This retrospective cross-sectional study examined the relationships among lung function (spirometry, plethysmography, DLCO), structural lung damage (modified Bhalla CT score), and quality of life (SGRQ) in 30 genetically confirmed PCD children (median age 11.6 years) evaluated during clinically stable periods over a 1-year window.
SGRQ total scores correlated negatively with FEV1/FVC (r = –0.502, p = 0.025) and FEF25-75 (r = –0.519, p = 0.016), and positively with exacerbation frequency. Modified Bhalla scores correlated with FEV1 (r = –0.637, p = 0.008), FVC, FEF25-75, and FEV1/FVC, but **not** with SGRQ scores. Mild obstruction was present in 48%, moderate in 24%, and severe in 20%; 80% had mild and 20% moderate structural damage on HRCT.
- Small sample (n = 30) from a single tertiary center limits generalizability. - SGRQ was not designed or validated for pediatric PCD; QOL-PCD was intentionally excluded. - Cross-sectional design prevents causal or temporal inferences about the sequence of structural, functional, and QoL changes.
In children with PCD, patient-reported symptom burden (SGRQ) tracks with spirometric decline and exacerbation frequency, but not with CT structural scores — so PFT, imaging, and QoL tools each capture distinct information and should be used together. Clinicians should not rely on spirometry alone to gauge disease progression, as structural damage may precede functional change.
