Bronchiectasis In Children Research Articles

Therapeutic Management of Bronchiectasis in Children and Adolescents: A Concise Narrative Review.

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.

Association between arachidonate lipoxygenase 15,c.-292 C > T gene polymorphism and non-cystic fibrosis bronchiectasis in children: a pilot study on the effects on airway lipoxin A4 and disease phenotype

BackgroundPersistent airway inflammation is a central feature of bronchiectasis. Arachidonate 15-lipoxygenase (ALOX-15) controls production of endogenous lipid mediators, including lipoxins that regulate airway inflammation. Mutations at various positions in ALOX-15 gene can influence airway disease development. We investigated association between ALOX-15,c.-292 C > T gene polymorphism and bronchiectasis unrelated to cystic fibrosis in Egyptian children. Also, lipoxin A4 (LXA4) level in bronchoalveolar lavage (BAL) was studied in relation to polymorphism genotypes and disease phenotypes determined by clinical, pulmonary functions, and radiological severity parameters.MethodsThis was an exploratory study that included 60 participants. Thirty children with non-cystic fibrosis bronchiectasis (NCFB) were compared with 30 age and sex-matched controls. ALOX-15,c.-292 C > T polymorphism was genotyped using TaqMan-based Real-time PCR. LXA4 was measured in BAL using ELISA method.ResultsThere was no significant difference between patients and controls regarding ALOX-15,c.-292 C > T polymorphism genotypes and alleles (OR = 1.75; 95% CI (0.53–5.7), P = 0.35) (OR = 1; 95% CI (0.48-2), p = 1). BAL LXA4 level was significantly lower in patients, median (IQR) of 576.9 (147.6–1510) ng/ml compared to controls, median (IQR) of 1675 (536.8–2542) (p = 0.002). Patients with severe bronchiectasis had a significantly lower LXA4 level (p < 0.001). There were significant correlations with exacerbations frequency (r=-0.54, p = 0.002) and FEV1% predicted (r = 0.64, p = 0.001). Heterozygous CT genotype carriers showed higher LXA4 levels compared to other genotypes(p = 0.005).ConclusionsLow airway LXA4 in children with NCFB is associated with severe disease phenotype and lung function deterioration. CT genotype of ALOX-15,c.-292 C > T polymorphism might be a protective genetic factor against bronchiectasis development and/or progression due to enhanced LXA4 production.

Radiographic Outcomes in Pediatric Bronchiectasis and Factors Associated with Reversibility.

Rationale: Conventionally considered irreversible, bronchiectasis has been demonstrated to be reversible in children in small studies. However, the factors associated with radiographic reversibility of bronchiectasis have yet to be defined. Objectives: In a large cohort of children with bronchiectasis, we aimed to determine: 1) if and to what extent bronchiectasis is reversible and 2) factors associated with radiographic chest high-resolution computed tomography (cHRCT) resolution. Methods: We identified children with bronchiectasis who had a repeat multidetector cHRCT scan between 2010 and 2021. We excluded those with cystic fibrosis, surgical pulmonary resection, traction bronchiectasis only, or lobar opacification. Measurements and Main Results: cHRCT scans were scored using the modified Reiff score (MRS) with a pediatric correction. Resolution was defined as an absence of abnormal bronchoarterial ratio (>0.8) on the second cHRCT scan. We included 142 children (median age, 5 years; IQR, 2.6-7.4). Inter- and intrarater agreement in MRSs was excellent (weighted κ = 0.83-0.86 and 0.95, respectively). There was radiographic resolution in 57 of 142 patients (40.1%), improvement in 56 of 142 (39.4%), and no change or worsening in 29 of 142 (20.4%). Pseudomonas aeruginosa (PsA) was absolutely associated with a lack of resolution. On multivariable regression, in those without PsA cultured, younger age at the time of diagnosis (risk ratio [RR], 0.94; 95% confidence interval [CI], 0.88-0.99), lower MRS (RR, 0.89; 95% CI, 0.82-0.97), and lower annual rate of exacerbations requiring intravenous antibiotic therapy (RR, 0.60; 95% CI, 0.37-0.98) increased the likelihood of radiographic resolution. Conclusions: This first large cohort confirms that bronchiectasis in children is often reversible with appropriate management. Younger children and those with lesser radiographic severity at diagnosis were most likely to exhibit radiographic reversibility, whereas those with PsA infection were least likely.

Paediatric Bronchiectasis in a Resource-Constrained Centre: A Case Series

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.

Evidence-based management guidelines for noncystic fibrosis bronchiectasis in children and adolescents.

Noncystic fibrosis bronchiectasis is a chronic respiratory disease that carries high socioeconomic and medical burdens and is caused by diverse respiratory illnesses. To improve clinical outcomes, early recognition, active treatment of exacerbations, and prevention of further exacerbations are essential. However, evidence for the treatment and prevention of acute exacerbation of noncystic fibrosis bronchiectasis, especially in children, is lacking. Therefore, the evidence- and consensus-based guidelines for medical and nonmedical treatment strategies for noncystic fibrosis bronchiectasis in children and adolescents were developed by the Korean Academy of Pediatric Allergy and Respiratory Disease using the methods recommended by the Grading of Recommendations Assessment, Development, and Evaluation working group with evidence published through July 2, 2020. This guideline encompasses evidence-based treatment recommendations as well as expert opinions, addressing crucial aspects of the treatment and management of non-cystic fibrosis bronchiectasis in children. This includes considerations for antibiotics and airway clearance strategies, particularly in areas where evidence may be limited. Large, well-designed, and controlled studies are required to accumulate further evidence of management strategies for noncystic fibrosis bronchiectasis in children and adolescents.

Pathophysiology of non-cystic fibrosis bronchiectasis in children and adolescents with asthma: A protocol for systematic review and meta-analysis.

The pathophysiological mechanisms by which asthma and bronchiectasis are associated are still unclear. The association of these two diseases can result in more severe symptoms and a greater number of exacerbations. The aim of this systematic review is to collect evidence of the pathophysiology of non-cystic fibrosis bronchiectasis with associated asthma in children and adolescents, aged 6-18 years old. A systematic and comprehensive search will be performed using eight main databases, PubMed, PubMed PMC, BVS/BIREME, Scopus, EMBASE, Cochrane Library, Scielo and Web of Science. Articles will be searched from the earliest available time to July 2023. The studied population will be composed of children and adolescents with asthma and non-cystic fibrosis bronchiectasis. From the data obtained, all articles found will be transferred to the Rayyan platform. Study selection will follow the Preferred Reporting Items for Systematic Reviews and Meta-Analysis Protocols Checklist (PRISMA P-2015). In addition, if sufficient data are available, a meta-analysis will be conducted. Two independent reviewers will conduct the studies selection, data extraction, and risk of bias assessment. The outcome measures will be to analyze if non-cystic fibrosis bronchiectasis is related to a specific inflammatory profile. A systematic review will provide better knowledge about the etiopathogenesis and causes of the association between asthma and bronchiectasis and its role in the severity and control of asthma. Identifying, selecting and critically evaluating studies on asthma and bronchiectasis, would be possible to illuminate the characteristics of children and adolescents with associated diagnoses and provide information to help individualized treatments in order to control and prevent complications. The findings of this study will be published in a peer-reviewed journal. The systematic review protocol was registered with the International Prospective Register of Systematic Reviews (PROSPERO) in July 2023 (registration number CRD42023440355).

Video-assisted thoracoscopic surgery for non-cystic fibrosis bronchiectasis in children.

Pediatric bronchiectasis is a common respiratory disease in children. The use of video-assisted thoracoscopic surgery (VATS) for its treatment remains controversial. The objective of our study was to compare and analyze the clinical efficacy of thoracoscopic surgery and thoracotomy in the treatment of pediatric bronchiectasis and summarize the surgical treatment experience of VATS in children with bronchiectasis. Retrospective single-center cohort study. A retrospective analysis was conducted on the clinical data of 46 pediatric patients who underwent surgery with bronchiectasis at the Children's Hospital of Chongqing Medical University from May 2015 to May 2023. The patients were divided into two groups: the VATS group (25 cases) and the thoracotomy group (21 cases). Comparative analysis was performed on various parameters including basic clinical data, surgical methods, operation time, intraoperative blood loss, transfusion status, postoperative pain, postoperative mechanical ventilation time, chest tube drainage time, length of hospital stay, incidence of complications, and follow-up information. There were no statistically significant differences between the two groups of patients in terms of age, weight, gender, etiology, duration of symptoms, site of onset, and comorbidities (p > 0.05). The operation time in the VATS group was longer than that in the thoracotomy group (p < 0.001). However, the VATS group had better outcomes in terms of intraoperative blood loss, transfusion status, postoperative pain, postoperative mechanical ventilation time, chest tube drainage time, and length of hospital stay (p < 0.05). The incidence of postoperative complications in the VATS group was lower than that in the thoracotomy group, although the difference was not statistically significant (p = 0.152). Follow-up data showed no statistically significant difference in the surgical treatment outcomes between the two groups (p = 0.493). The incidence of complications and mortality in surgical treatment of bronchiectasis is acceptable. Compared with thoracotomy surgery, VATS has advantages such as smaller trauma, less pain, faster recovery, and fewer complications. For suitable pediatric patients with bronchiectasis, VATS is a safe and effective surgical method.

The Respiratory Microbiome in Paediatric Chronic Wet Cough: What Is Known and Future Directions.

Chronic wet cough for longer than 4 weeks is a hallmark of chronic suppurative lung diseases (CSLD), including protracted bacterial bronchitis (PBB), and bronchiectasis in children. Severe lower respiratory infection early in life is a major risk factor of PBB and paediatric bronchiectasis. In these conditions, failure to clear an underlying endobronchial infection is hypothesised to drive ongoing inflammation and progressive tissue damage that culminates in irreversible bronchiectasis. Historically, the microbiology of paediatric chronic wet cough has been defined by culture-based studies focused on the detection and eradication of specific bacterial pathogens. Various 'omics technologies now allow for a more nuanced investigation of respiratory pathobiology and are enabling development of endotype-based models of care. Recent years have seen substantial advances in defining respiratory endotypes among adults with CSLD; however, less is understood about diseases affecting children. In this review, we explore the current understanding of the airway microbiome among children with chronic wet cough related to the PBB-bronchiectasis diagnostic continuum. We explore concepts emerging from the gut-lung axis and multi-omic studies that are expected to influence PBB and bronchiectasis endotyping efforts. We also consider how our evolving understanding of the airway microbiome is translating to new approaches in chronic wet cough diagnostics and treatments.

Bronchiectasis with Trans Mediastinal Herniation of Left Upper Lobe in 3-Year-Old Child: A Case Report

BACKGROUND: Bronchiectasis is a disorder characterized by destruction of smooth muscle and elastic tissue due to inflammation which leads to permanent dilation of bronchi and bronchioles. It may develop in association with Cystic Fibrosis, a single severe episode or recurrent episodes of pneumonia and exposure to tuberculosis. The principal conditions associated with bronchiectasis are obstruction and infection. Infections primarily originate from issues with airway clearance, which cause bronchi and bronchioles to enlarge irreversibly. Vertical airways are notably affected, while distal bronchi and bronchioles are more severely affected. The degree of disease activity and chronicity may affect the histological findings. Childhood bronchiectasis can proceed to severe consequences including persistent bacterial bronchitis, in developing nations like India due to a lack of effective diagnosis and treatment as a result of poor health care infrastructure in the rural areas as well as limited awareness on the part of general public and health care professionals. THE CASE: A 3.5-year-old Indian boy presented with productive cough and cold for 8 days associated with low grade fever. Patient was admitted through Out Patient Department (OPD) due to respiratory distress and facial swelling. Clinical exam revealed presence of crepitation, wheeze and pectus carinatum. Patient has history of multiple hospital admissions due to pneumonia and respiratory distress. There is history of exposure to tuberculosis. His mother had been diagnosed with tuberculosis when the child was 3 months old. She received anti-tubercular drugs and is now cured. HRCT thorax reveals collapse of basal segment of right lung, trans-mediastinal space shift of left upper lobe and bi-lateral bronchiectatic changes which include unusually thickened enlarged airways exhibiting the characteristic tram-track appearance. Echocardiogram findings show thickened pericardium, mild pericardial collection and trace tricuspid valve regurgitation. The case is unique since it is quite rare for a young child to have such a severe form of bronchiectasis. CONCLUSION: Diagnosis can be done with the help of radiological and clinical examination. However, High Resolution Computed Tomography (HRCT) scan is the investigation of choice. Early management is a key factor in minimizing more serious complication like severe hemoptysis and cor pulmonale. Current treatment modalities include antibiotics, bronchodilators, anti-inflammatory drugs and chest physical therapy. Severe cases may require injectable antibiotics. Bronchiectasis was thought to be an orphan disease that seldom progressed to severe consequences, especially after the introduction of newer antimicrobials. There exist guidelines that advocate treatments for bronchiectasis, and reports of therapy have been shown to be linked with clinical success. However, such guidelines do not exist in India at present. This case is noteworthy as it portrays bronchiectasis in an Indian child that has proceeded to the severe complication of transmediastinal herniation, demonstrating that it is relatively common albeit under-diagnosed in developing countries. Though is an irreversible disease in adults, in children if detected early there is almost complete restoration of pulmonary function and adequate exercise tolerance. Early diagnosis with suitable pharmacological and non-pharmacological management is critical for a positive outcome and prevention of sequelae like persistent bacterial bronchitis.

Chronic suppurative lung disease and bronchiectasis in children, adolescents and adults in Australia and New Zealand: TSANZ position statement summary.

Chronic suppurative lung disease and bronchiectasis in children, adolescents and adults in Australia and New Zealand: TSANZ position statement summary.

Pixel-level image analysis to derive the broncho-artery (BA) ratio employing HRCT scans: A computer-aided approach

Bronchiectasis in children is a major health issue which can be life-threatening if not diagnosed and effectively treated. In the diagnosis of bronchiectasis, an increased broncho-arterial (BA) ratio is considered a significant marker. The BA ratio is measured by evaluating BA pairs, using high-resolution computed tomography (HRCT) scans. Detecting BA pairs automatically is challenging due to the complex characteristics of BA pairs and the ambiguous appearance of the bronchi. This study proposes an effective computerized approach to detect BA pairs and assess BA ratio using HRCT scans of children and employing computer-aided techniques and novel custom-build algorithms. Attention is given to reconstructing broken bronchial walls and identifying discrete BA pairs using custom-built kernel based and patch-based algorithms for pixel-level image analysis. To detect BA pairs, the lung region is segmented in the HRCT slices and image preprocessing techniques, including noise reduction, binarizing, largest contour detection and a hole-filling algorithm, are applied. A histogram analysis method is introduced to clean the images. A kernel-based algorithm is proposed to reconstruct the pixel distribution if the bronchial wall is so that the bronchi can be detected precisely. Potential arteries are detected using balanced histogram thresholding, morphological opening and an approach based on four conditions related to the object area circularity, rectangular boundary box ratio and enclosing circle area ratio. Potential bronchi are detected through matching of object coordinates with potential arteries, hole-filling and four condition based approaches. The potential BA pairs are detected by matching the coordinates of potential bronchi with those of potential arteries as the artery and bronchus are adjacent to each other in BA pairs. Finally, from the potential BA pairs, actual BA pairs are identified using a custom-built patch algorithm. The study is conducted using 2471 HRCT slices of seven children, obtained from the Royal Darwin Hospital, Australia. The BA ratio is derived based on the ratio of diameters, major axis lengths, minor axis lengths, area, convex hull and equivalent diameter where the BA ratios are respectively 0.51–0.65, 0.49–0.59, 0.59–0.77, 0.25–0.42, 0.29–0.47, 1.5–2 and 0.50–0.65.

The Economic Burden of Bronchiectasis: A Systematic Review

Bronchiectasis, a previously neglected condition, now has renewed research interest. There are a few systematic reviews that have reported on the economic and societal burden of bronchiectasis in adults, but none have reported on children. We undertook this systematic review to estimate the economic burden of bronchiectasis in children and adults. What is the health care resource utilization and economic burden of bronchiectasis in adults and children? We performed a systematic review identifying publications from Embase, PubMed, Web of Science, Cochrane (trials, reviews, and editorials), and EconLit about the economic burden and health care utilization in adults and children with bronchiectasis between January 1, 2001, and October 10, 2022. We used a narrative synthesis approach and estimated aggregate costs for several countries. We identified 53 publications reporting on the economic burden and/or health care utilization of people with bronchiectasis. Total annual health care costs per adult patient ranged from 2021 $3,579 to $82,545 USD and were predominantly driven by hospitalization costs. Annual indirect costs including lost income because of illness (reported in only five studies) ranged from $1,311 to $2,898 USD. Total health care costs in children with bronchiectasis were $23,687 USD annually in the one study that estimated them. Additionally, one publication found that children with bronchiectasis missed 12 school days per year. We estimated aggregate annual health care costs for nine countries, ranging from $101.6 million per year in Singapore to $14.68 billion per year in the United States. We also estimated the aggregate cost of bronchiectasis in Australian children to be $17.77 million per year. This review highlights the substantial economic burden of bronchiectasis for patients and health systems. To our knowledge, it is the first systematic review to include the costs for children with bronchiectasis and their families. Future research to examine the economic impact of bronchiectasis in children and economically disadvantaged communities, and to further understand the indirect burden of bronchiectasis on individuals and the community, is needed.

An Automated Broncho-Arterial (BA) Pair Segmentation Process and Assessment of BA Ratios in Children with Bronchiectasis Using Lung HRCT Scans: A Pilot Study.

Bronchiectasis in children can progress to a severe lung condition if not diagnosed and treated early. The radiological diagnostic criteria for the diagnosis of bronchiectasis is an increased broncho-arterial (BA) ratio. From high-resolution computed tomography (HRCT) scans, the BA pairs must be detected first to derive the BA ratio. This study aims to identify potential BA pairs from HRCT scans of children undertaken to evaluate suppurative lung disease through an automated approach. After segmenting the lung regions, the HRCT scans are cleaned using a histogram analysis-based approach followed by a potential arteries identification process comprising four conditions based on imaging features. Potential arteries and their connected components are extracted, and potential bronchi are identified. Finally, the coordinates of potential arteries and potential bronchi are matched as the last step of BA pairs extraction. A total of 8-50 BA pairs are detected for each patient. Additionally, the area and several diameters of the bronchi and arteries are measured, and BA ratios based on these are calculated. Through this approach, the BA pairs of a CT scan datasets are detected and utilizing a deep learning model, a high classification test accuracy of 98.53% is achieved, validating the robustness of the proposed BA detection approach. The results show that visible BA pairs can be identified and segmented automatically, and the BA ratio calculated may help diagnose bronchiectasis with less effort and time.

MORPHOFUNCTIONAL RELATIONSHIPS OF CELLS IN THE BRONCH IN CHRONIC INFLAMMATION

It has been established that under conditions of chronic inflammation of the lungs with bronchiectasis in children, destructive changes predominate in the structures of innate and acquired immunity of the airways, indicating their inability to effectively perform a protective function.

Thoracic Society of Australia and New Zealand (TSANZ) position statement on chronic suppurative lung disease and bronchiectasis in children, adolescents and adults in Australia and New Zealand.

This position statement, updated from the 2015 guidelines for managing Australian and New Zealand children/adolescents and adults with chronic suppurative lung disease (CSLD) and bronchiectasis, resulted from systematic literature searches by a multi-disciplinary team that included consumers. The main statements are: Diagnose CSLD and bronchiectasis early; this requires awareness of bronchiectasis symptoms and its co-existence with other respiratory diseases (e.g., asthma, chronic obstructive pulmonary disease). Confirm bronchiectasis with a chest computed-tomography scan, using age-appropriate protocols and criteria in children. Undertake a baseline panel of investigations. Assess baseline severity, and health impact, and develop individualized management plans that include a multi-disciplinary approach and coordinated care between healthcare providers. Employ intensive treatment to improve symptom control, reduce exacerbation frequency, preserve lung function, optimize quality-of-life and enhance survival. In children, treatment also aims to optimize lung growth and, when possible, reverse bronchiectasis. Individualize airway clearance techniques (ACTs) taught by respiratory physiotherapists, encourage regular exercise, optimize nutrition, avoid air pollutants and administer vaccines following national schedules. Treat exacerbations with 14-day antibiotic courses based upon lower airway culture results, local antibiotic susceptibility patterns, clinical severity and patient tolerance. Patients with severe exacerbations and/or not responding to outpatient therapy are hospitalized for further treatments, including intravenous antibiotics and intensive ACTs. Eradicate Pseudomonas aeruginosa when newly detected in lower airway cultures. Individualize therapy for long-term antibiotics, inhaled corticosteroids, bronchodilators and mucoactive agents. Ensure ongoing care with 6-monthly monitoring for complications and co-morbidities. Undertake optimal care of under-served peoples, and despite its challenges, delivering best-practice treatment remains the overriding aim.

Global research status and trends of bronchiectasis in children from 2003 to 2022: A 20-year bibliometric analysis.

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.

Gastrointestinal factors associated with risk of bronchiectasis in children.

To evaluate gastrointestinal (GI) risk factors for bronchiectasis in children. We hypothesized that upper GI tract dysmotility would be associated with increased risk of bronchiectasis. Subjects in this retrospective cohort study included those evaluated for persistent pulmonary symptoms in the Aerodigestive Center at Boston Children's Hospital who underwent chest computed tomography (CT) between 2002 and 2019. To determine gastrointestinal predictors of bronchiectasis, baseline characteristics, comorbidities, enteral tube status, medications received, gastroesophageal reflux burden, adequacy of swallow function, esophageal dysmotility, gastric dysmotility, and neutrophil count on bronchoalveolar lavage (BAL) were compared between patients with and without bronchiectasis. Proportions were compared with Fisher's exact test and binary logistic regression with stepwise selection was used for multivariate analysis. ROC analyses were utilized to compare BAL neutrophils and bronchiectasis. Of 192 subjects, 24% were found to have evidence of bronchiectasis on chest CT at age 7.9 ± 0.5 years. Enteral tubes (OR 5.77, 95% CI 2.25-14.83, p < 0.001) and increased BAL neutrophil count (OR 5.79, 95% CI 1.87-17.94, p = 0.002) were associated with increased risk while neurologic comorbidities were associated with decreased risk (OR 0.24, 95% CI 0.09-0.66, p = 0.006). Gastroesophageal reflux was not found to be a significant risk factor. Neutrophil counts >10% had 72% sensitivity and 60% specificity for identifying bronchiectasis. Enteral tubes were associated with significantly increased risk of bronchiectasis but gastroesophageal reflux was not. Providers should consider obtaining chest CT to evaluate for bronchiectasis in children found to have unexplained elevated BAL neutrophil count.

Bronchiectasis in children following kidney transplantation in New Zealand.

Bronchiectasis is an acquired chronic respiratory condition with a relatively high incidence in New Zealand children. Bronchiectasis following kidney transplant has been reported internationally. This study aimed to identify the incidence rate of bronchiectasis following paediatric kidney transplantation. Secondary aims were to assess the impact on kidney allograft function and identify risk factors that might prompt earlier diagnosis. Case control study of children who developed bronchiectasis following kidney transplant in New Zealand. All children who were transplanted during the 16-year period from 2001 to 2016 were included. Each identified case was matched with two controls (children who did not develop bronchiectasis and received a kidney transplant within the closest time period to their matched case). Data were collected on baseline demographics, clinical variables, immunosuppression and allograft function. Of 95 children who had a kidney transplant during the specified time period, eight (8.4%) developed bronchiectasis at a median of 4 years post-transplant. The mean incidence rate of bronchiectasis was 526 cases per 100 000 paediatric kidney transplant population per year. The majority of children were Māori or Pasifika ethnicity and lived in areas of greater socio-economic deprivation. Immunosuppression burden and allograft function were not significantly different between groups. The incidence rate of bronchiectasis following paediatric kidney transplantation is substantially higher than the baseline paediatric incidence rate in New Zealand. A high index of suspicion for bronchiectasis and prompt investigation of children post kidney transplantation with a history of recurrent lower respiratory tract infection or chronic cough are advised.

Surgical treatment of bronchiectasis in children: An 11-year experience at a central health facility in KwaZulu-Natal, South Africa

&#x0D; &#x0D; &#x0D; &#x0D; Background. The surgical management of children with bronchiectasis has seldom been reported.Objective. To describe the presentation, surgical management and outcomes in children with bronchiectasis presenting for surgery. Methods. We retrospectively reviewed the electronic records of 0 - 13-year-old children who underwent pulmonary resection for bronchiectasis at Inkosi Albert Luthuli Central Hospital, Durban, South Africa, between January 2004 and December 2014. Clinical, radiological and preoperative bronchoscopic findings, as well as surgical and histological outcomes, were analysed.Results. Eighty-eight patients underwent surgical resection. The female/male ratio was 3:2, with a mean age at surgery of 8.2 (range 2 - 13) years; 39 patients were HIV infected and 39 were HIV uninfected. Tuberculosis (TB) (n=68; 77.2%) was the most common cause of bronchiectasis, and recurrent chest infection (n= 45; 51.1%) was the most common clinical finding. Radiological examination confirmed isolated left-sided disease in 40 children (45.4%), isolated right-sided disease in 28 (31.8%) and bilateral disease in 20 (22.7%). Saccular disease with fibrocavitation (n=35; 39.7%) was the most common morphological disease type. Preoperative bronchoalveolar lavage samples confirmed a bacterial cause in 27 patients (30.6%). The most common operative procedures were primary pneumonectomy in 33 patients (37.0%), lobectomy in 30 (34.0%) and bilobectomy in 13 (14.7%). Seventy-five patients were asymptomatic after the operation and complications occurred in 13. Two children (2.2%), one with sepsis and the other with intraoperative hypoxia, died. Seventy patients underwent complete resection. At 1 month after surgery, 89.2% of patients were asymptomatic, while 77.7% of symptomatic patients were HIV positive.Conclusions. Complete pulmonary resection in children with advanced-stage bronchiectasis is safe, with a low morbidity and mortality. Surgery in HIV-positive patients was not associated with worse outcomes and is not contraindicated. HIV- and TB-preventive measures could reduce the burden of childhood bronchiectasis.&#x0D; &#x0D; &#x0D; &#x0D;

Monitoring disease progression in childhood bronchiectasis.

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.