The relationship between quality of life, structural and functional status of the lung in children with primary ciliary dyskinesia

<bold>Introduction:</bold> Patients with Primary Ciliary Dyskinesia (PCD),a hereditary chronic disorder, are regularly monitored using spirometry and imaging techniques. Quality-of-life surveys tailored to PCD offer a more comprehensive understanding of social effects. Our study examines the relationship between lung function, structural status, and quality of life in PCD patients. <bold>Methods:</bold> Thirty patients with genetically diagnosed PCD who were admitted to our outpatient clinic for a check-up and had no signs or complaints of active infection were included in the study. Chest X-rays and thorax tomographies performed within the last year were scored with the Chrispin-Norman and modified Bhalla score by a radiologist who did not know about the patients' clinic. Pulmonary function tests(PFT) were recorded. St George's Respiratory Questionnaire(SGRQ) was administered to the patients. <bold>Results:</bold> The median value for FEV1 z-score was -1.6. Due to the Bhalla score, 80% of the patients had mild damage, and 20% had moderate damage. A negative correlation was determined between FEV1(p:0.001), FVC(p:0.007), FEF25-75(p:0.005), and FEV1/FVC (p:0.008) and Bhalla score. A positive correlation was detected between the number of exacerbations in the last year and the symptom scores(p:0.001), activity score(p:0.005),exposure score(p:0.004),and the total score of the SGQR(p;0.001). <bold>Conclusion:</bold> A correlation has been identified between the quality of life scale and PFT, as well as between the Bhalla score and PFT. Harmony between Bhalla scoring and spirometry emphasizes the importance of choosing spirometry to avoid unnecessary radiation.