Gaz exchange, nasal nitric oxyde levels and lung function tests in children with primary ciliary dyskinesia

Abstract

Introduction: Capillary partial pressure of O 2 (PcapO 2 ) was found to decrease with age in children with primary ciliary dyskinesia (PCD) while PcapCO 2 remained steady. Objectives: To further explore the relationship between PcapO 2 , nasal Nitric Oxide (nNO) output and lung function (LF) in children with PCD. Patients and methods: Retrospective monocentric study in children referred to our national paediatric reference center for rare lung diseases for PCD diagnostic work-up, and in whom ear lobe capillary blood gas was available along with nNO and/or LF measurements. Results: 30 PCD children were included (18 girls, median age 11.5 (0.7 to 18.2) years). Median PcapO 2 was -1.9 (-5.2 to 2.2) z-score, median nNO in 27 children was 61 (20.3 to 697) ppb and median FEV 1 in 24 children was -2 (-4.7 to 2.18) z-score. In univariate analysis, there was a significant relationship between PcapO 2 and nNO, age, and LF indices (see table). In multivariate analysis, there was a significant relationship between PcapO 2 and nNO (p=0.02), and FEF 75% (p Conclusion: In the study children, low PcapO 2 was correlated with decrease nNO output and one LF indice. This could explain the early occurrence of hypoxemia in the course of PCD disease.