry of alpha-1 antitrypsin deficiency. Alpha-1 antitrypsin deficiency registry study group","volume":"111","author":"McElvaney","year":"1997","journal-title":"Chest"},{"issue":"8421","key":"10.1016\/j.ccm.2016.04.011_bib33","doi-asserted-by":"crossref","first-page":"152","DOI":"10.1016\/S0140-6736(85)91916-6","article-title":"Smoking, lung function, and alpha-1 antitrypsin deficiency","volume":"1","author":"Janus","year":"1985","journal-title":"Lancet"},{"issue":"5","key":"10.1016\/j.ccm.2016.04.011_bib34","first-page":"945","article-title":"Clinical, epidemiologic, and pulmonary function studies in alpha-1 antitrypsin-deficient subjects of Pi Z type","volume":"114","author":"Rawlings","year":"1976","journal-title":"Am Rev Respir Dis"},{"issue":"2","key":"10.1016\/j.ccm.2016.04.011_bib35","doi-asserted-by":"crossref","first-page":"162","DOI":"10.3109\/15412555.2014.922068","article-title":"Lung function and CT densitometry in subjects with alpha-1 antitrypsin deficiency and healthy controls at 35 years of age","volume":"12","author":"Piitulainen","year":"2015","journal-title":"COPD"},{"issue":"11","key":"10.1016\/j.ccm.2016.04.011_bib36","doi-asserted-by":"crossref","first-page":"1172","DOI":"10.1164\/rccm.200406-761OC","article-title":"Pattern of emphysema distribution in alpha-1 antitrypsin deficiency influences lung function impairment","volume":"170","author":"Parr","year":"2004","journal-title":"Am J Respir Crit Care Med"},{"issue":"1","key":"10.1016\/j.ccm.2016.04.011_bib37","doi-asserted-by":"crossref","first-page":"49","DOI":"10.1164\/ajrccm.158.1.9712017","article-title":"Survival and FEV1 decline in individuals with severe deficiency of \u03b11-antitrypsin","volume":"158","author":"The Alpha-1 Antitrypsin Deficiency Registry Study Group","year":"1998","journal-title":"Am J Respir Crit Care Med"},{"issue":"2","key":"10.1016\/j.ccm.2016.04.011_bib38","doi-asserted-by":"crossref","first-page":"247","DOI":"10.1183\/09031936.99.13224799","article-title":"Decline in FEV1 related to smoking status in individuals with severe alpha-1 antitrypsin deficiency (PiZZ)","volume":"13","author":"Piitulainen","year":"1999","journal-title":"Eur Respir J"},{"issue":"6","key":"10.1016\/j.ccm.2016.04.011_bib39","doi-asserted-by":"crossref","first-page":"1338","DOI":"10.1183\/09031936.00061208","article-title":"Rate of progression of lung function impairment in alpha-1 antitrypsin deficiency","volume":"33","author":"Dawkins","year":"2009","journal-title":"Eur Respir J"},{"issue":"3","key":"10.1016\/j.ccm.2016.04.011_bib40","doi-asserted-by":"crossref","first-page":"387","DOI":"10.1016\/j.rmed.2012.12.003","article-title":"Age related development of respiratory abnormalities in non-index alpha-1 antitrypsin deficient studies","volume":"107","author":"Holme","year":"2013","journal-title":"Respir Med"},{"issue":"12","key":"10.1016\/j.ccm.2016.04.011_bib41","doi-asserted-by":"crossref","first-page":"1091","DOI":"10.1136\/thx.2008.095497","article-title":"Clinical course and prognosis of never-smokers with severe alpha-1 antitrypsin deficiency (PiZZ)","volume":"63","author":"Tanash","year":"2008","journal-title":"Thorax"},{"issue":"8","key":"10.1016\/j.ccm.2016.04.011_bib42","doi-asserted-by":"crossref","first-page":"556","DOI":"10.1136\/oem.2009.047589","article-title":"Outdoor air pollution is associated with rapid decline of lung function in alpha-1 antitrypsin deficiency","volume":"67","author":"Wood","year":"2010","journal-title":"Occup Environ Med"},{"issue":"5","key":"10.1016\/j.ccm.2016.04.011_bib43","doi-asserted-by":"crossref","first-page":"1116","DOI":"10.1378\/chest.10-0187","article-title":"Accelerated spirometric decline in New York City firefighters with alpha-1 antitrypsin deficiency","volume":"138","author":"Banauch","year":"2010","journal-title":"Chest"},{"key":"10.1016\/j.ccm.2016.04.011_bib44","doi-asserted-by":"crossref","first-page":"44","DOI":"10.1186\/1465-9921-11-44","article-title":"Survival in severe alpha-1 antitrypsin deficiency (PiZZ)","volume":"11","author":"Tanash","year":"2010","journal-title":"Respir Res"},{"issue":"4","key":"10.1016\/j.ccm.2016.04.011_bib45","doi-asserted-by":"crossref","first-page":"419","DOI":"10.1164\/rccm.201311-1984OC","article-title":"Clarification of the risk of chronic obstructive pulmonary disease in alpha-1 antitrypsin deficiency PiMZ heterozygotes","volume":"189","author":"Molloy","year":"2014","journal-title":"Am J Respir Crit Care Med"},{"issue":"5","key":"10.1016\/j.ccm.2016.04.011_bib46","doi-asserted-by":"crossref","first-page":"1125","DOI":"10.1378\/chest.10-0746","article-title":"Alpha-1) antitrypsin protease inhibitor MZ heterozygosity is associated with airflow obstruction in two large cohorts","volume":"138","author":"Sorheim","year":"2010","journal-title":"Chest"},{"issue":"12","key":"10.1016\/j.ccm.2016.04.011_bib47","doi-asserted-by":"crossref","first-page":"1215","DOI":"10.1164\/rccm.200703-489OC","article-title":"Prevalence and impact of bronchiectasis in alpha1-antitrypsin deficiency","volume":"176","author":"Parr","year":"2007","journal-title":"Am J Respir Crit Care Med"},{"issue":"2","key":"10.1016\/j.ccm.2016.04.011_bib48","doi-asserted-by":"crossref","first-page":"415","DOI":"10.1378\/chest.117.2.415","article-title":"Distribution of alpha(1)-antitrypsin alleles in patients with bronchiectasis","volume":"117","author":"Cuvelier","year":"2000","journal-title":"Chest"},{"issue":"6","key":"10.1016\/j.ccm.2016.04.011_bib49","first-page":"1504","article-title":"Molecular pathogenesis of liver disease in alpha-1antitrypsin deficiency","volume":"24","author":"Teckman","year":"1996","journal-title":"Hepatology"},{"issue":"6","key":"10.1016\/j.ccm.2016.04.011_bib50","doi-asserted-by":"crossref","first-page":"847","DOI":"10.1111\/j.1651-2227.1988.tb10767.x","article-title":"The natural history of liver disease in alpha-1 antitrypsin deficient children","volume":"77","author":"Sveger","year":"1988","journal-title":"Acta Paediatr Scand"},{"issue":"10","key":"10.1016\/j.ccm.2016.04.011_bib51","doi-asserted-by":"crossref","first-page":"1427","DOI":"10.4254\/wjh.v7.i10.1427","article-title":"Alpha-1 antitrypsin deficiency and the risk of hepatocellular carcinoma in end-stage liver disease","volume":"7","author":"Antoury","year":"2015","journal-title":"World J Hepatol"},{"issue":"12","key":"10.1016\/j.ccm.2016.04.011_bib52","doi-asserted-by":"crossref","first-page":"2668","DOI":"10.1002\/1097-0142(20000615)88:12<2668::AID-CNCR4>3.0.CO;2-G","article-title":"Is heterozygous alpha-1 antitrypsin deficiency type PIZ a risk factor for primary liver carcinoma?","volume":"88","author":"Zhou","year":"2000","journal-title":"Cancer"},{"issue":"5","key":"10.1016\/j.ccm.2016.04.011_bib53","doi-asserted-by":"crossref","first-page":"502","DOI":"10.1164\/rccm.201204-0739OC","article-title":"Prevalence and risk factors for liver involvement in individuals with PiZZ-related lung disease","volume":"187","author":"Dawwas","year":"2013","journal-title":"Am J Respir Crit Care Med"},{"issue":"11","key":"10.1016\/j.ccm.2016.04.011_bib54","doi-asserted-by":"crossref","first-page":"1278","DOI":"10.1016\/j.cgh.2012.07.007","article-title":"Liver test results do not identify liver disease in adults with alpha\u20141 antitrypsin deficiency","volume":"10","author":"Clark","year":"2012","journal-title":"Clin Gastroenterol Hepatol"},{"issue":"6","key":"10.1016\/j.ccm.2016.04.011_bib55","doi-asserted-by":"crossref","first-page":"1329","DOI":"10.1038\/ki.1993.186","article-title":"Alpha-1 antitrypsin genetic polymorphism in ANCA-positive systemic vasculitis","volume":"43","author":"Esnault","year":"1993","journal-title":"Kidney Int"},{"key":"10.1016\/j.ccm.2016.04.011_bib56","doi-asserted-by":"crossref","first-page":"331","DOI":"10.1007\/978-1-4757-9182-2_54","article-title":"Alpha-1 proteinase inhibitor and pulmonary haemorrhage in systemic vasculitis","volume":"336","author":"O'Donoghue","year":"1993","journal-title":"Adv Exp Med Biol"},{"issue":"12","key":"10.1016\/j.ccm.2016.04.011_bib57","doi-asserted-by":"crossref","first-page":"3760","DOI":"10.1002\/art.27742","article-title":"Alpha\u20141 antitrypsin deficiency-related alleles Z and S and the risk of Wegener's granulomatosis","volume":"62","author":"Mahr","year":"2010","journal-title":"Arthritis Rheum"},{"issue":"3","key":"10.1016\/j.ccm.2016.04.011_bib58","doi-asserted-by":"crossref","first-page":"844","DOI":"10.1038\/ki.1995.360","article-title":"The PiZ gene of alpha-1 antitrypsin as a determinant of outcome in PR3-ANCA-positive vasculitis","volume":"48","author":"Segelmark","year":"1995","journal-title":"Kidney Int"},{"issue":"4","key":"10.1016\/j.ccm.2016.04.011_bib59","doi-asserted-by":"crossref","first-page":"553","DOI":"10.1007\/s00296-013-2745-9","article-title":"Alpha-1 antitrypsin activity is markedly decreased in Wegener's granulomatosis","volume":"34","author":"Mota","year":"2014","journal-title":"Rheumatol Int"},{"issue":"10","key":"10.1016\/j.ccm.2016.04.011_bib60","first-page":"877","article-title":"Weber-Christian syndrome associated with an alpha-1 antitrypsin deficiency. Familial investigation","volume":"123","author":"Warter","year":"1972","journal-title":"Ann Med Interne (Paris)"},{"issue":"4","key":"10.1016\/j.ccm.2016.04.011_bib61","doi-asserted-by":"crossref","first-page":"645","DOI":"10.1016\/j.jaad.2003.12.053","article-title":"Alpha-1 antitrypsin associated panniculitis: the MS variant","volume":"51","author":"Geraminejad","year":"2004","journal-title":"J\u00a0Am Acad Dermatol"},{"issue":"12","key":"10.1016\/j.ccm.2016.04.011_bib62","doi-asserted-by":"crossref","first-page":"1655","DOI":"10.1001\/archderm.1987.01660360083017","article-title":"Panniculitis associated with severe alpha-1 antitrypsin deficiency. Treatment and review of the literature","volume":"123","author":"Smith","year":"1987","journal-title":"Arch Dermatol"},{"issue":"6","key":"10.1016\/j.ccm.2016.04.011_bib63","doi-asserted-by":"crossref","first-page":"1192","DOI":"10.1016\/S0190-9622(89)70328-5","article-title":"Clinical and pathologic correlations in 96 patients with panniculitis, including 15 patients with deficient levels of alpha-1 antitrypsin","volume":"21","author":"Smith","year":"1989","journal-title":"J\u00a0Am Acad Dermatol"},{"issue":"6","key":"10.1016\/j.ccm.2016.04.011_bib64","doi-asserted-by":"crossref","first-page":"1258","DOI":"10.1046\/j.1365-2133.2002.05095.x","article-title":"Severe panniculitis caused by homozygous ZZ alpha-1 antitrypsin deficiency treated successfully with human purified enzyme (prolastin)","volume":"147","author":"Chowdhury","year":"2002","journal-title":"Br J Dermatol"},{"issue":"3","key":"10.1016\/j.ccm.2016.04.011_bib65","doi-asserted-by":"crossref","first-page":"480","DOI":"10.1097\/00007890-199702150-00028","article-title":"Alpha-1 antitrypsin deficiency-associated panniculitis: Resolution with intravenous alpha-1 antitrypsin administration and liver transplantation","volume":"63","author":"O'Riordan","year":"1997","journal-title":"Transplantation"},{"issue":"13","key":"10.1016\/j.ccm.2016.04.011_bib66","doi-asserted-by":"crossref","first-page":"988","DOI":"10.1056\/NEJMcp012477","article-title":"Clinical practice. acute exacerbations of chronic obstructive pulmonary disease","volume":"346","author":"Stoller","year":"2002","journal-title":"N\u00a0Engl J Med"},{"issue":"26","key":"10.1016\/j.ccm.2016.04.011_bib67","doi-asserted-by":"crossref","first-page":"2689","DOI":"10.1056\/NEJMra030415","article-title":"Management of chronic obstructive pulmonary disease","volume":"350","author":"Sutherland","year":"2004","journal-title":"N\u00a0Engl J Med"},{"issue":"9823","key":"10.1016\/j.ccm.2016.04.011_bib68","doi-asserted-by":"crossref","first-page":"1341","DOI":"10.1016\/S0140-6736(11)60968-9","article-title":"Chronic obstructive pulmonary disease","volume":"379","author":"Decramer","year":"2012","journal-title":"Lancet"},{"issue":"21","key":"10.1016\/j.ccm.2016.04.011_bib69","doi-asserted-by":"crossref","first-page":"2059","DOI":"10.1056\/NEJMoa030287","article-title":"A\u00a0randomized trial comparing lung-volume-reduction surgery with medical therapy for severe emphysema","volume":"348","author":"Fishman","year":"2003","journal-title":"N\u00a0Engl J Med"},{"issue":"10","key":"10.1016\/j.ccm.2016.04.011_bib70","doi-asserted-by":"crossref","first-page":"2260","DOI":"10.1183\/09031936.97.10102260","article-title":"Does alpha-1 antitrypsin augmentation therapy slow the annual decline in FEV1 in patients with severe hereditary alpha-1 antitrypsin deficiency? Wissenschaftliche Arbeitsgemeinschaft zur Therapie von Lungenerkrankungen (WATL) Alpha1-AT Study Group","volume":"10","author":"Seersholm","year":"1997","journal-title":"Eur Respir J"},{"issue":"3","key":"10.1016\/j.ccm.2016.04.011_bib71","doi-asserted-by":"crossref","first-page":"737","DOI":"10.1378\/chest.119.3.737","article-title":"Wissenschaftliche Arbeitsgemeinschaft zur Therapie von Lungenerkrankungen. Longitudinal follow-up of patients with alpha-1 protease inhibitor deficiency before and during therapy with IV alpha-1 protease inhibitor","volume":"119","author":"Wencker","year":"2001","journal-title":"Chest"},{"key":"10.1016\/j.ccm.2016.04.011_bib72","doi-asserted-by":"crossref","first-page":"443","DOI":"10.2147\/COPD.S8577","article-title":"Alpha-1 antitrypsin augmentation therapy in deficient individuals enrolled in the Alpha-1 Foundation DNA and Tissue Bank","volume":"4","author":"Tonelli","year":"2009","journal-title":"Int J Chron Obstruct Pulmon Dis"},{"issue":"2","key":"10.1016\/j.ccm.2016.04.011_bib73","doi-asserted-by":"crossref","first-page":"76","DOI":"10.1159\/000196395","article-title":"Preliminary evidence that augmentation therapy diminishes degradation of cross-linked elastin in alpha-1 antitrypsin-deficient humans","volume":"62","author":"Stone","year":"1995","journal-title":"Respiration"},{"issue":"5 Pt 1","key":"10.1016\/j.ccm.2016.04.011_bib74","doi-asserted-by":"crossref","first-page":"1468","DOI":"10.1164\/ajrccm.160.5.9901055","article-title":"A\u00a0randomized clinical trial of Alpha\u20141 antitrypsin augmentation therapy","volume":"160","author":"Dirksen","year":"1999","journal-title":"Am J Respir Crit Care Med"},{"issue":"6","key":"10.1016\/j.ccm.2016.04.011_bib75","doi-asserted-by":"crossref","first-page":"2069","DOI":"10.1164\/ajrccm.162.6.2002032","article-title":"Short-term\u00a0supplementation therapy does not affect elastin degradation in severe alpha(1)-antitrypsin deficiency. The American-Italian AATD Study Group","volume":"162","author":"Gottlieb","year":"2000","journal-title":"Am J Respir Crit Care Med"},{"issue":"5","key":"10.1016\/j.ccm.2016.04.011_bib76","doi-asserted-by":"crossref","first-page":"1480","DOI":"10.1378\/chest.118.5.1480","article-title":"Augmentation therapy reduces frequency of lung infections in antitrypsin deficiency: a new hypothesis with supporting data","volume":"118","author":"Lieberman","year":"2000","journal-title":"Chest"},{"issue":"11","key":"10.1016\/j.ccm.2016.04.011_bib77","doi-asserted-by":"crossref","first-page":"1494","DOI":"10.1164\/rccm.2109013","article-title":"The effect of augmentation therapy on bronchial inflammation in alpha-1 antitrypsin deficiency","volume":"165","author":"Stockley","year":"2002","journal-title":"Am J Respir Crit Care Med"},{"issue":"6","key":"10.1016\/j.ccm.2016.04.011_bib78","doi-asserted-by":"crossref","first-page":"1345","DOI":"10.1183\/09031936.00159408","article-title":"Exploring the role of CT densitometry: a randomised study of augmentation therapy in alpha-1 antitrypsin deficiency","volume":"33","author":"Dirksen","year":"2009","journal-title":"Eur Respir J"},{"key":"10.1016\/j.ccm.2016.04.011_bib79","first-page":"687","article-title":"The effects of weekly augmentation therapy in patients with PiZZ alpha-1 antitrypsin deficiency","volume":"7","author":"Schmid","year":"2012","journal-title":"Int J Chron Obstruct Pulmon Dis"},{"issue":"2","key":"10.1016\/j.ccm.2016.04.011_bib80","doi-asserted-by":"crossref","first-page":"67","DOI":"10.1177\/1753465812438387","article-title":"Investigators of the rEXA study group. Reduction of severe exacerbations and hospitalization-derived costs in alpha-1-antitrypsin-deficient patients treated with alpha-1 antitrypsin augmentation therapy","volume":"6","author":"Barros-Tiz\u00f3n","year":"2012","journal-title":"Ther Adv Respir Dis"},{"issue":"4","key":"10.1016\/j.ccm.2016.04.011_bib81","doi-asserted-by":"crossref","first-page":"473","DOI":"10.3109\/15412555.2013.771163","article-title":"Alpha-1 antitrypsin augmentation therapy and biomarkers of elastin degradation","volume":"10","author":"Ma","year":"2013","journal-title":"COPD"},{"issue":"9991","key":"10.1016\/j.ccm.2016.04.011_bib82","doi-asserted-by":"crossref","first-page":"360","DOI":"10.1016\/S0140-6736(15)60860-1","article-title":"Intravenous augmentation treatment and lung density in severe alpha-1 antitrypsin deficiency (RAPID): a randomised, double-blind, placebo-controlled trial","volume":"386","author":"Chapman","year":"2015","journal-title":"Lancet"},{"issue":"6A","key":"10.1016\/j.ccm.2016.04.011_bib83","doi-asserted-by":"crossref","first-page":"52","DOI":"10.1016\/S0002-9343(88)80071-8","article-title":"Alpha-1 antitrypsin augmentation therapy for alpha-1 antitrypsin deficiency","volume":"84","author":"Hubbard","year":"1988","journal-title":"Am J Med"},{"issue":"3","key":"10.1016\/j.ccm.2016.04.011_bib84","doi-asserted-by":"crossref","first-page":"607","DOI":"10.1378\/chest.112.3.607","article-title":"Pharmacokinetic study of alpha-1antitrypsin infusion in alpha1-antitrypsin deficiency","volume":"112","author":"Barker","year":"1997","journal-title":"Chest"},{"issue":"6","key":"10.1016\/j.ccm.2016.04.011_bib85","doi-asserted-by":"crossref","first-page":"687","DOI":"10.3109\/15412555.2013.800852","article-title":"Safety and pharmacokinetics of 120 mg\/kg versus 60 mg\/kg weekly intravenous infusions of alpha-1 proteinase inhibitor in alpha-1 antitrypsin deficiency: a multicenter, randomized, double-blind, crossover study (SPARK)","volume":"10","author":"Campos","year":"2013","journal-title":"COPD"},{"issue":"4","key":"10.1016\/j.ccm.2016.04.011_bib86","doi-asserted-by":"crossref","first-page":"831","DOI":"10.1378\/chest.08-0868","article-title":"Alpha-1 antitrypsin augmentation therapy for PI\u2217MZ heterozygotes: a cautionary note","volume":"134","author":"Sandhaus","year":"2008","journal-title":"Chest"},{"issue":"6 Pt 1","key":"10.1016\/j.ccm.2016.04.011_bib87","doi-asserted-by":"crossref","first-page":"1718","DOI":"10.1164\/ajrccm.154.6.8970361","article-title":"Clinical features of individuals with PI\u2217SZ phenotype of alpha-1 antitrypsin deficiency. Alpha-1 Antitrypsin Deficiency Registry Study Group","volume":"154","author":"Turino","year":"1996","journal-title":"Am J Respir Crit Care Med"},{"issue":"2","key":"10.1016\/j.ccm.2016.04.011_bib88","doi-asserted-by":"crossref","first-page":"428","DOI":"10.1183\/09031936.98.11020428","article-title":"Long-term treatment of alpha-1antitrypsin deficiency-related pulmonary emphysema with human alpha-1 antitrypsin. Wissenschaftliche Arbeitsgemeinschaft zur Therapie von Lungenerkrankungen (WATL) Alpha1-AT Study Group","volume":"11","author":"Wencker","year":"1998","journal-title":"Eur Respir J"},{"issue":"10","key":"10.1016\/j.ccm.2016.04.011_bib89","doi-asserted-by":"crossref","first-page":"1387","DOI":"10.1164\/rccm.200209-1035OC","article-title":"Cost-effectiveness analysis of augmentation therapy for severe alpha-1 antitrypsin deficiency","volume":"167","author":"Gildea","year":"2003","journal-title":"Am J Respir Crit Care Med"},{"issue":"2","key":"10.1016\/j.ccm.2016.04.011_bib90","first-page":"109","article-title":"Alpha-1 antitrypsin deficiency targeted testing and augmentation therapy: a Canadian Thoracic Society clinical practice guideline","volume":"19","author":"Marciniuk","year":"2012","journal-title":"Can Respir J"},{"issue":"10","key":"10.1016\/j.ccm.2016.04.011_bib91","doi-asserted-by":"crossref","first-page":"1104","DOI":"10.1016\/j.healun.2011.08.004","article-title":"The registry of the International Society for Heart and Lung Transplantation: twenty-eighth adult lung and heart-lung transplant report\u20132011","volume":"30","author":"Christie","year":"2011","journal-title":"J\u00a0Heart Lung Transplant"},{"issue":"5","key":"10.1016\/j.ccm.2016.04.011_bib92","doi-asserted-by":"crossref","first-page":"1663","DOI":"10.1016\/S0003-4975(02)04064-X","article-title":"Thirteen-year experience in lung transplantation for emphysema","volume":"74","author":"Cassivi","year":"2002","journal-title":"Ann Thorac Surg"},{"issue":"11","key":"10.1016\/j.ccm.2016.04.011_bib93","doi-asserted-by":"crossref","first-page":"1834","DOI":"10.1016\/j.healun.2005.03.001","article-title":"The Copenhagen National Lung Transplant Group: survival after single lung, double lung, and heart-lung transplantation","volume":"24","author":"Burton","year":"2005","journal-title":"J\u00a0Heart Lung Transplant"},{"issue":"6","key":"10.1016\/j.ccm.2016.04.011_bib94","doi-asserted-by":"crossref","first-page":"906","DOI":"10.1093\/ejcts\/ezu550","article-title":"Lung transplantation for emphysema: impact of age on short- and long-term survival","volume":"48","author":"Inci","year":"2015","journal-title":"Eur J Cardiothorac Surg"},{"issue":"5","key":"10.1016\/j.ccm.2016.04.011_bib95","doi-asserted-by":"crossref","first-page":"1493","DOI":"10.1016\/j.jtcvs.2003.11.047","article-title":"Twenty-year experience of lung transplantation at a single center: Influence of recipient diagnosis on long-term survival","volume":"127","author":"de Perrot","year":"2004","journal-title":"J\u00a0Thorac Cardiovasc Surg"},{"issue":"6","key":"10.1016\/j.ccm.2016.04.011_bib96","doi-asserted-by":"crossref","first-page":"1930","DOI":"10.1016\/j.athoracsur.2014.07.030","article-title":"Survival benefit of lung transplantation for chronic obstructive pulmonary disease in Sweden","volume":"98","author":"Tanash","year":"2014","journal-title":"Ann Thorac Surg"},{"issue":"12","key":"10.1016\/j.ccm.2016.04.011_bib97","doi-asserted-by":"crossref","first-page":"1342","DOI":"10.1016\/j.healun.2011.07.003","article-title":"Survival benefit of lung transplantation in individuals with severe alpha1-anti-trypsin deficiency (PiZZ) and emphysema","volume":"30","author":"Tanash","year":"2011","journal-title":"J\u00a0Heart Lung Transplant"},{"issue":"3","key":"10.1016\/j.ccm.2016.04.011_bib98","doi-asserted-by":"crossref","first-page":"274","DOI":"10.1164\/rccm.201401-0031OC","article-title":"The natural history of lung function after lung transplantation for alpha\u20141 antitrypsin deficiency","volume":"190","author":"Banga","year":"2014","journal-title":"Am J Respir Crit Care Med"},{"issue":"1","key":"10.1016\/j.ccm.2016.04.011_bib99","doi-asserted-by":"crossref","first-page":"241","DOI":"10.1016\/j.athoracsur.2006.07.080","article-title":"National Emphysema Treatment Trial Research Group. Lung volume reduction surgery in patients with emphysema and alpha-1 antitrypsin deficiency","volume":"83","author":"Stoller","year":"2007","journal-title":"Ann Thorac Surg"},{"issue":"5","key":"10.1016\/j.ccm.2016.04.011_bib100","doi-asserted-by":"crossref","first-page":"1028","DOI":"10.1183\/09031936.98.12051028","article-title":"Two-year results after lung volume reduction surgery in alpha-1 antitrypsin deficiency versus smoker's emphysema","volume":"12","author":"Cassina","year":"1998","journal-title":"Eur Respir J"},{"issue":"4","key":"10.1016\/j.ccm.2016.04.011_bib101","doi-asserted-by":"crossref","first-page":"928","DOI":"10.1034\/j.1399-3003.1999.14d33.x","article-title":"Lung function after bilateral lower lobe lung volume reduction surgery for alpha-1 antitrypsin emphysema","volume":"14","author":"Gelb","year":"1999","journal-title":"Eur Respir J"},{"issue":"3","key":"10.1016\/j.ccm.2016.04.011_bib102","doi-asserted-by":"crossref","first-page":"408","DOI":"10.1016\/j.jtcvs.2004.03.040","article-title":"Long-term results after lung volume reduction surgery in patients with alpha-1 antitrypsin deficiency","volume":"128","author":"Tutic","year":"2004","journal-title":"J\u00a0Thorac Cardiovasc Surg"},{"issue":"2","key":"10.1016\/j.ccm.2016.04.011_bib103","first-page":"201","article-title":"Functional results of unilateral lung volume reduction surgery in alpha-1 antitrypsin deficient patients","volume":"1","author":"Dauriat","year":"2006","journal-title":"Int J Chron Obstruct Pulmon Dis"},{"issue":"2","key":"10.1016\/j.ccm.2016.04.011_bib104","doi-asserted-by":"crossref","first-page":"201","DOI":"10.1016\/j.thorsurg.2009.02.002","article-title":"Lung volume reduction surgery for patients with alpha-1 antitrypsin deficiency emphysema","volume":"19","author":"Donahue","year":"2009","journal-title":"Thorac Surg Clin"},{"issue":"1","key":"10.1016\/j.ccm.2016.04.011_bib105","doi-asserted-by":"crossref","first-page":"45","DOI":"10.1111\/j.1752-699X.2012.00280.x","article-title":"Endobronchial valve deployment in severe alpha-1 antitrypsin deficiency emphysema: a case series","volume":"7","author":"Tuohy","year":"2013","journal-title":"Clin Respir J"},{"issue":"4","key":"10.1016\/j.ccm.2016.04.011_bib106","doi-asserted-by":"crossref","first-page":"320","DOI":"10.1159\/000365662","article-title":"One- to four-year follow-up of endobronchial lung volume reduction in alpha-1 antitrypsin deficiency patients: a case series","volume":"88","author":"Hillerdal","year":"2014","journal-title":"Respiration"},{"issue":"38","key":"10.1016\/j.ccm.2016.04.011_bib107","doi-asserted-by":"crossref","first-page":"16363","DOI":"10.1073\/pnas.0904514106","article-title":"Sustained transgene expression despite T lymphocyte responses in a clinical trial of rAAV1-AAT gene therapy","volume":"106","author":"Brantly","year":"2009","journal-title":"Proc Natl Acad Sci U S A"},{"issue":"6","key":"10.1016\/j.ccm.2016.04.011_bib108","doi-asserted-by":"crossref","first-page":"1143","DOI":"10.1203\/01.pdr.0000189226.03684.fe","article-title":"Adeno-associated virus-based gene therapy for inherited disorders","volume":"58","author":"Flotte","year":"2005","journal-title":"Pediatr Res"},{"issue":"2","key":"10.1016\/j.ccm.2016.04.011_bib109","doi-asserted-by":"crossref","first-page":"133","DOI":"10.1165\/rcmb.2003-0276OC","article-title":"Inhibiting polymerization: new therapeutic strategies for Z alpha-1 antitrypsin-related emphysema","volume":"31","author":"Parfrey","year":"2004","journal-title":"Am J Respir Cell Mol Biol"},{"issue":"3","key":"10.1016\/j.ccm.2016.04.011_bib110","doi-asserted-by":"crossref","first-page":"931","DOI":"10.1016\/j.jmb.2004.07.078","article-title":"How small peptides block and reverse serpin polymerisation","volume":"342","author":"Zhou","year":"2004","journal-title":"J\u00a0Mol Biol"},{"issue":"4","key":"10.1016\/j.ccm.2016.04.011_bib111","doi-asserted-by":"crossref","first-page":"1796","DOI":"10.1073\/pnas.97.4.1796","article-title":"Chemical chaperones mediate increased secretion of mutant alpha-1 antitrypsin (alpha-1 AT) Z: a potential pharmacological strategy for prevention of liver injury and emphysema in alpha-1 AT deficiency","volume":"97","author":"Burrows","year":"2000","journal-title":"Proc Natl Acad Sci U S A"},{"issue":"3","key":"10.1016\/j.ccm.2016.04.011_bib112","doi-asserted-by":"crossref","first-page":"1987","DOI":"10.1074\/jbc.275.3.1987","article-title":"Glucosidase and mannosidase inhibitors mediate increased secretion of mutant alpha-1 antitrypsin Z","volume":"275","author":"Marcus","year":"2000","journal-title":"J\u00a0Biol Chem"},{"issue":"6","key":"10.1016\/j.ccm.2016.04.011_bib113","doi-asserted-by":"crossref","first-page":"659","DOI":"10.1165\/rcmb.4866","article-title":"Polyethylene glycol conjugation at Cys232 prolongs the half-life of alpha-1 proteinase inhibitor","volume":"27","author":"Cantin","year":"2002","journal-title":"Am J Respir Cell Mol Biol"},{"issue":"5988","key":"10.1016\/j.ccm.2016.04.011_bib114","doi-asserted-by":"crossref","first-page":"229","DOI":"10.1126\/science.1190354","article-title":"An autophagy-enhancing drug promotes degradation of mutant alpha-1 antitrypsin Z and reduces hepatic fibrosis","volume":"329","author":"Hidvegi","year":"2010","journal-title":"Science"},{"issue":"6","key":"10.1016\/j.ccm.2016.04.011_bib115","doi-asserted-by":"crossref","first-page":"700","DOI":"10.1258\/ebm.2010.009297","article-title":"Rapamycin reduces intrahepatic alpha-1 antitrypsin mutant Z protein polymers and liver injury in a mouse model","volume":"235","author":"Kaushal","year":"2010","journal-title":"Exp Biol Med (Maywood)"},{"issue":"10","key":"10.1016\/j.ccm.2016.04.011_bib116","doi-asserted-by":"crossref","first-page":"1239","DOI":"10.1089\/hum.2011.053","article-title":"Phase 2 clinical trial of a recombinant adeno-associated viral vector expressing alpha-1 antitrypsin: interim results","volume":"22","author":"Flotte","year":"2011","journal-title":"Hum Gene Ther"},{"issue":"3","key":"10.1016\/j.ccm.2016.04.011_bib117","doi-asserted-by":"crossref","first-page":"329","DOI":"10.1517\/14712598.2015.978854","article-title":"Current status of gene therapy for alpha-1 antitrypsin deficiency","volume":"15","author":"Loring","year":"2015","journal-title":"Expert Opin Biol Ther"},{"issue":"4","key":"10.1016\/j.ccm.2016.04.011_bib118","doi-asserted-by":"crossref","first-page":"411","DOI":"10.3109\/15412555.2012.758698","article-title":"Inhaled alpha-1 antitrypsin: Gauging patient interest in a new treatment","volume":"10","author":"Monk","year":"2013","journal-title":"COPD"}],"container-title":["Clinics in Chest Medicine"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S027252311630048X?httpAccept=text\/xml","content-type":"text\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S027252311630048X?httpAccept=text\/plain","content-type":"text\/plain","content-version":"vor","intended-application":"text-mining"}],"deposited":{"date-parts":[[2025,10,3]],"date-time":"2025-10-03T00:53:59Z","timestamp":1759452839000},"score":1,"resource":{"primary":{"URL":"https:\/\/linkinghub.elsevier.com\/retrieve\/pii\/S027252311630048X"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2016,9]]},"references-count":118,"journal-issue":{"issue":"3","published-print":{"date-parts":[[2016,9]]}},"alternative-id":["S027252311630048X"],"URL":"https:\/\/doi.org\/10.1016\/j.ccm.2016.04.011","relation":{},"ISSN":["0272-5231"],"issn-type":[{"value":"0272-5231","type":"print"}],"subject":[],"published":{"date-parts":[[2016,9]]},"assertion":[{"value":"Elsevier","name":"publisher","label":"This article is maintained by"},{"value":"\u03b11-Antitrypsin Deficiency","name":"articletitle","label":"Article Title"},{"value":"Clinics in Chest Medicine","name":"journaltitle","label":"Journal Title"},{"value":"https:\/\/doi.org\/10.1016\/j.ccm.2016.04.011","name":"articlelink","label":"CrossRef DOI link to publisher maintained version"},{"value":"article","name":"content_type","label":"Content Type"},{"value":"\u00a9 2016 Elsevier Inc. All rights reserved.","name":"copyright","label":"Copyright"}]}}