Archive
Safety and efficacy of the epithelial sodium channel blocker idrevloride in people with primary ciliary dyskinesia (CLEAN-PCD): a multinational, phase 2, randomised, double-blind, placebo-controlled crossover trial.
Ringshausen FC, Shapiro AJ, Nielsen KG, Mazurek H, Pifferi M, Donn KH, van der Eerden MM, Loebinger MR, Zariwala MA, Leigh MW, Knowles MR, Ferkol TW; CLEAN-PCD investigators and study team. Lancet Respir Med. 2023 Aug 31:S2213-2600(23)00226-6. doi: 10.1016/S2213-2600(23)00226-6. Epub ahead of print. PMID: 37660715.
Pulmonary Radioaerosol Mucociliary Clearance assessment: searching for genotype-specific differences and potential as an outcome measure in PCD”
June K. Marthin, Mathias G. Holgersen, Kim G. Nielsen, Jann Mortensen ERJ Open Research 2023; DOI: 10.1183/23120541.00685-2023
Quantitative 99mTc-albumin colloid nasal mucociliary clearance as an outcome in primary ciliary dyskinesia.
Marthin JK, Nielsen KG, Mortensen J. ERJ Open Res. 2023 Sep 11;9(5):00345-2023. doi: 10.1183/23120541.00345-2023. eCollection 2023 Sep. PMID: 37701364
Chronic airway disease in primary ciliary dyskinesia-spiced with geno-phenotype associations
Nielsen KG, Holgersen MG, Crowley S, Marthin JK.. Am J Med Genet C Semin Med Genet. 2022 Mar 29. doi: 10.1002/ajmg.c.31967. Epub ahead of print. PMID: 35352480.
Comparison of the Lung Clearance Index in Preschool Children With Primary Ciliary Dyskinesia and Cystic Fibrosis
Roehmel JF, Doerfler FJ, Koerner-Rettberg C, Brinkmann F, Schlegtendal A, Wetzke M, Rudolf I, Helms S, Große-Onnebrink J, Yu Y, Nuesslein T, Wojsyk-Banaszak I, Becker S, Eickmeier O, Sommerburg O, Omran H, Stahl M, Mall MA.. Chest. 2022 Mar 7:S0012-3692(22)00421-4. doi: 10.1016/j.chest.2022.02.052. Epub ahead of print. PMID: 35271842.
Limitations of Nasal Nitric Oxide Measurement for Diagnosis of Primary Ciliary Dyskinesia with Normal Ultrastructure
Raidt J, Krenz H, Tebbe J, Große-Onnebrink J, Olbrich H, Loges NT, Biebach L, Schmalstieg C, Keßler C, Wallmeier J, Dworniczak B, Pennekamp P, Dugas M, Werner C, Omran H.. Ann Am Thorac Soc. 2022 Feb 24. doi: 10.1513/AnnalsATS.202106-728OC. Epub ahead of print. PMID: 35202559.
Current and Future Treatments in Primary Ciliary Dyskinesia
Paff T, Omran H, Nielsen KG, Haarman EG.. Int J Mol Sci. 2021 Sep 11;22(18):9834. doi: 10.3390/ijms22189834. PMID: 34575997; PMCID: PMC8470068.
International BEAT-PCD consensus statement for infection prevention and control for primary ciliary dyskinesia in collaboration with ERN-LUNG PCD Core Network and patient representatives
Marthin JK, Lucas JS, Boon M, Casaulta C, Crowley S, Destouches DMS, Eber E, Escribano A, Haarman E, Hogg C, Maitre B, Marsh G, Martinu V, Moreno-Galdó A, Mussaffi H, Omran H, Pohunek P, Rindlisbacher B, Robinson P, Snijders D, Walker WT, Yiallouros P, Johansen HK, Nielsen KG.. ERJ Open Res. 2021 Aug 2;7(3):00301-2021. doi: 10.1183/23120541.00301-2021. PMID: 34350277; PMCID: PMC8326680.
Efficacy and safety of azithromycin maintenance therapy in primary ciliary dyskinesia (BESTCILIA): a multicentre, double-blind, randomised, placebo-controlled phase 3 trial.
Kobbernagel HE, Buchvald FF, Haarman EG, Casaulta C, Collins SA, Hogg C, Kuehni CE, Lucas JS, Moser CE, Quittner AL, Raidt J, Rosthøj S, Sørensen AL, Thomsen K, Werner C, Omran H, Nielsen KG. Lancet Respir Med. 2020 May;8(5):493-505. doi: 10.1016/S2213-2600(20)30058-8. PMID: 32380069.
Clinical features and management of children with primary ciliary dyskinesia in England
Rubbo B, Best S, Hirst RA, Shoemark A, Goggin P, Carr SB, Chetcuti P, Hogg C, Kenia P, Lucas JS, Moya E, Narayanan M, O’Callaghan C, Williamson M, Walker WT; English National Children’s PCD Management Service. Arch Dis Child. 2020 Aug;105(8):724-729. doi: 10.1136/archdischild-2019-317687. Epub 2020 Mar 10. PMID: 32156696.
One-year evolution and variability in multiple-breath washout indices in children and young adults with primary ciliary dyskinesia.
Kobbernagel HE, Green K, Ring AM, Buchvald FF, Rosthøj S, Gustafsson PM, Nielsen KG. Eur Clin Respir J. 2019 Mar 26;6(1):1591841. doi: 10.1080/20018525.2019.1591841. PMID: 30949311; PMCID: PMC6442098.
Mutations in C11orf70 Cause Primary Ciliary Dyskinesia with Randomization of Left/Right Body Asymmetry Due to Defects of Outer and Inner Dynein Arms
Höben IM, Hjeij R, Olbrich H, Dougherty GW, Nöthe-Menchen T, Aprea I, Frank D, Pennekamp P, Dworniczak B, Wallmeier J, Raidt J, Nielsen KG, Philipsen MC, Santamaria F, Venditto L, Amirav I, Mussaffi H, Prenzel F, Wu K, Bakey Z, Schmidts M, Loges NT, Omran H. Am J Hum Genet. 2018 May 3;102(5):973-984. doi: 10.1016/j.ajhg.2018.03.025. PMID: 29727693; PMCID: PMC5986731.
An international registry for primary ciliary dyskinesia.
Werner C, Lablans M, Ataian M, Raidt J, Wallmeier J, Große-Onnebrink J, Kuehni CE, Haarman EG, Leigh MW, Quittner AL, Lucas JS, Hogg C, Witt M, Priftis KN, Yiallouros P, Nielsen KG, Santamaria F, Ückert F, Omran H. Eur Respir J. 2016 Mar;47(3):849-59. doi: 10.1183/13993003.00776-2015. Epub 2015 Dec 8. PMID: 26659107.