Pediatric Pulmonology最新文献

Background: Sleep-disordered breathing (SDB) is an increasingly recognized yet underappreciated contributor to morbidity in people with cystic fibrosis (pwCF). While SDB, including nocturnal hypoxemia, hypoventilation, and obstructive sleep apnea (OSA), is well documented in high-income countries (HICs), evidence from low- and middle-income countries (LMICs) remains limited.

Methods: This narrative review synthesizes available evidence on the prevalence, clinical characteristics, diagnostic practices, and management approaches of SDB in pwCF living in LMICs, with a particular focus on studies using polysomnography (PSG) and overnight oximetry.

Results: Findings from upper-middle-income countries such as Brazil and Turkey indicate substantial rates of OSA and nocturnal desaturation, even among clinically stable individuals. Studies using PSG or overnight oximetry have linked these disturbances to lower lung function, poor nutritional status, and increased healthcare utilization. Despite the clinical relevance of these findings, access to diagnostic tools such as PSG and treatment modalities such as non-invasive ventilation remain severely limited.

Conclusions: SDB represents a clinically relevant but underdiagnosed comorbidity in pwCF residing in LMICs. There is an urgent need for increased clinical awareness, implementation of low-cost screening tools, and development of resource-adapted diagnostic and management strategies to improve comprehensive CF care in underserved regions.

Background: Our prior work revealed multilevel barriers to smoking cessation interventions among cystic fibrosis (CF) families and clinical providers. Here, we assess qualitatively the implementation of CEASE-CF, a multicomponent smoking cessation intervention tailored to the needs of CF caregivers and delivered by a tobacco treatment specialist (TTS) integrated into the CF care team (NCT04777344).

Methods: We conducted interviews with CF caregivers in the CEASE-CF intervention arm and with CF clinical care providers. Interviews were recorded and transcribed. Data analysis was completed by two independent coders using NVivo. Themes were generated using a content analysis approach with a constant comparative method.

Results: In 22 interviews with caregivers (n = 12) and providers (n = 10), four overarching themes emerged: (1) Increased caregiver awareness about the sources and harms of smoke exposure; (2) Increased caregiver self-efficacy for quitting; (3) Caregiver endorsement of the effectiveness of counseling by TTS; and (4) Provider endorsement of the integration of TTS in the CF care team.

Conclusion: CEASE-CF positively impacted caregivers by increasing their awareness of the sources and harms of smoke exposure, equipping them with tools to change habits, and enhancing their self-reported confidence and self-efficacy for quitting. Consistent and multifaceted cessation support provided by a trained TTS is practice-changing and can be successfully integrated in CF care delivery.

Introduction: Bronchiolitis is the leading cause of infant hospitalizations worldwide, primarily driven by respiratory syncytial virus (RSV).

Methods: This multicenter retrospective comparative study assessed the impact of immunoprophylaxis with nirsevimab against RSV on bronchiolitis-related hospitalizations in Italy during the 2024-2025 winter season.

Results: Data from nine Italian hospitals showed a substantial 48% decrease in bronchiolitis admissions compared to the previous season (438 vs. 832 admissions). Among hospitalized infants, only 23% had received immunoprophylaxis. RSV positivity dropped significantly among immunized patients (48%) versus non-immunized (73%, p < 0.0001), with fewer RSV-related coinfections.

Discussion: While indicators of illness severity-such as ICU admission, respiratory support needs, and complications-were generally lower, no statistically significant differences in disease course were observed between immunized and non-immunized hospitalized infants. A shift in viral epidemiology was noted, with a reduction in RSV dominance and increased detection of rhinovirus and enterovirus, suggesting a pathogen replacement effect. The 2024-2025 season also saw a lower intubation rate (< 0.5%), pointing to an overall milder disease course.

Conclusions: This study supports the effectiveness of nirsevimab in reducing RSV-associated hospitalizations and reshaping the virological landscape of bronchiolitis in Italy. Early and widespread implementation of immunoprophylaxis is recommended to maximize public health benefits.

Background: Delayed foreign body aspiration (FBA) mimicking a lung abscess is rare in children.

Case presentation: A 3-year-old girl presented with fever, cough, and a cavitary lung lesion initially diagnosed as lung abscess. Poor response to antibiotics prompted bronchoscopy, which revealed a pumpkin seed shell fragment causing obstruction and granulation. History revealed a choking episode eight months prior.

Results: The foreign body was removed bronchoscopically, and the patient recovered fully.

Conclusions: Occult FBA should be considered in children with focal cavitary lung lesions, especially when unresponsive to antibiotics for lung abscess.

Introduction: Pediatric asthma is characterized by airway inflammation, hyperresponsiveness, and reversible obstruction. While spirometry is pivotal in asthma management, traditional spirometric indices may inadequately assess Small Airway Disease. This study aimed to explore the prevalence and characteristics of spirometric patterns in asthmatic children and evaluate non-traditional indices as tools for assessing airway dysfunction and asthma control.

Methods: This retrospective study included 162 asthmatic children aged 3-15 years who underwent spirometry. Asthma control was assessed using the Asthma Control Questionnaire (ACQ). Spirometric measurements included Forced Expiratory Volume in 1 s (FEV₁), Forced Vital Capacity (FVC), FEV₁/FVC ratio, Forced Expiratory Volumes at 0.5, 2, and 3 s (FEV_0.5, FEV₂, and FEV₃), and Forced Expiratory Time (FET). FEV_0.5/FVC, FEV₂/FVC, and FEV₃/FVC ratios were calculated. ROC curve analysis evaluated the diagnostic utility of spirometric indices for distinguishing controlled from uncontrolled asthma.

Results: Preserved Ratio Impaired Spirometry (PRISm) was more prevalent (11.90%) than obstructive ventilatory defects (6%), reflecting the heterogeneity of asthma phenotypes. Uncontrolled asthma was associated with prolonged FET and lower FEV_0.5/FVC, FEV₁/FVC, FEV₂/FVC, and FEV₃/FVC ratios. Inverse correlations were observed between ACQ scores and non-traditional indices, particularly FEV_0.5/FVC (r = -0.572). FEV_0.5/FVC exhibited the highest diagnostic accuracy (AUC = 0.805, sensitivity = 89.9%, specificity = 67.7%), surpassing traditional indices.

Conclusion: Non-traditional spirometric indices, particularly FEV0.5/FVC, enhance the evaluation of airway dysfunction and asthma control in pediatric populations. Incorporating these indices into routine assessments offers a broader understanding of asthma phenotypes, supporting personalized management strategies.

Background: For people with cystic fibrosis (pwCF) carrying susceptible mutations, elexacaftor/tezacaftor/ivacaftor (ETI) has changed the course of their lives. Organ manifestations critically affecting both respiratory and digestive tracts have been found to improve considerably. Consequently, ETI-associated reductions in sinonasal symptoms (SNS) deriving from the upper respiratory tract are consistently reported. However, although substantial ETI-related declines in the burden of abdominal symptoms (AS) have been reported using the CFAbd-Score, their correlation with SNS changes has not been studied.

Aims: Assessing correlations between ETI-related changes in pwCF SNS and AS.

Methods: The German adapted version of the Sinonasal-Outcome-Test 20 (SNOT-20-GAV) and the CFAbd-Score, the first CF-specific gastrointestinal (GI) patient-reported outcome measure developed and validated following FDA guidelines, were simultaneously administered. PwCF completing both paired questionnaires, at least once before and after ETI initiation, were included.

Results: In two German CF-centers, n = 31 pwCF (18 f, mean-age 13 years) were included, completing n = 106 paired questionnaires. During ETI therapy, significant decreases (p < 0.05) were observed for the mean total CFAbd-Score (10.9→5.8pts[-47%]) as well as all sub-scores ('pain': -61%, 'disorders of bowel movement': -56%, 'GERD': -54%, 'disorders of appetite': -50% and 'QoL-impairment':-67%). Analogously, SNS significantly improved (total SNOT: 10.1→3.4pts[-66%] and its 3 domains: primary and secondary nasal symptoms, and SNS-related QoL [-68%/-81%/-54%]). However, the correlation between changes in symptoms related to upper-respiratory and GI tracts was only modest.

Conclusion: The burden of symptoms captured with the CFAbd-Score and the SNOT-20-GAV significantly and markedly decreased after ETI initiation. The modest association between changes in both organ-specific symptom scores supports the previously reported individualized electrophysiological responses observed in nasal-potential and intestinal-current measurements.

Introduction: Pediatric patients with tracheobronchomalacia may require tracheostomy for long-term mechanical ventilation. Ventilator instability can persist despite a stable tracheostomy tube and is characterized by desaturations, rising pressure requirements, or the need for cardiopulmonary resuscitation. There is a paucity of data on the management of ventilatory instability in tracheostomy and ventilator-dependent pediatric patients.

Objectives: To evaluate inpatient airway outcomes after tracheobronchoscopy in pediatric patients with tracheostomy and ventilatory dependence.

Methods: Retrospective chart review of tracheostomy- and ventilator-dependent pediatric patients who underwent inpatient bedside tracheobronchoscopy at an academic tertiary referral center from 2017 to 2023. Primary endpoints are positive end expiratory pressure (PEEP) adjustment, tracheostomy tube change, otolaryngologic operative intervention, or medication addition.

Results: There were 132 patients (median 6 months, IQR [4.7-8.4]) who underwent 204 tracheobronchoscopies for ventilatory instability or about 1.5 ± 0.6 bronchoscopies per admission. Of 157 endoscopies that met criteria, 56 (36%) had PEEP adjustment (1.7 ± 2.2 cmH₂O), 57 (36%) had tracheostomy tube size change, 4 (3%) required operations, and 19 (12%) received a medication course. Seventy-three (46%) patients had an improved hospital status. PEEP adjustment (6.9 vs. 4.8 cmH₂O, p = 0.04) and medical management (9.6 vs. 6.4 cmH₂O, p = 0.03) were associated with 24 h PIP range improvement but not FiO₂ improvement. Trach change was not associated with improvement. Patients who underwent tracheostomy change were more likely to receive subsequent bronchoscopy (21% vs. 8%, p = 0.02) and less likely to have improved hospital status (36% vs. 52%, p = 0.04).

Conclusions: Ventilation instability in tracheostomy-dependent children has variable causes. Airway management can be first directed by flexible tracheobronchoscopy for intervention.