ArticlePediatric pulmonology2026
The Relationship Between Structural and Functional Pulmonary Status and Quality of Life in Children With Primary Ciliary Dyskinesia.
Article in Pediatric pulmonology, 2026. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Not yet cited in PubMed.
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4 authors.
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Abstract
introductionPrimary ciliary dyskinesia (PCD) is a genetic disorder characterized by impaired mucociliary clearance, leading to recurrent airway infections and bronchiectasis. Monitoring disease progression requires both functional and structural assessments of the lungs. This study aims to investigate the relationship between lung function, structural abnormalities, and quality of life in patients with PCD. MATERIALS AND
methodsThe study included 30 genetically confirmed PCD patients under routine follow-up and had no active infection at the time of evaluation. Demographic and clinical data were recorded. Pulmonary function tests (PFT), including spirometry, plethysmography, and diffusion capacity for carbon monoxide, performed within the past year, were analyzed. Chest computed tomography (CT) scans were evaluated using the modified Bhalla scoring system. The St. George's Respiratory Questionnaire (SGRQ) was administered during the most recent follow-up.
resultsThe median age of the 30 PCD patients (male-to-female ratio: 17:13) was 11.6 years. The median FEV
conclusionsPulmonary functional impairment and reduced health-related quality of life in children with PCD are closely related to the perceived burden of disease. Although structural abnormalities on HRCT do not directly affect quality-of-life scores, they correlate with functional impairment.
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