Pediatric Pulmonology最新文献

Introduction: Primary ciliary dyskinesia (PCD) management has not been systematically evaluated and is largely empirical.

Methods: Pediatric participants with PCD were enrolled in a prospective, longitudinal, multicenter, observational study. Therapies were recorded at annual visits and categorized by type. Age-related trends in prevalence of therapies were described by serial cross-sectional analyses. Generalized estimating equations analyzed covariates affecting prevalence of certain therapies and whether these covariates impacted oral antibiotic courses.

Results: A total of 137 participants completed 897 visits over 13 years. All but one received ≥ 1 antibiotic courses during study participation, most often cephalosporins (74%) or amoxicillin-clavulanate (73%). Thirty-one percent reported chronic azithromycin use. Per participant, there was an average of 2.3 (SD = 2.2) oral antibiotic courses annually. The rate of reported antibiotic courses at the 6 United States sites was 2.6 times higher compared to the Canadian site (p < 0.001). As patients got older, they were more likely to report use of amoxicillin-clavulanate (p < 0.001), chronic azithromycin (p < 0.001), fluroquinolones (p < 0.001), inhaled steroids with long-acting beta-agonists (p = 0.010), and hypertonic saline (p < 0.001). Compared to outer dynein arm defects, those with inner dynein arm/microtubular disorganization defects reported increased use of chronic azithromycin (p = 0.011) and inhaled steroids (p = 0.015).

Discussion: Older participants and those with inner dynein arm/microtubular disorganization defects reported more therapies likely due to disease progression and more severe phenotypes, respectively. We report that a wide range of therapies are used in PCD without disease-specific studies defining benefits and risks.

Background: Children with invasive long-term mechanical ventilation (LTMV) dependence are a complex, heterogeneous population with wide variability in respiratory outcomes. Limited data exist on their ventilator weaning trajectories and respiratory characteristics as they progress toward liberation from the ventilator.

Objective: To describe a population of children with invasive LTMV dependence who have successfully liberated from ventilator support, focusing on ventilator parameters as potential early predictors of liberation.

Methods: This was a retrospective study of children who initiated chronic ventilator support at < 12 months of age at our institution, received ventilator support through a tracheostomy tube, were followed through our outpatient clinic, and were fully liberated from mechanical ventilation. Our primary outcome was age at liberation from ventilator support. Multiple covariates were described, including baseline descriptors, health system utilization descriptors, disease markers, and care milestones.

Results: Seventy-eight patients were identified. The median age of tracheostomy was 3.8 [IQR: 3.0-4.8] months. The median age of first hospital discharge to home care was 9.3 months [IQR: 7.5-12], with a median of 44 hospital encounters after initial discharge. These patients were liberated at a median age of 23.9 [18.3-32.2] months. Age at liberation from the ventilator was highly variable within our institution.

Conclusion: The most significant variation in outcome was introduced after hospital discharge and appears to be largely independent of lung disease severity as indicated by ventilatory support. No single covariate was strongly correlated with liberation outcome. Further studies are needed to identify underlying pathophysiology that may contribute to the varied weaning trajectories to better define objective weaning strategies.

Background: Risk factors for progression of adenovirus (AdV)-associated bronchiolitis (AdV-B) to post-infectious bronchiolitis obliterans (PIBO) are poorly defined. We aimed to investigate this in a multicenter cohort.

Methods: A multicenter hospital-based analysis included children admitted with AdV-B in Jerusalem during 2016-2022. A case-control analysis included AdV-PIBO patients in Jerusalem during 2005-2023. Cases were compared to randomly assigned controls admitted with AdV-B without progression to AdV-PIBO. Multivariate analysis with logistic regression was used.

Results: The annual incidence of AdV-B admissions and AdV-PIBO increased during 2016-2022, during which 1522 children were admitted with AdV-B and 8 developed AdV-PIBO (0.5%). Of 30 AdV-PIBO cases identified during 2005-2023, available data were compared for 25 of them (72% boys; mean age ± standard deviation 1.2 ± 0.8 years) and 139 controls (66% boys; mean age 1.0 ± 0.6 years, p = 0.5 for age). Jewish ethnicity was more common in the AdV-PIBO versus control group (92% vs. 66%, p = 0.009), as were oxygen supplementation (84% vs. 45%, p < 0.001), noninvasive ventilation (20% vs. 4%, p = 0.004), consolidations on chest X-ray (44% vs. 19%, p = 0.011), and lymphopenia (92% vs. 46%, p < 0.001). Combining Jewish ethnicity, lymphopenia, consolidations, and prolonged admission as independent risk factors yielded positive and negative predictive values of 68.8% and 90.5%, respectively.

Conclusion: These findings emphasize the urgent need for targeted preventive and management strategies. The identification of Jewish ethnicity as a risk factor may imply a genetic contribution to Adv-PIBO risk.

Background: Spirometry is the gold standard for assessing airway function for clinical studies; however, obtaining high-quality data in young children remains challenging. Since the forced oscillation technique (FOT) requires less subject cooperations, there has been increasing interest in FOT, particularly in young children. We evaluated whether spirometry and FOT in young children provides comparable ability to detect a treatment effect.

Methods: We recently reported in a randomized controlled trial that vitamin C compared to placebo treatment of mothers who smoked during pregnancy (MSDP) results in the offspring having significantly higher forced expiratory flows (FEFs) at 5-years of age, as well as significantly less wheeze at 4-6 years of age. In these same offspring, we also measured respiratory impedance using FOT at 8-Hz impedance at 3, 4, and 5 years of age.

Results: Although spirometry demonstrated significantly increased FEFs in vitamin C compared to placebo-treatment group at 5 years of age (p < 0.001), we were not able to detect a similar treatment effect using FOT impedance.

Conclusions: It may be challenging to obtain technically successful spirometry in preschool children; however, FEFs may provide a better outcome than single-frequency FOT impedance to assess improvements in airway function in these young subjects.

A 6-month-old child with stridor from 3 months of age presented with interim worsening following a respiratory infection. Airway evaluation revealed a smooth, polypoidal mass herniating in and out of the airway with each respiratory cycle, from the anterior surface of the upper trachea. Computerized tomography showed a non-enhancing cystic mass in anterior neck protruding into the upper trachea through an anterior tracheal defect. Surgical resection was performed and pathological examination revealed a bronchogenic cyst. Tracheal bronchogenic cyst is a rare cause of stridor and can occur anywhere along the tracheobronchial tree.

Viral lower respiratory tract infections (LRTI) are ubiquitous in early life. They are disproportionately severe in infants and toddlers (0-2 years), leading to more than 100,000 hospitalizations in the United States per year. The recent relative resilience to severe Coronavirus disease (COVID-19) observed in young children is surprising. These observations, taken together, underscore current knowledge gaps in the pathogenesis of viral lower respiratory tract diseases in young children and respiratory developmental immunology. Further, early-life respiratory viral infections could have a lasting impact on lung development with potential life-long pulmonary sequelae. Modern molecular methods, including high-resolution spatial and single-cell technologies, in concert with longitudinal observational studies beginning in the prenatal period and continuing into early childhood, promise to elucidate developmental pulmonary and immunophenotypes following early-life viral infections and their impact on trajectories of future respiratory health. In November 2019, under the auspices of a multi-disciplinary Workshop convened by the National Heart Lung Blood Institute and the Eunice Kennedy Shriver National Institute of Child Health and Human Development, experts came together to highlight the challenges of respiratory viral infections, particularly in early childhood, and emphasize the knowledge gaps in immune, virological, developmental, and clinical factors that contribute to disease severity and long-term pulmonary morbidity from viral LRTI in children. We hope that the scientific community will view these challenges in clinical care on pulmonary health trajectories and disease burden not as a window of susceptibility but as a window of opportunity.

Objective: This retrospective study aimed to analyze the clinical characteristics, ventilatory strategies, and effectiveness of ventilation in pediatric patients with central apneas treated at the Sleep Medicine and Long-Term Ventilation Unit of the Bambino Gesù Children's Hospital in Rome from 2012 to 2022.

Methods: Among all ventilated patients at our Center from January 2012 to December 2022, we retrospectively included children with a cAHI ≥ 1 events/h on baseline poly(somno)graphic study. Additional parameters assessed included the underlying disease, type of ventilation (non-invasive vs. invasive), age at ventilation onset, ventilation mode, and transcutaneous capnometry parameters. To assess the effectiveness of ventilation on central apneas, we compared the cAHI at baseline and on ventilation.

Results: Sixty-seven patients met the inclusion criteria for central apnea (cAHI > 1 events/h). Diagnoses included hypoxic-ischemic encephalopathy, 15 (22.4%); Ondine syndrome, 14 (20.9%); polymalformative syndrome, 10 (14.9%); Prader-Willi syndrome, 8 (11.9%); brain tumor, 6 (9.0%); Down syndrome, 4 (6.0%); ROHHAD syndrome, 2 (3.0%); other infrequent pathologies were, Arnold-Chiari II, primary central apnea, epilepsy, lisosomal diseases, hydrocephalus, myopathy, obesity, Rett Syndrome. Pressure-supported ventilation (PSV) was the most common mode used (45 out 67 patients, 67.2%), followed by pressure-controlled ventilation (PCV) (15 out 67 patients, 22.4%) and continuous positive airway pressure (CPAP) (7 out 67 patients, 10.4%). Statistically significant improvement (p < 0.05) in cAHI was observed in patients with polymalformative syndrome (3.5 vs. 0.3, p = 0.01), hypoxic-ischemic encephalopathy (3.1 vs. 0.1, p = < 0.01), and Prader-Willi syndrome (3.5 vs. 0.1, p = 0.03), while there was no significant improvementn in children with brain tumor (6.2 vs. 1.5, p = 0.21).

Conclusion: Central apneas are present in children with various underlying pathologies. Ventilatory strategies tailored to the specific diagnosis and severity of central apneas yield significant improvements in cAHI. PSV was the preferred ventilation mode in this study and there was notable effectiveness across different diagnostic categories. PCV was employed in most severe cases. CPAP was exclusively used in patients with predominantly obstructive sleep apneas.