A Descriptive Study of Non-Cystic Fibrosis Bronchiectasis in a Pediatric Population from Central and Southern Italy

Abstract

Background: Non-cystic fibrosis (CF) bronchiectasis is now identified more often than in the past. Objectives: It was the aim of this study to assess the high-resolution computed tomography (HRCT) localization and extent of bronchiectasis and to determine whether asthma status, atopy and bronchiectasis distribution are associated with the etiology of bronchiectasis. Methods: We retrospectively analyzed clinical, laboratory, functional and HRCT data of 105 children with non-CF bronchiectasis at 2 tertiary respiratory units in Italy. Forty cases had bronchiectasis associated with ongoing underlying conditions, namely primary ciliary dyskinesia, primary immunodeficiency or aspiration. Results: Age at the onset of chronic cough/wheeze and at the first X-ray-documented pneumonia as well as atopy prevalence were lower in patients with ongoing underlying conditions than in those without (p = 0.049, p = 0.003 and p = 0.0008, respectively). In most cases, bronchiectasis was multilobar, and a mean of 2.5 lobes were involved. The right side was more often involved than the left (88 vs. 70%; p = 0.002), and the upper lobes were relatively spared (p < 0.000001). Right lung involvement and multilobar disease were more prevalent in children younger than 2 years at first pneumonia (p < 0.05). Conclusions: Clinical information combined with laboratory data provides additional insights into the characteristics of non-CF bronchiectasis in a large population of Italian children. This study highlights the need for longitudinal evaluations, also using HRCT, of severe and non-resolving pneumonia in children.

Antibiotics for bronchiectasis exacerbations in children: rationale and study protocol for a randomised placebo-controlled trial

“…Despite the considerable global burden, bronchiectasis services receive disproportionately fewer allocated resources (clinical and research) when compared with other chronic respiratory diseases [3,54,55]. The marked paucity of RCTs [21,55] is reflected in the existence of only a single (small) published placebo-controlled RCT in children with bronchiectasis [21,56].…”

“…Although regarded as an ‘orphan disease’ in affluent countries, reports of prevalence of bronchiectasis are increasing [1,2]. Bronchiectasis remains a major contributor to chronic respiratory morbidity [2,3] and mortality [1,4] in both Indigenous [5] and non-Indigenous populations [6]. In our recently completed multicentre study of children newly referred for chronic cough and managed in accordance to a standardised protocol [7], 31 (9%) of the 346 children had bronchiectasis proven on radiology [8].…”

Respiratory viruses in exacerbations of non-cystic fibrosis bronchiectasis in children

“…Non-cystic fibrosis (non-CF) bronchiectasis is being increasingly diagnosed and recognised as an important contributor to chronic lung disease in both adults and children in developing1 and developed countries 2. Pulmonary exacerbations increase morbidity,3 and are associated with progressive deterioration in lung function4 and poor quality of life,5 but little research data are available.…”

Computed tomography dose optimisation in cystic fibrosis: A review

“…There may also be a role for lowdose protocols in nonCF bronchiectasis, which accounts for 10% of referrals to tertiary respiratory centres [15] . In 50% of these new referrals, patients are misdiagnosed with asthma until the true diagnosis is confirmed at CT [93,94] . Once a diagnosis of CF is established, some experts suggest that lowdose CT should be performed biannually for assessment of lung parenchyma and bronchoalveolar structures in place of chest radiography [95] .…”