{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,16]],"date-time":"2026-07-16T23:06:17Z","timestamp":1784243177010,"version":"3.55.0"},"reference-count":50,"publisher":"Elsevier BV","issue":"3","license":[{"start":{"date-parts":[[2020,9,1]],"date-time":"2020-09-01T00:00:00Z","timestamp":1598918400000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/tdm\/userlicense\/1.0\/"},{"start":{"date-parts":[[2020,9,1]],"date-time":"2020-09-01T00:00:00Z","timestamp":1598918400000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/legal\/tdmrep-license"}],"funder":[{"name":"Adverum"},{"name":"CSA Medical"},{"DOI":"10.13039\/100008322","name":"CSL Behring","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100008322","id-type":"DOI","asserted-by":"publisher"}]},{"name":"Grifols"},{"name":"MatRx"},{"DOI":"10.13039\/100004336","name":"Novartis","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100004336","id-type":"DOI","asserted-by":"publisher"}]},{"name":"Nuvaira"},{"name":"Pulmonx"},{"DOI":"10.13039\/100007723","name":"Takeda","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100007723","id-type":"DOI","asserted-by":"publisher"}]},{"name":"Vertex"}],"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":[[2020,9]]},"DOI":"10.1016\/j.ccm.2020.05.003","type":"journal-article","created":{"date-parts":[[2020,8,12]],"date-time":"2020-08-12T18:11:50Z","timestamp":1597255910000},"page":"339-345","update-policy":"https:\/\/doi.org\/10.1016\/elsevier_cm_policy","source":"Crossref","is-referenced-by-count":31,"title":["Alpha-1 Antitrypsin Deficiency Associated COPD"],"prefix":"10.1016","volume":"41","author":[{"given":"Charlie","family":"Strange","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"78","reference":[{"key":"10.1016\/j.ccm.2020.05.003_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","volume":"15","author":"Laurell","year":"1963","journal-title":"Scand J Clin Lab Invest"},{"issue":"11","key":"10.1016\/j.ccm.2020.05.003_bib2","doi-asserted-by":"crossref","first-page":"939","DOI":"10.1136\/thx.53.11.939","article-title":"Environmental correlates of impaired lung function in non-smokers with severe alpha 1-antitrypsin deficiency (PiZZ)","volume":"53","author":"Piitulainen","year":"1998","journal-title":"Thorax"},{"issue":"11","key":"10.1016\/j.ccm.2020.05.003_bib3","doi-asserted-by":"crossref","first-page":"1120","DOI":"10.1111\/j.1651-2227.1998.tb00918.x","article-title":"Effect of environmental and clinical factors on lung function and respiratory symptoms in adolescents with alpha1-antitrypsin deficiency","volume":"87","author":"Piitulainen","year":"1998","journal-title":"Acta Paediatr"},{"issue":"6A","key":"10.1016\/j.ccm.2020.05.003_bib4","doi-asserted-by":"crossref","first-page":"3","DOI":"10.1016\/S0002-9343(88)80065-2","article-title":"Natural history of alpha-1-protease inhibitor deficiency","volume":"84","author":"Hutchison","year":"1988","journal-title":"Am J Med"},{"issue":"5872","key":"10.1016\/j.ccm.2020.05.003_bib5","doi-asserted-by":"crossref","first-page":"329","DOI":"10.1038\/298329a0","article-title":"Structure and variation of human alpha 1-antitrypsin","volume":"298","author":"Carrell","year":"1982","journal-title":"Nature"},{"issue":"1","key":"10.1016\/j.ccm.2020.05.003_bib6","doi-asserted-by":"crossref","first-page":"26","DOI":"10.1172\/JCI113580","article-title":"Multiple tissues express alpha 1-antitrypsin in transgenic mice and man","volume":"82","author":"Carlson","year":"1988","journal-title":"J\u00a0Clin Invest"},{"issue":"6","key":"10.1016\/j.ccm.2020.05.003_bib7","doi-asserted-by":"crossref","first-page":"1952","DOI":"10.1172\/JCI112524","article-title":"Expression of the alpha-1-antitrypsin gene in mononuclear phagocytes of normal and alpha-1-antitrypsin-deficient individuals","volume":"77","author":"Mornex","year":"1986","journal-title":"J\u00a0Clin Invest"},{"issue":"2","key":"10.1016\/j.ccm.2020.05.003_bib8","doi-asserted-by":"crossref","first-page":"93","DOI":"10.1042\/bst0300093","article-title":"Gene regulation of the serine proteinase inhibitors alpha1-antitrypsin and alpha1-antichymotrypsin","volume":"30","author":"Kalsheker","year":"2002","journal-title":"Biochem Soc Trans"},{"issue":"4","key":"10.1016\/j.ccm.2020.05.003_bib9","doi-asserted-by":"crossref","first-page":"828","DOI":"10.1093\/ajcn\/76.4.828","article-title":"Alpha(1)-antitrypsin and antichymotrypsin in human milk: origin, concentrations, and stability","volume":"76","author":"Chowanadisai","year":"2002","journal-title":"Am J Clin Nutr"},{"issue":"10","key":"10.1016\/j.ccm.2020.05.003_bib10","first-page":"759","article-title":"Corneal ulceration and the serum antiproteases. I. Alpha 1-antitrypsin","volume":"12","author":"Berman","year":"1973","journal-title":"Invest Ophthalmol"},{"issue":"1","key":"10.1016\/j.ccm.2020.05.003_bib11","doi-asserted-by":"crossref","first-page":"37","DOI":"10.1016\/S0167-4889(98)00018-4","article-title":"Local control of alpha1-proteinase inhibitor levels: regulation of alpha1-proteinase inhibitor in the human cornea by growth factors and cytokines","volume":"1403","author":"Boskovic","year":"1998","journal-title":"Biochim Biophys Acta"},{"issue":"31","key":"10.1016\/j.ccm.2020.05.003_bib12","doi-asserted-by":"crossref","first-page":"16499","DOI":"10.1016\/S0021-9258(18)37620-8","article-title":"Distinct and additive effects of elastase and endotoxin on expression of alpha 1 proteinase inhibitor in mononuclear phagocytes","volume":"263","author":"Perlmutter","year":"1988","journal-title":"J\u00a0Biol Chem"},{"issue":"1","key":"10.1016\/j.ccm.2020.05.003_bib13","doi-asserted-by":"crossref","first-page":"246","DOI":"10.1164\/ajrccm.157.1.9702033","article-title":"Alpha 1-antitrypsin and protease complexation is induced by lipopolysaccharide, interleukin-1beta, and tumor necrosis factor-alpha in monocytes","volume":"157","author":"Knoell","year":"1998","journal-title":"Am J Respir Crit Care Med"},{"issue":"3","key":"10.1016\/j.ccm.2020.05.003_bib14","doi-asserted-by":"crossref","first-page":"373","DOI":"10.1042\/cs0720373","article-title":"Lung lavage fluid from patients with alpha 1-proteinase inhibitor deficiency or chronic obstructive bronchitis: anti-elastase function and cell profile","volume":"72","author":"Morrison","year":"1987","journal-title":"Clin Sci (Lond)"},{"issue":"12","key":"10.1016\/j.ccm.2020.05.003_bib15","doi-asserted-by":"crossref","first-page":"1059","DOI":"10.1136\/thx.2005.057943","article-title":"Alpha-1-antitrypsin deficiency: optimal therapeutic regimen based on population pharmacokinetics","volume":"61","author":"Soy","year":"2006","journal-title":"Thorax"},{"issue":"6","key":"10.1016\/j.ccm.2020.05.003_bib16","doi-asserted-by":"crossref","first-page":"1774","DOI":"10.1172\/JCI113519","article-title":"Elastase regulates the synthesis of its inhibitor, alpha 1-proteinase inhibitor, and exaggerates the defect in homozygous PiZZ alpha 1 PI deficiency","volume":"81","author":"Perlmutter","year":"1988","journal-title":"J\u00a0Clin Invest"},{"issue":"2","key":"10.1016\/j.ccm.2020.05.003_bib17","doi-asserted-by":"crossref","first-page":"301","DOI":"10.1016\/0014-5793(81)80805-8","article-title":"Human alpha 1-antitrypsin: carbohydrate attachment and sequence homology","volume":"135","author":"Carrell","year":"1981","journal-title":"FEBS Lett"},{"issue":"3","key":"10.1016\/j.ccm.2020.05.003_bib18","doi-asserted-by":"crossref","first-page":"210","DOI":"10.1002\/prot.340220303","article-title":"Structural basis for serpin inhibitor activity","volume":"22","author":"Wright","year":"1995","journal-title":"Proteins"},{"issue":"11","key":"10.1016\/j.ccm.2020.05.003_bib19","doi-asserted-by":"crossref","first-page":"1222","DOI":"10.1164\/rccm.200512-1842OC","article-title":"A\u00a0novel antiapoptotic role for alpha1-antitrypsin in the prevention of pulmonary emphysema","volume":"173","author":"Petrache","year":"2006","journal-title":"Am J Respir Crit Care Med"},{"issue":"4","key":"10.1016\/j.ccm.2020.05.003_bib20","doi-asserted-by":"crossref","first-page":"1155","DOI":"10.2353\/ajpath.2006.060058","article-title":"alpha-1 antitrypsin inhibits caspase-3 activity, preventing lung endothelial cell apoptosis","volume":"169","author":"Petrache","year":"2006","journal-title":"Am J Pathol"},{"issue":"2","key":"10.1016\/j.ccm.2020.05.003_bib21","doi-asserted-by":"crossref","first-page":"382","DOI":"10.1378\/chest.14-0520","article-title":"The relationship of the fibrinogen cleavage biomarker Aalpha-Val360 with Disease Severity and Activity in alpha1-antitrypsin deficiency","volume":"148","author":"Carter","year":"2015","journal-title":"Chest"},{"issue":"3","key":"10.1016\/j.ccm.2020.05.003_bib22","doi-asserted-by":"crossref","DOI":"10.1183\/23120541.00095-2019","article-title":"A\u00a0specific proteinase 3 activity footprint in alpha1-antitrypsin deficiency","volume":"5","author":"Newby","year":"2019","journal-title":"ERJ Open Res"},{"issue":"7","key":"10.1016\/j.ccm.2020.05.003_bib23","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":"6","key":"10.1016\/j.ccm.2020.05.003_bib24","first-page":"1154","article-title":"Characterization of the molecular basis of the alpha 1-antitrypsin F allele","volume":"48","author":"Okayama","year":"1991","journal-title":"Am J Hum Genet"},{"issue":"1","key":"10.1016\/j.ccm.2020.05.003_bib25","doi-asserted-by":"crossref","first-page":"151","DOI":"10.1186\/s12931-018-0826-8","article-title":"Real world evaluation of a novel lateral flow assay (AlphaKit(R) QuickScreen) for the detection of alpha-1-antitrypsin deficiency","volume":"19","author":"Greulich","year":"2018","journal-title":"Respir Res"},{"issue":"4","key":"10.1016\/j.ccm.2020.05.003_bib26","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 alpha1-antitrypsin deficiency PiMZ heterozygotes","volume":"189","author":"Molloy","year":"2014","journal-title":"Am J Respir Crit Care Med"},{"issue":"2","key":"10.1016\/j.ccm.2020.05.003_bib27","doi-asserted-by":"crossref","first-page":"469","DOI":"10.1164\/ajrccm.163.2.2006158","article-title":"Susceptibility genes for rapid decline of lung function in the lung health study","volume":"163","author":"Sandford","year":"2001","journal-title":"Am J Respir Crit Care Med"},{"key":"10.1016\/j.ccm.2020.05.003_bib28","doi-asserted-by":"crossref","DOI":"10.1164\/rccm.202002-0262OC","article-title":"Clarifying the risk of lung disease in SZ alpha1-antitrypsin deficiency","author":"Franciosi","year":"2020","journal-title":"Am J Respir Crit Care Med"},{"key":"10.1016\/j.ccm.2020.05.003_bib29","doi-asserted-by":"crossref","first-page":"367","DOI":"10.1016\/S0954-6111(98)90278-5","article-title":"Alpha-1 antitrypsin deficiency in Europe: geographicaal distribution of Pi types S and Z","volume":"92","author":"Hutchinson","year":"1998","journal-title":"Respir Med"},{"issue":"5","key":"10.1016\/j.ccm.2020.05.003_bib30","doi-asserted-by":"crossref","first-page":"1818","DOI":"10.1378\/chest.122.5.1818","article-title":"Worldwide racial and ethnic distribution of alpha1-antitrypsin deficiency: summary of an analysis of published genetic epidemiologic surveys","volume":"122","author":"de Serres","year":"2002","journal-title":"Chest"},{"issue":"3","key":"10.1016\/j.ccm.2020.05.003_bib31","first-page":"668","article-title":"The diagnosis and management of alpha-1 antitrypsin deficiency in the adult","volume":"3","author":"Sandhaus","year":"2016","journal-title":"Chronic Obstr Pulm Dis"},{"issue":"4","key":"10.1016\/j.ccm.2020.05.003_bib32","doi-asserted-by":"crossref","first-page":"1989","DOI":"10.1378\/chest.128.4.1989","article-title":"Delay in diagnosis of alpha1-antitrypsin deficiency: a continuing problem","volume":"128","author":"Stoller","year":"2005","journal-title":"Chest"},{"issue":"5","key":"10.1016\/j.ccm.2020.05.003_bib33","doi-asserted-by":"crossref","first-page":"1501","DOI":"10.1183\/09031936.00111213","article-title":"Circulating polymers in alpha1-antitrypsin deficiency","volume":"43","author":"Tan","year":"2014","journal-title":"Eur Respir J"},{"issue":"2","key":"10.1016\/j.ccm.2020.05.003_bib34","doi-asserted-by":"crossref","first-page":"377","DOI":"10.1016\/S0002-9440(10)62261-4","article-title":"Polymers of Z alpha1-antitrypsin co-localize with neutrophils in emphysematous alveoli and are chemotactic in\u00a0vivo","volume":"166","author":"Mahadeva","year":"2005","journal-title":"Am J Pathol"},{"issue":"5","key":"10.1016\/j.ccm.2020.05.003_bib35","doi-asserted-by":"crossref","first-page":"1952","DOI":"10.1378\/chest.125.5.1952","article-title":"Z\u00a0alpha1-antitrypsin polymerizes in the lung and acts as a neutrophil chemoattractant","volume":"125","author":"Mulgrew","year":"2004","journal-title":"Chest"},{"issue":"1\u20132","key":"10.1016\/j.ccm.2020.05.003_bib36","doi-asserted-by":"crossref","first-page":"113","DOI":"10.1016\/j.cell.2018.12.002","article-title":"Activated PMN exosomes: pathogenic Entities causing matrix Destruction and disease in the lung","volume":"176","author":"Genschmer","year":"2019","journal-title":"Cell"},{"issue":"2","key":"10.1016\/j.ccm.2020.05.003_bib37","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":"12","key":"10.1016\/j.ccm.2020.05.003_bib38","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":"9","key":"10.1016\/j.ccm.2020.05.003_bib39","doi-asserted-by":"crossref","first-page":"1971","DOI":"10.1016\/j.rmed.2007.04.003","article-title":"Respiratory symptoms and lung function in 30-year-old individuals with alpha-1-antitrypsin deficiency","volume":"101","author":"Bernspang","year":"2007","journal-title":"Respir Med"},{"issue":"8","key":"10.1016\/j.ccm.2020.05.003_bib40","doi-asserted-by":"crossref","first-page":"1384","DOI":"10.1016\/j.rmed.2005.11.017","article-title":"Asthma and allergy in alpha-1 antitrypsin deficiency","volume":"100","author":"Eden","year":"2006","journal-title":"Respir Med"},{"issue":"6\u20137","key":"10.1016\/j.ccm.2020.05.003_bib41","first-page":"1101","article-title":"Fragments of extracellular matrix as mediators of inflammation","volume":"40","author":"Adair-Kirk","year":"2007","journal-title":"Int J Biochem Cell Biol"},{"issue":"9991","key":"10.1016\/j.ccm.2020.05.003_bib42","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 alpha1 antitrypsin deficiency (RAPID): a randomised, double-blind, placebo-controlled trial","volume":"386","author":"Chapman","year":"2015","journal-title":"Lancet"},{"issue":"1","key":"10.1016\/j.ccm.2020.05.003_bib43","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 alpha1-antitrypsin. The alpha-1-antitrypsin deficiency registry study group","volume":"158","year":"1998","journal-title":"Am J Respir Crit Care Med"},{"issue":"6","key":"10.1016\/j.ccm.2020.05.003_bib44","doi-asserted-by":"crossref","first-page":"1097","DOI":"10.1183\/09031936.01.00056501","article-title":"High-resolution computed tomography scanning in alpha1-antitrypsin deficiency: relationship to lung function and health status","volume":"17","author":"Dowson","year":"2001","journal-title":"Eur Respir J"},{"issue":"12","key":"10.1016\/j.ccm.2020.05.003_bib45","doi-asserted-by":"crossref","first-page":"1020","DOI":"10.1136\/thorax.58.12.1020","article-title":"Predictors of mortality in alpha1-antitrypsin deficiency","volume":"58","author":"Dawkins","year":"2003","journal-title":"Thorax"},{"issue":"2","key":"10.1016\/j.ccm.2020.05.003_bib46","first-page":"401","article-title":"Evaluation of tamoxifen as a therapy to augment alpha-1-antitrypsin concentrations in Z homozygous alpha-1-antitrypsin-deficient subjects","volume":"135","author":"Wewers","year":"1987","journal-title":"Am Rev Respir Dis"},{"issue":"9","key":"10.1016\/j.ccm.2020.05.003_bib47","doi-asserted-by":"crossref","first-page":"1259","DOI":"10.1001\/jama.1988.03410090091037","article-title":"Biochemical efficacy and safety of monthly augmentation therapy for alpha 1-antitrypsin deficiency","volume":"260","author":"Hubbard","year":"1988","journal-title":"JAMA"},{"issue":"4","key":"10.1016\/j.ccm.2020.05.003_bib48","doi-asserted-by":"crossref","first-page":"204","DOI":"10.1097\/01.GIM.0000132669.09819.79","article-title":"Genetic testing for alpha1-antitrypsin deficiency","volume":"6","author":"Strange","year":"2004","journal-title":"Genet Med"},{"issue":"3","key":"10.1016\/j.ccm.2020.05.003_bib49","first-page":"259","article-title":"A\u00a0future for neonatal alpha1-antitrypsin screening?","volume":"89","author":"Sveger","year":"2000","journal-title":"Acta Paediatr"},{"issue":"1","key":"10.1016\/j.ccm.2020.05.003_bib50","doi-asserted-by":"crossref","first-page":"22","DOI":"10.1207\/s15324796abm3301_3","article-title":"Does genetic testing result in behavioral health change? Changes in smoking behavior following testing for alpha-1 antitrypsin deficiency","volume":"33","author":"Carpenter","year":"2007","journal-title":"Ann Behav Med"}],"container-title":["Clinics in Chest Medicine"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S0272523120300290?httpAccept=text\/xml","content-type":"text\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S0272523120300290?httpAccept=text\/plain","content-type":"text\/plain","content-version":"vor","intended-application":"text-mining"}],"deposited":{"date-parts":[[2025,10,3]],"date-time":"2025-10-03T00:57:19Z","timestamp":1759453039000},"score":1,"resource":{"primary":{"URL":"https:\/\/linkinghub.elsevier.com\/retrieve\/pii\/S0272523120300290"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2020,9]]},"references-count":50,"journal-issue":{"issue":"3","published-print":{"date-parts":[[2020,9]]}},"alternative-id":["S0272523120300290"],"URL":"https:\/\/doi.org\/10.1016\/j.ccm.2020.05.003","relation":{},"ISSN":["0272-5231"],"issn-type":[{"value":"0272-5231","type":"print"}],"subject":[],"published":{"date-parts":[[2020,9]]},"assertion":[{"value":"Elsevier","name":"publisher","label":"This article is maintained by"},{"value":"Alpha-1 Antitrypsin Deficiency Associated COPD","name":"articletitle","label":"Article Title"},{"value":"Clinics in Chest Medicine","name":"journaltitle","label":"Journal Title"},{"value":"https:\/\/doi.org\/10.1016\/j.ccm.2020.05.003","name":"articlelink","label":"CrossRef DOI link to publisher maintained version"},{"value":"article","name":"content_type","label":"Content Type"},{"value":"\u00a9 2020 Elsevier Inc. All rights reserved.","name":"copyright","label":"Copyright"}]}}