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

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...

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Autores principales: 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, Kuehni, Claudia E., Yiallouros, Panayiotis K.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: BioMed Central 2019
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6751891/
https://www.ncbi.nlm.nih.gov/pubmed/31533829
http://dx.doi.org/10.1186/s12931-019-1183-y
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author 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
Kuehni, Claudia E.
Yiallouros, Panayiotis K.
author_facet 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
Kuehni, Claudia E.
Yiallouros, Panayiotis K.
author_sort Kouis, Panayiotis
collection PubMed
description 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.
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spelling pubmed-67518912019-09-23 Prevalence and course of disease after lung resection in primary ciliary dyskinesia: a cohort & nested case-control study 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 Kuehni, Claudia E. Yiallouros, Panayiotis K. Respir Res Research 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. BioMed Central 2019-09-18 2019 /pmc/articles/PMC6751891/ /pubmed/31533829 http://dx.doi.org/10.1186/s12931-019-1183-y Text en © The Author(s). 2019 Open Access This article is distributed under the terms of the Creative Commons Attribution 4.0 International License (http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution, and reproduction in any medium, provided you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons license, and indicate if changes were made. The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated.
spellingShingle Research
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
Kuehni, Claudia E.
Yiallouros, Panayiotis K.
Prevalence and course of disease after lung resection in primary ciliary dyskinesia: a cohort & nested case-control study
title Prevalence and course of disease after lung resection in primary ciliary dyskinesia: a cohort & nested case-control study
title_full Prevalence and course of disease after lung resection in primary ciliary dyskinesia: a cohort & nested case-control study
title_fullStr Prevalence and course of disease after lung resection in primary ciliary dyskinesia: a cohort & nested case-control study
title_full_unstemmed Prevalence and course of disease after lung resection in primary ciliary dyskinesia: a cohort & nested case-control study
title_short Prevalence and course of disease after lung resection in primary ciliary dyskinesia: a cohort & nested case-control study
title_sort prevalence and course of disease after lung resection in primary ciliary dyskinesia: a cohort & nested case-control study
topic Research
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6751891/
https://www.ncbi.nlm.nih.gov/pubmed/31533829
http://dx.doi.org/10.1186/s12931-019-1183-y
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