{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,6,30]],"date-time":"2026-06-30T07:22:34Z","timestamp":1782804154386,"version":"3.54.5"},"reference-count":45,"publisher":"Springer Science and Business Media LLC","issue":"1","content-domain":{"domain":["link.springer.com"],"crossmark-restriction":false},"short-container-title":["Ital J Pediatr"],"published-print":{"date-parts":[[2017,12]]},"DOI":"10.1186\/s13052-017-0351-2","type":"journal-article","created":{"date-parts":[[2017,4,11]],"date-time":"2017-04-11T23:48:01Z","timestamp":1491954481000},"update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":24,"title":["Lung structure and function similarities between primary ciliary dyskinesia and mild cystic fibrosis: a pilot study"],"prefix":"10.1186","volume":"43","author":[{"given":"Marco","family":"Maglione","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Silvia","family":"Montella","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Carmine","family":"Mollica","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Vincenzo","family":"Carnovale","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Paola","family":"Iacotucci","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Fabiola","family":"De Gregorio","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Antonella","family":"Tosco","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mariarosaria","family":"Cervasio","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Valeria","family":"Raia","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-9375-7429","authenticated-orcid":false,"given":"Francesca","family":"Santamaria","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2017,4,12]]},"reference":[{"key":"351_CR1","doi-asserted-by":"crossref","first-page":"850","DOI":"10.1136\/archdischild-2013-304831","volume":"99","author":"JS Lucas","year":"2014","unstructured":"Lucas JS, Burgess A, Mitchison HM, Moya E, Williamson M, Hogg C, National PCD, Service UK. Diagnosis and management of primary ciliary dyskinesia. Arch Dis Child. 2014;99:850\u20136.","journal-title":"Arch Dis Child"},{"key":"351_CR2","doi-asserted-by":"crossref","first-page":"115","DOI":"10.1002\/ppul.23304","volume":"51","author":"AJ Shapiro","year":"2016","unstructured":"Shapiro AJ, Zariwala MA, Ferkol T, Davis SD, Sagel SD, Dell SD, Genetic Disorders of Mucociliary Clearance Consortium, et al. Diagnosis, monitoring, and treatment of primary ciliary dyskinesia: PCD foundation consensus recommendations based on state of the art review. Pediatr Pulmonol. 2016;51:115\u201332.","journal-title":"Pediatr Pulmonol"},{"key":"351_CR3","doi-asserted-by":"crossref","first-page":"307","DOI":"10.1002\/ppul.1950200509","volume":"20","author":"M Desai","year":"1995","unstructured":"Desai M, Weller PH, Spencer DA. Clinical benefit from nebulized human recombinant DNase in Kartagener\u2019s syndrome. Pediatr Pulmonol. 1995;20:307\u20138.","journal-title":"Pediatr Pulmonol"},{"key":"351_CR4","doi-asserted-by":"crossref","first-page":"413","DOI":"10.1164\/ajrccm.154.2.8756815","volume":"154","author":"PJ Wills","year":"1996","unstructured":"Wills PJ, Wodehouse T, Corkery K, Mallon K, Wilson R, Cole PJ. Short-term recombinant human DNase in bronchiectasis. Effect on clinical state and in vitro sputum transportability. Am J Respir Crit Care Med. 1996;154:413\u20137.","journal-title":"Am J Respir Crit Care Med"},{"key":"351_CR5","doi-asserted-by":"crossref","first-page":"571","DOI":"10.1172\/JCI0215217","volume":"109","author":"MR Knowles","year":"2002","unstructured":"Knowles MR, Boucher RC. Mucus clearance as a primary innate defense mechanism for mammalian airways. J Clin Invest. 2002;109:571\u20137.","journal-title":"J Clin Invest"},{"key":"351_CR6","first-page":"39","volume":"18","author":"D Polineni","year":"2016","unstructured":"Polineni D, Davis SD, Dell SD. Treatment recommendations in Primary Ciliary Dyskinesia. Paediatr Respir Rev. 2016;18:39\u201345.","journal-title":"Paediatr Respir Rev"},{"key":"351_CR7","doi-asserted-by":"crossref","first-page":"948","DOI":"10.1183\/09031936.05.00035205","volume":"26","author":"R Pellegrino","year":"2005","unstructured":"Pellegrino R, Viegi G, Brusasco V, Crapo RO, Burgos F, Casaburi R, et al. Interpretative strategies for lung function tests. Eur Respir J. 2005;26:948\u201368.","journal-title":"Eur Respir J"},{"key":"351_CR8","doi-asserted-by":"crossref","first-page":"908","DOI":"10.1542\/peds.101.5.908","volume":"101","author":"F Santamaria","year":"1998","unstructured":"Santamaria F, Grillo G, Guidi G, Rotondo A, Raia V, de Ritis G, et al. Cystic fibrosis: when should high-resolution computed tomography of the chest Be obtained? Pediatrics. 1998;101:908\u201313.","journal-title":"Pediatrics"},{"key":"351_CR9","doi-asserted-by":"crossref","first-page":"480","DOI":"10.1378\/chest.130.2.480","volume":"130","author":"F Santamaria","year":"2006","unstructured":"Santamaria F, Montella S, Camera L, Palumbo C, Greco L, Boner AL. Lung structure abnormalities, but normal lung function in pediatric bronchiectasis. Chest. 2006;130:480\u20136.","journal-title":"Chest"},{"key":"351_CR10","doi-asserted-by":"crossref","first-page":"498","DOI":"10.1002\/ppul.21569","volume":"47","author":"M Maglione","year":"2012","unstructured":"Maglione M, Bush A, Montella S, Mollica C, Manna A, Esposito A, et al. Progression of lung disease in primary ciliary dyskinesia: is spirometry less accurate than CT? Pediatr Pulmonol. 2012;47:498\u2013504.","journal-title":"Pediatr Pulmonol"},{"key":"351_CR11","doi-asserted-by":"crossref","first-page":"1490","DOI":"10.1007\/s00247-014-2964-6","volume":"44","author":"HA Tiddens","year":"2014","unstructured":"Tiddens HA, Rosenow T. What did we learn from two decades of chest computed tomography in cystic fibrosis? Pediatr Radiol. 2014;44:1490\u20135.","journal-title":"Pediatr Radiol"},{"key":"351_CR12","doi-asserted-by":"crossref","first-page":"716","DOI":"10.1007\/s00330-006-0373-4","volume":"17","author":"M Puderbach","year":"2007","unstructured":"Puderbach M, Eichinger M, Gahr J, Ley S, Tuengerthal S, Schm\u00e4hl A, et al. Proton MRI appearance of cystic fibrosis: comparison to CT. Eur Radiol. 2007;17:716\u201324.","journal-title":"Eur Radiol"},{"key":"351_CR13","doi-asserted-by":"crossref","first-page":"532","DOI":"10.1097\/RLI.0b013e3181b4c1ba","volume":"44","author":"S Montella","year":"2009","unstructured":"Montella S, Santamaria F, Salvatore M, Pignata C, Maglione M, Iacotucci P, et al. Assessment of chest high-field magnetic resonance imaging in children and young adults with noncystic fibrosis chronic lung disease: comparison to high-resolution computed tomography and correlation with pulmonary function. Invest Radiol. 2009;44:532\u20138.","journal-title":"Invest Radiol"},{"key":"351_CR14","doi-asserted-by":"crossref","first-page":"1185","DOI":"10.1007\/s10875-013-9933-y","volume":"33","author":"S Montella","year":"2013","unstructured":"Montella S, Mollica C, Finocchi A, Pession A, Pietrogrande MC, Trizzino A, et al. Non invasive assessment of lung disease in ataxia telangiectasia by high-field magnetic resonance imaging. J Clin Immunol. 2013;33:1185\u201391.","journal-title":"J Clin Immunol"},{"key":"351_CR15","doi-asserted-by":"crossref","first-page":"738","DOI":"10.1378\/chest.13-1162","volume":"145","author":"M Cohen-Cymberknoh","year":"2014","unstructured":"Cohen-Cymberknoh M, Simanovsky N, Hiller N, Gileles Hillel A, Shoseyov D, Kerem E. Differences in disease expression between primary ciliary dyskinesia and cystic fibrosis with and without pancreatic insufficiency. Chest. 2014;145:738\u201344.","journal-title":"Chest"},{"key":"351_CR16","doi-asserted-by":"crossref","first-page":"780","DOI":"10.1164\/rccm.200512-1919OC","volume":"174","author":"MD Schluchter","year":"2006","unstructured":"Schluchter MD, Konstan MW, Drumm ML, Yankaskas JR, Knowles MR. Classifying severity of cystic fibrosis lung disease using longitudinal pulmonary function data. Am J Respir Crit Care Med. 2006;174:780\u20136.","journal-title":"Am J Respir Crit Care Med"},{"key":"351_CR17","doi-asserted-by":"crossref","first-page":"285","DOI":"10.1016\/j.jcf.2007.11.003","volume":"7","author":"R Soni","year":"2008","unstructured":"Soni R, Dobbin CJ, Milross MA, Young IH, Bye PP. Gas exchange in stable patients with moderate-to-severe lung disease from cystic fibrosis. J Cyst Fibros. 2008;7:285\u201391.","journal-title":"J Cyst Fibros"},{"key":"351_CR18","doi-asserted-by":"crossref","first-page":"589","DOI":"10.1016\/S0022-3476(98)70344-0","volume":"132","author":"BJ Rosenstein","year":"1998","unstructured":"Rosenstein BJ, Cutting GR. The diagnosis of cystic fibrosis: a consensus statement. Cystic Fibrosis Foundation Consensus Panel. J Pediatr. 1998;132:589\u201395.","journal-title":"J Pediatr"},{"key":"351_CR19","doi-asserted-by":"crossref","first-page":"663","DOI":"10.1586\/ers.12.60","volume":"6","author":"A Bush","year":"2012","unstructured":"Bush A, Hogg C. Primary ciliary dyskinesia: recent advances in epidemiology, diagnosis, management and relationship with the expanding spectrum of ciliopathy. Expert Rev Respir Med. 2012;6:663\u201382.","journal-title":"Expert Rev Respir Med"},{"key":"351_CR20","doi-asserted-by":"crossref","first-page":"1601090","DOI":"10.1183\/13993003.01090-2016","volume":"49","author":"JS Lucas","year":"2017","unstructured":"Lucas JS, Barbato A, Collins SA, Goutaki M, Behan L, Caudri D, et al. European Respiratory Society guidelines for the diagnosis of primary ciliary dyskinesia. Eur Respir J. 2017;49:1601090. https:\/\/doi.org\/10.1183\/13993003.01090-2016 .","journal-title":"Eur Respir J"},{"key":"351_CR21","first-page":"92","volume":"15","author":"HA Tiddens","year":"2014","unstructured":"Tiddens HA, Stick SM, Davis S. Multi-modality monitoring of cystic fibrosis lung disease: the role of chest computed tomography. Paediatr Respir Rev. 2014;15:92\u20137.","journal-title":"Paediatr Respir Rev"},{"key":"351_CR22","doi-asserted-by":"crossref","first-page":"319","DOI":"10.1183\/09031936.05.00034805","volume":"26","author":"MR Miller","year":"2005","unstructured":"Miller MR, Hankinson J, Brusasco V, Burgos F, Casaburi R, Coates A, ATS\/ERS Task Force, et al. Standardisation of spirometry. Eur Respir J. 2005;26:319\u201338.","journal-title":"Eur Respir J"},{"key":"351_CR23","doi-asserted-by":"crossref","first-page":"1324","DOI":"10.1183\/09031936.00080312","volume":"40","author":"PH Quanjer","year":"2012","unstructured":"Quanjer PH, Stanojevic S, Cole TJ, Baur X, Hall GL, Culver BH, ERS Global Lung Function Initiative, et al. Multi-ethnic reference values for spirometry for the 3-95-yr age range: the global lung function 2012 equations. Eur Respir J. 2012;40:1324\u201343.","journal-title":"Eur Respir J"},{"key":"351_CR24","doi-asserted-by":"crossref","first-page":"68","DOI":"10.1136\/thorax.58.1.68","volume":"58","author":"I Horv\u00e1th","year":"2003","unstructured":"Horv\u00e1th I, Loukides S, Wodehouse T, Csisz\u00e9r E, Cole PJ, Kharitonov SA, et al. Comparison of exhaled and nasal nitric oxide and exhaled carbon monoxide levels in bronchiectatic patients with and without primary ciliary dyskinesia. Thorax. 2003;58:68\u201372.","journal-title":"Thorax"},{"key":"351_CR25","doi-asserted-by":"crossref","first-page":"351","DOI":"10.1378\/chest.07-2812","volume":"134","author":"F Santamaria","year":"2008","unstructured":"Santamaria F, Montella S, Tiddens HA, Guidi G, Casotti V, Maglione M, et al. Structural and functional lung disease in primary ciliary dyskinesia. Chest. 2008;134:351\u20137.","journal-title":"Chest"},{"key":"351_CR26","doi-asserted-by":"crossref","first-page":"163","DOI":"10.1089\/pai.2009.0022","volume":"22","author":"KJ Mackerness","year":"2009","unstructured":"Mackerness KJ, Jose PJ, Bush A. Differences in airway inflammation in cystic fibrosis and primary ciliary dyskinesia. Pediatr Asthma Allergy Immunol. 2009;22:163\u20138.","journal-title":"Pediatr Asthma Allergy Immunol"},{"key":"351_CR27","doi-asserted-by":"crossref","first-page":"545","DOI":"10.1164\/rccm.201304-0800OC","volume":"188","author":"SJ Irving","year":"2013","unstructured":"Irving SJ, Ives A, Davies G, Donovan J, Edey AJ, Gill SS, et al. Lung clearance index and high-resolution computed tomography scores in primary ciliary dyskinesia. Am J Respir Crit Care Med. 2013;188:545\u20139.","journal-title":"Am J Respir Crit Care Med"},{"key":"351_CR28","doi-asserted-by":"crossref","first-page":"229","DOI":"10.1164\/rccm.201402-0249LE","volume":"190","author":"P Montuschi","year":"2014","unstructured":"Montuschi P, Paris D, Montella S, Melck D, Mirra V, Santini G, et al. Nuclear magnetic resonance-based metabolomics discriminates primary ciliary dyskinesia from cystic fibrosis. Am J Respir Crit Care Med. 2014;190:229\u201333.","journal-title":"Am J Respir Crit Care Med"},{"key":"351_CR29","doi-asserted-by":"crossref","first-page":"e115584","DOI":"10.1371\/journal.pone.0115584","volume":"9","author":"O Joensen","year":"2014","unstructured":"Joensen O, Paff T, Haarman EG, Skovgaard IM, Jensen P\u00d8, Bjarnsholt T, et al. Exhaled breath analysis using electronic nose in cystic fibrosis and primary ciliary dyskinesia patients with chronic pulmonary infections. PLoS One. 2014;9:e115584.","journal-title":"PLoS One"},{"key":"351_CR30","doi-asserted-by":"crossref","first-page":"1248","DOI":"10.1183\/09031936.00001010","volume":"36","author":"CE Kuehni","year":"2010","unstructured":"Kuehni CE, Frischer T, Strippoli MP, Maurer E, Bush A, Nielsen KG, ERS Task Force on Primary Ciliary Dyskinesia in Children, et al. Factors influencing age at diagnosis of primary ciliary dyskinesia in European children. Eur Respir J. 2010;36:1248\u201358.","journal-title":"Eur Respir J"},{"key":"351_CR31","doi-asserted-by":"crossref","first-page":"1262","DOI":"10.1164\/rccm.200811-1731OC","volume":"181","author":"JK Marthin","year":"2010","unstructured":"Marthin JK, Petersen N, Skovgaard LT, Nielsen KG. Lung function in patients with primary ciliary dyskinesia: a cross-sectional and 3-decade longitudinal study. Am J Respir Crit Care Med. 2010;181:1262\u20138.","journal-title":"Am J Respir Crit Care Med"},{"key":"351_CR32","doi-asserted-by":"crossref","first-page":"1243","DOI":"10.1002\/ppul.22984","volume":"49","author":"M Maglione","year":"2014","unstructured":"Maglione M, Bush A, Nielsen KG, Hogg C, Montella S, Marthin JK, et al. Multicenter analysis of body mass index, lung function, and sputum microbiology in primary ciliary dyskinesia. Pediatr Pulmonol. 2014;49:1243\u201350.","journal-title":"Pediatr Pulmonol"},{"key":"351_CR33","doi-asserted-by":"crossref","first-page":"441","DOI":"10.1183\/13993003.00209-2016","volume":"48","author":"A Shah","year":"2016","unstructured":"Shah A, Shoemark A, MacNeill SJ, Bhaludin B, Rogers A, Bilton D, et al. A longitudinal study characterising a large adult primary ciliary dyskinesia population. Eur Respir J. 2016;48:441\u201350.","journal-title":"Eur Respir J"},{"key":"351_CR34","doi-asserted-by":"crossref","first-page":"10260","DOI":"10.1073\/pnas.1106695108","volume":"108","author":"OA Itani","year":"2011","unstructured":"Itani OA, Chen JH, Karp PH, Ernst S, Keshavjee S, Parekh K, et al. Human cystic fibrosis airway epithelia have reduced Cl- conductance but not increased Na\u2009+\u2009conductance. Proc Natl Acad Sci U S A. 2011;108:10260\u20135.","journal-title":"Proc Natl Acad Sci U S A"},{"key":"351_CR35","doi-asserted-by":"crossref","first-page":"116","DOI":"10.1080\/01926230601060025","volume":"35","author":"A Livraghi","year":"2007","unstructured":"Livraghi A, Randell SH. Cystic fibrosis and other respiratory diseases of impaired mucus clearance. Toxicol Pathol. 2007;35:116\u201329.","journal-title":"Toxicol Pathol"},{"key":"351_CR36","doi-asserted-by":"crossref","first-page":"1992","DOI":"10.1056\/NEJMra043184","volume":"352","author":"SM Rowe","year":"2005","unstructured":"Rowe SM, Miller S, Sorscher EJ. Cystic fibrosis. N Engl J Med. 2005;352:1992\u20132001.","journal-title":"N Engl J Med"},{"key":"351_CR37","doi-asserted-by":"crossref","first-page":"118","DOI":"10.1378\/chest.129.1.118","volume":"129","author":"A Bush","year":"2006","unstructured":"Bush A, Payne D, Pike S, Jenkins G, Henke MO, Rubin BK. Mucus properties in children with primary ciliary dyskinesia: comparison with cystic fibrosis. Chest. 2006;129:118\u201323.","journal-title":"Chest"},{"key":"351_CR38","doi-asserted-by":"crossref","first-page":"787","DOI":"10.1183\/09031936.00051509","volume":"35","author":"M Pifferi","year":"2010","unstructured":"Pifferi M, Bush A, Di Cicco M, Pradal U, Ragazzo V, Macchia P, et al. Health-related quality of life and unmet needs in patients with primary ciliary dyskinesia. Eur Respir J. 2010;35:787\u201394.","journal-title":"Eur Respir J"},{"key":"351_CR39","doi-asserted-by":"crossref","first-page":"1224","DOI":"10.1002\/ppul.23307","volume":"50","author":"A Shakkottai","year":"2015","unstructured":"Shakkottai A, Kidwell KM, Townsend M, Nasr SZ. A five-year retrospective analysis of adherence in cystic fibrosis. Pediatr Pulmonol. 2015;50:1224\u20139.","journal-title":"Pediatr Pulmonol"},{"key":"351_CR40","doi-asserted-by":"crossref","first-page":"1482","DOI":"10.1183\/09031936.00073911","volume":"39","author":"MP Strippoli","year":"2012","unstructured":"Strippoli MP, Frischer T, Barbato A, Snijders D, Maurer E, Lucas JS, ERS Task Force on Primary Ciliary Dyskinesia in Children, et al. Management of primary ciliary dyskinesia in European children: recommendations and clinical practice. Eur Respir J. 2012;39:1482\u201391.","journal-title":"Eur Respir J"},{"key":"351_CR41","doi-asserted-by":"crossref","first-page":"316","DOI":"10.1164\/rccm.201409-1672OC","volume":"191","author":"SD Davis","year":"2015","unstructured":"Davis SD, Ferkol TW, Rosenfeld M, Lee HS, Dell SD, Sagel SD, et al. Clinical features of childhood primary ciliary dyskinesia by genotype and ultrastructural phenotype. Am J Respir Crit Care Med. 2015;191:316\u201324.","journal-title":"Am J Respir Crit Care Med"},{"key":"351_CR42","doi-asserted-by":"crossref","first-page":"80","DOI":"10.1136\/thx.2005.045146","volume":"61","author":"PA Jong de","year":"2006","unstructured":"de Jong PA, Lindblad A, Rubin L, Hop WC, de Jongste JC, Brink M, et al. Progression of lung disease on computed tomography and pulmonary function tests in children and adults with cystic fibrosis. Thorax. 2006;61:80\u20135.","journal-title":"Thorax"},{"key":"351_CR43","doi-asserted-by":"crossref","first-page":"2277","DOI":"10.1056\/NEJMra072149","volume":"357","author":"DJ Brenner","year":"2007","unstructured":"Brenner DJ, Hall EJ. Computed tomography--an increasing source of radiation exposure. N Engl J Med. 2007;357:2277\u201384.","journal-title":"N Engl J Med"},{"key":"351_CR44","doi-asserted-by":"crossref","first-page":"87","DOI":"10.1111\/j.1440-1843.2011.02067.x","volume":"17","author":"S Montella","year":"2012","unstructured":"Montella S, Maglione M, Bruzzese D, Mollica C, Pignata C, Aloj G, et al. Magnetic resonance imaging is an accurate and reliable method to evaluate non-cystic fibrosis paediatric lung disease. Respirology. 2012;17:87\u201391.","journal-title":"Respirology"},{"key":"351_CR45","doi-asserted-by":"crossref","first-page":"3","DOI":"10.1111\/j.1440-1843.2011.02079.x","volume":"17","author":"AM Owrangi","year":"2012","unstructured":"Owrangi AM, Parraga G. Chest MRI in children: why bother? Respirology. 2012;17:3\u20134.","journal-title":"Respirology"}],"container-title":["Italian Journal of Pediatrics"],"original-title":[],"language":"en","link":[{"URL":"http:\/\/link.springer.com\/content\/pdf\/10.1186\/s13052-017-0351-2.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2017,6,25]],"date-time":"2017-06-25T10:41:16Z","timestamp":1498387276000},"score":1,"resource":{"primary":{"URL":"http:\/\/ijponline.biomedcentral.com\/articles\/10.1186\/s13052-017-0351-2"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2017,4,12]]},"references-count":45,"journal-issue":{"issue":"1","published-print":{"date-parts":[[2017,12]]}},"alternative-id":["351"],"URL":"https:\/\/doi.org\/10.1186\/s13052-017-0351-2","relation":{},"ISSN":["1824-7288"],"issn-type":[{"value":"1824-7288","type":"electronic"}],"subject":[],"published":{"date-parts":[[2017,4,12]]},"article-number":"34"}}