Human respiratory syncytial virus (HRSV) is a major cause of respiratory disease. It is classified into two antigenic subgroups. While several authors have studied the potential link between HRSV infection and later development of recurrent wheezing (RW) or asthma, no previous studies have assessed the role of HRSV subtypes. The aim of this study was to assess the medium-term respiratory morbidity in children and adolescents with prior severe bronchiolitis due to HRSV-A or HRSV-B infection. From September 2023 to June 2025 an observational, prospective, cohort longitudinal study was conducted, including children and adolescents (5-16 years) with a history of hospital admission due to single HRSV infection. Patients underwent a structured questionnaire, measurement of exhaled nitric oxide, spirometry and skin prick testing for allergic sensitization. A total of 185 patients were recruited (129 HRSV-A, 56 HRSV-B). No differences in the variables regarding hospital admission for bronchiolitis were detected. The prevalence of current asthma in both groups was similar (HRSV-A 17.2% vs. 25.5%, p=0.19). Patients with prior HRSV-B bronchiolitis reported more severe respiratory symptoms, particularly speech-limiting wheezing (HRSV-A 1.5% vs. HRSV-B 8.9%, p=0.039, OR 2.62 (95%CI 1.3-5.4)) and more frequent nocturnal dry cough not associated with infections (HRSV-A 7.7% vs. HRSV-B 16.9%, p=0.044, OR 1.78, (95%CI 1.78-2.9)). Multivariate analysis revealed HRSV-B prior bronchiolitis to be an independent risk factor for medical diagnoses of asthma and nocturnal cough. Schoolchildren and adolescents with prior severe bronchiolitis due to HRSV-B have poorer respiratory outcome compared to those with HRSV-A, with more frequent intercrisis symptoms.

Introduction: Congenital diaphragmatic hernia (CDH) is a rare and severe condition requiring mechanical ventilation before and after surgery. Neurally adjusted ventilatory assist (NAVA) has demonstrated benefits in neonates with CDH, including improved patient-ventilator synchrony, reduced peak inspiratory pressure, and reduced sedation-analgesia requirements. The aim of this study was to evaluate the impact of NAVA initiation on sedation-analgesia, comfort scores, and ventilatory parameters in neonates with CDH. Patients and Methods: This retrospective single-center study included all neonates with CDH who were ventilated with NAVA from 2014 to 2022. Sedation-analgesia drug doses, comfort scores, and ventilatory parameters were compared 24 h before and after initiation of NAVA. Results: 28 NAVA ventilation periods were analyzed in 21 patients. NAVA initiation was associated with significant reductions in midazolam and fentanyl doses from (median [interquartile range]) 33 [26–75] to 17 [0–34] µg/kg/h (n = 21, p = 0.0004) and from 1.10 [1.00–1.25] to 0.00 [0.00–0.95] µg/kg/h (n = 11, p = 0.006), respectively. Ketamine doses also decreased, but non-significantly, from 0.90 [0.65–1.00] to 0.15 [0.00–0.54] mg/kg/h (p = 0.10). Morphine and dexmedetomidine doses and comfort scores (COMFORT-B, DAN, EDIN) were similar before and after NAVA initiation. Peak inspiratory pressure and fraction of inspired oxygen decreased significantly after NAVA initiation, respectively from 21 [19–22] to 16 [13–20] cmH 2O (n = 13, p = 0.020) and from 0.30 [0.25–0.41] to 0.25 [0.21–0.31] (n = 28, p = 0.013) . Conclusion: In this group of neonates with CDH, NAVA initiation was associated with reduced sedation-analgesia doses and ventilatory parameters with no decrease in patient comfort. These results support the use of NAVA to wean neonates with CDH from mechanical ventilation.

Background: Children with Cystic fibrosis (CF) are at risk of fat-soluble vitamin deficiencies, studies have shown Elexacaftor/Tezacaftor/Ivacaftor (ETI) therapy to substantially improve nutritional status. This study aimed to investigate the effect of ETI on fat-soluble vitamin levels A, D and E, for children aged 6-16 years with CF. Method: We performed a retrospective cohort study of children with CF. Data collected included demographics, clinical characteristics, lung function, respiratory colonisation, serum vitamin A, D and E and vitamin supplementation dosages. Seven timepoints were decided for statistical analysis: T1-T3 – pre-ETI and T4-T7 – at 6 month intervals post-ETI. The significance of changes in vitamin levels and supplementation was assessed using a linear mixed-effects model. A p-value <0.05 was used to indicate significance. Results: Fifty-six children met the inclusion criteria, with 262, 257 and 252 complete observations for vitamins A, D and E, respectively. Median vitamin D levels increased from 77nmol/L to 85nmol/L (p<0.001). There was no significant increase in median vitamin A levels however post-ETI there was a significant association between supplementation dose and serum vitamin A levels (p=0.008). There were no significant changes in median vitamin E. Over the timepoints there was an increase in the proportion of patients receiving no vitamin supplementation. Conclusions: This study demonstrates ETI initiation increases vitamin D levels and lowers the requirement for fat-soluble vitamins supplementation. Whilst ETI did not cause significant increases in vitamin A and E, it appears there was a shift in vitamin A making supplementation more effective on ETI therapy.

Background/Objective: Pediatric Home Mechanical Ventilator (HMV) Programs have supported children with chronic respiratory failure since the 1970s, yet optimal caregiver training duration remains unclear. This study evaluated the safety and effectiveness of a standardized 4-week HMV discharge pathway (HMV MAP) by analyzing hospital length of stay (LOS) and 30-day revisit rates. Methods: A retrospective cohort study was conducted at a single center, including 59 children (ages 0–21) with tracheostomy and chronic home ventilator use who completed the HMV MAP training between 2016–2021. Data were extracted from electronic medical records. Existing home ventilator-dependent patients re-admitted for any reason, HMV MAP patients on palliative, hospice and/or concurrent care for home, as well as patients discharged to long-term care facilities/subacute care facilities were excluded. Results: Of 59 eligible patients, the most common indication for long-term mechanical ventilation was chronic respiratory failure due to respiratory insufficiency. The HMV MAP median LOS was 37 days (IQR 12-92). There were 30 patients who had extended LOS (eLOS HMV MAP). Of these, 21 were due to non-respiratory causes, with 14 being medical-related and 7 being social-related. Among patients discharged in 4 weeks, 0% had ED visits within 48 hours of discharge, 17% were readmitted within 30 days for respiratory complications and 6.9% for non-respiratory complications; compared to 6.7%, 10% and 10% respectively for eLOS 4W HMV MAP. Conclusions: The HMV MAP appears safe and effective, with low early ED visits and readmission rates. Most delays were due to non-respiratory factors, suggesting the pathway may benefit from flexibility to accommodate medical and social complexities.

Introduction Infants with severe bronchopulmonary dysplasia (BPD) are at increased risk of hypoxemia and often fail hypoxic challenge testing (HCT). The predictive value of clinical characteristics and structural lung abnormalities on HCT outcomes is unclear. Methods This prospective cohort study included preterm-born infants (≤32 weeks gestation) with severe BPD enrolled in a standardized follow-up program. Perinatal and neonatal data were extracted from medical records. At 6 months corrected gestational age, structural lung abnormalities were quantified on chest CT using PRAGMA-BPD scoring. HCT was performed using a sealed body plethysmograph and failure was defined as inability to maintain oxygen saturation ≥ 85% for 20 minutes. Univariate and multivariable logistic regression analyses identified predictors of HCT failure. Results Among 156 infants, 28.8% failed HCT. Of the perinatal and neonatal factors, patent ductus arteriosus (odds ratio 2.73, 95% confidence interval 1.29–6.14), pulmonary hypertension (2.57, 0.99–6.62), and longer duration of supplemental oxygen therapy (odds ratio per interquartile range increase (OR IQR) 2.40, 1.55–3.71) were associated with failure. For structural lung abnormalities, higher composite PRAGMA-BPD scores (OR IQR 2.18, 1.36–3.48), higher hyper-attenuation (OR IQR 1.75, 1.17–2.63) and hypo-attenuation (OR IQR 1.45, 1.08–1.98) scores predicted failure. In multivariable analysis, only longer duration of supplemental oxygen therapy (OR IQR 2.04, 1.22–3.42) and higher composite PRAGMA-BPD scores (OR IQR 1.95, 1.15–3.31) remained independent predictors. Conclusion Duration of supplemental oxygen therapy and structural lung abnormalities independently predict HCT failure, highlighting the clinical relevance of both structural and functional impairments in severe BPD.

Introduction: Novel therapeutics and the rapid expansion of telehealth have reshaped cystic fibrosis (CF) care. Their impact on visit patterns and equitable access across the CF population remains unclear. We characterized changes in visit patterns among people with CF from 2017–2022 and the association of sociodemographic and clinical factors with visit frequency in 2022. Methods: This observational cohort study analyzed 2017-2022 US CF Foundation Patient Registry data for people aged 6-65 years. We described trends in care utilization and used longitudinal mixed-effects models to evaluate predictors of between-visit interval (BVI), adjusting for confounding identified by directed acyclic graphs separately for each predictor to ensure accurate, predictor-specific effect estimates. Results: The study included 28,340 people and 463,745 encounters. Average BVI increased 22.6% from 2019-2022, with larger increases among adults. During this span, BVI differences between F508del homozygotes and heterozygotes decreased significantly. Starting elexacaftor/tezacaftor/ivacaftor (ETI) initially shortened BVI, followed by sustained increases. Non-white and Hispanic individuals had shorter BVI than white, non-Hispanic counterparts. After adjusting for confounding and clinic-level variation, insurance changes and greater residential distance from CF centers were associated with longer BVI. Conclusion: Advances in CF therapeutics and COVID-19–related care adaptations have both markedly influenced visit frequency, indicating evolving care models responsive to the needs of people with CF. Persistent disparities by race, insurance status, and geographic location reveal ongoing challenges in achieving equitable access to routine CF care. Targeted strategies to address these inequities and enhance integrated care are essential to optimize outcomes for people with CF.

Background Children born preterm with chronic lung disease of Immaturity (CLDI) frequently present with obstructive lung function, yet optimal bronchodilator therapy remains uncertain. Previous studies focused on β2-agonists, while evidence for muscarinic antagonist therapy is limited. Methods We conducted a prospectively planned cross-sectional study of preterm-born children (<35 weeks) with CLDI and obstructive spirometry (FEV1 z-score < –1.64). Of 73 screened, 64 were enrolled and 55 completed the protocol. Each attended three randomized crossover visits with salbutamol, ipratropium bromide, or fenoterol/ipratropium combination. Spirometry was performed before and after administration. Results Overall, 48 children demonstrated bronchodilator responsiveness to at least one agent. Response rates were 56.4% for salbutamol (95% CI 43–70), 58.2% for ipratropium (95% CI 45–71), and 65.5% for the combination (95% CI 53–78), with no significant differences (McNemar’s test, p = 0.45). Exclusive responses were observed in 9.1% to salbutamol (95% CI 3.0 - 20.0), 5.5% to ipratropium (95% CI 1.1 – 15.1), and 9.1% to the combination (95% CI 3.0 - 20.0). Among children with reversibility, repeated-measures ANOVA showed no significant effect of drug (F = 0.1, p = 0.9), phenotype (F = 2.46, p = 0.12), or their interaction (F = 1.1, p = 0.34). Conclusions In our cohort, 87% of preterm-born children with CLDI exhibit reversible bronchial obstruction, with a substantial subset responding exclusively to a single bronchodilator. Due to the fact that single-drug testing may underestimate reversibility, individualized bronchodilator testing should be incorporated into care, and its long-term implications merit further study.

not-yet-known not-yet-known not-yet-known unknown Introduction and Objective: Physical activity (PA) is associated with improved asthma outcomes. Black girls face higher rates of asthma morbidity and are less likely to meet recommended PA than their White and Black male peers. To address these health disparities, it is essential to understand beliefs and behaviors related to PA among Black girls with asthma. Methods: For this qualitative study, Black girls with asthma and their mothers or female caregivers were recruited through flyers and direct outreach to patients at one academic medical center. Semi-structured interviews focused on knowledge of PA recommendations, perceived risks and benefits of PA, barriers and facilitators to PA, and maternal influences on PA. Transcripts were coded iteratively through deductive thematic analysis. Findings: Twenty girls (age: mean=9.9 years, SD=1.33, range=8-12) and their caregivers participated. Most viewed asthma as a limitation to PA and could not identify a beneficial relationship between PA and asthma. Nonetheless, girls were enthusiastic about PA and shared strategies for managing asthma symptoms while exercising. Facilitators included outdoor access and social support, while barriers included program costs and safety concerns. Many girls said they would be more active with their mother/caregiver. Conclusions: Despite personal and structural barriers to PA, Black girls with asthma view PA as important for physical and social wellbeing. Mothers/female caregivers play a major role in motivating and creating opportunities for PA. Our findings inform efforts to promote PA in a vulnerable yet understudied population, including expanding asthma management education and leveraging mother-daughter relationships to facilitate engagement in PA.

Background and objective: Severe asthma in preschool children is difficult to manage. Inhaled corticosteroids (ICS) are first-line therapy, but many remain uncontrolled, and evidence for additional treatments is scarce. We evaluated the real-world effectiveness and safety of adding long-acting beta agonists (LABA) to inhaled corticosteroids (ICS) in preschool children with severe uncontrolled asthma. Methods: We conducted a retrospective observational study (2021–2024) of children aged 6–72 months with severe asthma uncontrolled on medium- to high-dose ICS, treated with LABA add-on. We collected demographics and clinical data. Primary outcomes were rates of severe exacerbations defined as systemic corticosteroid [SCS] use and hospitalizations and uncontrolled symptoms; secondary outcomes included rate of pediatric intensive care unit (PICU) admissions and reported adverse events. Outcomes six months before and after LABA initiation, were compared. Results: Fifty-three children were included (mean age 39 ± 17.6 months, 43% male, atopy in 50.9%). Six-months following LABA initiation, hospitalizations declined from 39.6% to 1.9% (p<0.001), SCS use from 84.9% to 50.9% (p<0.001), and daily symptoms from 88.7% to 39.6% (p<0.001) compared to 6-months before. PICU admissions fell from 9.4% to 0%. No adverse effects were reported. Subgroup analyses confirmed consistent benefits across age, sex, atopic status, and smoke exposure. Conclusion: In this real-world cohort of preschool children with severe, difficult-to-control asthma, LABA add-on therapy to ICS was associated with marked clinical improvement and no safety concerns. These findings support the cautious use of LABA in this population and highlight the need for further prospective studies to guide practice.

Background: Acute bronchiolitis outbreaks are seasonal winter phenomena caused by various viruses, primarily respiratory syncytial virus (RSV). These outbreaks lead to high hospitalization rates in infants, placing a significant burden on pediatric services. During the SARS-CoV-2 pandemic, public health measures were implemented to curb viral transmission. Objectives: Assess the impact of the COVID-19 pandemic and barrier measures on the incidence, severity and virology of hospitalized acute bronchiolitis cases. Methods: This is a retrospective study, including infants under 12 months of age, hospitalized for acute bronchiolitis at the University Hospital of Clermont-Ferrand (France) between September 2018 and August 2023. The five epidemic seasons were compared. Results: 1420 hospital stays were included. A significant decrease in bronchiolitis hospitalizations was observed in 2020–2021 compared to 2018–2019 (p=0.040), 2021–2022 (p=0.018), and 2022–2023 (p=0.001). The epidemic peak in 2020–2021 was delayed to July and involved fewer cases (36 vs. 110.8 in other years). No significant difference was found in hospital stay duration across the five epidemic seasons (p=0.109). Lockdowns measures were statistically associated with reduced hospitalization cases (p=0.012). Conclusion: Lockdown measures limited respiratory viruses spread, particularly RSV. A marked decrease in the number of hospitalized acute bronchiolitis was observed during the 2020–2021 season, along with an unusual reduction and delay in the epidemic peak during summer. In 2021–2022, a resurgence of cases was noted, with a distribution of hospitalizations similar to pre-COVID-19 seasons. SARS-CoV-2 was rarely detected, confirming its minor role in acute bronchiolitis epidemics compared to other respiratory viruses.

Title: One Size May Not Fit All: A Case SeriesAuthors: Anjali Daulat, DO1; Joseph Walter, MD2; Michelle Condren, PharmD1Institutions: University of Oklahoma School of Community Medicine, Department of Pediatrics1, Tulsa, OK; Saint Francis Children’s Hospital Physicians, Tulsa, OK 741362Email ID: anjali-daulat@ou.eduOne Size May Not Fit All: A Case SeriesTo the editor,Although highly effective CFTR modulators like ivacaftor, elexacaftor/tezacaftor/ivacaftor (ETI), and vanzecaftor/tezacaftor/ivacaftor (VTI) have proven to be clinically beneficial, adverse effects may require dose reductions. Once ETI was approved in children with CF ages 2-5, reports of behavioral issues emerged after treatment initiation, including hyperactivity, mood disorders and sleep disturbances 3. This raises the question of whether reducing the dose allows for continued and similar clinical benefits. In adults, a dose-reduction study demonstrated resolution of neuropsychiatric adverse effects (AEs) with maintained clinical benefit despite a modest increase in sweat chloride concentration (SCC) from 38 mmol/L to 44 mmol/L1. This supports the possibility that lower doses may still provide sweat chloride reduction and clinical benefits.This case series highlights 9 children with CF who had modified ETI dosing due to adverse effects with follow-up sweat chloride testing to determine their response to reduced doses. Per Oklahoma State University Institutional policy and IRB review, this case series was deemed exempt. Thus, informed consent was not obtained from each patient. A HIPAA waiver of consent was authorized due to being low risk to the patient population.Table #1 summarizes each patient case including reason for dose adjustment, and sweat chloride results on modified dosing. Of note, 2 of the 9 patients (case #7 and #8), were started at ½ dosing due to pre-existing behavior concerns within themselves or within a sibling on ETI. Of the 9 patients, 6 (cases #1-3, #7-9) had modified dosing due to neuropsychiatric effects and 3 had modified dosing due to hepatic and GI effects (cases #4-6). Case #2 for example, required multiple adjustments over time, correlating improved behavioral symptoms with lower dosing. Many neuropsychiatric concerns were improved or resolved with reduced dosing while maintaining clinical benefit, as seen through lower sweat chlorides compared to those values at diagnosis.  Injury to the hepatic and GI system prompted adjustments with improved or resolved the concerns with continued reduction of their sweat chloride.Keywords: case series, cystic fibrosis, pediatricTable 1. Case summaries

Sweat testing remains a fundamental diagnostic tool for cystic fibrosis (CF), with the classic sweat chloride test (SCT) established as the gold standard since 1959. Despite its wide use, SCT presents challenges including limited accessibility in low- and middle-income countries, difficulties in obtaining adequate sweat samples, especially from infants, and ambiguous borderline results that require further functional or genetic analysis. Newer diagnostic methods, such as nasal potential difference (NPD) and intestinal current measurement (ICM), provide sensitive and specific assessments of CFTR function, but are technically complex and require specialized expertise, restricting their use in resource-limited settings. The beta-adrenergic sweat test (BAST) emerges as a promising, less invasive alternative, directly measuring CFTR-mediated sweat production using evaporimetry or sweat bubble imaging techniques. BAST shows advantages including ease of use, rapid results, and the ability to distinguish between CF, CFTR-related disorders, heterozygotes, and unaffected individuals. Furthermore, recent advances in wearable sensor technologies and microneedle patches indicate future potential for continuous, real-time, and noninvasive sweat monitoring, which could revolutionize CF diagnostics and patient management. This review summarizes the strengths and limitations of current and emerging sweat test modalities, highlighting BAST and wearable sensors as impactful tools for improving CF diagnosis and monitoring, especially in settings with limited resources.

To the Editor:

Introduction: Respiratory oscillometry (OSC) is a pulmonary function test that measures respiratory system impedance and is well-suited for young children. Previous studies have suggested that there may be differences between impedance values obtained on different instruments, however, no studies comparing instruments have been done in healthy children. Methods: We performed both impulse oscillometry (IOS) and airwave oscillometry (AOS), in random order, in a cohort of healthy children with no history of cardiopulmonary disease or recent illness. We used linear regression analysis and Bland Altman plots to compare results. Results: Eighty children ages 4 – 18 years completed study visits. Compared with AOS, the IOS mean and median values for resistance at 5 Hz (R5) were higher (p < 0.001), reactance at 5 Hz (X5) mean and median values were less negative (p < 0.001), and both the area under the reactance curve (AX) and resonant frequency (Fres) were lower (p < 0.001). Bland Altman analysis revealed proportional bias between the two systems. Conclusions: Our results in healthy children show differences between results obtained on different OSC instruments. This may be due to differences in the type of pressure signal produced by the instrument, or, in the case of IOS, may be related to a stress-relaxation response. Our results suggest that normal values obtained on one instrument may not be comparable to results obtained on another.

Background and objective: Early-life insults affect lung function trajectories throughout life. In Nepal there is a high burden of disease from lower respiratory tract infections, and high levels of exposures to household and environmental pollution. To enable assessment of these factors on lung development we aimed to assess the feasibility of establishing a pulmonary function laboratory for tidal breath flow-volume loops (TBFVL) in infants and children in Dhulikhel, Nepal. Methods: A pulmonary function laboratory was established and TBFVL testing, meeting international quality standards, were performed on healthy children 1-24 months of age, between October 2024 and May 2025, in Dhulikhel, Nepal. Results were interpreted by local staff after initial training and ongoing oversight by international experts. TBFVL indices with coefficient of variation (CoV) were reported and inter-interpreter level of agreement evaluated using Bland-Altman plots. Results: Eighty-six children were included, of which 80 (93%) had successful tests. Fifty-five (69%) 0-<4 months, 12 (15%) 4-<12 months and 13 (16%) 12-24 months of age. The CoV ranged from 4.7 (4.4, 5,2) for tidal volume to 19.5 (15.8, 24.1) for the ratio of the proportion of time to reach peak tidal expiratory flow to total expiratory time (T PTEF/TE), with CoV being similar across age groups. Agreement between local and expert international assessors was good, with the largest mean inter-interpreter difference observed for T PTEF/TE. Conclusion: Establishing a pulmonary function laboratory and achieving high-quality TBFVL measurements with expert-level interpretation is feasible in Nepal and contributes to the knowledge of normal pulmonary function values in Nepali children.

Treprostinil for the treatment of bronchopulmonary dysplasia-associated pulmonary hypertension (BPD-PH) has previously been described in small cohort studies, often used later in the course after failure to improve on other therapies. We retrospectively describe the clinical course and outcomes of 18 infants (gestational age 26.3±2.6 weeks) who received parenteral treprostinil to treat BPD-PH, including changes in echocardiographic and cardiac catheterization parameters. All patients had moderate-to-severe BPD and PH, with a mean pulmonary arterial pressure of 45.6 ± 12.7 mm Hg at cardiac catheterization prior to treprostinil. Treprostinil was initiated at a median postmenstrual age of 53.5 (IQR 45.7, 62.6) weeks. Echocardiograms after 3 months of treatment showed preserved right ventricular function (tricuspid annular plane systolic excursion (TAPSE) average 1.40 ± 0.32) and improvement of PH severity. At repeat catheterization, mean pulmonary arterial pressure (delta -16.4 ± 12.2, p < 0.01) and indexed pulmonary vascular resistance (delta -4.2 ± 3.3, p < 0.01) significantly improved. Ten of 18 infants (55.6%) survived to discharge. Our study shows a potential benefit of treprostinil use in moderate-to-severe BPD-PH; larger studies are needed to validate our findings and guide decision-making around treprostinil initiation and duration.

Title: A pediatric patient with PCD and broncholith formation.Swetha Gannarapu, MDUniversity of Texas Southwestern Medical Center, Department of Pediatrics, Division of Pulmonology and Sleep Medicine, Dallas TexasSwetha.gannarapu@utsouthwestern.eduMuhanned Abu-Hijleh, MDUniversity of Texas, Southwestern Medical Center, Department of Medicine, Division of Pulmonary and Critical Care MedicineMuhanned.abu-hijleh@utsouthwestern.eduFolashade Afolabi, MDUniversity of Texas Southwestern Medical Center, Department of Pediatrics, Division of Pulmonology and Sleep Medicine, Dallas, TexasFolashade.afolabi@utsouthwestern.eduAndrew S. Gelfand, MDUniversity of Texas Southwestern Medical Center, Department of Pediatrics, Division of Pulmonology and Sleep Medicine, Dallas, TexasAndrew.gelfand@utsouthwestern.eduYadira M. Rivera-Sanchez, MD (corresponding author)University of Texas Southwestern Medical Center, Department of Pediatrics, Division of Pulmonology and Sleep Medicine, Dallas, TexasYadira.rivera-sanchez@utsouthwestern.eduTo the editor:We present a 17-year-old male of Hispanic descent with a diagnosis of PCD who manifested with symptoms of recurrent fever and bronchiectasis exacerbations, signs and symptoms of increased cough, sputum production with sputum discoloration, and fatigue with an associated decrease in baseline lung function. PCD diagnosis had been made at the age of 11 with nasal nitric oxide (nNO) levels of 9 nL/min and transmission electron microscopy (TEM) of nasal cilia, revealing the partial absence of the outer dynein arms (ODA). PCD genetic panel was inconclusive, revealing three homozygous variants of unknown significance (VUS) in DNAH5, CCDC65, and DNAH8 (Table 1). Relevant past medical history included a left lower lobe (LLL) lobectomy at the age of 12 for recurrent bronchiectasis exacerbations requiring hospitalization due to failure to respond to oral antibiotics and ventilation-perfusion (V/Q) scan findings of almost absent perfusion to the LLL.The patient’s lung function remained normal but showed a decrease from baseline in the forced expiratory volume in 1 second (FEV1). Flexible bronchoscopy showed an endobronchial polypoid lesion with surrounding friable mucosa at the level of the LLL lobectomy stump. BAL cultures were positive for Aspergillus niger and Pseudomonas aeruginosa (Pa). The patient had a known colonization with Pa.For findings of Aspergillus niger in association with recurrent fevers, respiratory symptoms, and failure to respond to antibacterials, Voriconazole was started and continued for 4 months. It was discontinued after 4 months due to side effects, including photosensitivity. Inhaled Tobramycin, alternated with inhaled Aztreonam, were continued as per the baseline plan of care. Repeat flexible bronchoscopy again showed the same endobronchial polypoid lesion at the level of the LLL lobectomy stump with friable surrounding mucosa. Since our institution does not have a pediatric interventional bronchoscopy service, the patient was referred to the adult interventional pulmonary program for flexible bronchoscopy with biopsy consideration. Differential considerations at that time included an Aspergilloma, a broncholith, and a foreign body. Therapeutic aspiration was performed to remove thick mucoid secretions, primarily in the left tracheobronchial tree, with relatively long remaining left lower lobe airways, including the superior segment airway and basilar segments airways with distal stump. The polypoid lesion had a white, rough surface and was wedged at the level of the superior segment airway.  The polypoid abnormality was extracted as a single entity using a large jaw flexible forceps without significant resistance or bleeding (Figure 1). The entire lesion measured 7 mm x 5 mm. The clinical and pathology findings were consistent with a broncholith.Post-operative clinic visits have shown improvement in respiratory symptoms.

Introduction: Tracheostomy-dependent pediatric patients face an increased risk of respiratory infections due to bacterial colonization by Pseudomonas aeruginosa (PA). Inhaled antibiotics, such as tobramycin, were developed as targeted treatments to reduce infection rates and the need for systemic antibiotics; however, limited research explores their use in tracheostomy-dependent children, particularly their impact on healthcare utilization and optimal dosing. Methods: We conducted a retrospective cohort study at Children’s National Hospital in Washington, DC, including tracheostomy-dependent patients aged 0 to 21 years from January 1, 2003, to June 30, 2023. Patients with cystic fibrosis (CF), bronchiectasis, or primary ciliary dyskinesia were excluded. The primary outcome measured emergency department visits, regular floor admissions, ICU admissions, and systemic antibiotic use six months before and after initiating inhaled tobramycin. The secondary outcome assessed changes in PA positivity rates. Results: A total of 42 children were included, with a median age of 2 years; 64.3% were male. Neurologic disorders (45.2%) and bronchopulmonary dysplasia (26.2%) were the most common reasons for tracheostomy. Inhaled tobramycin therapy was associated with reduced regular admissions (p = 0.04), ICU admissions (p = 0.006), and systemic antibiotic use (p = 0.05). PA positivity decreased from 40.5% to 30.9% post-treatment (p = 0.001). Conclusion: This study the largest in non-CF, tracheostomy-dependent children demonstrates that inhaled tobramycin significantly reduces hospitalizations, ICU admissions, and antibiotic use. Although 40% of patients received doses between 160 mg and 300 mg every other month, dose variability did not significantly impact clinical outcomes.