Abstract

The most frequent cause of bronchiectasis in children is cystic fibrosis. Several underlying conditions in patients with non-CF bronchiectasis should be excluded: recurrent respiratory infection (RRI), aspiration, ciliary dyskinesia, primary and secondary immunodeficiency, congenital malformations and others. We retrospectively analyzed 155 children with non-CF bronchiectasis attending our department in the last 20 years. In our series, 62.5% had RRI, 13.8% specific diseases (bronchopulmonary dysplasia 4.5%, malformations 7.4%, post infectious bronchiolitis obliterans 1.3%, alpha1antitripsin deficiency 0.6%), 9.1% inhalation (gastroesophageal reflux 1.3%, esophageal atresia 3.2%, tracheoesophageal fistula 1.9%, neurological disorders 4.5%), 4.9% immunological defects, 3.7% cardiac malformations with pulmonary overflow, 2.6% ciliary dyskinesia, 2.1% had other causes, 1.3% were idiopathic. 38.1% of patients with RRI had the first infection within the first year of life and 5.2% required admission in intensive care unit. Diagnosis was made with chest CT scan at average age of 5.8 years, after a mean of 3.3 chest X-ray per patient. We analyzed CT scan applying modifiedReiff score, based on the number of lobes involved (including lingula) and the severity of bronchial dilatation (min 0 max 18). In our series the mean value was 2.36. In our series the prevalence of comorbidities and specific underlying diseases reflect those in literature. Most of our patients presented RRI as main cause of non-CF bronchiectasis and it could be possible that idiopathic bronchiectasis in adults may origin during childhood, but further studies are necessary to confirm this hypothesis.

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