{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,20]],"date-time":"2026-09-20T14:19:05Z","timestamp":1789913945151,"version":"4.0.1"},"reference-count":48,"publisher":"Oxford University Press (OUP)","issue":"10","license":[{"start":{"date-parts":[[2014,5,1]],"date-time":"2014-05-01T00:00:00Z","timestamp":1398902400000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/academic.oup.com\/pages\/standard-publication-reuse-rights"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2014,5,15]]},"DOI":"10.1164\/rccm.201402-0203up","type":"journal-article","created":{"date-parts":[[2014,5,15]],"date-time":"2014-05-15T09:55:46Z","timestamp":1400147746000},"page":"1181-1186","source":"Crossref","is-referenced-by-count":24,"title":["Update in Cystic Fibrosis 2013"],"prefix":"10.1093","volume":"189","author":[{"given":"Manu","family":"Jain","sequence":"first","affiliation":[{"name":"Division of Pulmonary and Critical Care Medicine, Department of Medicine, Feinberg School of Medicine, Northwestern University, Chicago, Illinois"},{"name":"Division of Pulmonary Medicine, Department of Pediatrics, Lurie Children\u2019s Hospital, Chicago, Illinois"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Christopher H.","family":"Goss","sequence":"additional","affiliation":[{"name":"Division of Pulmonary and Critical Care Medicine, Department of Medicine, University of Washington, Seattle, Washington; and"},{"name":"Division of Pulmonary Medicine, Department of Pediatrics, Seattle Children\u2019s Hospital, Seattle, Washington"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"286","published-online":{"date-parts":[[2014,5,15]]},"reference":[{"key":"2026032100384556300_bib1","article-title":"Cystic Fibrosis Foundation patient registry 2012 annual data report","author":"Cystic Fibrosis Foundation","year":"2013"},{"key":"2026032100384556300_bib2","doi-asserted-by":"crossref","first-page":"593","DOI":"10.1164\/rccm.201204-0785PP","article-title":"Personalized medicine in cystic fibrosis: dawning of a new era","volume":"186","author":"Clancy","year":"2012","journal-title":"Am J Respir Crit Care Med"},{"key":"2026032100384556300_bib3","doi-asserted-by":"crossref","first-page":"1219","DOI":"10.1164\/rccm.201301-0153OC","article-title":"Efficacy and safety of ivacaftor in patients aged 6 to 11 years with cystic fibrosis with a G551D mutation","volume":"187","author":"Davies","year":"2013","journal-title":"Am J Respir Crit Care Med"},{"key":"2026032100384556300_bib4","doi-asserted-by":"crossref","first-page":"1663","DOI":"10.1056\/NEJMoa1105185","article-title":"A CFTR potentiator in patients with cystic fibrosis and the G551D mutation","volume":"365","author":"Ramsey","year":"2011","journal-title":"N Engl J Med"},{"key":"2026032100384556300_bib5","doi-asserted-by":"crossref","first-page":"109","DOI":"10.1038\/nature11130","article-title":"Reduced airway surface pH impairs bacterial killing in the porcine cystic fibrosis lung","volume":"487","author":"Pezzulo","year":"2012","journal-title":"Nature"},{"key":"2026032100384556300_bib6","doi-asserted-by":"crossref","first-page":"2598","DOI":"10.1172\/JCI2865","article-title":"Activation of NF-kappaB by adherent Pseudomonas aeruginosa in normal and cystic fibrosis respiratory epithelial cells","volume":"101","author":"DiMango","year":"1998","journal-title":"J Clin Invest"},{"key":"2026032100384556300_bib7","doi-asserted-by":"crossref","first-page":"8119","DOI":"10.1073\/pnas.131087598","article-title":"Submucosal gland secretions in airways from cystic fibrosis patients have normal [Na(+)] and pH but elevated viscosity","volume":"98","author":"Jayaraman","year":"2001","journal-title":"Proc Natl Acad Sci USA"},{"key":"2026032100384556300_bib8","first-page":"49","article-title":"Lysosomal enzymes from polymorphonuclear leukocytes and proteinase inhibitors in patients with cystic fibrosis","volume":"134","author":"Goldstein","year":"1986","journal-title":"Am Rev Respir Dis"},{"key":"2026032100384556300_bib9","doi-asserted-by":"crossref","first-page":"1478","DOI":"10.1172\/JCI115738","article-title":"Neutrophil elastase in respiratory epithelial lining fluid of individuals with cystic fibrosis induces interleukin-8 gene expression in a human bronchial epithelial cell line","volume":"89","author":"Nakamura","year":"1992","journal-title":"J Clin Invest"},{"key":"2026032100384556300_bib10","doi-asserted-by":"crossref","first-page":"L542","DOI":"10.1152\/ajplung.00180.2010","article-title":"A new role for bicarbonate in mucus formation","volume":"299","author":"Chen","year":"2010","journal-title":"Am J Physiol Lung Cell Mol Physiol"},{"key":"2026032100384556300_bib11","doi-asserted-by":"crossref","first-page":"1434","DOI":"10.1164\/rccm.201307-1268OC","article-title":"Air trapping and airflow obstruction in newborn cystic fibrosis piglets","volume":"188","author":"Adam","year":"2013","journal-title":"Am J Respir Crit Care Med"},{"key":"2026032100384556300_bib12","doi-asserted-by":"crossref","first-page":"29ra31","DOI":"10.1126\/scitranslmed.3000928","article-title":"Cystic fibrosis pigs develop lung disease and exhibit defective bacterial eradication at birth","volume":"2","author":"Stoltz","year":"2010","journal-title":"Sci Transl Med"},{"key":"2026032100384556300_bib13","doi-asserted-by":"crossref","first-page":"1391","DOI":"10.1164\/rccm.201311-1941ED","article-title":"Trouble from the start: airway structural anomalies present at birth in the cystic fibrosis piglet","volume":"188","author":"Teague","year":"2013","journal-title":"Am J Respir Crit Care Med"},{"key":"2026032100384556300_bib14","doi-asserted-by":"crossref","first-page":"213","DOI":"10.1093\/hmg\/2.3.213","article-title":"Expression of the cystic fibrosis gene in human foetal tissues","volume":"2","author":"Trezise","year":"1993","journal-title":"Hum Mol Genet"},{"key":"2026032100384556300_bib15","doi-asserted-by":"crossref","first-page":"1963","DOI":"10.1056\/NEJMoa1301725","article-title":"Risk factors for bronchiectasis in children with cystic fibrosis","volume":"368","author":"Sly","year":"2013","journal-title":"N Engl J Med"},{"key":"2026032100384556300_bib16","doi-asserted-by":"crossref","first-page":"1139","DOI":"10.1164\/rccm.200508-1330OC","article-title":"Cystic fibrosis transmembrane conductance regulator function is suppressed in cigarette smokers","volume":"173","author":"Cantin","year":"2006","journal-title":"Am J Respir Crit Care Med"},{"key":"2026032100384556300_bib17","doi-asserted-by":"crossref","first-page":"1321","DOI":"10.1164\/rccm.201304-0733OC","article-title":"Cigarette smoke induces systemic defects in cystic fibrosis transmembrane conductance regulator function","volume":"188","author":"Raju","year":"2013","journal-title":"Am J Respir Crit Care Med"},{"key":"2026032100384556300_bib18","doi-asserted-by":"crossref","first-page":"1277","DOI":"10.1164\/rccm.201311-1947ED","article-title":"Are the smoking-induced diseases an acquired form of cystic fibrosis?","volume":"188","author":"Crystal","year":"2013","journal-title":"Am J Respir Crit Care Med"},{"key":"2026032100384556300_bib19","doi-asserted-by":"crossref","first-page":"174","DOI":"10.1164\/rccm.200607-1029OC","article-title":"A novel host defense system of airways is defective in cystic fibrosis","volume":"175","author":"Moskwa","year":"2007","journal-title":"Am J Respir Crit Care Med"},{"key":"2026032100384556300_bib20","doi-asserted-by":"crossref","first-page":"857","DOI":"10.1164\/rccm.201203-0507OC","article-title":"Sputum biomarkers of inflammation and lung function decline in children with cystic fibrosis","volume":"186","author":"Sagel","year":"2012","journal-title":"Am J Respir Crit Care Med"},{"key":"2026032100384556300_bib21","doi-asserted-by":"crossref","first-page":"822","DOI":"10.1164\/rccm.200609-1354OC","article-title":"Association between pulmonary function and sputum biomarkers in cystic fibrosis","volume":"175","author":"Mayer-Hamblett","year":"2007","journal-title":"Am J Respir Crit Care Med"},{"key":"2026032100384556300_bib22","doi-asserted-by":"crossref","first-page":"170","DOI":"10.1164\/rccm.201205-0875OC","article-title":"Neutrophil elastase degrades cystic fibrosis transmembrane conductance regulator via calpains and disables channel function in vitro and in vivo","volume":"187","author":"Le Gars","year":"2013","journal-title":"Am J Respir Crit Care Med"},{"key":"2026032100384556300_bib23","doi-asserted-by":"crossref","first-page":"120","DOI":"10.1164\/rccm.201211-2011ED","article-title":"The yin and yang of cystic fibrosis transmembrane conductance regulator function: implications for chronic lung disease","volume":"187","author":"Vij","year":"2013","journal-title":"Am J Respir Crit Care Med"},{"key":"2026032100384556300_bib24","doi-asserted-by":"crossref","first-page":"922","DOI":"10.1165\/rcmb.2010-0224OC","article-title":"Lack of cystic fibrosis transmembrane conductance regulator in CD3+ lymphocytes leads to aberrant cytokine secretion and hyperinflammatory adaptive immune responses","volume":"44","author":"Mueller","year":"2011","journal-title":"Am J Respir Cell Mol Biol"},{"key":"2026032100384556300_bib25","doi-asserted-by":"crossref","first-page":"164","DOI":"10.1136\/thoraxjnl-2011-200585","article-title":"Distinct patterns of inflammation in the airway lumen and bronchial mucosa of children with cystic fibrosis","volume":"67","author":"Regamey","year":"2012","journal-title":"Thorax"},{"key":"2026032100384556300_bib26","doi-asserted-by":"crossref","first-page":"621","DOI":"10.1164\/rccm.201206-1150OC","article-title":"A Th17- and Th2-skewed cytokine profile in cystic fibrosis lungs represents a potential risk factor for Pseudomonas aeruginosa infection","volume":"187","author":"Tiringer","year":"2013","journal-title":"Am J Respir Crit Care Med"},{"key":"2026032100384556300_bib27","doi-asserted-by":"crossref","first-page":"609","DOI":"10.1164\/rccm.201207-1346OC","article-title":"Th17\/Treg imbalance in murine cystic fibrosis is linked to indoleamine 2,3-dioxygenase deficiency but corrected by kynurenines","volume":"187","author":"Iannitti","year":"2013","journal-title":"Am J Respir Crit Care Med"},{"key":"2026032100384556300_bib28","doi-asserted-by":"crossref","first-page":"564","DOI":"10.1164\/rccm.201212-2201ED","article-title":"T-cell immunotherapy in cystic fibrosis: weighing the risk\/reward","volume":"187","author":"Robinson","year":"2013","journal-title":"Am J Respir Crit Care Med"},{"key":"2026032100384556300_bib29","doi-asserted-by":"crossref","first-page":"1338","DOI":"10.1164\/rccm.201305-0986OC","article-title":"Hypoxia promotes danger-mediated inflammation via receptor for advanced glycation end products in cystic fibrosis","volume":"188","author":"Iannitti","year":"2013","journal-title":"Am J Respir Crit Care Med"},{"key":"2026032100384556300_bib30","doi-asserted-by":"crossref","first-page":"1280","DOI":"10.1164\/rccm.201310-1908ED","article-title":"Hypoxia, receptor for advanced glycation end products, and cystic fibrosis: a pathway to chronic inflammation?","volume":"188","author":"McGuire","year":"2013","journal-title":"Am J Respir Crit Care Med"},{"key":"2026032100384556300_bib31","doi-asserted-by":"crossref","first-page":"752","DOI":"10.1164\/rccm.200911-1646OC","article-title":"Lung clearance index at 4 years predicts subsequent lung function in children with cystic fibrosis","volume":"183","author":"Aurora","year":"2011","journal-title":"Am J Respir Crit Care Med"},{"key":"2026032100384556300_bib32","doi-asserted-by":"crossref","first-page":"545","DOI":"10.1164\/rccm.201304-0800OC","article-title":"Lung clearance index and high-resolution computed tomography scores in primary ciliary dyskinesia","volume":"188","author":"Irving","year":"2013","journal-title":"Am J Respir Crit Care Med"},{"key":"2026032100384556300_bib33","doi-asserted-by":"crossref","first-page":"862","DOI":"10.1164\/rccm.201109-1631OC","article-title":"Early cystic fibrosis lung disease detected by bronchoalveolar lavage and lung clearance index","volume":"185","author":"Belessis","year":"2012","journal-title":"Am J Respir Crit Care Med"},{"key":"2026032100384556300_bib34","doi-asserted-by":"crossref","first-page":"456","DOI":"10.1164\/rccm.201302-0219OC","article-title":"Lung clearance index as an outcome measure for clinical trials in young children with cystic fibrosis: a pilot study using inhaled hypertonic saline","volume":"188","author":"Subbarao","year":"2013","journal-title":"Am J Respir Crit Care Med"},{"key":"2026032100384556300_bib35","doi-asserted-by":"crossref","first-page":"2269","DOI":"10.1001\/jama.2012.5214","article-title":"Inhaled hypertonic saline in infants and children younger than 6 years with cystic fibrosis: the ISIS randomized controlled trial","volume":"307","author":"Rosenfeld","year":"2012","journal-title":"JAMA"},{"key":"2026032100384556300_bib36","doi-asserted-by":"crossref","first-page":"409","DOI":"10.1164\/rccm.201306-1023ED","article-title":"Early intervention for newborns screened for cystic fibrosis","volume":"188","author":"Beydon","year":"2013","journal-title":"Am J Respir Crit Care Med"},{"key":"2026032100384556300_bib37","doi-asserted-by":"crossref","first-page":"630","DOI":"10.1016\/S2213-2600(13)70182-6","article-title":"Assessment of clinical response to ivacaftor with lung clearance index in cystic fibrosis patients with a G551D-CFTR mutation and preserved spirometry: a randomised controlled trial","volume":"1","author":"Davies","year":"2013","journal-title":"Lancet Respir Med"},{"key":"2026032100384556300_bib38","doi-asserted-by":"crossref","first-page":"1484","DOI":"10.1002\/emmm.201302699","article-title":"Discovery of novel potent \u0394F508-CFTR correctors that target the nucleotide binding domain","volume":"5","author":"Odolczyk","year":"2013","journal-title":"EMBO Mol Med"},{"key":"2026032100384556300_bib39","doi-asserted-by":"crossref","first-page":"83","DOI":"10.1164\/rccm.201303-0427OC","article-title":"Inhalation treatment with glutathione in patients with cystic fibrosis: a randomized clinical trial","volume":"188","author":"Griese","year":"2013","journal-title":"Am J Respir Crit Care Med"},{"key":"2026032100384556300_bib40","doi-asserted-by":"crossref","first-page":"637","DOI":"10.1056\/NEJM199409083311003","article-title":"Effect of aerosolized recombinant human DNase on exacerbations of respiratory symptoms and on pulmonary function in patients with cystic fibrosis","volume":"331","author":"Fuchs","year":"1994","journal-title":"N Engl J Med"},{"key":"2026032100384556300_bib41","doi-asserted-by":"crossref","first-page":"703","DOI":"10.1164\/rccm.201305-0912OC","article-title":"Poly-L-lysine compacts DNA, kills bacteria, and improves protease inhibition in cystic fibrosis sputum","volume":"188","author":"Dubois","year":"2013","journal-title":"Am J Respir Crit Care Med"},{"key":"2026032100384556300_bib42","doi-asserted-by":"crossref","first-page":"634","DOI":"10.1164\/rccm.201307-1371ED","article-title":"Faster, higher, stronger","volume":"188","author":"Rubin","year":"2013","journal-title":"Am J Respir Crit Care Med"},{"key":"2026032100384556300_bib43","doi-asserted-by":"crossref","first-page":"231","DOI":"10.1164\/ajrccm.185.2.231","article-title":"Respiratory outbreak of Mycobacterium abscessus subspecies massiliense in a lung transplant and cystic fibrosis center","volume":"185","author":"Aitken","year":"2012","journal-title":"Am J Respir Crit Care Med"},{"key":"2026032100384556300_bib44","doi-asserted-by":"crossref","first-page":"1551","DOI":"10.1016\/S0140-6736(13)60632-7","article-title":"Whole-genome sequencing to identify transmission of Mycobacterium abscessus between patients with cystic fibrosis: a retrospective cohort study","volume":"381","author":"Bryant","year":"2013","journal-title":"Lancet"},{"key":"2026032100384556300_bib45","doi-asserted-by":"crossref","first-page":"1749","DOI":"10.1001\/jama.290.13.1749","article-title":"Azithromycin in patients with cystic fibrosis chronically infected with Pseudomonas aeruginosa: a randomized controlled trial","volume":"290","author":"Saiman","year":"2003","journal-title":"JAMA"},{"key":"2026032100384556300_bib46","doi-asserted-by":"crossref","first-page":"807","DOI":"10.1164\/rccm.201307-1200OC","article-title":"Epidemiology of nontuberculous mycobacterial infections and associated chronic macrolide use among persons with cystic fibrosis","volume":"188","author":"Binder","year":"2013","journal-title":"Am J Respir Crit Care Med"},{"key":"2026032100384556300_bib47","doi-asserted-by":"crossref","first-page":"1335","DOI":"10.1164\/rccm.201303-0429OC","article-title":"Survival benefit of lung transplant for cystic fibrosis since lung allocation score implementation","volume":"187","author":"Thabut","year":"2013","journal-title":"Am J Respir Crit Care Med"},{"key":"2026032100384556300_bib48","doi-asserted-by":"crossref","first-page":"1283","DOI":"10.1164\/rccm.201304-0736ED","article-title":"Should adults with cystic fibrosis receive lung transplants? Outcomes versus progression of disease","volume":"187","author":"Garrity","year":"2013","journal-title":"Am J Respir Crit Care Med"}],"container-title":["American Journal of Respiratory and Critical Care Medicine"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/academic.oup.com\/ajrccm\/article-pdf\/189\/10\/1181\/67291124\/ajrccm_189_10_1181.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"syndication"},{"URL":"https:\/\/academic.oup.com\/ajrccm\/article-pdf\/189\/10\/1181\/67291124\/ajrccm_189_10_1181.pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,3,21]],"date-time":"2026-03-21T04:40:06Z","timestamp":1774068006000},"score":1,"resource":{"primary":{"URL":"https:\/\/academic.oup.com\/ajrccm\/article\/189\/10\/1181\/8506301"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2014,5]]},"references-count":48,"journal-issue":{"issue":"10","published-online":{"date-parts":[[2014,5,15]]},"published-print":{"date-parts":[[2014,5,15]]}},"URL":"https:\/\/doi.org\/10.1164\/rccm.201402-0203up","relation":{},"ISSN":["1073-449X","1535-4970"],"issn-type":[{"value":"1073-449X","type":"print"},{"value":"1535-4970","type":"electronic"}],"subject":[],"published-other":{"date-parts":[[2014,5]]},"published":{"date-parts":[[2014,5]]}}}