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The Capital Region of Denmark - a part of Copenhagen University Hospital
  1. 2020
  2. Published

    Primary ciliary dyskinesia patients have the same P. aeruginosa clone in sinuses and lungs

    Arndal, E., Johansen, H. K., Haagensen, J. A. J., Bartell, J. A., Marvig, R. L., Alanin, M., Aanæs, K., Høiby, N., Nielsen, K. G., Backer, V. & von Buchwald, C., 2020, In : European Respiratory Journal. Supplement. p. e1901472

    Research output: Contribution to journalJournal articleResearchpeer-review

  3. E-pub ahead of print

    Randomization of Left-right Asymmetry and Congenital Heart Defects: The Role of DNAH5 in Humans and Mice

    Nöthe-Menchen, T., Wallmeier, J., Pennekamp, P., Höben, I. M., Olbrich, H., Loges, N. T., Raidt, J., Dougherty, G. W., Hjeij, R., Dworniczak, B., Omran, H. & Nielsen, K. G., 2020, In : Circulation. Genomic and precision medicine.

    Research output: Contribution to journalJournal articleResearchpeer-review

  4. E-pub ahead of print

    SPEF2- and HYDIN-mutant Cilia Lack the Central Pair Associated Protein SPEF2 Aiding PCD Diagnostics

    Cindrić, S., Dougherty, G. W., Olbrich, H., Hjeij, R., Loges, N. T., Amirav, I., Philipsen, M. C., Marthin, J. K., Nielsen, K. G., Sutharsan, S., Raidt, J., Werner, C., Pennekamp, P., Dworniczak, B. & Omran, H., 2020, In : American Journal of Respiratory Cell and Molecular Biology.

    Research output: Contribution to journalJournal articleResearchpeer-review

  5. Published

    The Controversies and Difficulties of Diagnosing Primary Ciliary Dyskinesia

    Shoemark, A., Rubbo, B., Haarman, E., Hirst, R. A., Hogg, C., Jackson, C. L., Nielsen, K. G., Papon, J-F., Robinson, P., Walker, W. T. & Lucas, J. S., 2020, In : American Journal of Respiratory and Critical Care Medicine. 201, 1, p. 120-122 3 p.

    Research output: Contribution to journalJournal articleResearchpeer-review

  6. 2019
  7. Published

    The impact of mannose-binding lectin polymorphisms on lung function in primary ciliary dyskinesia

    Videbaek, K., Buchvald, F., Holgersen, M. G., Henriksen, A., Eriksson, F., Garred, P. & Nielsen, K. G., 1 Aug 2019, In : Pediatric Pulmonology. 54, 8, p. 1182-1189 8 p.

    Research output: Contribution to journalJournal articleResearchpeer-review

  8. Published

    Long-Term Lung Function and Exercise Capacity in Postinfectious chILD

    Sisman, Y., Buchvald, F. F., Ring, A. M., Wassilew, K. & Nielsen, K. G., 1 Mar 2019, In : Pediatric, Allergy, Immunology, and Pulmonology. 32, 1, p. 4-11 8 p.

    Research output: Contribution to journalJournal articleResearchpeer-review

  9. Published

    Lung clearance index for early detection of pulmonary complications after allo-HSCT in children

    Uhlving, H. H., Skov, L., Buchvald, F., Heilmann, C., Grell, K., Ifversen, M., Green, K., Müller, K. & Nielsen, K. G., 2019, In : Pediatric Pulmonology. 54, 7, p. 1029-1038 10 p.

    Research output: Contribution to journalJournal articleResearchpeer-review

  10. Published

    One-year evolution and variability in multiple-breath washout indices in children and young adults with primary ciliary dyskinesia

    Kobbernagel, H. E., Green, K., Ring, A. M., Buchvald, F. F., Rosthøj, S., Gustafsson, P. M. & Nielsen, K. G., 2019, In : European Clinical Respiratory Journal. 6, 1, p. 1591841

    Research output: Contribution to journalJournal articleResearchpeer-review

  11. Published

    Prevalence and course of disease after lung resection in primary ciliary dyskinesia: a cohort & nested case-control study

    Israeli PCD Consortium, 2019, In : Respiratory Research. 20, 1, p. 212

    Research output: Contribution to journalJournal articleResearchpeer-review

  12. Published

    Proof of Concept: Very Rapid Tidal Breathing Nasal Nitric Oxide Sampling Discriminates Primary Ciliary Dyskinesia from Healthy Subjects

    Holgersen, M. G., Marthin, J. K. & Nielsen, K. G., 2019, In : Lung. 197, 2, p. 209-216 8 p.

    Research output: Contribution to journalJournal articleResearchpeer-review

  13. Published

    Pulmonary exacerbations in patients with primary ciliary dyskinesia: an expert consensus definition for use in clinical trials

    Lucas, J. S., Gahleitner, F., Amorim, A., Boon, M., Brown, P., Constant, C., Cook, S., Crowley, S., Destouches, D. M. S., Eber, E., Mussaffi, H., Haarman, E., Harris, A., Koerner-Rettberg, C., Kuehni, C. E., Latzin, P., Loebinger, M. R., Lorent, N., Maitre, B., Moreno-Galdó, A., Nielsen, K. G., Özçelik, U., Philipsen, L. K. D., Pohunek, P., Polverino, E., Rademacher, J., Robinson, P., Snijders, D., Yiallouros, P. & Carr, S. B., 2019, In : ERJ Open Research. 5, 1

    Research output: Contribution to journalJournal articleResearchpeer-review

  14. 2018
  15. Published

    The accessibility of topical treatment in the paranasal sinuses on operated cystic fibrosis patients assessed by scintigraphy

    Aanaes, K., Alanin, M. C., Nielsen, K. G., Moller Jorgensen, M., von Buchwald, C., Hoiby, N., Johansen, H. K., Johannesen, H. H. & Mortensen, J., 1 Sep 2018, In : Rhinology. 56, 3, p. 268-273 6 p.

    Research output: Contribution to journalJournal articleResearchpeer-review

  16. Published
  17. Published

    Ventilation inhomogeneity and NO and CO diffusing capacity in ex-premature school children

    Sørensen, J. K., Buchvald, F., Berg, A. K., Robinson, P. D. & Nielsen, K. G., Jul 2018, In : Respiratory medicine. 140, p. 94-100 7 p.

    Research output: Contribution to journalJournal articleResearchpeer-review

  18. Published

    Fitness and lung function in children with primary ciliary dyskinesia and cystic fibrosis

    Ring, A. M., Buchvald, F. F., Holgersen, M. G., Green, K. & Nielsen, K. G., Jun 2018, In : Respiratory medicine. 139, p. 79-85 7 p.

    Research output: Contribution to journalJournal articleResearchpeer-review

  19. Published
  20. Published

    Mutations in C11orf70 Cause Primary Ciliary Dyskinesia with Randomization of Left/Right Body Asymmetry Due to Defects of Outer and Inner Dynein Arms

    Höben, I. M., Hjeij, R., Olbrich, H., Dougherty, G. W., Nöthe-Menchen, T., Aprea, I., Frank, D., Pennekamp, P., Dworniczak, B., Wallmeier, J., Raidt, J., Nielsen, K. G., Philipsen, M. C., Santamaria, F., Venditto, L., Amirav, I., Mussaffi, H., Prenzel, F., Wu, K., Bakey, Z., Schmidts, M., Loges, N. T. & Omran, H., 2018, In : American Journal of Human Genetics. 102, 5, p. 973-984 12 p.

    Research output: Contribution to journalJournal articleResearchpeer-review

  21. Published

    The effect of time-of-day and chest physiotherapy on multiple breath washout measures in children with clinically stable cystic fibrosis

    Voldby, C., Green, K., Rosthøj, S., Kongstad, T., Philipsen, L., Buchvald, F., Skov, M., Pressler, T., Gustafsson, P. & Nielsen, K. G., 2018, In : P L o S One. 13, 1, p. e0190894

    Research output: Contribution to journalJournal articleResearchpeer-review

  22. Published
  23. Published

    Variation in treatment strategies for the eradication of Pseudomonas aeruginosa in primary ciliary dyskinesia across European centers

    Crowley, S., Holgersen, M. G. & Nielsen, K. G., 2018, In : Chronic Respiratory Disease. 16, p. 1479972318787919

    Research output: Contribution to journalJournal articleResearchpeer-review

  24. 2017
  25. Published

    Clinical care of children with primary ciliary dyskinesia

    Lucas, J. S., Alanin, M. C., Collins, S., Harris, A., Johansen, H. K., Nielsen, K. G., Papon, J. F., Robinson, P. & Walker, W. T., Oct 2017, In : Expert Review of Respiratory Medicine. 11, 10, p. 779-790 12 p.

    Research output: Contribution to journalJournal articleResearchpeer-review

  26. Published
  27. Published

    Cardiopulmonary Exercise Testing in Fontan Patients With and Without Isomerism (Heterotaxy) as Compared to Patients With Primary Ciliary Dyskinesia and Subjects With Structurally Normal Hearts

    Loomba, R. S., Danduran, M., Nielsen, K. G., Ring, A. M., Kovach, J. & Anderson, R. H., 2017, In : Pediatric Cardiology. 38, 2, p. 410-417 8 p.

    Research output: Contribution to journalJournal articleResearchpeer-review

  28. Published

    Patient-specific three-dimensional explant spheroids derived from human nasal airway epithelium: a simple methodological approach for ex vivo studies of primary ciliary dyskinesia

    Marthin, J. K., Stevens, E. M., Larsen, L. A., Christensen, S. T. & Nielsen, K. G., 2017, In : Cilia. 6, p. 3

    Research output: Contribution to journalJournal articleResearchpeer-review

  29. Published
  30. 2016
  31. Published

    Specific airway resistance in preschool children: why not panting after all?

    Ioan, I., Demoulin-Alexikova, S., Coutier, L., Bonabel, C., Kirkby, J., Nielsen, K. G., Tomalak, W., Thompson, B., Schweitzer, C., Robinson, P. D. & Marchal, F., Dec 2016, In : European Respiratory Journal. 48, 6, p. 1804-1807 4 p.

    Research output: Contribution to journalJournal articleResearchpeer-review

  32. Published

    Exercise-induced inspiratory symptoms in school children

    Buchvald, F., Phillipsen, L. D., Hjuler, T. & Nielsen, K. G., Nov 2016, In : Pediatric Pulmonology. 51, 11, p. 1200-1205 6 p.

    Research output: Contribution to journalJournal articleResearchpeer-review

  33. Published

    Sinus surgery postpones chronic Gram-negative lung infection: cohort study of 106 patients with cystic fibrosis

    Alanin, M. C., Aanaes, K., Høiby, N., Pressler, T., Skov, M., Nielsen, K. G., Taylor-Robinson, D., Waldmann, E., Johansen, H. K. & von Buchwald, C., Sep 2016, In : Rhinology. 54, 3, p. 206-13 8 p.

    Research output: Contribution to journalJournal articleResearchpeer-review

  34. Published

    Study protocol, rationale and recruitment in a European multi-centre randomized controlled trial to determine the efficacy and safety of azithromycin maintenance therapy for 6 months in primary ciliary dyskinesia

    Kobbernagel, H. E., Buchvald, F. F., Haarman, E. G., Casaulta, C., Collins, S. A., Hogg, C., Kuehni, C. E., Lucas, J. S., Omran, H., Quittner, A. L., Werner, C. & Nielsen, K. G., 22 Jul 2016, In : BMC Pulmonary Medicine. 16, 1, p. 104

    Research output: Contribution to journalJournal articleResearchpeer-review

  35. Published

    Bacterial evolution in PCD and CF patients follows the same mutational steps

    Sommer, L. M., Alanin, M. C., Marvig, R. L., Nielsen, K. G., Høiby, N., von Buchwald, C., Molin, S. & Johansen, H. K., 28 Jun 2016, In : Scientific Reports. 6, p. 28732

    Research output: Contribution to journalJournal articleResearchpeer-review

  36. Published

    Abbreviation modalities of nitrogen multiple-breath washout tests in school children with obstructed lung disease

    Green, K., Ejlertsen, J. S., Madsen, A., Buchvald, F. F., Kongstad, T., Kobbernagel, H., Gustafsson, P. M. & Nielsen, K. G., Jun 2016, In : Pediatric Pulmonology. 51, 6, p. 624-32 9 p.

    Research output: Contribution to journalJournal articleResearchpeer-review

  37. Published

    Childhood asthma and physical activity: a systematic review with meta-analysis and Graphic Appraisal Tool for Epidemiology assessment

    Lochte, L., Nielsen, K. G., Petersen, P. E. & Platts-Mills, T. A. E., 18 Apr 2016, In : BMC Pediatrics. 16, p. 50

    Research output: Contribution to journalJournal articleResearchpeer-review

  38. Published

    An international registry for primary ciliary dyskinesia

    Werner, C., Lablans, M., Ataian, M., Raidt, J., Wallmeier, J., Große-Onnebrink, J., Kuehni, C. E., Haarman, E. G., Leigh, M. W., Quittner, A. L., Lucas, J. S., Hogg, C., Witt, M., Priftis, K. N., Yiallouros, P., Nielsen, K. G., Santamaria, F., Ückert, F. & Omran, H., Mar 2016, In : European Respiratory Journal. 47, 3, p. 849-59 11 p.

    Research output: Contribution to journalJournal articleResearchpeer-review

  39. Published

    Pulmonary function and fitness years after treatment for hypersensitivity pneumonitis during childhood

    Sisman, Y., Buchvald, F., Blyme, A. K., Mortensen, J. & Nielsen, K. G., 2016, In : Pediatric Pulmonology. 51, 8, p. 830-7

    Research output: Contribution to journalJournal articleResearchpeer-review

  40. 2015
  41. Published
  42. Published

    Clinical value of measurement of pulmonary radioaerosol mucociliary clearance in the work up of primary ciliary dyskinesia

    Munkholm, M., Nielsen, K. G. & Mortensen, J., Dec 2015, In : EJNMMI Research. 5, 1, p. 118

    Research output: Contribution to journalJournal articleResearchpeer-review

  43. Published
  44. Published

    Simultaneous sinus and lung infections in patients with primary ciliary dyskinesia

    Alanin, M. C., Johansen, H. K., Aanaes, K., Høiby, N., Pressler, T., Skov, M., Nielsen, K. G. & von Buchwald, C., Jan 2015, In : Acta Oto-Laryngologica. 135, 1, p. 58-63 6 p.

    Research output: Contribution to journalJournal articleResearchpeer-review

  45. Published

    Small airways dysfunction in long-term survivors of pediatric stem cell transplantation

    Uhlving, H. H., Mathiesen, S., Buchvald, F., Green, K., Heilmann, C., Gustafsson, P., Müller, K. & Nielsen, K. G., 2015, In : Pediatric Pulmonology. 50, 7, p. 704-12

    Research output: Contribution to journalJournal articleResearchpeer-review

  46. Published

    The development of diabetes among Danish cystic fibrosis patients over the last two decades

    Knudsen, K. B., Mathiesen, E. R., Eriksen, V., Skov, M., Nielsen, K. G., Johannesen, J. & Pressler, T., 2015, In : Pediatric Diabetes. 16, 3, p. 219-226 7 p.

    Research output: Contribution to journalJournal articleResearchpeer-review

  47. 2014
  48. Published

    Multicenter analysis of body mass index, lung function, and sputum microbiology in primary ciliary dyskinesia

    Maglione, M., Bush, A., Nielsen, K. G., Hogg, C., Montella, S., Marthin, J. K., Di Giorgio, A. & Santamaria, F., Dec 2014, In : Pediatric Pulmonology. 49, 12, p. 1243-50 8 p.

    Research output: Contribution to journalJournal articleResearchpeer-review

  49. Published

    Aerobic exercise capacity at long-term follow-up after paediatric allogeneic haematopoietic SCT

    Mathiesen, S., Uhlving, H. H., Buchvald, F., Hanel, B., Nielsen, K. G. & Müller, K., Nov 2014, In : Bone Marrow Transplantation. 49, 11, p. 1393-9 7 p.

    Research output: Contribution to journalJournal articleResearchpeer-review

  50. Published

    CCDC151 mutations cause primary ciliary dyskinesia by disruption of the outer dynein arm docking complex formation

    Hjeij, R., Onoufriadis, A., Watson, C. M., Slagle, C. E., Klena, N. T., Dougherty, G. W., Kurkowiak, M., Loges, N. T., Diggle, C. P., Morante, N. F. C., Gabriel, G. C., Lemke, K. L., Li, Y., Pennekamp, P., Menchen, T., Konert, F., Marthin, J. K., Mans, D. A., Letteboer, S. J. F., Werner, C., Burgoyne, T., Westermann, C., Rutman, A., Carr, I. M., O'Callaghan, C., Moya, E., Chung, E. M. K., Sheridan, E., Nielsen, K. G., Roepman, R., Bartscherer, K., Burdine, R. D., Lo, C. W., Omran, H., Mitchison, H. M. & UK10K Consortium, 4 Sep 2014, In : American Journal of Human Genetics. 95, 3, p. 257-74 18 p.

    Research output: Contribution to journalJournal articleResearchpeer-review

  51. Published

    Diffus lungesygdom hos børn

    Buchvald, F. & Nielsen, K. G., 18 Aug 2014, In : Ugeskrift for læger [online]. 176, 34

    Research output: Contribution to journalJournal articleResearchpeer-review

  52. Published

    Pulmonary function and autoantibodies in a long-term follow-up of juvenile dermatomyositis patients

    Mathiesen, P. R., Buchvald, F., Nielsen, K. G., Herlin, T., Friis, T. & Nielsen, S., Apr 2014, In : Rheumatology. 53, 4, p. 644-649 5 p.

    Research output: Contribution to journalJournal articleResearchpeer-review

  53. Published

    Exhaled breath analysis using electronic nose in cystic fibrosis and primary ciliary dyskinesia patients with chronic pulmonary infections

    Joensen, O., Paff, T., Haarman, E. G., Skovgaard, I., Jensen, P. Ø., Bjarnsholt, T. & Nielsen, K. G., 2014, In : P L o S One. 9, 12, p. e115584

    Research output: Contribution to journalJournal articleResearchpeer-review

  54. Published

    New insights into the aspects of pulmonary diffusing capacity in Fontan patients

    Idorn, L., Hanel, B., Jensen, A. S., Juul, K., Reimers, J. I., Nielsen, K. G. & Søndergaard, L., 2014, In : Cardiology in the Young. 24, p. 311-320

    Research output: Contribution to journalJournal articleResearchpeer-review

  55. Published

    The single-breath diffusing capacity of CO and NO in healthy children of European descent

    Thomas, A., Hanel, B., Marott, J. L., Buchvald, F., Mortensen, J. & Nielsen, K. G., 2014, In : P L o S One. 9, 12, p. e113177 22 p.

    Research output: Contribution to journalJournal articleResearchpeer-review

  56. 2013
  57. Published
  58. Published

    Enzyme replacement therapy for alpha-mannosidosis: 12 months follow-up of a single centre, randomised, multiple dose study

    Borgwardt, L. G., Dali, C. I., Fogh, J., Månsson, J., Olsen, K. J., Beck, H., Nielsen, K. G., Nielsen, L. H., Olsen, S. O. E., Riise Stensland, H. M. F., Nilssen, O., Wibrand, F., Thuesen, A-M., Pearl, T., Haugsted, U., Saftig, P., Blanz, J., Jones, S., Tylki-Szymanska, A., Guffon-Fouiloux, N., Beck, M. V. & Lund, A. M., Nov 2013, In : Journal of Inherited Metabolic Disease. 36, 6, p. 1015-24 10 p.

    Research output: Contribution to journalJournal articleResearchpeer-review

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