Primary ciliary dyskinesia (PCD) has been considered a relatively mild disease, especially compared to cystic fibrosis (CF), but studies on lung function in PCD patients have been few and small.This study compared lung function from spirometry of PCD patients to normal reference values and to published data from CF patients. We calculated z-scores and % predicted values for forced expiratory volume in 1 s (FEV) and forced vital capacity (FVC) using the Global Lung Function Initiative 2012 values for 991 patients from the international PCD Cohort. We then assessed associations with age, sex, country, diagnostic certainty, organ laterality, body mass index and age at diagnosis in linear regression models. Lung function in PCD patients was reduced compared to reference values in both sexes and all age groups. Children aged 6-9 years had the smallest impairment (FEV z-score -0.84 (-1.03 to -0.65), FVC z-score -0.31 (-0.51 to -0.11)). Compared to CF patients, FEV was similarly reduced in children (age 6-9 years PCD 91% (88-93%); CF 90% (88-91%)), but less impaired in young adults (age 18-21 years PCD 79% (76-82%); CF 66% (65-68%)). The results suggest that PCD affects lung function from early in life, which emphasises the importance of early standardised care for all patients.

Type Scientific Poster Presentation Topic Pediatric RadiologyPurpose Cystic fibrosis (CF) is characterized by chronic respiratory infections and functional impairment of the lung. Lung function tests such as nitrogen multiple breath washout (N2-MBW), are sensitive in detecting ventilation inhomogeneity, but cannot determine its exact origin. Novel magnetic resonance imaging (MRI) methods such as matrix pencil decomposition MRI can visualize functional changes in the lung without the administration of contrast agents and the need for breathing maneuvers.Objectives: To examine the correlation between novel functional MRI and lung function tests in patients with CF. Methods and MaterialsMethods: Forty patients with CF (mean age 11.7 years, range 6-18) underwent MRI and lung function tests on the same day. Functional MRI provided semiquantitative measures of the perfusion (RQ) and ventilation (RFV) impairment as percentages of the affected lung volume. Morphological MRI was evaluated using a CF-specific score. N2-MBW provided information about global (lung clearance index, LCI) ventilation inhomogeneity. ResultsResults: MRI detected functional impairment in all patients with CF: RFV ranged from 19% to 38% and RQ ranged from 16% to 35%. RFV and RQ were strongly correlated with LCI (r=0.76, p<0.001; r=0.85, p<0.001, respectively), as well as total morphology scores and sub-scores. ConclusionConclusions: Non-invasive functional MRI is a promising method to detect and visualize perfusion and ventilation impairment in CF without the need of contrast agents or breath holding maneuvers. AffirmationsAuthors agreement: I confirm that all authors indicated within the author block of this abstract have been informed about this abstract submission and agreed to the same. (I confirm) Mandatory presenter registration: I understand that if the presenting author has not paid his registration fee by April 24, 2017, he will not be permitted to display the poster or hold the presentation and the abstract will not be published. (I agree)Copyright: I affirm that if my poster contains any material that has been previously published, I was entitled to use this material by applicable law or have obtained a transferable license from the copyright holder. (I confirm) Seite