Paediatric Pulmonology

Related Articles

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

Respir Res. 2019 Sep 18;20(1):212

Authors: Kouis P, Goutaki M, Halbeisen FS, Gioti I, Middleton N, Amirav I, Israeli PCD Consortium, Barbato A, Italian PCD Consortium, Behan L, Boon M, Emiralioglu N, Haarman EG, Karadag B, Koerner-Rettberg C, Lazor R, Swiss PCD Group, Loebinger MR, Maitre B, French Reference Centre for Rare Lung Diseases, Mazurek H, Morgan L, Nielsen KG, Omran H, Özçelik U, Price M, Pogorzelski A, Snijders D, PCD Italian Consortium, Thouvenin G, French Reference Centre for Rare Lung Diseases, Werner C, Zivkovic Z, Kuehni CE, Yiallouros PK

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.

PMID: 31533829 [PubMed - in process]

Library News

Image result for new

 

WMUH Library Discovery Tool

Check out our guide for a brief overview, how to access and use the Discovery tool. Trouble accessing or have questions?  Please contact us

BMJ Best Practice, clinical decision support tool is now available. Access it on the Trust intranet without any password, to access remotely login with NHS OpenAthens, download the app to access on mobile devices anywhere. See the  user guide for details.

Accessing Articles
Articles from journals marked in green are freely available or available in print in the library, or are available by using your NHS Athens account. You may need to click on 'Log in with Athens' to get an Athens login box.

If you don't have an NHS Athens account, you can register online, and if you do this on an NHS PC, you'll receive a confirmation email the same day.

Journals marked in orange aren't available online, but we hold print copies in the Library.

Journals marked in red aren't available online or in the Library but we can order articles  via our Inter Library Loan Service. There is a small charge for this. Please contact the library on ext 5968 or email Library.InfoService@chelwest.nhs.uk  for more information.

Quick Links
qrcode.14118297[1]