{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,1]],"date-time":"2026-09-01T23:48:58Z","timestamp":1788306538979,"version":"build-2803163510"},"reference-count":30,"publisher":"Wiley","issue":"7","license":[{"start":{"date-parts":[[2009,6,9]],"date-time":"2009-06-09T00:00:00Z","timestamp":1244505600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Pediatric Pulmonology"],"published-print":{"date-parts":[[2009,7]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Background<\/jats:title><jats:p>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.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>A retrospective review of case notes of children with primary immunodeficiency was undertaken to identify patients with confirmed bronchiectasis. Children who had two high\u2010resolution computed tomography scans of the chest (HRCT chest) with an interval of at least 2 years were identified. The HRCT\u2010chest scans at diagnosis and follow up were scored using a Bhalla score. Spirometry results (FEV<jats:sub>1<\/jats:sub>, FVC, and FEV<jats:sub>1<\/jats:sub>:FVC ratios) were related to HRCT\u2010chest scores, where available. Statistical analysis was by Wilcoxon signed rank test and Spearman's rank order correlation.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Eighteen subjects were studied. The diagnosis of primary immunodeficiency was established at median (range) age 3.4 (1\u201313) years, and bronchiectasis at 9.3 (3.1\u201313.8) years. There was no significant difference between baseline and follow\u2010up median (range) HRCT\u2010chest scores (6 [1\u201313] and 7.5 [0\u201315], <jats:italic>P<\/jats:italic>\u2009=\u20090.21) respectively. The follow\u2010up FEV<jats:sub>1<\/jats:sub> and FVC percent predicted median (range) were significantly higher than baseline (86% [49\u2013124%] vs. 75% [36\u201393%], <jats:italic>P<\/jats:italic>\u2009&lt;\u20090.005, and 86% [47\u2013112%] vs. 78% [31\u201396%], <jats:italic>P<\/jats:italic>\u2009&lt;\u20090.05), respectively; there was no significant difference between baseline and follow\u2010up FEV<jats:sub>1<\/jats:sub>:FVC ratios. There was no significant correlation between HRCT\u2010chest score changes and FEV<jats:sub>1<\/jats:sub> or FVC changes.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions<\/jats:title><jats:p>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\u2013675. \u00a9 2009 Wiley\u2010Liss, Inc.<\/jats:p><\/jats:sec>","DOI":"10.1002\/ppul.21036","type":"journal-article","created":{"date-parts":[[2009,6,9]],"date-time":"2009-06-09T19:18:59Z","timestamp":1244575139000},"page":"669-675","source":"Crossref","is-referenced-by-count":64,"title":["Bronchiectasis secondary to primary immunodeficiency in children: Longitudinal changes in structure and 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