{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,3,7]],"date-time":"2026-03-07T14:32:25Z","timestamp":1772893945097,"version":"3.50.1"},"reference-count":49,"publisher":"Springer Science and Business Media LLC","issue":"6","license":[{"start":{"date-parts":[[2006,11,1]],"date-time":"2006-11-01T00:00:00Z","timestamp":1162339200000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.springer.com\/tdm"},{"start":{"date-parts":[[2006,11,1]],"date-time":"2006-11-01T00:00:00Z","timestamp":1162339200000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/www.springer.com\/tdm"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Curr Allergy Asthma Rep"],"published-print":{"date-parts":[[2006,11]]},"DOI":"10.1007\/s11882-006-0030-7","type":"journal-article","created":{"date-parts":[[2007,6,3]],"date-time":"2007-06-03T00:27:45Z","timestamp":1180830465000},"page":"513-517","source":"Crossref","is-referenced-by-count":8,"title":["Primary ciliary dyskinesia and upper airway diseases"],"prefix":"10.1007","volume":"6","author":[{"given":"Marcus P.","family":"Kennedy","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Lawrence E.","family":"Ostrowski","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"297","reference":[{"key":"30_CR1","doi-asserted-by":"crossref","first-page":"498","DOI":"10.1007\/BF02141468","volume":"83","author":"M Kartagener","year":"1933","unstructured":"Kartagener M: Zur pathogenese der bronchiectasien. I Mitteilung: Bronchiectasien bei situs viscerum inversus. Beitr Klin Tuberk 1933, 83:498\u2013501.","journal-title":"Beitr Klin Tuberk"},{"key":"30_CR2","doi-asserted-by":"publisher","first-page":"1","DOI":"10.1056\/NEJM197707072970101","volume":"196","author":"R Eliasson","year":"1977","unstructured":"Eliasson R, Mossberg B, Camner P, Afzelius BA: The immotile cilia syndrome: a congenital ciliary abnormality as an etiologic factor in chronic airway infections and male sterility. N Engl J Med 1977, 196:1\u20136.","journal-title":"N Engl J Med"},{"key":"30_CR3","doi-asserted-by":"publisher","first-page":"476","DOI":"10.1016\/S0140-6736(81)90811-4","volume":"2","author":"MA Sleigh","year":"1981","unstructured":"Sleigh MA: Primary ciliary dyskinesia. Lancet 1981, 2:476.","journal-title":"Lancet"},{"key":"30_CR4","doi-asserted-by":"crossref","unstructured":"Badano JL, Mitsuma N, Beales PL, Katsanis N: The ciliopathies:an emerging class of human genetic disorders. Annu Rev Genomics Hum Genet 2006 [Epub ahead of print].","DOI":"10.1146\/annurev.genom.7.080505.115610"},{"key":"30_CR5","doi-asserted-by":"publisher","first-page":"2528","DOI":"10.1097\/01.ASN.0000141055.57643.E0","volume":"15","author":"GJ Pazour","year":"2004","unstructured":"Pazour GJ: Intraflagellar transport and cilia-dependent renal disease: the ciliary hypothesis of polycystic kidney disease. J Am Soc Nephrol 2004, 15:2528\u20132536.","journal-title":"J Am Soc Nephrol"},{"key":"30_CR6","doi-asserted-by":"publisher","first-page":"628","DOI":"10.1038\/nature02030","volume":"425","author":"SJ Ansley","year":"2003","unstructured":"Ansley SJ, Badano JL, Blacque OE, et al.: Basal body dysfunction is a likely cause of pleiotropic Bardet-Biedl syndrome. Nature 2003, 425:628\u2013633.","journal-title":"Nature"},{"key":"30_CR7","doi-asserted-by":"crossref","first-page":"56","DOI":"10.1378\/chest.61.1.56","volume":"61","author":"K Katsuhara","year":"1972","unstructured":"Katsuhara K, Kawamoto S, Wakabayashi T, Belsky JL: Situs inversus totalis and Kartagener\u2019s syndrome in a Japanese population. Chest 1972, 61:56\u201361.","journal-title":"Chest"},{"key":"30_CR8","first-page":"361","volume":"2","author":"J Torgersen","year":"1950","unstructured":"Torgersen J: Situs inversus, asymmetry, and twinning. Am J Hum Genet 1950, 2:361\u2013370.","journal-title":"Am J Hum Genet"},{"key":"30_CR9","doi-asserted-by":"publisher","first-page":"451","DOI":"10.1074\/mcp.M200037-MCP200","volume":"1","author":"LE Ostrowski","year":"2002","unstructured":"Ostrowski LE, Blackburn K, Radde KM, et al.: A proteomic analysis of human cilia: identification of novel components. Mol Cell Proteomics 2002, 1:451\u2013465.","journal-title":"Mol Cell Proteomics"},{"key":"30_CR10","doi-asserted-by":"publisher","first-page":"158","DOI":"10.1002\/ppul.20329","volume":"41","author":"B Mahut","year":"2006","unstructured":"Mahut B, Escudier E, de Blic J, et al.: Impairment of nitric oxide output of conducting airways in primary ciliary dyskinesia. Pediatr Pulmonol 2006, 41:158\u2013163.","journal-title":"Pediatr Pulmonol"},{"key":"30_CR11","doi-asserted-by":"publisher","first-page":"459","DOI":"10.1164\/rccm.200303-365OC","volume":"169","author":"PG Noone","year":"2004","unstructured":"Noone PG, Leigh MW, Sannuti A, et al.: Primary ciliary dyskinesia: diagnostic and phenotypic features. Am J Respir Crit Care Med 2004, 169:459\u2013467. Largest cohort of PCD (N = 78) patients analyzed to date with analysis of clinical presentation and diagnostic tests.","journal-title":"Am J Respir Crit Care Med"},{"key":"30_CR12","doi-asserted-by":"publisher","first-page":"1402","DOI":"10.1183\/09031936.99.13614069","volume":"13","author":"B Karadag","year":"1999","unstructured":"Karadag B, James AJ, Gultekin E, et al.: Nasal and lower airway level of nitric oxide in children with primary ciliary dyskinesia. Eur Respir J 1999, 13:1402\u20131405.","journal-title":"Eur Respir J"},{"key":"30_CR13","doi-asserted-by":"publisher","first-page":"1030","DOI":"10.1086\/319511","volume":"68","author":"C Guichard","year":"2001","unstructured":"Guichard C, Harricane MC, Lafitte JJ, et al.: Axonemal dynein intermediate-chain gene (DNAI1) mutations result in situs inversus and primary ciliary dyskinesia (Kartagener syndrome). Am J Hum Genet 2001, 68:1030\u20131035.","journal-title":"Am J Hum Genet"},{"key":"30_CR14","doi-asserted-by":"publisher","first-page":"1508","DOI":"10.1086\/302683","volume":"65","author":"G Pennarun","year":"1999","unstructured":"Pennarun G, Escudier E, Chapelin C, et al.: Loss-of-function mutations in a human gene related to Chlamydomonas reinhardtii dynein IC78 result in primary ciliary dyskinesia. Am J Hum Genet 1999, 65:1508\u20131519.","journal-title":"Am J Hum Genet"},{"key":"30_CR15","doi-asserted-by":"crossref","first-page":"577","DOI":"10.1165\/ajrcmb.25.5.4619","volume":"25","author":"M Zariwala","year":"2001","unstructured":"Zariwala M, Noone PG, Sannuti A, et al.: Germline mutations in an intermediate chain dynein cause primary ciliary dyskinesia. Am J Respir Cell Mol Biol 2001, 25:577\u2013583.","journal-title":"Am J Respir Cell Mol Biol"},{"key":"30_CR16","doi-asserted-by":"publisher","first-page":"120","DOI":"10.1164\/rccm.200601-084OC","volume":"174","author":"N Hornef","year":"2006","unstructured":"Hornef N, Olbrich H, Horvath J, et al.: DNAH5 mutations are a common cause of primary ciliary dyskinesia with outer dynein arm defects. Am J Respir Crit Care Med 2006, 174:120\u2013126.","journal-title":"Am J Respir Crit Care Med"},{"key":"30_CR17","doi-asserted-by":"publisher","first-page":"143","DOI":"10.1038\/ng817","volume":"30","author":"H Olbrich","year":"2002","unstructured":"Olbrich H, Haffner K, Kispert A, et al.: Mutations in DNAH5 cause primary ciliary dyskinesia and randomization of left-right asymmetry. Nat Genet 2002, 30:143\u2013144.","journal-title":"Nat Genet"},{"key":"30_CR18","unstructured":"Zariwala M, Leigh MW, Ceppa F, et al.: Mutations of DNAI1 in primary ciliary dyskinesia: evidence of founder effects in a common mutation. Am J Respir Crit Care Med, In press."},{"key":"30_CR19","doi-asserted-by":"publisher","first-page":"667","DOI":"10.1080\/080352502760069089","volume":"91","author":"ME Coren","year":"2002","unstructured":"Coren ME, Meeks M, Morrison I, et al.: Primary ciliary dyskinesia: age at diagnosis and symptom history. Acta Paediatr 2002, 91:667\u2013669.","journal-title":"Acta Paediatr"},{"key":"30_CR20","first-page":"193","volume":"79","author":"M Kartagener","year":"1962","unstructured":"Kartagener M, Stucki P: Bronchiectasis with situs inversus. Arch Pediatr 1962, 79:193\u2013207.","journal-title":"Arch Pediatr"},{"key":"30_CR21","doi-asserted-by":"publisher","first-page":"653","DOI":"10.1055\/s-2004-815661","volume":"24","author":"MW Leigh","year":"2003","unstructured":"Leigh MW: Primary ciliary dyskinesia. Semin Respir Crit Care Med 2003, 24:653\u2013662. Excellent review of pathophysiology, diagnosis, and management of PCD.","journal-title":"Semin Respir Crit Care Med"},{"key":"30_CR22","doi-asserted-by":"publisher","first-page":"439","DOI":"10.1080\/07853890510011985","volume":"37","author":"KS van\u2019s Gravesande","year":"2005","unstructured":"van\u2019s Gravesande KS, Omran H: Primary ciliary dyskinesia: clinical presentation, diagnosis and genetics. Ann Med 2005, 37:439\u2013449.","journal-title":"Ann Med"},{"key":"30_CR23","doi-asserted-by":"publisher","first-page":"857","DOI":"10.1007\/PL00008354","volume":"159","author":"D Holzmann","year":"2000","unstructured":"Holzmann D, Felix H: Neonatal respiratory distress syndrome \u2014a sign of primary ciliary dyskinesia?\n                           Eur J Pediatr 2000, 159:857\u2013860.","journal-title":"Eur J Pediatr"},{"key":"30_CR24","doi-asserted-by":"crossref","first-page":"651","DOI":"10.1148\/radiology.154.3.3969467","volume":"154","author":"HR Nadel","year":"1985","unstructured":"Nadel HR, Stringer DA, Levison H, J et al.: The immotile cilia syndrome: radiological manifestations. Radiology 1985, 154:651\u2013655.","journal-title":"Radiology"},{"key":"30_CR25","first-page":"399","volume":"3","author":"MP Kennedy","year":"2006","unstructured":"Kennedy MP, Leigh MW, Dell S, et al.: Primary ciliary dyskinesia and situs ambiguus\/heterotaxy: organ laterality defects other than situs inversus totalis. Proc Am Thorac Soc 2006, 3:399","journal-title":"Proc Am Thorac Soc"},{"key":"30_CR26","doi-asserted-by":"publisher","first-page":"155","DOI":"10.1002\/(SICI)1096-8628(19990115)82:2<155::AID-AJMG11>3.0.CO;2-T","volume":"82","author":"PG Noone","year":"1999","unstructured":"Noone PG, Bali D, Carson JL, et al.: Discordant organ laterality in monozygotic twins with primary ciliary dyskinesia. Am J Med Genet 1999, 82:155\u2013160.","journal-title":"Am J Med Genet"},{"key":"30_CR27","doi-asserted-by":"publisher","first-page":"96","DOI":"10.1038\/nature00849","volume":"418","author":"S Nonaka","year":"2002","unstructured":"Nonaka S, Shiratori H, Saijoh Y, Hamada H: Determination of left-right patterning of the mouse embryo by artificial nodal flow. Nature 2002, 418:96\u201399.","journal-title":"Nature"},{"key":"30_CR28","doi-asserted-by":"publisher","first-page":"829","DOI":"10.1016\/S0092-8674(00)81705-5","volume":"95","author":"S Nonaka","year":"1998","unstructured":"Nonaka S, Tanaka Y, Okada Y, et al.: Randomization of left-right asymmetry due to loss of nodal cilia generating leftward flow of extraembryonic fluid in mice lacking KIF3B motor protein. Cell 1998, 95:829\u2013837.","journal-title":"Cell"},{"key":"30_CR29","doi-asserted-by":"publisher","first-page":"9","DOI":"10.1002\/ppul.1950160103","volume":"16","author":"VG Engesaeth","year":"1993","unstructured":"Engesaeth VG, Warner JO, Bush A: New associations of primary ciliary dyskinesia syndrome. Pediatr Pulmonol 1993, 16:9\u201312.","journal-title":"Pediatr Pulmonol"},{"key":"30_CR30","doi-asserted-by":"publisher","first-page":"427","DOI":"10.1159\/000086260","volume":"72","author":"K Chmura","year":"2005","unstructured":"Chmura K, Chan ED, Noone PG, et al.: A middle-aged woman with recurrent respiratory infections. Respiration 2005, 72:427\u2013430.","journal-title":"Respiration"},{"key":"30_CR31","doi-asserted-by":"publisher","first-page":"401","DOI":"10.1016\/S0022-3476(82)80439-3","volume":"100","author":"DV Schidlow","year":"1982","unstructured":"Schidlow DV, Katz SM, Turtz MG, et al.: Polysplenia and Kartagener syndromes in a sibship: association with abnormal respiratory cilia. J Pediatr 1982, 100:401\u2013403.","journal-title":"J Pediatr"},{"key":"30_CR32","doi-asserted-by":"publisher","first-page":"399","DOI":"10.1016\/S0022-3476(82)80438-1","volume":"100","author":"S Teichberg","year":"1982","unstructured":"Teichberg S, Markowitz J, Silverberg M, et al.: Abnormal cilia in a child with the polysplenia syndrome and extrahepatic biliary atresia. J Pediatr 1982, 100:399\u2013401.","journal-title":"J Pediatr"},{"key":"30_CR33","first-page":"A390","volume":"2","author":"MP Kennedy","year":"2005","unstructured":"Kennedy MP, Noone PG, Leigh MW, et al.: High-resolution computed tomographic imaging in patients with primary ciliary dyskinesia (PCD). Proc Am Thorac Soc 2005, 2:A390.","journal-title":"Proc Am Thorac Soc"},{"key":"30_CR34","doi-asserted-by":"publisher","first-page":"97","DOI":"10.1007\/BF02388082","volume":"17","author":"S de la Rocha Reyes","year":"1987","unstructured":"Reyes de la Rocha S, Pysher TJ, Leonard JC: Dyskinetic cilia syndrome: clinical, radiographic and scintigraphic findings. Pediatr Radiol 1987, 17:97\u2013103.","journal-title":"Pediatr Radiol"},{"key":"30_CR35","doi-asserted-by":"crossref","unstructured":"Kennedy MP, Noone PG, Carson J, et al.: Calcium stone lithoptysis in primary ciliary dyskinesia. Respir Med 2006 [Epub ahead of print].","DOI":"10.1016\/j.rmed.2006.04.007"},{"key":"30_CR36","doi-asserted-by":"publisher","first-page":"684","DOI":"10.1136\/thx.49.7.684","volume":"49","author":"NC Munro","year":"1994","unstructured":"Munro NC, Currie DC, Lindsay KS, et al.: Fertility in men with primary ciliary dyskinesia presenting with respiratory infection. Thorax 1994, 49:684\u2013687.","journal-title":"Thorax"},{"key":"30_CR37","doi-asserted-by":"publisher","first-page":"5329","DOI":"10.1242\/dev.02153","volume":"132","author":"B Banizs","year":"2005","unstructured":"Banizs B, Pike MM, Millican CL, et al.: Dysfunctional cilia lead to altered ependyma and choroid plexus function, and result in the formation of hydrocephalus. Development 2005, 132:5329\u20135339.","journal-title":"Development"},{"key":"30_CR38","doi-asserted-by":"publisher","first-page":"2133","DOI":"10.1093\/hmg\/ddh219","volume":"13","author":"I Ibanez-Tallon","year":"2004","unstructured":"Ibanez-Tallon I, Pagenstecher A, Fliegauf M, et al.: Dysfunction of axonemal dynein heavy chain Mdnah5 inhibits ependymal flow and reveals a novel mechanism for hydrocephalus formation. Hum Mol Genet 2004, 13:2133\u20132141.","journal-title":"Hum Mol Genet"},{"key":"30_CR39","doi-asserted-by":"publisher","first-page":"1343","DOI":"10.1164\/rccm.200411-1583OC","volume":"171","author":"M Fliegauf","year":"2005","unstructured":"Fliegauf M, Olbrich H, Horvath J, et al.: Mislocalization of DNAH5 and DNAH9 in respiratory cells from patients with primary ciliary dyskinesia. Am J Respir Crit Care Med 2005, 171:1343\u20131349.","journal-title":"Am J Respir Crit Care Med"},{"key":"30_CR40","doi-asserted-by":"publisher","first-page":"418","DOI":"10.1203\/01.pdr.0000200809.21364.e2","volume":"59","author":"H Olbrich","year":"2006","unstructured":"Olbrich H, Horvath J, Fekete A, et al.: Axonemal localization of the dynein component DNAH5 is not altered in secondary ciliary dyskinesia. Pediatr Res 2006, 59:418\u2013422.","journal-title":"Pediatr Res"},{"key":"30_CR41","doi-asserted-by":"publisher","first-page":"586","DOI":"10.1136\/thorax.57.7.586","volume":"57","author":"I Narang","year":"2002","unstructured":"Narang I, Ersu R, Wilson NM, Bush A: Nitric oxide in chronic airway inflammation in children: diagnostic use and pathophysiological significance. Thorax 2002, 57:586\u2013589.","journal-title":"Thorax"},{"key":"30_CR42","doi-asserted-by":"publisher","first-page":"281","DOI":"10.1164\/rccm.200207-640OC","volume":"168","author":"M Deja","year":"2003","unstructured":"Deja M, Busch T, Bachmann S, et al.: Reduced nitric oxide in sinus epithelium of patients with radiologic maxillary sinusitis and sepsis. Am J Respir Crit Care Med 2003, 168:281\u2013286.","journal-title":"Am J Respir Crit Care Med"},{"key":"30_CR43","doi-asserted-by":"publisher","first-page":"1699","DOI":"10.1378\/chest.128.3.1699","volume":"128","author":"B Degano","year":"2005","unstructured":"Degano B, Genestal M, Serrano E, et al.: Effect of treatment on maxillary sinus and nasal nitric oxide concentrations in patients with nosocomial maxillary sinusitis. Chest 2005, 128:1699\u20131705.","journal-title":"Chest"},{"key":"30_CR44","doi-asserted-by":"publisher","first-page":"982","DOI":"10.1183\/09031936.98.12040982","volume":"12","author":"A Bush","year":"1998","unstructured":"Bush A, Cole P, Hariri M, et al.: Primary ciliary dyskinesia: diagnosis and standards of care. Eur Respir J 1998, 12:982\u2013988.","journal-title":"Eur Respir J"},{"key":"30_CR45","doi-asserted-by":"crossref","first-page":"1329","DOI":"10.1378\/chest.113.5.1329","volume":"113","author":"AE O\u2019Donnell","year":"1998","unstructured":"O\u2019Donnell AE, Barker AF, Ilowite JS, Fick RB: Treatment of idiopathic bronchiectasis with aerosolized recombinant human DNase I. rhDNase Study Group. Chest 1998, 113:1329\u20131334.","journal-title":"Chest"},{"key":"30_CR46","first-page":"371","volume":"352","author":"S Loukides","year":"1998","unstructured":"Loukides S, Kharitonov S, Wodehouse T, et al.: Effect of arginine on mucociliary function in primary ciliary dyskinesia. 1998, 352:371\u2013372.","journal-title":"Effect of arginine on mucociliary function in primary ciliary dyskinesia"},{"key":"30_CR47","doi-asserted-by":"publisher","first-page":"1061","DOI":"10.1016\/j.ijporl.2005.02.013","volume":"69","author":"A Majithia","year":"2005","unstructured":"Majithia A, Fong J, Hariri M, Harcourt J: Hearing outcomes in children with primary ciliary dyskinesia\u2014a longitudinal study. Int J Pediatr Otorhinolaryngol 2005, 69:1061\u20131064.","journal-title":"Int J Pediatr Otorhinolaryngol"},{"key":"30_CR48","first-page":"96","volume":"127","author":"N Mygind","year":"1983","unstructured":"Mygind N, Pedersen M: Nose-, sinus- and ear-symptoms in 27 patients with primary ciliary dyskinesia. Eur J Respir Dis Suppl 1983, 127:96\u2013101.","journal-title":"Eur J Respir Dis Suppl"},{"issue":"5 Suppl","key":"30_CR49","doi-asserted-by":"publisher","first-page":"S147","DOI":"10.1067\/mai.2001.118891","volume":"108","author":"J Bousquet","year":"2001","unstructured":"Bousquet J, Van Cauwenberge P, Khaltaev N: Allergic rhinitis and its impact on asthma. J Allergy Clin Immunol 2001, 108(5 Suppl):S147-S334.","journal-title":"J Allergy Clin Immunol"}],"container-title":["Current Allergy and Asthma Reports"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1007\/s11882-006-0030-7.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/article\/10.1007\/s11882-006-0030-7\/fulltext.html","content-type":"text\/html","content-version":"vor","intended-application":"text-mining"},{"URL":"http:\/\/link.springer.com\/content\/pdf\/10.1007\/s11882-006-0030-7","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"},{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1007\/s11882-006-0030-7.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2022,5,15]],"date-time":"2022-05-15T11:44:50Z","timestamp":1652615090000},"score":1,"resource":{"primary":{"URL":"https:\/\/link.springer.com\/10.1007\/s11882-006-0030-7"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2006,11]]},"references-count":49,"journal-issue":{"issue":"6","published-print":{"date-parts":[[2006,11]]}},"alternative-id":["30"],"URL":"https:\/\/doi.org\/10.1007\/s11882-006-0030-7","relation":{},"ISSN":["1529-7322","1534-6315"],"issn-type":[{"value":"1529-7322","type":"print"},{"value":"1534-6315","type":"electronic"}],"subject":[],"published":{"date-parts":[[2006,11]]}}}