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; ... (2019). Prevalence and course of disease after lung resection in primary ciliary dyskinesia: a cohort & nested case-control study. Respiratory research, 20(1), p. 212. BioMed Central 10.1186/s12931-019-1183-y

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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.

Item Type:

Journal Article (Original Article)

Division/Institute:

04 Faculty of Medicine > Pre-clinic Human Medicine > Institute of Social and Preventive Medicine (ISPM)

UniBE Contributor:

Goutaki, Myrofora; Halbeisen, Florian Samuel and Kühni, Claudia

Subjects:

600 Technology > 610 Medicine & health
300 Social sciences, sociology & anthropology > 360 Social problems & social services

ISSN:

1465-9921

Publisher:

BioMed Central

Funders:

[4] Swiss National Science Foundation

Language:

English

Submitter:

Beatrice Minder Wyssmann

Date Deposited:

20 Sep 2019 14:14

Last Modified:

26 Oct 2019 04:10

Publisher DOI:

10.1186/s12931-019-1183-y

PubMed ID:

31533829

Uncontrolled Keywords:

Ciliary motility disorders (MeSH) Kartagener syndrome (MeSH) Lobectomy

BORIS DOI:

10.7892/boris.133410

URI:

https://boris.unibe.ch/id/eprint/133410

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