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Title: Prevalence and course of disease after lung resection in primary ciliary dyskinesia: A cohort & nested case-control study
Authors: Kouis, Panayiotis 
Goutaki, Myrofora 
Halbeisen, Florian S. 
Gioti, Ifigeneia 
Middleton, Nicos 
Amirav, Israel 
Barbato, Angelo 
Behan, Laura 
Boon, Mieke 
Emiralioglu, Nagehan 
Haarman, Eric G. 
Karadag, Bulent 
Koerner-Rettberg, Cordula 
Lazor, Romain 
Loebinger, Michael R. 
Maitre, Bernard 
Mazurek, Henryk 
Morgan, Lucy 
Nielsen, Kim Gjerum 
Omran, Heymut 
Özçelik, Ugur 
Price, Mareike 
Pogorzelski, Andrzej 
Snijders, Deborah 
Thouvenin, Guillaume 
Werner, Claudius 
Zivkovic, Zorica M. 
Kuehni, Claudia E. 
Yiallouros, Panayiotis K. 
Major Field of Science: Medical and Health Sciences
Field Category: Clinical Medicine
Keywords: Ciliary motility disorders (MeSH);Kartagener syndrome (MeSH);Lobectomy
Issue Date: 18-Sep-2019
Source: Respiratory Research, 2019, vol. 20, no. 1, articl. no. 212
Volume: 20
Issue: 1
Journal: Respiratory Research 
Abstract: Background: Lung resection is a controversial and understudied therapeutic modality in Primary Ciliary Dyskinesia (PCD). We assessed the prevalence of lung resection in PCD across countries and compared disease course in lobectomised and non-lobectomised patients. Methods: In the international iPCD cohort, we identified lobectomised and non-lobectomised age and sex-matched PCD patients and compared their characteristics, lung function and BMI cross-sectionally and longitudinally. Results: Among 2896 patients in the iPCD cohort, 163 from 20 centers (15 countries) underwent lung resection (5.6%). Among adult patients, prevalence of lung resection was 8.9%, demonstrating wide variation among countries. Compared to the rest of the iPCD cohort, lobectomised patients were more often females, older at diagnosis, and more often had situs solitus. In about half of the cases (45.6%) lung resection was performed before presentation to specialized PCD centers for diagnostic work-up. Compared to controls (n = 197), lobectomised patients had lower FVC z-scores (-2.41 vs-1.35, p = 0.0001) and FEV1 z-scores (-2.79 vs-1.99, p = 0.003) at their first post-lung resection assessment. After surgery, lung function continued to decline at a faster rate in lobectomised patients compared to controls (FVC z-score slope:-0.037/year Vs-0.009/year, p = 0.047 and FEV1 z-score slope:-0.052/year Vs-0.033/year, p = 0.235), although difference did not reach statistical significance for FEV1. Within cases, females and patients with multiple lobe resections had lower lung function. Conclusions: Prevalence of lung resection in PCD varies widely between countries, is often performed before PCD diagnosis and overall is more frequent in patients with delayed diagnosis. After lung resection, compared to controls most lobectomised patients have poorer and continuing decline of lung function despite lung resection. Further studies benefiting from prospective data collection are needed to confirm these findings.
ISSN: 1465-993X
DOI: 10.1186/s12931-019-1183-y
Rights: © The Author(s)
Attribution-NonCommercial-NoDerivs 3.0 United States
Type: Article
Affiliation : University of Cyprus 
University of Bern 
Cyprus University of Technology 
University of Alberta Edmonton 
University of Padova 
University Hospital Southampton NHS Foundation Trust 
University of Southampton 
University Hospital Gasthuisberg Leuven 
Hacettepe University 
Vrije Universiteit Brussel 
Marmara University 
University Children's Hospital of Ruhr 
University Bochum 
Lausanne University Hospital 
National Reference Centre for Rare Pulmonary Diseases 
Royal Brompton and Harefield NHS Foundation Trust 
Université Paris Est Créteil 
Instytut Gruźlicy i ChoróbPłuc 
University of Sydney 
Copenhagen University Hospital 
University Hospital Muenster 
Hannover Medical School 
Sorbonne Universités 
Medical Centre dr Dragisa Misovic 
Business Academy in Novi Sad 
Shakolas Educational Center of Clinical Medicine 
Tel Aviv Medical Center 
Helios Hospital Schwerin 
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