Update in Work Package 1
Standardization and analysis of the identified clinical and epidemiological PCD datasets advances as scheduled in WP1. The largest number so far of cross-sectional and longitudinal datasets of PCD patients from all over the world has been identified and anonymous data are continuously being cleaned and pooled into one standardized dataset. First analyses of the cross-sectional data have begun and a publication on height and body mass index (BMI) of PCD patients is currently under preparation.(Progress in Work Package 1)
So far, researchers involved with WP1 have collected data from over 2500 PCD patients across 18 countries, creating a large PCD meta-cohort. Data collection and data cleaning is ongoing, as more centres contribute datasets from national registries or through RedCap, in the developed standardised format.
The first paper aims to describe age and sex‐adjusted height and BMI of PCD patients, considering both international (WHO) and national reference values, and determine risk factors for poor growth. First analysis results from 1498 patients from 17 countries have been presented at the 1st Young Researchers’ Meeting on PCD in Bern, March 2015 and smaller height was reported in PCD patients compared to reference population. The next papers will focus on lung function, clinical symptoms in patients from different age groups and on the levels of diagnostic certainty. Possible topics for additional publications as well as procedures and a template concept sheet have been discussed with collaborators and data providers.