engleski

MEASUREMENTS OF NITRIC OXIDE IN CHILDREN WITH PRIMARY AND SECONDARY CILIARY DYSKINESIA

Introduction: Primary ciliary dyskinesia (PCD) is an inherited disease related to ciliary disfunction, with heterogenity in clinical presentation and in ciliary ultrastuctural defect. Up to present day, three types of cilia are known: primary, nodal and motile. Cilia are complex, likely involving more than 1000 gene products. Early diagnosis is important to prevent disease progression. Nasal nitric oxide (nNO) measurement is an established first line test in the work-up for PCD. Objective: To examine the usefulness of nasal (nNO) and bronchial exhaled nitric oxide (FeNO) measurements in detecting PCD in children. Method: Measurements were done in 5 PCD children and 9 children with secondary ciliary dyskinesia (SCD), aged between 5 and 16 years. They were diagnosed with PCD or SCD syndrome based on their clinical presentation and electron microscopy findings of bronchial biopsy sample. Results: No significant differences were found on the basis of age or ventilatory function test between the PCD patients and SCD groups. Values of FeNO were significantly lower in PCD group (5-14, 9 ppb), compared to group with SCD (7-46 ppb). Values of nNO in PCD group varied from 10 to 2819 ppb, and for SCD group varied from 19, 4 to 2136 ppb. Diagnosis of recurrent otitis was established in 4 patients (28, 6%), recurrent sinusitis in 9 (64, 3%), chronic productive cough in 9 (64, 3%), and bronchitis in 8 (57%). Conclusion: The measurements of FeNO appears to be a useful tool for screening children with PCD and SCD, so it should be performed before nasal biopsy and electron microscopy studies.

primary ciliary dyskinesia secondary ciliary dyskinesia bronchial exhaled nitric oxide ( FeNO)

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