Non-cystic fibrosis bronchiectasis in children and adolescents: Neglected and emerging issues

Abstract

Pediatric non-cystic fibrosis (CF) bronchiectasis is characterized by endobronchial suppuration, airway neutrophilic inflammation and poor mucus clearance and is associated with persistent productive cough due to recurrent airway infections. Most recommendations are based on expert opinion or extrapolated from CF practice. The present narrative review aims to address some issues on the management of children or adolescents with non CF-bronchiectasis that still require attention, and analyze what available literature offers to reply to open questions. We focused on the potential offered by technological advances on lung disease assessment through novel chest imaging techniques and new or old pulmonary function tests. We also summarized the main novelties in the disease prevention and treatment. Finally, a novel diagnostic algorithm is proposed, that might help physicians in the daily clinical decision-making process. Future directions for research on pediatric non-CF bronchiectasis should include larger study populations and longer prospective clinical trials, as well as new clinical and laboratory endpoints to determine the underlying mechanisms of lung disease progression and support the role of new and existing treatments.

Surgical treatment of non-cystic fibrosis bronchiectasis in Central Europe

“…A congenital form of non-CF-bronchiectasis has been observed (4,5), but acquired variations are far more abundant. Repeated respiratory tract infections, especially by tuberculosis, measles or pertussis, are the main cause for developing bronchiectasis, even though bronchial obstruction by tumor or foreign body aspiration may play a role.…”

“…Repeated respiratory tract infections, especially by tuberculosis, measles or pertussis, are the main cause for developing bronchiectasis, even though bronchial obstruction by tumor or foreign body aspiration may play a role. Additionally, bronchiectasis has been seen in allergic bronchopulmonary aspergillosis, primary ciliary dyskinesia, α1-antitrypsin deficiency, congenital and acquired immunodeficiency states, intralobar or extralobar sequestrations, sarcoidosis and non-tuberculous mycobacteria (NTM)-Infection (2)(3)(4)(5). Chronic obstructive pulmonary disease (COPD) patients showing evidence of bronchiectasis are known to often present with more severe symptoms, a higher frequency of exacerbation, lower lung function and increased mortality (6).…”

Effectiveness of hypertonic saline nebulization in airway clearance in children with non‐cystic fibrosis bronchiectasis: A randomized control trial

“…Therefore treatment of underlying cause and providing optimum supportive care should happen parallelly 9,10 . To date, most of the treatment recommendations for non‐CF bronchiectasis are extrapolated from studies done on pediatric patients with CF or adults with bronchiectasis 11–13 . Multiple airway clearance techniques have been introduced without their value been well established 14 …”

“…9,10 To date, most of the treatment recommendations for non-CF bronchiectasis are extrapolated from studies done on pediatric patients with CF or adults with bronchiectasis. [11][12][13] Multiple airway clearance techniques have been introduced without their value been well established. 14 Hypertonic saline (HS) has many mucolytic mechanisms that improve the invitro transportability of the mucus.…”

Effectiveness of Hypertonic Saline Nebulization in Airway Clearance in Children with Non-Cystic Fibrosis Bronchiectasis: A randomized control trial

“…9,10 To date, most of the treatment recommendations for non-CF bronchiectasis are extrapolated from studies done on paediatric patients with CF or adults with bronchiectasis. [11][12][13] Multiple airway clearance techniques have been introduced without their value been well established. 14 HS has many mucolytic mechanisms that improve the in vitro transportability of the mucus.…”