Pulmonary function evaluation in pediatric patients with primary immunodeficiency complicated by bronchiectasis.

Area of Science:

• Pediatric Pulmonology
• Immunology
• Radiology

Background:

• Primary immunodeficiency (PID) is associated with recurrent respiratory infections, potentially impacting lung function.
• The relationship between airway abnormalities on chest CT, dyspnea severity, and pulmonary function test (PFT) results in pediatric PID patients requires further investigation.

Purpose of the Study:

• To investigate the association between airway structural abnormalities on chest CT, dyspnea severity, and PFT parameters in children with PID.
• To evaluate the utility of chest CT scans in assessing bronchiectasis and pulmonary function impairment in pediatric PID.

Main Methods:

• Nineteen children diagnosed with PID were enrolled.
• Chest CT scans (modified Reiff scores), PFT parameters, and mMRC dyspnea scale were analyzed.
• Spearman's rank correlation test was used to assess relationships between variables.

Main Results:

• Thirteen out of nineteen children had bronchiectasis on chest CT.
• Modified Reiff scores negatively correlated with FEV1 (% predicted) and FEV1/FVC ratio.
• Dyspnea severity (mMRC scale) negatively correlated with FEV1 and FVC, and positively with RV and RV/TLC, indicating airflow obstruction and air trapping.

Conclusions:

• Chest CT is valuable for diagnosing bronchiectasis and assessing pulmonary function impairment in pediatric PID.
• Airflow obstruction and air trapping due to bronchiectasis contribute to reduced quality of life in these patients.