Longitudinal variation of nasal nitric oxide in patients with Primary Ciliary Dyskinesia and healthy controls

Introduction Primary Ciliary Dyskinesia (PCD) is a rare genetic disorder affecting several organs due to malfunctioning of motile cilia. Nasal nitric oxide (nNO) is significantly decreased in PCD and is part of the diagnostic process. However, the longitudinal variation of nNO levels remains unknown. The aim of our study was to investigate the longitudinal variation of nNO levels in patients with PCD and healthy controls.

Methods NNO was measured using the Ecomedics device Analyzer CLD 88sp. 20 patients with PCD and 20 healthy controls (0-64 years) with at least four nNO measurements in one year were included. The measurements were comprised of three breathing manoeuvres – tidal breathing (TB), breath hold (BH) and exhalation against resistance (ER). Data were analysed regarding the coefficient of variation between the measurements and differences between the two groups.

Results For patients with PCD TB, BH and ER measurement had an average variation of 32.16% (standard deviation (SD): 11.97); 29.45% (SD: 15.04) and 29.88% (SD: 14.82) respectively. Healthy controls had an average variation in TB, BH and ER measurement of 22.59% (SD: 11.97); 18.7% (SD: 11.2) and 19.47% (SD: 9.73) respectively. The difference of the variation between both groups was significant for TB (p=0.015), BH (p=0.043) and ER (p=0.023) measurements.

Conclusion Our data show that intraindividual nNO values were more variable over the course of one year in PCD patients than in healthy controls. This data supports further investigation of nasal NO as a possible non-invasive quantitative biomarker of disease activity and response to therapy for patients with PCD.

Publication History

Article published online:
21 September 2022