CLINICAL UTILITY OF HIGH-RESOLUTION PULMONARY COMPUTED-TOMOGRAPHY IN CHILDREN WITH ANTIBODY DEFICIENCY DISORDERS

Objective. To assess the value of high-resolution computed tomography (HRCT) in determining the extent and significance of lung disease in c hildren with antibody deficiency states. Materials and methods. Sevent y HRCT scans performed on 37 children with various antibody deficiency disorders over a 5-year period were retrospectively scored using a pr eviously described demerit scoring system (0-25 with 0 = worst, 25 = b est). Points are subtracted from 25 with increasing severity of diseas e. The potential correlations between CT scores and clinical factors, including age at diagnosis, age at CT, type of immunoglobulin deficien cy, length of respiratory symptoms before diagnosis, number of pneumon ias before diagnosis, type, length and success of therapy, patient com pliance and pulmonary function tests (PFTs), were assessed, Results. O f the 37 children, a demonstrated 22 abnormal scans (CT score less tha n or equal to 22), All nine demonstrated bronchiectasis with a lower l obe and right middle lobe predominance, Statistically significant corr elations were seen between severity of lung disease (CT score) and len gth of respiratory symptoms before diagnosis (p = 0.01), success of th erapy (P = 0.001) and PFTs (P = 0.0008) Of seven children who were fol lowed with repeated scans, 4 of the 7 demonstrated CT scores which imp roved on high-dose intravenous immunoglobulin replacement therapy Conc lusion. HRCT is a useful adjunct to demonstrate the extent and severit y of lung disease at diagnosis and during therapy. Correlation with cl inical factors suggests a higher risk group needing more aggressive ma nagement.