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Bronchiectasis secondary to primary immunodeficiency in children: Longitudinal changes in structure and function
Author(s) -
Haidopoulou Katerina,
Calder Alistair,
Jones Alison,
Jaffe Adam,
Sonnappa Samatha
Publication year - 2009
Publication title -
pediatric pulmonology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.866
H-Index - 106
eISSN - 1099-0496
pISSN - 8755-6863
DOI - 10.1002/ppul.21036
Subject(s) - bronchiectasis , medicine , spirometry , high resolution computed tomography , pulmonary function testing , lung , asthma
Background Primary immunodeficiency is a common cause of bronchiectasis in children. The term bronchiectasis suggests an irreversible process; however, disease progression following treatment is controversial. The aim of this study was to evaluate the progression of bronchiectasis in children with primary immunodeficiency after institution of treatment. Methods A retrospective review of case notes of children with primary immunodeficiency was undertaken to identify patients with confirmed bronchiectasis. Children who had two high‐resolution computed tomography scans of the chest (HRCT chest) with an interval of at least 2 years were identified. The HRCT‐chest scans at diagnosis and follow up were scored using a Bhalla score. Spirometry results (FEV 1 , FVC, and FEV 1 :FVC ratios) were related to HRCT‐chest scores, where available. Statistical analysis was by Wilcoxon signed rank test and Spearman's rank order correlation. Results Eighteen subjects were studied. The diagnosis of primary immunodeficiency was established at median (range) age 3.4 (1–13) years, and bronchiectasis at 9.3 (3.1–13.8) years. There was no significant difference between baseline and follow‐up median (range) HRCT‐chest scores (6 [1–13] and 7.5 [0–15], P = 0.21) respectively. The follow‐up FEV 1 and FVC percent predicted median (range) were significantly higher than baseline (86% [49–124%] vs. 75% [36–93%], P < 0.005, and 86% [47–112%] vs. 78% [31–96%], P < 0.05), respectively; there was no significant difference between baseline and follow‐up FEV 1 :FVC ratios. There was no significant correlation between HRCT‐chest score changes and FEV 1 or FVC changes. Conclusions Bronchiectasis secondary to primary immunodeficiency in childhood is not always a progressive condition, suggesting a potential to slow or prevent disease progression with appropriate treatment. Pediatr Pulmonol. 2009; 44:669–675. © 2009 Wiley‐Liss, Inc.