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Pulmonary function evaluation in pediatric patients with primary immunodeficiency complicated by bronchiectasis
Chun-Che Chiu1, Chao-Jan Wang2, Wen-I Lee3
1Division of Pediatric Pulmonology, Department of Pediatrics, Chang Gung Memorial Hospital at Linkou, and Chang Gung University, Taoyuan, Taiwan.
Insights
Primary immunodeficiency in children often leads to recurrent respiratory infections and lung damage. Chest CT scans can diagnose bronchiectasis and assess pulmonary function decline, correlating with dyspnea and reduced quality of life.
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.
Background:
Primary immunodeficiency (PID) accompanying with recurrent respiratory infections is thought to have a devastating effect on lung function. However, the associations between the airway structural abnormalities on chest computed tomography (CT), severity of dyspnea, and deterioration of pulmonary function test (PFT) have not been fully addressed.
Methods:
Children diagnosed with PID in a tertiary referred center in northern Taiwan were enrolled. Demographic and clinical data including age, sex, age at diagnosis of PID, and follow-up period were collected. Chest CT images (modified Reiff scores), parameters of PFT, and life quality questionnaires (mMRC dyspnea scale) were analyzed and correlated using Spearman's rank correlation test.
Results:
A total of nineteen children with PID were enrolled and thirteen patients were diagnosed as having bronchiectasis based on chest CT scans. Modified Reiff scores of chest CT scan were negatively correlated with FEV1 (% predicted) and FEV1/FVC ratio (P < 0.05). A strongly negative correlation was found between the mMRC dyspnea scale and FEV1 (% predicted) and FVC (% predicted), but positively correlated with RV (% predicted) and RV/TLC ratio (P < 0.05). Furthermore, there was a negative correlation between FVC (% predicted) with increasing follow-up period (P < 0.05).
Conclusions:
In pediatric patients with PID, chest CT scan appears to be a good tool for not only the diagnosis of bronchiectasis, but also the degree of pulmonary function impairment. Further quality of life impairments could be particularly due to the airflow obstruction and air trapping related to bronchiectasis.
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