Abstract
Rationale: Primary ciliary dyskinesia (PCD), an inherited lung disease, is characterized by abnormal ciliary function leading to progressive bronchiectasis. There is wide variability in respiratory disease severity at birth and later in life. Objectives: To evaluate the association between neonatal hospital length of stay (neonatal-LOS) and supplemental oxygen duration (SuppO(2)) with lung function in pediatric PCD. We hypothesized that longer neonatal-LOS and SuppO(2) are associated with worse lung function (i.e., forced expiratory volume in 1 second percent predicted [FEV(1)pp]). Methods: We performed a secondary analysis of the Genetic Disorders of Mucociliary Clearance Consortium prospective longitudinal multicenter cohort study. Participants enrolled, during 2006-2011, were <19 years old with a confirmed PCD diagnosis and followed annually for 5 years. The exposure variables were neonatal-LOS and SuppO(2), counted in days since birth. The outcome, FEV(1)pp, was measured annually by spirometry. The associations of neonatal-LOS and SuppO(2) with FEV(1)pp were evaluated with a linear mixed-effects model with repeated measures and random intercepts, adjusted for age and ciliary ultrastructural defects. Results: Included were 123 participants (male, 47%; mean enrollment age, 8.3 yr [range, 0 to 18 yr]) with 578 visits (median follow-up, 5 yr). The median neonatal-LOS was 9 d (range, 1 to 90 d), and median SuppO(2) was 5 d (range, 0 to 180 d). Neonatal-LOS was associated with worse lung function (-0.27 FEV(1)pp/d [95% confidence interval, -0.53 to -0.01]; P = 0.04). SuppO(2) was not associated with lung function. Conclusions: Neonatal-LOS is associated with worse lung function in pediatric PCD, independent of age and ultrastructural defects. Future research on the mechanisms of neonatal respiratory distress and its management may help us understand the variability of lung health outcomes in PCD.