{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,22]],"date-time":"2026-10-22T11:46:34Z","timestamp":1792669594363,"version":"4.5.0"},"reference-count":90,"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.2016.04.008","type":"journal-article","created":{"date-parts":[[2016,7,2]],"date-time":"2016-07-02T05:48:54Z","timestamp":1467438534000},"page":"449-461","update-policy":"https:\/\/doi.org\/10.1016\/elsevier_cm_policy","source":"Crossref","is-referenced-by-count":210,"title":["Primary Ciliary Dyskinesia"],"prefix":"10.1016","volume":"37","author":[{"given":"Michael R.","family":"Knowles","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Maimoona","family":"Zariwala","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Margaret","family":"Leigh","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"78","reference":[{"key":"10.1016\/j.ccm.2016.04.008_bib1","doi-asserted-by":"crossref","first-page":"913","DOI":"10.1164\/rccm.201301-0059CI","article-title":"Primary ciliary dyskinesia. Recent advances in diagnostics, genetics, and characterization of clinical disease","volume":"188","author":"Knowles","year":"2013","journal-title":"Am J Respir Crit Care Med"},{"key":"10.1016\/j.ccm.2016.04.008_bib2","doi-asserted-by":"crossref","first-page":"169","DOI":"10.1055\/s-0035-1546748","article-title":"Primary ciliary dyskinesia","volume":"36","author":"Lobo","year":"2015","journal-title":"Semin Respir Crit Care Med"},{"key":"10.1016\/j.ccm.2016.04.008_bib3","doi-asserted-by":"crossref","first-page":"36","DOI":"10.12703\/P7-36","article-title":"Unique among ciliopathies: primary ciliary dyskinesia, a motile cilia disorder","volume":"7","author":"Praveen","year":"2015","journal-title":"F1000Prime Rep"},{"key":"10.1016\/j.ccm.2016.04.008_bib4","first-page":"18","article-title":"Genetics and biology of primary ciliary dyskinesia","volume":"18","author":"Horani","year":"2016","journal-title":"Paediatr Respir Rev"},{"key":"10.1016\/j.ccm.2016.04.008_bib5","doi-asserted-by":"crossref","first-page":"489","DOI":"10.1007\/BF02141468","article-title":"Zur pathogenese der bronkiectasien: bronkiectasien bei situs viscerum inversus","volume":"82","author":"Kartagener","year":"1933","journal-title":"Beitr Klin Tuberk"},{"key":"10.1016\/j.ccm.2016.04.008_bib6","doi-asserted-by":"crossref","first-page":"317","DOI":"10.1126\/science.1084576","article-title":"A\u00a0human syndrome caused by immotile cilia","volume":"193","author":"Afzelius","year":"1976","journal-title":"Science"},{"key":"10.1016\/j.ccm.2016.04.008_bib7","doi-asserted-by":"crossref","first-page":"17","DOI":"10.3109\/00016924709135208","article-title":"Transposition of viscera, bronchiectasis and nasal polyps; a genetical analysis and a contribution to the problem of constitution","volume":"28","author":"Torgersen","year":"1947","journal-title":"Acta Radiol"},{"key":"10.1016\/j.ccm.2016.04.008_bib8","doi-asserted-by":"crossref","first-page":"56","DOI":"10.1378\/chest.61.1.56","article-title":"Situs inversus totalis and Kartagener's syndrome in a Japanese population","volume":"61","author":"Katsuhara","year":"1972","journal-title":"Chest"},{"key":"10.1016\/j.ccm.2016.04.008_bib9","doi-asserted-by":"crossref","first-page":"1418","DOI":"10.1183\/09031936.00175614","article-title":"Diagnosis of primary ciliary dyskinesia: searching for a gold standard","volume":"44","author":"Lucas","year":"2014","journal-title":"Eur Respir J"},{"key":"10.1016\/j.ccm.2016.04.008_bib10","doi-asserted-by":"crossref","first-page":"722","DOI":"10.1111\/jpc.12791","article-title":"Presentation of primary ciliary dyskinesia in children: 30 years' experience","volume":"51","author":"Hosie","year":"2015","journal-title":"J\u00a0Paediatr Child Health"},{"key":"10.1016\/j.ccm.2016.04.008_bib11","doi-asserted-by":"crossref","DOI":"10.1513\/AnnalsATS.201511-748OC","article-title":"Clinical features and associated likelihood of primary ciliary dyskinesia in children and adolescents","author":"Leigh","year":"2016","journal-title":"Ann Am Thorac Soc"},{"key":"10.1016\/j.ccm.2016.04.008_bib12","doi-asserted-by":"crossref","first-page":"438","DOI":"10.1513\/pats.201103-024SD","article-title":"Update of respiratory tract disease in children with primary ciliary dyskinesia","volume":"8","author":"Sagel","year":"2011","journal-title":"Proc Am Thorac Soc"},{"key":"10.1016\/j.ccm.2016.04.008_bib13","doi-asserted-by":"crossref","first-page":"574","DOI":"10.1513\/AnnalsATS.201305-110OC","article-title":"Standardizing nasal nitric oxide measurement as a test for primary ciliary dyskinesia","volume":"10","author":"Leigh","year":"2013","journal-title":"Ann Am Thorac Soc"},{"key":"10.1016\/j.ccm.2016.04.008_bib14","unstructured":"Zariwala MA, Knowles MR, Leigh MW. Primary ciliary dyskinesia. In: Pagon RA, Adam MP, Ardinger HH, et\u00a0al, editors. GeneReviews\u00ae [Internet]. Seattle (WA); 1993. Updated September 2015."},{"issue":"2","key":"10.1016\/j.ccm.2016.04.008_bib15","doi-asserted-by":"crossref","first-page":"115","DOI":"10.1002\/ppul.23304","article-title":"Diagnosis, monitoring, and treatment of primary ciliary dyskinesia: PCD foundation consensus recommendations based on state of the art review","volume":"51","author":"Shapiro","year":"2016","journal-title":"Pediatr Pulmonol"},{"key":"10.1016\/j.ccm.2016.04.008_bib16","doi-asserted-by":"crossref","first-page":"401","DOI":"10.1146\/annurev.physiol.69.040705.141253","article-title":"Regulation of mammalian ciliary beating","volume":"69","author":"Salathe","year":"2007","journal-title":"Annu Rev Physiol"},{"key":"10.1016\/j.ccm.2016.04.008_bib17","doi-asserted-by":"crossref","first-page":"377","DOI":"10.1146\/annurev.physiol.69.040705.141236","article-title":"Overview of structure and function of mammalian cilia","volume":"69","author":"Satir","year":"2007","journal-title":"Annu Rev Physiol"},{"key":"10.1016\/j.ccm.2016.04.008_bib18","doi-asserted-by":"crossref","first-page":"518","DOI":"10.1016\/S0091-6749(03)01799-8","article-title":"Ciliary beat pattern is associated with specific ultrastructural defects in primary ciliary dyskinesia","volume":"112","author":"Chilvers","year":"2003","journal-title":"J\u00a0Allergy Clin Immunol"},{"key":"10.1016\/j.ccm.2016.04.008_bib19","doi-asserted-by":"crossref","first-page":"151","DOI":"10.1016\/S0070-2153(08)00806-5","article-title":"Cilia multifunctional organelles at the center of vertebrate left-right asymmetry","volume":"85","author":"Basu","year":"2008","journal-title":"Curr Top Dev Biol"},{"key":"10.1016\/j.ccm.2016.04.008_bib20","doi-asserted-by":"crossref","first-page":"1533","DOI":"10.1056\/NEJMra1010172","article-title":"Ciliopathies","volume":"364","author":"Hildebrandt","year":"2011","journal-title":"N\u00a0Engl J Med"},{"key":"10.1016\/j.ccm.2016.04.008_bib21","doi-asserted-by":"crossref","first-page":"571","DOI":"10.1172\/JCI0215217","article-title":"Mucus clearance as a primary innate defense mechanism for mammalian airways","volume":"109","author":"Knowles","year":"2002","journal-title":"J\u00a0Clin Invest"},{"key":"10.1016\/j.ccm.2016.04.008_bib22","doi-asserted-by":"crossref","first-page":"379","DOI":"10.1146\/annurev-physiol-021014-071931","article-title":"Cilia dysfunction in lung disease","volume":"77","author":"Tilley","year":"2015","journal-title":"Annu Rev Physiol"},{"key":"10.1016\/j.ccm.2016.04.008_bib23","doi-asserted-by":"crossref","first-page":"L170","DOI":"10.1152\/ajplung.00105.2012","article-title":"Human airway ciliary dynamics","volume":"304","author":"Sears","year":"2013","journal-title":"Am J Physiol Lung Cell Mol Physiol"},{"key":"10.1016\/j.ccm.2016.04.008_bib24","doi-asserted-by":"crossref","first-page":"924","DOI":"10.1016\/j.cub.2009.04.018","article-title":"The PCP pathway instructs the planar orientation of ciliated cells in the Xenopus larval skin","volume":"19","author":"Mitchell","year":"2009","journal-title":"Curr Biol"},{"key":"10.1016\/j.ccm.2016.04.008_bib25","doi-asserted-by":"crossref","first-page":"339","DOI":"10.1136\/thoraxjnl-2014-206578","article-title":"Lung structure-function correlation in patients with primary ciliary dyskinesia","volume":"70","author":"Boon","year":"2015","journal-title":"Thorax"},{"key":"10.1016\/j.ccm.2016.04.008_bib26","doi-asserted-by":"crossref","first-page":"351","DOI":"10.1378\/chest.07-2812","article-title":"Structural and functional lung disease in primary ciliary dyskinesia","volume":"134","author":"Santamaria","year":"2008","journal-title":"Chest"},{"key":"10.1016\/j.ccm.2016.04.008_bib27","doi-asserted-by":"crossref","first-page":"986","DOI":"10.1016\/j.crad.2007.04.015","article-title":"Primary ciliary dyskinesia in the paediatric population: range and severity of radiological findings in a cohort of patients receiving tertiary care","volume":"62","author":"Jain","year":"2007","journal-title":"Clin Radiol"},{"key":"10.1016\/j.ccm.2016.04.008_bib28","doi-asserted-by":"crossref","first-page":"603","DOI":"10.1183\/09031936.00108410","article-title":"Inner dynein arm defects causing primary ciliary dyskinesia: repeat testing required","volume":"38","author":"O'Callaghan","year":"2011","journal-title":"Eur Respir J"},{"key":"10.1016\/j.ccm.2016.04.008_bib29","doi-asserted-by":"crossref","first-page":"72","DOI":"10.1038\/ng.726","article-title":"CCDC39 is required for assembly of inner dynein arms and the dynein regulatory complex and for normal ciliary motility in humans and dogs","volume":"43","author":"Merveille","year":"2011","journal-title":"Nat Genet"},{"key":"10.1016\/j.ccm.2016.04.008_bib30","doi-asserted-by":"crossref","first-page":"79","DOI":"10.1038\/ng.727","article-title":"The coiled-coil domain containing protein CCDC40 is essential for motile cilia function and left-right axis formation","volume":"43","author":"Becker-Heck","year":"2011","journal-title":"Nat Genet"},{"key":"10.1016\/j.ccm.2016.04.008_bib31","doi-asserted-by":"crossref","first-page":"527","DOI":"10.1080\/00016480410016270","article-title":"The secondary nature of\u00a0ciliary (dis)orientation in secondary and primary ciliary dyskinesia","volume":"124","author":"Jorissen","year":"2004","journal-title":"Acta Otolaryngol"},{"key":"10.1016\/j.ccm.2016.04.008_bib32","doi-asserted-by":"crossref","first-page":"646","DOI":"10.1038\/ng.2961","article-title":"Mutations in CCNO result in congenital mucociliary clearance disorder with reduced generation of multiple motile cilia","volume":"46","author":"Wallmeier","year":"2014","journal-title":"Nat Genet"},{"key":"10.1016\/j.ccm.2016.04.008_bib33","doi-asserted-by":"crossref","first-page":"4418","DOI":"10.1038\/ncomms5418","article-title":"MCIDAS mutations result in a mucociliary clearance disorder with reduced generation of multiple motile cilia","volume":"5","author":"Boon","year":"2014","journal-title":"Nat Commun"},{"key":"10.1016\/j.ccm.2016.04.008_bib34","doi-asserted-by":"crossref","first-page":"465","DOI":"10.2350\/14-06-1504-OA.1","article-title":"Ciliary inclusion disease: report of a new primary ciliary dyskinesia variant","volume":"17","author":"Wartchow","year":"2014","journal-title":"Pediatr Dev Pathol"},{"key":"10.1016\/j.ccm.2016.04.008_bib35","doi-asserted-by":"crossref","first-page":"483","DOI":"10.1002\/ppul.21402","article-title":"Diagnostic yield of nasal scrape biopsies in primary ciliary dyskinesia: a multicenter experience","volume":"46","author":"Olin","year":"2011","journal-title":"Pediatr Pulmonol"},{"key":"10.1016\/j.ccm.2016.04.008_bib36","first-page":"A1217","article-title":"The diagnostic dilemma of primary ciliary dyskinesia: findings and experience of the genetic disorders of mucociliary clearance consortium","volume":"183","author":"Daniels","year":"2011","journal-title":"Am J Respir Crit Care Med"},{"key":"10.1016\/j.ccm.2016.04.008_bib37","doi-asserted-by":"crossref","first-page":"1057","DOI":"10.1183\/09031936.00046209","article-title":"A\u00a020-year experience of electron microscopy in the diagnosis of primary ciliary dyskinesia","volume":"35","author":"Papon","year":"2010","journal-title":"Eur Respir J"},{"key":"10.1016\/j.ccm.2016.04.008_bib38","doi-asserted-by":"crossref","first-page":"433","DOI":"10.1136\/thoraxjnl-2011-200301","article-title":"Mutations of DNAH11 in patients with primary ciliary dyskinesia with normal ciliary ultrastructure","volume":"67","author":"Knowles","year":"2012","journal-title":"Thorax"},{"key":"10.1016\/j.ccm.2016.04.008_bib39","doi-asserted-by":"crossref","first-page":"333","DOI":"10.1136\/thorax.58.4.333","article-title":"Functional analysis of cilia and ciliated epithelial ultrastructure in healthy children and young adults","volume":"58","author":"Chilvers","year":"2003","journal-title":"Thorax"},{"key":"10.1016\/j.ccm.2016.04.008_bib40","doi-asserted-by":"crossref","first-page":"853","DOI":"10.1111\/j.1651-2227.1999.tb00061.x","article-title":"Ciliary motility at light microscopy: a screening technique for ciliary defects","volume":"88","author":"Santamaria","year":"1999","journal-title":"Acta Paediatr"},{"key":"10.1016\/j.ccm.2016.04.008_bib41","doi-asserted-by":"crossref","first-page":"401","DOI":"10.1183\/09031936.00153308","article-title":"Disrupted ciliated epithelium shows slower ciliary beat frequency and increased dyskinesia","volume":"34","author":"Thomas","year":"2009","journal-title":"Eur Respir J"},{"key":"10.1016\/j.ccm.2016.04.008_bib42","doi-asserted-by":"crossref","first-page":"295","DOI":"10.1152\/japplphysiol.00629.2010","article-title":"Primary ciliary dyskinesia: evaluation using cilia beat frequency assessment via spectral analysis of digital microscopy images","volume":"111","author":"Olm","year":"2011","journal-title":"J\u00a0Appl Physiol (1985)"},{"key":"10.1016\/j.ccm.2016.04.008_bib43","doi-asserted-by":"crossref","first-page":"1441","DOI":"10.1378\/chest.10-0175","article-title":"Ciliated air-liquid cultures as an aid to diagnostic testing of primary ciliary dyskinesia","volume":"138","author":"Hirst","year":"2010","journal-title":"Chest"},{"key":"10.1016\/j.ccm.2016.04.008_bib44","doi-asserted-by":"crossref","first-page":"186","DOI":"10.1378\/chest.10-1920","article-title":"Cooling of cilia allows functional analysis of the beat pattern for diagnostic testing","volume":"140","author":"Smith","year":"2011","journal-title":"Chest"},{"key":"10.1016\/j.ccm.2016.04.008_bib45","doi-asserted-by":"crossref","first-page":"543","DOI":"10.1378\/chest.11-3253","article-title":"Ciliary beat pattern analysis below 37 degrees C may increase risk of primary ciliary dyskinesia misdiagnosis","volume":"142","author":"Jackson","year":"2012","journal-title":"Chest"},{"key":"10.1016\/j.ccm.2016.04.008_bib46","doi-asserted-by":"crossref","first-page":"299ra124","DOI":"10.1126\/scitranslmed.aaa1233","article-title":"Automated identification of abnormal respiratory ciliary motion in nasal biopsies","volume":"7","author":"Quinn","year":"2015","journal-title":"Sci Transl Med"},{"key":"10.1016\/j.ccm.2016.04.008_bib47","doi-asserted-by":"crossref","first-page":"459","DOI":"10.1164\/rccm.200303-365OC","article-title":"Primary ciliary dyskinesia: diagnostic and phenotypic features","volume":"169","author":"Noone","year":"2004","journal-title":"Am J Respir Crit Care Med"},{"key":"10.1016\/j.ccm.2016.04.008_bib48","doi-asserted-by":"crossref","first-page":"912","DOI":"10.1164\/rccm.200406-710ST","article-title":"ATS\/ERS recommendations for standardized procedures for the online and offline measurement of exhaled lower respiratory nitric oxide and nasal nitric oxide, 2005","volume":"171","author":"American Thoracic Society","year":"2005","journal-title":"Am J Respir Crit Care Med"},{"key":"10.1016\/j.ccm.2016.04.008_bib49","doi-asserted-by":"crossref","first-page":"153","DOI":"10.1186\/s12890-015-0147-3","article-title":"Diagnostic accuracy of nasal nitric oxide for establishing diagnosis of primary ciliary dyskinesia: a meta-analysis","volume":"15","author":"Kouis","year":"2015","journal-title":"BMC Pulm Med"},{"key":"10.1016\/j.ccm.2016.04.008_bib50","doi-asserted-by":"crossref","first-page":"420","DOI":"10.1016\/j.jpeds.2011.03.007","article-title":"Diagnostic value of nasal nitric oxide measured with non-velum closure techniques for children with\u00a0primary ciliary dyskinesia","volume":"159","author":"Mateos-Corral","year":"2011","journal-title":"J\u00a0Pediatr"},{"key":"10.1016\/j.ccm.2016.04.008_bib51","doi-asserted-by":"crossref","first-page":"381","DOI":"10.1038\/ng.1106","article-title":"Mutations in axonemal dynein assembly factor DNAAF3 cause primary ciliary dyskinesia","volume":"44","author":"Mitchison","year":"2012","journal-title":"Nat Genet"},{"key":"10.1016\/j.ccm.2016.04.008_bib52","doi-asserted-by":"crossref","first-page":"563","DOI":"10.1165\/rcmb.2014-0483OC","article-title":"Immunofluorescence analysis and diagnosis of primary ciliary dyskinesia with radial spoke defects","volume":"53","author":"Frommer","year":"2015","journal-title":"Am J Respir Cell Mol Biol"},{"key":"10.1016\/j.ccm.2016.04.008_bib53","doi-asserted-by":"crossref","first-page":"966","DOI":"10.1378\/chest.06-2951","article-title":"Pulmonary radioaerosol mucociliary clearance in diagnosis of primary ciliary dyskinesia","volume":"132","author":"Marthin","year":"2007","journal-title":"Chest"},{"key":"10.1016\/j.ccm.2016.04.008_bib54","doi-asserted-by":"crossref","first-page":"707","DOI":"10.1164\/rccm.201311-2047OC","article-title":"Mutations in RSPH1 cause primary ciliary dyskinesia with a unique clinical and ciliary phenotype","volume":"189","author":"Knowles","year":"2014","journal-title":"Am J Respir Crit Care Med"},{"key":"10.1016\/j.ccm.2016.04.008_bib55","doi-asserted-by":"crossref","first-page":"5727","DOI":"10.1038\/ncomms6727","article-title":"Cryo-electron tomography reveals ciliary defects underlying human RSPH1 primary ciliary dyskinesia","volume":"5","author":"Lin","year":"2014","journal-title":"Nat Commun"},{"key":"10.1016\/j.ccm.2016.04.008_bib56","doi-asserted-by":"crossref","first-page":"316","DOI":"10.1164\/rccm.201409-1672OC","article-title":"Clinical features of childhood primary ciliary dyskinesia by genotype and ultrastructural phenotype","volume":"191","author":"Davis","year":"2015","journal-title":"Am J Respir Crit Care Med"},{"key":"10.1016\/j.ccm.2016.04.008_bib57","doi-asserted-by":"crossref","first-page":"1160","DOI":"10.1542\/peds.2014-0808","article-title":"Primary ciliary dyskinesia and neonatal respiratory distress","volume":"134","author":"Mullowney","year":"2014","journal-title":"Pediatrics"},{"key":"10.1016\/j.ccm.2016.04.008_bib58","doi-asserted-by":"crossref","first-page":"1232","DOI":"10.2214\/AJR.06.0965","article-title":"High-resolution CT of patients with primary ciliary dyskinesia","volume":"188","author":"Kennedy","year":"2007","journal-title":"AJR Am J Roentgenol"},{"issue":"1093","key":"10.1016\/j.ccm.2016.04.008_bib59","first-page":"e1091","article-title":"A\u00a0longitudinal study of lung bacterial pathogens in patients with primary ciliary dyskinesia","volume":"21","author":"Alanin","year":"2015","journal-title":"Clin Microbiol Infect"},{"key":"10.1016\/j.ccm.2016.04.008_bib60","doi-asserted-by":"crossref","first-page":"498","DOI":"10.1002\/ppul.21569","article-title":"Progression of lung disease in primary ciliary dyskinesia: is spirometry less accurate than CT?","volume":"47","author":"Maglione","year":"2012","journal-title":"Pediatr Pulmonol"},{"key":"10.1016\/j.ccm.2016.04.008_bib61","doi-asserted-by":"crossref","first-page":"1262","DOI":"10.1164\/rccm.200811-1731OC","article-title":"Lung function in patients with primary ciliary dyskinesia: a cross-sectional and 3-decade longitudinal study","volume":"181","author":"Marthin","year":"2010","journal-title":"Am J Respir Crit Care Med"},{"key":"10.1016\/j.ccm.2016.04.008_bib62","doi-asserted-by":"crossref","first-page":"2376","DOI":"10.1183\/09031936.97.10102376","article-title":"Longitudinal study of lung function in a cohort of primary ciliary dyskinesia","volume":"10","author":"Ellerman","year":"1997","journal-title":"Eur Respir J"},{"key":"10.1016\/j.ccm.2016.04.008_bib63","doi-asserted-by":"crossref","first-page":"422","DOI":"10.1007\/s004310050843","article-title":"Primary ciliary dyskinesia: evolution of pulmonary function","volume":"157","author":"Hellinckx","year":"1998","journal-title":"Eur J Pediatr"},{"key":"10.1016\/j.ccm.2016.04.008_bib64","doi-asserted-by":"crossref","first-page":"49","DOI":"10.1136\/thoraxjnl-2011-200726","article-title":"Ventilation inhomogeneity in children with primary ciliary dyskinesia","volume":"67","author":"Green","year":"2012","journal-title":"Thorax"},{"key":"10.1016\/j.ccm.2016.04.008_bib65","doi-asserted-by":"crossref","first-page":"87","DOI":"10.1111\/j.1440-1843.2011.02067.x","article-title":"Magnetic resonance imaging is an accurate and reliable method to evaluate non-cystic fibrosis paediatric lung disease","volume":"17","author":"Montella","year":"2012","journal-title":"Respirology"},{"key":"10.1016\/j.ccm.2016.04.008_bib66","doi-asserted-by":"crossref","first-page":"2793","DOI":"10.1161\/CIRCULATIONAHA.107.699256","article-title":"Heterotaxia, congenital heart disease, and primary ciliary dyskinesia","volume":"115","author":"Brueckner","year":"2007","journal-title":"Circulation"},{"key":"10.1016\/j.ccm.2016.04.008_bib67","doi-asserted-by":"crossref","first-page":"1176","DOI":"10.1378\/chest.13-1704","article-title":"Laterality defects other than situs inversus totalis in primary ciliary dyskinesia: insights into situs ambiguus and heterotaxy","volume":"146","author":"Shapiro","year":"2014","journal-title":"Chest"},{"key":"10.1016\/j.ccm.2016.04.008_bib68","first-page":"25","article-title":"Congenital heart disease and primary ciliary dyskinesia","volume":"18","author":"Harrison","year":"2016","journal-title":"Paediatr Respir Rev"},{"key":"10.1016\/j.ccm.2016.04.008_bib69","doi-asserted-by":"crossref","first-page":"637","DOI":"10.1016\/j.jtcvs.2010.07.082","article-title":"Increased postoperative and respiratory complications in patients with\u00a0congenital heart disease associated with heterotaxy","volume":"141","author":"Swisher","year":"2011","journal-title":"J\u00a0Thorac Cardiovasc Surg"},{"key":"10.1016\/j.ccm.2016.04.008_bib70","doi-asserted-by":"crossref","first-page":"2178","DOI":"10.1161\/CIRCULATIONAHA.112.103861","article-title":"Impact of genetic diagnosis on clinical management of patients with congenital heart disease: cilia point the way","volume":"125","author":"Brueckner","year":"2012","journal-title":"Circulation"},{"key":"10.1016\/j.ccm.2016.04.008_bib71","doi-asserted-by":"crossref","first-page":"H2102","DOI":"10.1152\/ajpheart.01118.2011","article-title":"Congenital heart disease and the specification of left-right asymmetry","volume":"302","author":"Francis","year":"2012","journal-title":"Am J Physiol Heart Circ Physiol"},{"key":"10.1016\/j.ccm.2016.04.008_bib72","doi-asserted-by":"crossref","first-page":"684","DOI":"10.1136\/thx.49.7.684","article-title":"Fertility\u00a0in men with primary ciliary dyskinesia presenting with respiratory infection","volume":"49","author":"Munro","year":"1994","journal-title":"Thorax"},{"key":"10.1016\/j.ccm.2016.04.008_bib73","doi-asserted-by":"crossref","first-page":"55","DOI":"10.1093\/humrep\/12.1.55","article-title":"Function and structure of cilia in the fallopian tube of an infertile woman with Kartagener's syndrome","volume":"12","author":"Halbert","year":"1997","journal-title":"Hum Reprod"},{"key":"10.1016\/j.ccm.2016.04.008_bib74","doi-asserted-by":"crossref","first-page":"660","DOI":"10.1016\/S0140-6736(12)60953-2","article-title":"Azithromycin for prevention of exacerbations in non-cystic fibrosis bronchiectasis (EMBRACE): a randomised, double-blind, placebo-controlled trial","volume":"380","author":"Wong","year":"2012","journal-title":"Lancet"},{"key":"10.1016\/j.ccm.2016.04.008_bib75","doi-asserted-by":"crossref","first-page":"356","DOI":"10.1016\/j.rmed.2011.11.018","article-title":"Outcomes of Pseudomonas eradication therapy in patients with non-cystic fibrosis bronchiectasis","volume":"106","author":"White","year":"2012","journal-title":"Respir Med"},{"key":"10.1016\/j.ccm.2016.04.008_bib76","doi-asserted-by":"crossref","first-page":"24","DOI":"10.2174\/157488712799363280","article-title":"Use of antibiotics in bronchiectasis","volume":"7","author":"King","year":"2012","journal-title":"Rev Recent Clin Trials"},{"key":"10.1016\/j.ccm.2016.04.008_bib77","doi-asserted-by":"crossref","first-page":"134605","DOI":"10.1155\/2012\/134605","article-title":"The role of macrolides in childhood non-cystic fibrosis-related bronchiectasis","volume":"2012","author":"Masekela","year":"2012","journal-title":"Mediators Inflamm"},{"key":"10.1016\/j.ccm.2016.04.008_bib78","first-page":"39","article-title":"Treatment recommendations in primary ciliary dyskinesia","volume":"18","author":"Polineni","year":"2016","journal-title":"Paediatr Respir Rev"},{"issue":"2","key":"10.1016\/j.ccm.2016.04.008_bib79","doi-asserted-by":"crossref","DOI":"10.1002\/14651858.CD001392.pub2","article-title":"Prolonged antibiotics for purulent bronchiectasis in children and adults","author":"Evans","year":"2007","journal-title":"Cochrane Database Syst Rev"},{"key":"10.1016\/j.ccm.2016.04.008_bib80","doi-asserted-by":"crossref","first-page":"491","DOI":"10.1164\/rccm.201005-0756OC","article-title":"A\u00a0randomized controlled trial of nebulized gentamicin in non-cystic fibrosis bronchiectasis","volume":"183","author":"Murray","year":"2011","journal-title":"Am J Respir Crit Care Med"},{"key":"10.1016\/j.ccm.2016.04.008_bib81","doi-asserted-by":"crossref","first-page":"144","DOI":"10.1164\/ajrccm.160.1.9806146","article-title":"Effect of aerosolized uridine-5'-triphosphate on airway clearance with cough in patients with primary ciliary dyskinesia","volume":"160","author":"Noone","year":"1999","journal-title":"Am J Respir Crit Care Med"},{"key":"10.1016\/j.ccm.2016.04.008_bib82","doi-asserted-by":"crossref","first-page":"1831","DOI":"10.1016\/j.rmed.2011.07.019","article-title":"Nebulised 7% hypertonic saline improves lung function and quality of life in bronchiectasis","volume":"105","author":"Kellett","year":"2011","journal-title":"Respir Med"},{"key":"10.1016\/j.ccm.2016.04.008_bib83","doi-asserted-by":"crossref","first-page":"1329","DOI":"10.1378\/chest.113.5.1329","article-title":"Treatment of idiopathic bronchiectasis with aerosolized recombinant human DNase I. rhDNase Study Group","volume":"113","author":"O'Donnell","year":"1998","journal-title":"Chest"},{"key":"10.1016\/j.ccm.2016.04.008_bib84","doi-asserted-by":"crossref","first-page":"1541","DOI":"10.1378\/chest.109.6.1541","article-title":"Is resection of bronchiectasis beneficial in patients with primary ciliary dyskinesia?","volume":"109","author":"Smit","year":"1996","journal-title":"Chest"},{"key":"10.1016\/j.ccm.2016.04.008_bib85","doi-asserted-by":"crossref","first-page":"375","DOI":"10.1183\/09031936.00216214","article-title":"A\u00a0quality-of-life measure for adults with primary ciliary dyskinesia: QOL-PCD","volume":"46","author":"Lucas","year":"2015","journal-title":"Eur Respir J"},{"key":"10.1016\/j.ccm.2016.04.008_bib86","doi-asserted-by":"crossref","first-page":"32","DOI":"10.1097\/ACI.0b013e32834eccc6","article-title":"Managing upper respiratory tract complications of primary ciliary dyskinesia in children","volume":"12","author":"Campbell","year":"2012","journal-title":"Curr Opin Allergy Clin Immunol"},{"key":"10.1016\/j.ccm.2016.04.008_bib87","doi-asserted-by":"crossref","first-page":"1264","DOI":"10.1183\/09031936.00176608","article-title":"Primary ciliary dyskinesia: a consensus statement on diagnostic and treatment approaches in children","volume":"34","author":"Barbato","year":"2009","journal-title":"Eur\u00a0Respir J"},{"key":"10.1016\/j.ccm.2016.04.008_bib88","doi-asserted-by":"crossref","first-page":"1121","DOI":"10.1001\/archoto.2010.183","article-title":"Otologic features in children with primary ciliary dyskinesia","volume":"136","author":"Pruliere-Escabasse","year":"2010","journal-title":"Arch Otolaryngol Head Neck Surg"},{"key":"10.1016\/j.ccm.2016.04.008_bib89","first-page":"189","article-title":"Primary ciliary dyskinesia: ultrastructural defects and clinical features","volume":"33","author":"Min","year":"1995","journal-title":"Rhinology"},{"key":"10.1016\/j.ccm.2016.04.008_bib90","doi-asserted-by":"crossref","first-page":"1269","DOI":"10.1288\/00005537-199311000-00010","article-title":"A\u00a0treatment for primary ciliary dyskinesia: efficacy of functional endoscopic sinus surgery","volume":"103","author":"Parsons","year":"1993","journal-title":"Laryngoscope"}],"container-title":["Clinics in Chest Medicine"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S0272523116300454?httpAccept=text\/xml","content-type":"text\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S0272523116300454?httpAccept=text\/plain","content-type":"text\/plain","content-version":"vor","intended-application":"text-mining"}],"deposited":{"date-parts":[[2025,10,3]],"date-time":"2025-10-03T00:52:20Z","timestamp":1759452740000},"score":1,"resource":{"primary":{"URL":"https:\/\/linkinghub.elsevier.com\/retrieve\/pii\/S0272523116300454"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2016,9]]},"references-count":90,"journal-issue":{"issue":"3","published-print":{"date-parts":[[2016,9]]}},"alternative-id":["S0272523116300454"],"URL":"https:\/\/doi.org\/10.1016\/j.ccm.2016.04.008","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":"Primary Ciliary Dyskinesia","name":"articletitle","label":"Article Title"},{"value":"Clinics in Chest Medicine","name":"journaltitle","label":"Journal Title"},{"value":"https:\/\/doi.org\/10.1016\/j.ccm.2016.04.008","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"}]}}