Lung function from school age to adulthood in primary ciliary dyskinesia
| العنوان: | Lung function from school age to adulthood in primary ciliary dyskinesia |
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| المؤلفون: | Nagehan Emiralioglu, Francesca Santamaria, Malena C, Myrofora Goutaki, Panayiotis K. Yiallouros, Bulent Karadag, Ben D. Spycher, Michael R. Loebinger, Claudia E. Kuehni, June K. Marthin, Philipp Latzin, Pedersen Esl, Eric G. Haarman, Mieke Boon, Suzanne Crowley, Cordula Koerner-Rettberg, Lucy Morgan, Guillaume Thouvenin, Nicolaus Schwerk, Florian S. Halbeisen, Israel Amirav, Petr Pohunek, Jane S. Lucas, Kim G. Nielsen, Mazurek H |
| المساهمون: | S Halbeisen, Florian, L Pedersen, Eva S, Goutaki, Myrofora, D Spycher, Ben, Amirav, Israel, Boon, Mieke, Cohen-Cymberknoh, Malena, Crowley, Suzanne, Emiralioglu, Nagehan, G Haarman, Eric, Karadag, Bulent, Koerner-Rettberg, Cordula, Latzin, Philipp, R Loebinger, Michael, S Lucas, Jane, Mazurek, Henryk, Morgan, Lucy, Marthin, June, Pohunek, Petr, Santamaria, Francesca, Schwerk, Nicolau, Thouvenin, Guillaume, Yiallouros, Panayioti, G Nielsen, Kim, E Kuehni, Claudia, Pediatrics, Amsterdam Reproduction & Development (AR&D), Halbeisen F. S. , Pedersen E. S. , Goutaki M., Spycher B. D. , Amirav I., Boon M., Cohen-Cymberknoh M., Crowley S., EMİRALİOĞLU ORDUKAYA N., Haarman E. G. , et al. |
| المصدر: | Halbeisen, Florian S; Pedersen, Eva S L; Goutaki, Myrofora; Spycher, Ben D; Amirav, Israel; Boon, Mieke; Malena, Cohen-Cymberknoh; Crowley, Suzanne; Emiralioglu, Nagehan; Haarman, Eric G; Karadag, Bulent; Koerner-Rettberg, Cordula; Latzin, Philipp; Loebinger, Michael R; Lucas, Jane S; Mazurek, Henryk; Morgan, Lucy; Marthin, June; Pohunek, Petr; Santamaria, Francesca; ... (2022). Lung function from school age to adulthood in primary ciliary dyskinesia. European respiratory journal, 60(4), p. 2101918. European Respiratory Society 10.1183/13993003.01918-2021 <http://dx.doi.org/10.1183/13993003.01918-2021Test> Halbeisen, F S, Pedersen, E S L, Goutaki, M, Spycher, B D, Amirav, I, Boon, M, Cohen-Cymberknoh, M, Crowley, S, Emiralioglu, N, Haarman, E G, Karadag, B, Koerner-Rettberg, C, Latzin, P, Loebinger, M R, Lucas, J S, Mazurek, H, Morgan, L, Marthin, J, Pohunek, P, Santamaria, F, Schwerk, N, Thouvenin, G, Yiallouros, P, Nielsen, K G & Kuehni, C E 2022, ' Lung function from school age to adulthood in primary ciliary dyskinesia ', European Respiratory Journal, vol. 60, no. 4, 2101918 . https://doi.org/10.1183/13993003.01918-2021Test European Respiratory Journal, 60(4):2101918. European Respiratory Society |
| بيانات النشر: | European Respiratory Society (ERS), 2022. |
| سنة النشر: | 2022 |
| مصطلحات موضوعية: | Adult, Pulmonary and Respiratory Medicine, Vital capacity, Pediatrics, medicine.medical_specialty, Adolescent, Vital Capacity, 610 Medicine & health, Sağlık Bilimleri, Clinical Medicine (MED), SOLUNUM SİSTEMİ, Cohort Studies, FEV1/FVC ratio, Young Adult, 360 Social problems & social services, Forced Expiratory Volume, Health Sciences, Medicine, Humans, Klinik Tıp (MED), Chest Diseases and Allergy, Young adult, Child, Lung, Primary ciliary dyskinesia, Internal Medicine Sciences, Klinik Tıp, business.industry, RESPIRATORY SYSTEM, Dahili Tıp Bilimleri, Göğüs Hastalıkları ve Allerji, CLINICAL MEDICINE, respiratory system, medicine.disease, Tıp, respiratory tract diseases, medicine.anatomical_structure, Cohort, Laterality, Akciğer ve Solunum Tıbbı, business, Cohort study, Ciliary Motility Disorders |
| الوصف: | Primary ciliary dyskinesia (PCD) presents with symptoms early in life and the disease course may be progressive, but longitudinal data on lung function are scarce. This multinational cohort study describes lung function trajectories in children, adolescents, and young adults with PCD. We analysed data from 486 patients with repeated lung function measurements obtained between the age of 6 and 24 years from the international PCD Cohort (iPCD) and calculated z-scores for forced expiratory volume in the first second (FEV1) and forced vital capacity (FVC) using the Global Lung Function Initiative 2012 references. We described baseline lung function and change of lung function over time and described their associations with possible determinants in mixed-effects linear regression models. Overall, FEV1 and FVC z-scores declined over time (average crude annual FEV1 decline was -0.07 z-scores) but not at the same rate for all patients. FEV1 z-scores improved over time in 21% of patients, remained stable in 40% and declined in 39%. Low BMI was associated with poor baseline lung function and with further decline. Results differed by country and ultrastructural defect, but we found no evidence of differences by sex, calendar year of diagnosis, age at diagnosis, diagnostic certainty, or laterality defect. Our study shows that on average lung function in PCD declines throughout the entire period of lung growth, from childhood to young adult age, even among patients treated in specialized centres. It is essential to develop strategies to reverse this tendency and improve prognosis.Take home messageLung function in children with PCD is reduced by age 6 years and further declines during the growth period. It is essential to develop strategies to improve prognosis. |
| وصف الملف: | application/pdf; Electronic-Print; text |
| تدمد: | 1399-3003 0903-1936 |
| الوصول الحر: | https://explore.openaire.eu/search/publication?articleId=doi_dedup___::ff2017aee79fb324168f9e4699b39660Test https://doi.org/10.1183/13993003.01918-2021Test |
| حقوق: | OPEN |
| رقم الانضمام: | edsair.doi.dedup.....ff2017aee79fb324168f9e4699b39660 |
| قاعدة البيانات: | OpenAIRE |
| تدمد: | 13993003 09031936 |
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