Pediatric Pulmonology最新文献

Background: The benefits of Elexecaftor-Tezacaftor-Ivacaftor (ETI) therapy on the health and wellbeing of people with CF (pwCF) are well documented. Since approval, however, a growing number of potential side effects have emerged in reports from clinical practice. With current understanding of ETI tolerability limited to data from clinical trials, the prevalence of side effects and their impact on care decision making remains poorly categorized.

Methods: A 10-question survey was developed and distributed to patients 18 years or older who were treated at the Massachusetts General Hospital CF centers. Reports of side effects were measured across 12 distinct categories, and dose adjustments and discontinuation due to side effects were collected. If a patient reported no side effects, they did not have to complete the entire survey.

Results: Among 92 respondents initiated on ETI, 51 respondents (55.4%) reported potential side effects and 41 (44.5%) respondents reported no adverse events. The most commonly reported side effects were mental health, changes in appearance, and gastrointestinal complaints, which were reported by 22.8%, 30.4%, and 21.7% of patients, respectively. Eighteen (19.6%) respondents modified their dosing in response to side effects, and six discontinued treatment permanently (6.52%) due to persistent side effects.

Conclusions: Responses demonstrated marked heterogeneity, with most respondents reporting at least one side effect following initiation. Dose modification was commonly utilized to mitigate adverse effects, however few respondents had to discontinue treatment. These findings demonstrate the importance of monitoring for potential drug-related side effects of ETI in clinical settings.

Introduction: A minority of asthmatic children develop persistent airflow limitation (PAL), associated with an increased risk of chronic airflow obstruction and poor prognosis. This study aimed to identify risk factors for PAL and develop a prediction model to identify high-risk asthmatic children.

Methods: This retrospective study included 2072 children (5-16 years) with asthma. After a 2-year follow-up, patients were categorized into non-PAL, reversible PAL (RPAL), and irreversible PAL (IPAL) groups. Logistic regression (LR) was used to identify independent risk factors for RPAL and IPAL. A prediction model based on multivariate LR was developed and validated to identify asthmatic children at high risk of developing PAL. A nomogram was created for visualization.

Results: Among the 2072 asthmatic patients, 14.72% (n = 305) developed PAL. Asthma exacerbation history (OR 1.80, 95% CI 1.03-3.01) and poor adherence (OR 1.83, 95% CI 1.26-2.65) were independent risk factors of RPAL. Independent risk factors for IPAL were BMI over 19.0 kg/m² (OR: 1.81, 95% CI: 1.03-3.21) and a history of pneumonia (OR: 2.40, 95% CI: 1.30-4.26). The prediction model incorporated nine variables and showed good discriminatory ability, with AUC values of 0.79 (95% CI: 0.76-0.81) for the training set, 0.76 (95% CI: 0.76-0.77) for internal validation, and 0.73 (95% CI: 0.64-0.81) for temporal validation.

Conclusion: Asthma exacerbation history and poor adherence were independent risk factors for developing RPAL. BMI over 19.0 kg/m² and a history of pneumonia were risk factors for IPAL. Our prediction model effectively identified asthmatic children at high risk of developing PAL.

Introduction: Identifying the asthmatic early to prevent permanent airway remodeling and the progression of the disease is desirable. In children, baseline impulse oscillometry has been found effective in identifying asthma in some studies but not others.

Objective: The purpose of our study was to utilize a meta-analysis to determine whether there were significant peripheral airway differences between asthmatic and non-asthmatic children across ethnicity/race, utilizing baseline impulse oscillometry (IOS) to establish its usefulness as a diagnostic tool in this age group.

Methods: This was a comprehensive search of published literature on pediatric oscillometric studies evaluating younger children (mean age ranging from 4.3 to 6 years) and older children (mean age ranging from 8.7 to 11.4 years) from the United States, Europe, Asia, and Middle East. Inclusion criteria required the primary variable resistance at 5 Hertz (R5) (kPa/L/s) or (cmH₂O/L/s) for both control and asthmatic subjects, and excluded studies if asthmatics had uncontrolled disease.

Results: Our data show that there are significantly higher R5 and area of reactance (AX) and lower reactance at 5 Hertz (X5) in both younger and older asthmatic children compared to healthy controls from various countries.

Conclusions: This meta-analysis firmly establishes that baseline oscillometry metrics, resistance and reactance, are effective in identifying the asthmatic child across age and the ethnicities/races evaluated.

Background: An inspiration-synchronized vibrating mesh nebulizer (VMN) has been reported to improve aerosol delivery during adult mechanical ventilation. A prototype VMN generating smaller particles was developed. We aimed to compare the aerosol delivery efficiency of small-particle and conventional VMNs in inspiration-synchronized and continuous modes during neonatal and pediatric mechanical ventilation.

Methods: A critical care ventilator with heated humidified circuits connected to an endotracheal tube (ETT) and passive test lung was set to pediatric and neonate parameters. Albuterol (2.5 mg/ml, 1 ml) was administered using both small-particle and conventional VMNs in inspiration-synchronization and continuous modes. For the pediatric model, VMN was placed at the humidifier inlet, inspiratory limb at Y-piece, and between Y-piece and ETT (Y-ETT). For the neonatal model, VMN was placed at the humidifier inlet and between Y-ETT. Each setup was repeated five times. Albuterol collected on the filter distal to the ETT was eluted and assayed with UV spectrophotometry (276 nm).

Results: The inspiration-synchronized VMN generated higher inhaled doses compared to continuous VMN across all nebulizer placements, particle sizes, and aerosol generation models (all p < .05). The highest inhaled doses (42.2 ± 2.0% and 40.7 ± 1.0% for pediatric and neonate, respectively) were observed with the small-particle inspiration-synchronized VMN placed at Y-ETT. In the pediatric model, the inhaled dose with inspiration-synchronized conventional VMN was similar, independent of nebulizer placements (24.4 ~ 27.0%). In contrast, the inhaled dose was greatest with continuous VMN placed at the humidifier inlet. With the neonatal model, VMN placed at Y-ETT yielded higher doses than the humidifier inlet, and small-particle VMNs outperformed conventional VMNs across all settings (all p < .05).

Conclusion: The prototype small-particle VMN positioned between Y-piece and ETT in an inspiration-synchronized mode optimized aerosol delivery during mechanical ventilation in both pediatric and neonatal models.

Background: Regular airway clearance techniques (ACTs) and exercise are recommended for children with bronchiectasis, but current clinical practice and their predictors are unknown.

Objective: We aimed to describe current use of ACTs and exercise among Australian children with bronchiectasis and identify associated predictors.

Methods: Physiotherapy-specific data of 397 children (median age = 8 were extracted from the Australian Bronchiectasis Registry. A multivariate analysis was undertaken to identify predictors associated with the use of regular ACTs and physical exercise.

Results: Regular ACTs were undertaken by 118 (30%) children while 192 (48%) engaged in regular exercise. Physical exercise was the most common ACT modality (n = 83, 20%). The likelihood of regular ACT increased in children whose sputum isolated Pseudomonas aeruginosa (OR = 3.88, 95% CI 1.69-8.89) and was 50% higher for every respiratory exacerbation in the previous 12-months that required hospitalization (OR = 1.50, 95% CI 1.15-1.95). For every year older in age, children had increased odds of engaging in physical exercise (OR = 1.21, 95% CI 1.08-1.34) or using an ACT device (OR = 1.21, 95% CI 1.05-1.34). Regular exercise was twice as likely in the presence of bibasal bronchiectasis (OR = 2.43, 95% CI 1.14-5.16), yet less likely in those with ≥1 hospitalizations in the previous 12-months (OR = 0.76, 0.95% CI 0.57-1.03).

Conclusion: Approximately one-third of children with bronchiectasis undertake regular ACTs while physical exercise was undertaken in approximately one in two children. Age, frequent respiratory exacerbations requiring hospitalization and the extent of disease are predictors of undertaking regular ACTs and exercise. Identification of these factors may assist in tailoring ACT, exercise and ACT modality prescription in clinical practice.

Objectives: Pediatric granulomatosis with polyangiitis (GPA) is associated with several pulmonary manifestations. This study aims to describe these manifestations at time of diagnosis and longitudinally at a tertiary-care pediatric hospital.

Methods: We performed a retrospective chart review of patients with GPA treated at our facility between 1 January 2010 through 31 December 2021. We collected baseline demographics, reported symptoms, imaging findings, pulmonary function tests (PFTs), and laboratory data at time of diagnosis. Data were collected using 6-month observation intervals to follow recurrence of respiratory manifestations, testing during recurrence, and resultant treatment modifications.

Results: Of 13 patients treated for GPA during the study period, 12 developed respiratory tract involvement. A total of 87 6-month observation periods were analyzed. At time of diagnosis, 83% (10/12) of subjects reported respiratory symptoms, 92% (11/12) had abnormal chest computed tomography (CT) imaging, and 42% (5/12) had abnormal PFTs. Fewer than half of the patients were seen by pulmonology within 6 months of diagnosis. Eight subjects (75%) had respiratory manifestations during subsequent observation periods. Chest CT or PFTs were obtained in 23/44 (52%) of observations periods with respiratory symptoms, with pulmonary consultation in only 9/44 (20%).

Conclusions: This is the first US study to describe respiratory manifestations in pediatric GPA patients longitudinally, finding they are common and frequently recurrent. Our cohort had almost universally abnormal imaging at diagnosis regardless of respiratory symptoms. Early collaboration with pediatric pulmonology in the care of GPA patients may allow rheumatology teams to efficiently evaluate recurrent symptoms and address concomitant lung disease.