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

ABSTRACT

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.