Pediatric Pulmonology最新文献

Objectives and hypothesis: Tonic diaphragmatic activity is common during non-invasive ventilation (NIV), suggesting efforts to increase end-expiratory lung volume. We assessed the feasibility and physiological impact of NeuroPAP, a novel NIV mode continuously adjusting the delivered positive pressure proportionally to diaphragm electric activity (Edi) during both inspiration and expiration, in infants with respiratory failure. We hypothesized NeuroPAP would enable dynamic control of end-expiratory pressures (PEEP).

Methodology: This prospective crossover study enrolled premature neonates (25-34 weeks) and infants with bronchiolitis supported by NIV-NAVA. Subjects underwent three ventilation phases: NIV-NAVA, NeuroPAP, and repeat NIV-NAVA. Ventilation pressures, Edi, cardio-respiratory events, neural breathing patterns, and systemic and cerebral oxygenation were assessed.

Results: A total of 15 infants with bronchiolitis and 8 premature neonates were included. The overall median PEEP was unchanged between modes, but PEEP was actively adjusted, with increased breath-to-breath variability of PEEP in NeuroPAP (p < 0.001). Compared to pre-study settings, individual PEEP increased in NeuroPAP in 7, decreased in 9, and was unchanged in 7 patients. In NeuroPAP, the breathing pattern was phasic 74% of the time and tonic 16% of the time, compared to 61% (p = 0.31) and 23% (p = 0.36) in NIV-NAVA. Respiratory rate was lower in NeuroPAP in the neonates (p = 0.006). The estimated PaO₂/FiO₂ ratio was higher in the post-NeuroPAP NIV-NAVA period in the bronchiolitis group (p = 0.006). Edi, heart rate, cerebral NIRS, or cardio-respiratory events were unchanged.

Conclusion: In infants with respiratory failure, NeuroPAP allowed dynamic control and personalization of PEEP. The clinical impact of this warrants further evaluation.

Background: Preschool children with refractory respiratory symptoms often undergo diagnostic bronchoscopy to exclude anatomical and functional abnormalities and to detect suspected chronic lower respiratory tract infections by bronchoalveolar lavage (BAL). The objective of this retrospective analysis was to analyze BAL fluid findings with respect to bacterial colonization and cytology. Additionally, we aimed to determine associations with bacterial colonization of the airways and allergic sensitization status and symptoms as well as to identify comorbidities like gastroesophageal reflux disease (GERD) or eosinophilic esophagitis (EoE).

Methods: In a retrospective analysis, the electronic medical records of 355 children aged 1-5 years who underwent bronchoscopy for refractory respiratory symptoms between 2010 and 2019 were evaluated. Differential cytology and bacterial cultures from BAL, laboratory parameters, oesophagogastroduodenoscopy (OGDS) and histology from esophageal biopsies were analyzed.

Results: A positive bacterial culture from BAL fluid was found in 214 children (61.7%). Of these, 105 children (49%) subsequently received antibiotic treatment. The most frequently identified bacteria were Haemophilus influenzae (34%), Streptococcus pneumoniae (25%) and Moraxella catarrhalis (16%). The percentage of neutrophils in differential cell counts from BAL samples was significantly higher with positive bacterial cultures compared to negative cultures (29.2 + 28.1% vs. 21.2 + 25.4%, p = 0.02). Children with insufficient S. pneumoniae antibody titers had significantly more positive cultures for S. pneumoniae in BAL fluid (28.3% vs. 12.8%; p = 0.0024). GERD was identified in 115 children (32%) and EoE was diagnosed in nine children (2.8%).

Conclusion: Bronchoscopy is a valuable diagnostic tool in the evaluation of persistent respiratory symptoms in preschool children. Bacterial colonization of the airways was common and associated with significantly elevated airway neutrophil counts.

Challenge: Cystic fibrosis (CF) is a global challenge. The epidemiological knowledge is incomplete and focused on patient registries in the United States, Canada, Europe, Australia and New Zealand, Brazil, and South Africa. To complete the global picture of CF, we have to learn from each individual with CF, as well as each cohort and population.

Solution: Structured, harmonized, quality-controlled, and sustainable patient registries are needed worldwide to complete this picture and give every person with CF the opportunity to be part of it.

Requirements: Commitment, trust, transparency, and independence are key elements to building and running a sustainable registry. Combining the experience of existing registries with the commitment of persons with CF and caregivers around the globe could build the basis for more complete data collection. In doing so, we must strike a balance between the quantity and quality of data. The European CF Society Patient Registry Partnership Project for CF registries in low- and middle-income countries outside the World Health Organization European Region is an example of bridging the gaps and allowing broader registry participation.

Conclusion: "Do not call the global CF registry a dream, call it plan," and let's start with the first steps and get involved in the global CF community.

Background: Severe asthma (SA) in children is a complex condition with high morbidity and healthcare costs. Mepolizumab, an anti-interleukin-5 biologic, is approved for severe eosinophilic asthma (SEA) in patients ≥ 6 years, yet long-term real-world pediatric data remain limited.

Aim: To assess the long-term safety and effectiveness of mepolizumab in pediatric SEA, focusing on lung function, oral corticosteroid (OCS) use, and exacerbation rates.

Methods: This retrospective, multicenter study examined the medical records of 33 patients with SEA (aged 6-17 years) who received mepolizumab treatment at three tertiary centers in Turkiye. Inclusion criteria included GINA-defined SA, ≥ 2 severe exacerbations in the previous year requiring OCS, high-dose ICS plus a second controller, and elevated eosinophil counts. Data on exacerbations, pulmonary function tests (PFTs), OCS use, ACT scores, eosinophils, and adverse events were collected over 24 months.

Results: At baseline, patients showed poor asthma control (median ACT: 13), impaired lung function (FEV1%: 62%), and frequent exacerbations (median: 7/year). Mepolizumab significantly reduced exacerbation rates (median: 7 to 0-0.05 at 12/24 months, p = 0.005) and OCS use (87.9% OCS-free by 3 months). ACT scores improved (median: 13-25, p < 0.001), as did FEV1% (62% to 89%, p < 0.001) at 24 months. Eosinophil counts decreased markedly (460 to 50 cells/µL, p < 0.001). The treatment was well-tolerated; one patient discontinued due to anaphylaxis and four due to lack of efficacy.

Conclusion: Mepolizumab showed sustained effectiveness and good tolerability in pediatric SEA, significantly reducing exacerbations and OCS use while improving asthma control and lung function. These findings support its real-world utility and underscore the need for careful patient selection and monitoring.

Background: The primary risk factor determining the progression of tuberculosis (TB) infection is the host's immune status. However, reports of TB cases in children diagnosed with primary immunodeficiency (PID), also referred to as inborn errors of immunity (IEI), remain scarce. In this study, we describe the impact of PID/IEI on childhood TB disease.

Methods: In this retrospective cohort study, data of patients aged 1 month to 18 years who were diagnosed with TB between January 2012 and January 2025 were collected. TB patients were compared according to PID status. Additionally, radiological, histopathological, and microbiological diagnostic findings, as well as clinical features and treatments of TB patients with PID, were evaluated.

Results: A total of 217 TB patients were included, with a median age of 118 months (IQR: 42-169.5). PID was detected in 5.5% (n = 12) of the patients. In 6 (50%) of the PID patients, the immunodeficiency was not known before the TB diagnosis. The median age of patients with PID was 17 months (IQR: 10.3-58.5), which was significantly lower compared to other patients (p = 0.001). The diagnosis of extrapulmonary TB was significantly more common among PID patients (p = 0.049). Treatment durations in patients with PID ranged from 6 to 24 months, and no mortality was observed.

Conclusion: Investigating PID in children diagnosed with TB may be a critical step in enabling early diagnosis and treatment before the development of potentially fatal complications. We also believe that expanding immunological investigations will contribute to a better understanding of childhood TB pathogenesis.

Introduction: Respiratory syncytial virus (RSV) is a leading cause of lower respiratory tract infections (LRTIs) in young children. Additionally, RSV is associated with long-term respiratory morbidities. This study evaluates acute and long-term healthcare utilization (HCU) in infants hospitalized with RSV-associated LRTI (RSV-LRTI) compared to non-RSV LRTI (Non-RSV-LRTI) in a nationwide cohort.

Methods: A retrospective case-control study used data from Clalit Healthcare Services (CHS), Israel's largest healthcare provider. Infants born between 2015 and 2023, admitted before 12 months of age with LRTI during the RSV season, were included. PCR confirmed RSV-LRTI cases, while controls had negative RSV PCR and positive PCR for other respiratory viruses. Acute HCU was assessed within 30 days post-discharge. Long-term respiratory-related HCU was evaluated up to 6 years of age. Statistical analyses included Poisson regression, adjusting for demographic and clinical potential confounders.

Results: A total of 8626 infants were included, with 4,951 in the RSV-LRTI group and 3675 in the Non-RSV-LRTI group. The adjusted acute HCUs were higher in RSV-LRTI cases with increased systemic steroid use (IRR = 2.25, 95%CI: 1.94-2.62, p < 0.001) and short-acting beta-agonist use (IRR = 3.80, 95%CI: 3.36-4.30, p < 0.001). The adjusted long-term HCU trends persisted, with significantly higher use of respiratory-related medications and pediatric pulmonologist visits (IRR = 1.21, 95%CI: 1.06-1.40, p = 0.006) in the RSV-LRTI group.

Conclusion: This nationwide study highlights the substantial acute and long-term HCU burden associated with RSV-LRTI compared to Non-RSV-LRTI, underscoring the need for effective preventive strategies for all infants, especially in the first months of life.