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An international registry for primary ciliary dyskinesia

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Harvard

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 2016, 'An international registry for primary ciliary dyskinesia' European Respiratory Journal, vol. 47, no. 3, pp. 849-59. https://doi.org/10.1183/13993003.00776-2015

APA

Werner, C., Lablans, M., Ataian, M., Raidt, J., Wallmeier, J., Große-Onnebrink, J., ... Omran, H. (2016). An international registry for primary ciliary dyskinesia. European Respiratory Journal, 47(3), 849-59. https://doi.org/10.1183/13993003.00776-2015

CBE

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. 2016. An international registry for primary ciliary dyskinesia. European Respiratory Journal. 47(3):849-59. https://doi.org/10.1183/13993003.00776-2015

MLA

Vancouver

Werner C, Lablans M, Ataian M, Raidt J, Wallmeier J, Große-Onnebrink J et al. An international registry for primary ciliary dyskinesia. European Respiratory Journal. 2016 Mar;47(3):849-59. https://doi.org/10.1183/13993003.00776-2015

Author

Werner, Claudius ; Lablans, Martin ; Ataian, Maximilian ; Raidt, Johanna ; Wallmeier, Julia ; Große-Onnebrink, Jörg ; Kuehni, Claudia E ; Haarman, Eric G ; Leigh, Margaret W ; Quittner, Alexandra L ; Lucas, Jane S ; Hogg, Claire ; Witt, Michal ; Priftis, Kostas N ; Yiallouros, Panayiotis ; Nielsen, Kim G ; Santamaria, Francesca ; Ückert, Frank ; Omran, Heymut. / An international registry for primary ciliary dyskinesia. In: European Respiratory Journal. 2016 ; Vol. 47, No. 3. pp. 849-59.

Bibtex

@article{0450d284fb6342679d7a1c18367b9670,
title = "An international registry for primary ciliary dyskinesia",
abstract = "Primary ciliary dyskinesia (PCD) is a rare autosomal recessive disorder leading to chronic upper and lower airway disease. Fundamental data on epidemiology, clinical presentation, course and treatment strategies are lacking in PCD. We have established an international PCD registry to realise an unmet need for an international platform to systematically collect data on incidence, clinical presentation, treatment and disease course.The registry was launched in January 2014. We used internet technology to ensure easy online access using a web browser under www.pcdregistry.eu. Data from 201 patients have been collected so far. The database is comprised of a basic data form including demographic and diagnostic information, and visit forms designed to monitor the disease course.To establish a definite PCD diagnosis, we used strict diagnostic criteria, which required two to three diagnostic methods in addition to classical clinical symptoms. Preliminary analysis of lung function data demonstrated a mean annual decline of percentage predicted forced expiratory volume in 1 s of 0.59{\%} (95{\%} CI 0.98-0.22).Here, we present the development of an international PCD registry as a new promising tool to advance the understanding of this rare disorder, to recruit candidates for research studies and ultimately to improve PCD care.",
keywords = "Adolescent, Adult, Age Distribution, Aged, Child, Child, Preschool, Disease Progression, Europe, Female, Forced Expiratory Volume, Humans, Incidence, Infant, Internet, Intersectoral Collaboration, Kartagener Syndrome, Male, Middle Aged, North America, Patient Selection, Registries, Young Adult, Journal Article, Observational Study, Research Support, Non-U.S. Gov't",
author = "Claudius Werner and Martin Lablans and Maximilian Ataian and Johanna Raidt and Julia Wallmeier and J{\"o}rg Gro{\ss}e-Onnebrink and Kuehni, {Claudia E} and Haarman, {Eric G} and Leigh, {Margaret W} and Quittner, {Alexandra L} and Lucas, {Jane S} and Claire Hogg and Michal Witt and Priftis, {Kostas N} and Panayiotis Yiallouros and Nielsen, {Kim G} and Francesca Santamaria and Frank {\"U}ckert and Heymut Omran",
note = "Copyright {\circledC}ERS 2016.",
year = "2016",
month = "3",
doi = "10.1183/13993003.00776-2015",
language = "English",
volume = "47",
pages = "849--59",
journal = "European Respiratory Journal",
issn = "0903-1936",
publisher = "European Respiratory Society",
number = "3",

}

RIS

TY - JOUR

T1 - An international registry for primary ciliary dyskinesia

AU - Werner, Claudius

AU - Lablans, Martin

AU - Ataian, Maximilian

AU - Raidt, Johanna

AU - Wallmeier, Julia

AU - Große-Onnebrink, Jörg

AU - Kuehni, Claudia E

AU - Haarman, Eric G

AU - Leigh, Margaret W

AU - Quittner, Alexandra L

AU - Lucas, Jane S

AU - Hogg, Claire

AU - Witt, Michal

AU - Priftis, Kostas N

AU - Yiallouros, Panayiotis

AU - Nielsen, Kim G

AU - Santamaria, Francesca

AU - Ückert, Frank

AU - Omran, Heymut

N1 - Copyright ©ERS 2016.

PY - 2016/3

Y1 - 2016/3

N2 - Primary ciliary dyskinesia (PCD) is a rare autosomal recessive disorder leading to chronic upper and lower airway disease. Fundamental data on epidemiology, clinical presentation, course and treatment strategies are lacking in PCD. We have established an international PCD registry to realise an unmet need for an international platform to systematically collect data on incidence, clinical presentation, treatment and disease course.The registry was launched in January 2014. We used internet technology to ensure easy online access using a web browser under www.pcdregistry.eu. Data from 201 patients have been collected so far. The database is comprised of a basic data form including demographic and diagnostic information, and visit forms designed to monitor the disease course.To establish a definite PCD diagnosis, we used strict diagnostic criteria, which required two to three diagnostic methods in addition to classical clinical symptoms. Preliminary analysis of lung function data demonstrated a mean annual decline of percentage predicted forced expiratory volume in 1 s of 0.59% (95% CI 0.98-0.22).Here, we present the development of an international PCD registry as a new promising tool to advance the understanding of this rare disorder, to recruit candidates for research studies and ultimately to improve PCD care.

AB - Primary ciliary dyskinesia (PCD) is a rare autosomal recessive disorder leading to chronic upper and lower airway disease. Fundamental data on epidemiology, clinical presentation, course and treatment strategies are lacking in PCD. We have established an international PCD registry to realise an unmet need for an international platform to systematically collect data on incidence, clinical presentation, treatment and disease course.The registry was launched in January 2014. We used internet technology to ensure easy online access using a web browser under www.pcdregistry.eu. Data from 201 patients have been collected so far. The database is comprised of a basic data form including demographic and diagnostic information, and visit forms designed to monitor the disease course.To establish a definite PCD diagnosis, we used strict diagnostic criteria, which required two to three diagnostic methods in addition to classical clinical symptoms. Preliminary analysis of lung function data demonstrated a mean annual decline of percentage predicted forced expiratory volume in 1 s of 0.59% (95% CI 0.98-0.22).Here, we present the development of an international PCD registry as a new promising tool to advance the understanding of this rare disorder, to recruit candidates for research studies and ultimately to improve PCD care.

KW - Adolescent

KW - Adult

KW - Age Distribution

KW - Aged

KW - Child

KW - Child, Preschool

KW - Disease Progression

KW - Europe

KW - Female

KW - Forced Expiratory Volume

KW - Humans

KW - Incidence

KW - Infant

KW - Internet

KW - Intersectoral Collaboration

KW - Kartagener Syndrome

KW - Male

KW - Middle Aged

KW - North America

KW - Patient Selection

KW - Registries

KW - Young Adult

KW - Journal Article

KW - Observational Study

KW - Research Support, Non-U.S. Gov't

U2 - 10.1183/13993003.00776-2015

DO - 10.1183/13993003.00776-2015

M3 - Journal article

VL - 47

SP - 849

EP - 859

JO - European Respiratory Journal

JF - European Respiratory Journal

SN - 0903-1936

IS - 3

ER -

ID: 49874218