Joint Annual Meeting of the Schweizerische Gesellschaft für Kardiologie / Société Suisse de Cardiologie. Schweizerische Gesellschaft für Herz- und thorakale Gefässchirurgie / Société Suisse de Chirurgie Cardiaque et Vasculaire Thoracique. Schweizerische Gesellschaft für Pneumologie / Société Suisse de Pneumologie. June 15-17, 2016, Lausanne: Abstracts
Data on lung function in patients with PCD are few and contradictory. Within the EU project BESTCILIA, we compared lung function (FEV 1 ) of patients with PCD to the Global lung function initiative (GLI) 2012 reference values and to published data from UK patients with Cystic Fibrosis (CF; Goss et al. Thorax 2015). Methods: We calculated z-scores and %predicted values for FEV 1 using the GLI reference, and used a multilevel linear regression model, adjusted for age, sex and study centre, to
... unt for repeated measurements. Results: We obtained 4683 FEV 1 measurements of 648 PCD patients from 15 centres. The median age was 16 years (range 6-70), 295 (46%) were females. In the multilevel model FEV 1 z-scores (95% CI) were significantly lower than GLI values (FEV 1 -1.5 (-1.6 to -1.4); p < 0.005). Both sexes and all age groups were affected, with a smaller difference for children aged <10 years (FEV 1 z score -1.18 (-1.3 to -1.05). Compared to published data for CF, FEV 1 %predicted (95% CI) was similar in children (e.g. age 6-9: PCD: 87% (86-89%); CF: 90% (88-91%)); but better in adult PCD patients (age 18-21: PCD: 76% (73-80%); CF: 66% (65-68%)). Conclusion: This is the largest study ever conducted on spirometry in patients with PCD. FEV 1 was significantly reduced compared to normal reference values for all age groups and both sexes. Young children with PCD and CF both had a low FEV 1 , which later in life remained relatively stable in PCD unlike in CF. In the ongoing study, we will analyse determinants of lung function, particularly age at diagnosis, time since diagnosis, and diagnostic certainty.