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

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

SUBMITTER: Kouis P 

PROVIDER: S-EPMC6751891 | biostudies-literature | 2019 Sep

REPOSITORIES: biostudies-literature