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 (FEV1, FVC, and FEV1: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 FEV1 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 FEV1 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.
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 (FEV1, FVC, and FEV1: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 FEV1 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 FEV1 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.
Authors: Andrew J Collaro; Anne B Chang; Julie M Marchant; Ian B Masters; Leanne T Rodwell; Allan J Takken; Margaret S McElrea Journal: Lung Date: 2020-02-20 Impact factor: 2.584
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Authors: Anne B Chang; Keith Grimwood; Colin F Robertson; Andrew C Wilson; Peter P van Asperen; Kerry-Ann F O'Grady; Theo P Sloots; Paul J Torzillo; Emily J Bailey; Gabrielle B McCallum; Ian B Masters; Catherine A Byrnes; Mark D Chatfield; Helen M Buntain; Ian M Mackay; Peter S Morris Journal: Trials Date: 2012-08-31 Impact factor: 2.279
Authors: Anne B Chang; Jeanette Boyd; Leanne Bell; Vikas Goyal; I Brent Masters; Zena Powell; Christine Wilson; Angela Zacharasiewicz; Efthymia Alexopoulou; Andrew Bush; James D Chalmers; Rebecca Fortescue; Adam T Hill; Bulent Karadag; Fabio Midulla; Gabrielle B McCallum; Deborah Snijders; Woo-Jung Song; Thomy Tonia; Keith Grimwood; Ahmad Kantar Journal: ERJ Open Res Date: 2021-07-19
Authors: Anne B Chang; Keith Grimwood; Andrew C Wilson; Peter P van Asperen; Catherine A Byrnes; Kerry-Ann F O'Grady; Theo P Sloots; Colin F Robertson; Paul J Torzillo; Gabrielle B McCallum; Ian B Masters; Helen M Buntain; Ian M Mackay; Jacobus Ungerer; Joanne Tuppin; Peter S Morris Journal: Trials Date: 2013-02-20 Impact factor: 2.279