Bronchiectasis in children: TABLE 1

Objectives: The objective of the study was to describe the disease burden, etiology, and clinical spectrum of bronchiectasis in children attending a tertiary hospital in Cape Town, South Africa. Materials and Methods: Data were collected by chart review of all patients aged 3 months to 15 years attending the respiratory clinic at Red Cross War Memorial Children’s Hospital between January 2019 and December 2019. We included children who had a diagnosis of bronchiectasis based on a history of a recurrent (>3 episodes/year) or persistent (>4 weeks) wet or productive cough and a clinical phenotype characterized by any of the following: Exertion dyspnea, recurrent chest infections, growth failure, finger clubbing, and chest deformity associated with radiographic features of bronchiectasis on chest radiograph or chest tomography reported by a pediatric radiologist. Patients with cystic fibrosis were excluded from the study. Results: Of 337 children seen at the respiratory clinic during the study period, 58 (17.2%) had bronchiectasis that was diagnosed at a mean age of 34 months (standard deviation 26), and 32 (55.0%) were female. The most common causes of bronchiectasis were post-infectious 25 (43.1%), and underlying immunodeficiencies 19 (32.8%), including 16/58 (27.6%) who were living with human immunodeficiency virus (HIV) and 3 (5.1%) with primary immunodeficiency. Other causes included aspiration syndrome 8 (13.8%) and anatomical abnormalities 4 (6.9%). Of the participants with post-infectious bronchiectasis, tuberculosis (TB) was the most common organism isolated 16 (64.0%), and most common in children living with HIV (11/16, 68.8%). Cough was common in 48 (82.8%), with wet cough being predominant in 41 (85.4%), course crepitations were found in 37 (63.8%), hyperinflation in 24 (41.4%), finger clubbing in 21 (36.2%), wheeze in 16 (29.3%), and exertional dyspnea in 7 (12.0%). Conclusion: Bronchiectasis is common in South African children, usually resulting from previous pneumonia episodes, with TB being the most common infective cause. The importance of early diagnosis and treatment of underlying causes, especially infectious diseases in low-middle-income settings, to prevent bronchiectasis is highlighted.

European Respiratory Society guidelines for the management of children and adolescents with bronchiectasis

Introduction: Bronchiectasis, characterized by airway dilation, mucus hypersecretion, and recurrent exacerbations, is increasingly recognized in children and adolescents. Recent guidelines from the European Respiratory Society (ERS) and Thoracic Society of Australia and New Zealand (TSANZ) emphasize early diagnosis and optimized management. This review explores therapeutic strategies for pediatric bronchiectasis. Materials and methods: Our review involved a comprehensive search of English-language literature in the PubMed and EMBASE databases until December 2023, focusing on observational studies, interventions, reviews, and guidelines in pediatric bronchiectasis. Results: Management strategies encompass airway clearance techniques, mucoactive agents, pulmonary rehabilitation, bronchodilators and inhaled corticosteroids tailored to individual needs and age-appropriate techniques. Antibiotics play key roles in preventing exacerbations, eradicating pathogens, and managing acute exacerbations, which are guided by culture sensitivities and symptoms. Long-term antibiotic prophylaxis, particularly macrolides, aims to reduce exacerbations, although concerns about antibiotic resistance persist. Vaccinations, including pneumococcal and influenza vaccines, are crucial for preventing infections and complications. Surgery and lung transplantation are reserved to severe, refractory cases after failure of medical therapies. Conclusions: The optimal management of pediatric bronchiectasis requires a multidisciplinary approach, including physiotherapy, pharmacotherapy, and vaccinations, tailored to individual needs and guided by evidence-based guidelines. Further research is needed to refine diagnostic and therapeutic strategies and improve outcomes for affected children and adolescents.

This study aims to analyze the research hotspots, evolution, and developing trends in pediatric bronchiectasis over the past 20 years using bibliometric analysis and visualization tools to identify potential new research directions. Publications related to bronchiectasis in children were retrieved from the Web of Science Core Collection (WoSCC) database from 2003 to 2022. Knowledge maps were performed through VOSviewer1.6.18 and CiteSpace6.1 R2. A total of 2,133 publications were searched, while only 1,351 original articles written in English between 2003 and 2022 were incorporated. After removing duplicates, we finally included 1,350 articles published by 6,593 authors from 1,865 institutions in 80 countries/regions in 384 different academic journals with an average citation frequency of 24.91 times. The number of publications shows an extremely obvious binomial growth trend. The majority of publications originated from the United States, Australia, and England. The institutes in Australia, especially Charles Darwin University, published the most articles associated with pediatric bronchiectasis. In addition, Pediatric Pulmonology was the most published journal. In terms of authors, Chang AB was the most productive author, while Gangell CL had the highest average citation frequency. The five keywords that have appeared most frequently during the last two decades were "children," "cystic fibrosis," "bronchiectasis," "ct," and "pulmonary-function." According to keyword analysis, early diagnosis and intervention and optimal long-term pediatric-specific management were the most concerned topics for researchers. This bibliometric analysis indicates that bronchiectasis in children has drawn increasing attention in the last two decades as its recognition continues to rise, providing scholars in the field with significant information on current topical issues and research frontiers.

Associations between peak oxygen uptake, lung function, and bronchiectasis in children with cystic fibrosis in the era of CFTR modulators.

ObjectiveChildren with tracheomalacia can develop chronic lower airway infection and neutrophilic inflammation. It is plausible children with tracheomalacia are at increased risk of developing bronchiectasis. We hypothesised that compared with...

Bronchiectasis (not related to cystic fibrosis) is a chronic lung disease caused by a range of etiologies but characterized by abnormal airway dilatation, recurrent respiratory symptoms, impaired quality of life and reduced life expectancy. Patients typically experience episodes of chronic wet cough and recurrent pulmonary exacerbations requiring hospitalization. Early diagnosis and management of childhood bronchiectasis are essential to prevent respiratory decline, optimize quality of life, minimize pulmonary exacerbations, and potentially reverse bronchial disease. Disease monitoring potentially allows for (1) the early detection of acute exacerbations, facilitating timely intervention, (2) tracking the rate of disease progression for prognostic purposes, and (3) quantifying the response to therapies. This narrative review article will discuss methods for monitoring disease progression in children with bronchiectasis, including lung imaging, respiratory function, patient-reported outcomes, respiratory exacerbations, sputum biomarkers, and nutritional outcomes.

Bronchiectasis denotes fixed and often irreversible dilatation of the bronchial wall caused by persistent inflammation and infection. Children with bronchiectasis in developing countries suffer recurrent hospitalisation and poor quality of life and usually succumb to the condition. These children are poorly recognised, misdiagnosed, and under-reported. We report five cases of computerised tomography-diagnosed paediatric bronchiectasis managed at the Edward Francis Small Teaching Hospital (EFSTH), Banjul, The Gambia. The ages of the children at presentation ranged from 3 to 14 years, and they all had recurrent chest infections. Four out of the five cases had Pulmonary Tuberculosis, among whom two cases had HIV/TB co-infections, and the only non-TB, non-HIV case had features of Down syndrome with congenital heart lesions. All the cases had growth impairment; digital clubbing was observed in four, and low peripheral oxygen saturation in room air in three of the five children. These cases are reported to increase the index of suspicion among clinicians working in resource-limited settings to consider the diagnosis of bronchiectasis in children with recurrent chest infections. Early diagnosis and prompt management, including appropriate follow-up care, will improve the quality of life of these children and ensure better management outcomes.

ABSTRACTIntroduction: The social and medical costs of bronchiectasis in children are becoming considerable due to its increasing prevalence. Early identification and intensive treatment of bronchiectasis are needed to decrease the morbidity and mortality associated with bronchiectasis in children.Area covered: This review presents the current pharmacotherapeutic strategies for treating bronchiectasis in children with a focus on non-cystic fibrosis bronchiectasis.Expert opinion: Evidence for the effectiveness of diverse treatment strategies in bronchiectasis is lacking, particularly in children, although the disease burden is substantial for bronchiectasis. Most treatment strategies for non-cystic fibrosis bronchiectasis in children have been extrapolated from those in adults with bronchiectasis or children with cystic fibrosis. Antibiotics combined with an active airway clearance therapy via the inhalation of mucoactive agents can stabilize bronchiectasis. The timely and intensive administration of antibiotics during acute exacerbation of bronchiectasis is essential to prevent its progression in children. To suppress the bacterial loads in the airway, systemic or inhaled antibiotics can be administered intermittently or continuously. However, studies on these protocols, including the appropriate duration and effective dosages are lacking. Long-term administration of azithromycin for 12–24 months may reduce the exacerbation frequency with the increased carriage rate of azithromycin-resistant bacteria.

IntroductionPrimary Ciliary Dyskinesia (PCD) is a rare genetic disease, affecting ~400 children in England. Ciliary structural defects cause abnormal function, impairing mucociliary clearance. Mucus build-up and infection cause lung disease,...

• Consensus recommendations for managing chronic suppurative lung disease (CSLD) and bronchiectasis, based on systematic reviews, were developed for Australian and New Zealand children and adults during a multidisciplinary workshop. • The diagnosis of bronchiectasis requires a high-resolution computed tomography scan ofthe chest. People with symptoms of bronchiectasis, but non-diagnostic scans, have CSLD, which may progress to radiological bronchiectasis. • CSLD/bronchiectasis is suspected when chronic wet cough persists beyond 8 weeks. Initial assessment requires specialist expertise. Specialist referral is also required for children who have either two or more episodes of chronic (> 4 weeks) wet cough per year that respond to antibiotics, or chest radiographic abnormalities persisting for at least 6 weeks after appropriate therapy. • Intensive treatment seeks to improve symptom control, reduce frequency of acute pulmonary exacerbations, preserve lung function, and maintain a good quality of life. • Antibiotic selection for acute infective episodes is based on results of lower airway culture, local antibiotic susceptibility patterns, clinical severity and patient tolerance. Patients whose condition does not respond promptly or adequately to oral antibiotics are hospitalised for more intensive treatments, including intravenous antibiotics. • Ongoing treatment requires regular and coordinated primary health care and specialist review, including monitoring for complications and comorbidities. • Chest physiotherapy and regular exercise should be encouraged, nutrition optimised, environmental pollutants (including tobacco smoke) avoided, and vaccines administered according to national immunisation schedules. • Individualised long-term use of oral or nebulised antibiotics, corticosteroids, bronchodilators and mucoactive agents may provide a benefit, but are not recommended routinely.

Bronchiectasis in Childhood

Improving the Diagnosis and Treatment of Paediatric Bronchiectasis Through Research and Translation

Childhood bronchiectasis (BE) is a chronic lung condition that remains under recognized, marked by irreversible widening of the bronchi, frequent respiratory infections, a persistent wet cough, and progressive lung damage, often leading to significant health burdens. To evaluate children with cystic fibrosis (CF) and non-CF BE and to examine the association between clinical presentation and radiological severity of BE using the Bhalla scoring system. Retrospective observational study. Children aged 0-18 years with a CT-confirmed diagnosis of BE were enrolled. Data on demographics, clinical characteristics, and imaging results were collected retrospectively from medical records. The Bhalla score was used to assess BE severity. Patients were categorized into CF-related and non-CF BE groups. The two groups were compared with respect to clinical features, growth z-scores, hospitalization frequency, and pulmonary function test outcomes. A total of 157 patients were analyzed. Among them, CF accounted for 23.6% of cases, and while the leading causes in the non-CF group were post-infectious BE (28%), immunodeficiency (19.8%), and primary ciliary dyskinesia (12.8%). The CF group presented at an earlier age, had a longer follow-up period, and experienced more frequent hospitalizations (p < 0.001). In the CF group, weight, height, and body mass index z-scores significantly improved from the initial to the final assessment (p = 0.010, p = 0.006, and p = 0.026, respectively), whereas no such improvement was observed in the non-CF group. Severe Bhalla scores were more frequently observed in the CF group (p < 0.001). Among CF patients, Bhalla scores showed a strong correlation with forced expiratory volume in one second (FEV1) in univariate analysis (r = 0.846, p < 0.001), though this was not significant in multivariable analysis (p = 0.434). In the non-CF group, there was no correlation between Bhalla scores and final FEV1 values (p = 0.148, r = 0.212). The results underscore distinct clinical trajectories between CF and non-CF BE in children. Improvements in CF patients suggest the effectiveness of structured clinical management, whereas inconsistent outcomes in non-CF patients point to the need for standardized follow-up protocols. While the Bhalla score may indicate the extent of structural lung disease in CF, it does not independently predict lung function, and therefore should be used as a supplementary, not solitary, measure of disease severity.

Pediatric bronchiectasis: An orphan disease ending in pneumonectomy: A case report