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Update in Work Package 1

WP1 work, whose main aim is to analyse existing clinical and epidemiological PCD datasets, advances as scheduled. A large number of cross-sectional and longitudinal datasets of PCD patients from all over the world has been identified. Anonymous data from eligible studies have been obtained and are currently being cleaned and pooled into a standardized dataset. First analyses on the cross-sectional data have begun.

So far, data from over 2000 PCD patients across 15 countries have been collected. The datasets have been carefully examined and proved considerably heterogeneous. In order to obtain comparable data which could be pooled in a meta-analysis we proposed a standardised dataset with a detailed variable list. This has been achieved through the implementation of a RedCap database, which allows to enter and extract data via a secure online platform. All collaborators only have access to their own data. The database also allows for migration of retrospective data into the WP2 European registry.

Data collection through Redcap and data cleaning is ongoing and analysis plans are being set up. The first paper will look at cross-sectional data on height and body mass index (BMI) in both paediatric and adult PCD patients and compare these to international reference values (WHO growth reference data). This allows to assess whether physical growth is affected in PCD and to determine risk factors for poor growth and underweight. An abstract presenting preliminary results has been accepted for presentation at the ERS annual conference in 2014 in Munich. The next papers will focus on clinical symptoms in patients from different age groups on the levels of diagnostic certainty and lung function.