Non-Cystic Fibrosis Bronchiectasis in children: a single center experience

Abstract

Background: Non-cystic-fibrosis bronchiectasis (NCFB) is still one of the most common causes of childhood morbidity. Objective: the study aimed to determine aetiology, clinical presentation, lobar distribution and microbiologic colonization of NCFB in children. Methods: we performed a retrospective review of clinical, laboratory and instrumental data from 132 non-CF patients with diagnosis of bronchiectasis. All the included patients were evaluated from 2010 to 2018. Results: Mean age at respiratory symptoms’ onset and diagnosis were 9.7 years (± 4.6 SD) and 10.6 years (±5.95 SD), respectively. The aetiology was as follows: post-infectious (39%), primary ciliary dyskinesia (30%), esophageal atresia with trachea-esophageal fistula (8%), secondary (7%) and primary immune deficiency (6%), bronchial malformation (3%), isolated asthma (3%), chronic aspiration (2%), pulmonary fibrosis with TBX4 gene mutation (1%), broncorrea in patient with CFTR polymorphism (1%). Chronic cough (45%), recurrent pneumonia (25%), single pneumonia episode (16%), recurrent wheezing (11%), were the commonest respiratory problems. Left and right lower lobes, medium lobe were commonly involved by cilindric bronchiectasis. H. Influenza (35, 31%), S. Aureus (20, 18%), P. Aeruginosa (20, 18%), were the more frequently isolated bacteria. 51 and 13 patients needed prophylactic oral (azithromycin) and inhaled antibiotics (tobramycin or colistin), respectively. Conclusions: this is the largest study in pediatric NCFB. As the management of NCFB affected patients remains problematic, a better definition of clinical, radiological and microbiological features of NCFB is required to improve their management. Reference: 1. BMC Pediatrics 2014;14: 299