Handheld lung ultrasound for assessing cystic fibrosis lung disease in children: a diaphragm-ıntegrated scoring approach.

UNLABELLED: Lung ultrasound (LUS) is a radiation-free imaging modality with potential value for monitoring cystic fibrosis (CF) lung disease. However, data on handheld LUS performed by pediatric pulmonologists, as well as the integration of diaphragmatic assessment into LUS scoring, remain limited. This prospective cross-sectional study included clinically stable children with CF (cwCF). LUS was performed independently by a pediatric radiologist using a conventional ultrasound device and by a pediatric pulmonologist using a handheld device. Three LUS scores were evaluated: radiologist-assessed LUS score(LUS-PR), pulmonologist-assessed handheld LUS score(LUS-PP), and diaphragm-integrated LUS score(LUS-DM). Correlations with Modified Chrispin-Norman chest radiography(CXR) scores, spirometry, and clinical variables were analyzed. 35 cwCF were included; the mean age was 7.2 ± 4.8 years, and 22 patients (62.9%) were female. The mean CXR, LUS-PR, LUS-PP, and LUS-DM scores were 3.00 ± 3.31, 2.37 ± 1.93, 2.00 ± 1.82, and 2.77 ± 1.93, respectively. CXR score correlated with LUS-PR (r = 0.55, p < 0.001), LUS-PP (r = 0.49,p = 0.003), and LUS-DM (r = 0.50, p = 0.002). LUS-PR and LUS-PP were strongly correlated (r = 0.91, p < 0.001), with excellent interobserver agreement (ICC = 0.91). FVC z-score correlated with both LUS-PP (r =  - 0.49, p = 0.046) and LUS-DM (r =  - 0.54,p = 0.025), while FEV₁ z-score correlated significantly with LUS-DM (r =  - 0.53,p = 0.030). Chronic airway infection was associated with LUS-DM (r = 0.38, p = 0.024), but not with LUS-PP.

CONCLUSION: Handheld LUS performed by a pediatric pulmonologist showed excellent agreement with radiologist-assessed LUS and correlated with radiographic disease severity in clinically stable children with CF. The diaphragm-integrated LUS-DM score was feasible, and exploratory analyses showed associations with spirometric z-scores and chronic airway infection, supporting further validation of diaphragm-integrated handheld LUS for monitoring cwCF.

WHAT IS KNOWN: • Children with cystic fibrosis require repeated monitoring of structural lung disease throughout follow-up. • Lung ultrasound is a radiation-free bedside imaging tool that may reflect lung involvement in pediatric cystic fibrosis.

WHAT IS NEW: • Handheld LUS by a pediatric pulmonologist showed excellent agreement with radiologist-assessed LUS and correlated with CXR severity. • Diaphragm-integrated LUS was feasible and exploratory analyses showed associations with spirometric z-scores and chronic airway infection.