Pediatric Pulmonology最新文献

Rationale: Primary ciliary dyskinesia (PCD) is a heterogeneous genetic disorder characterized by structural and functional abnormalities of motile cilia, leading to chronic oto-sino-pulmonary symptoms and progressive lung damage. Markers of early lung disease in PCD may help to identify individuals who may benefit from closer monitoring or earlier, more aggressive interventions. Multiple Breath Washout (MBW) offers a noninvasive assessment of ventilation distribution inhomogeneity. Whether MBW could serve as a marker of early lung disease in PCD or could be used as an efficacy endpoint in clinical trials in PCD remains to be established.

Methods: This narrative review evaluates current literature on the role of MBW in early detection and tracking of PCD-related lung disease progression, focusing on its sensitivity compared to spirometry and to the results obtained in different PCD genotypes and phenotypes.

Results: Current evidence suggests that LCI outperforms spirometry in detecting early lung abnormalities, but it may also be overly sensitive in this population. The role of LCI in long-term monitoring remains uncertain, requiring more longitudinal data. Alternative MBW indices, such as S_cond and S_acin, might offer additional insights into the source of ventilation heterogeneity but need further validation. Correlation of MBW with imaging is inconsistent, underscoring the need for integrated approaches.

Conclusion: MBW, particularly LCI, shows promise as a noninvasive sensitive marker of early lung disease in PCD. However, its long-term utility in tracking PCD lung disease remain unclear. Further genotype-stratified, longitudinal studies are needed to confirm its clinical value and optimize its application in PCD management.

Background: Childhood interstitial lung disease (chILD) is rare, heterogeneous, and presents major treatment challenges. This bibliometric study analyzes research trends and hotspots specifically within the domain of chILD treatment research.

Methods: Using bibliometrics, this study utilized data from the Web of Science Core Collection spanning from 2004 to 2024. Various research hotspots were analyzed using VOSviewer, CiteSpace, and the R package "bibliometric."

Results: A total of 577 articles were examined. Prominent journals contributing to this field included Pediatric Pulmonology, Thorax, and the European Respiratory Journal. Griese Matthias, Schwerk Nicolaus, and Clement Annick emerged as the most productive and cited authors. The study identified five key research directions: (1) genetic and molecular mechanisms, (2) clinical management, (3) neonatal risk factors, (4) autoimmune-related factors and immunomodulatory therapies, and (5) fibrosis progression. Keyword analysis showed a recent surge (2022-2024) in research on autoimmune and inflammatory factors, with notable bursts in "polymyositis" and "juvenile dermatomyositis," alongside growing attention to immunosuppressive therapies such as "mycophenolate-mofetil." In contrast, pulmonary fibrosis and imaging-based diagnosis demonstrated limited recent activity, as "thin-section CT" and "high-resolution CT" maintained only a secondary role, reflecting a shift in focus toward immune-mediated mechanisms and therapeutic innovation.

Conclusion: Although the total number of chILD publications remains small, increasing attention to the field highlights the need for further contributions. Along with advances in understanding molecular pathogenesis, the recent surge of immunomodulatory therapies reflects a promising shift toward precision medicine through the identification and targeting of cellular mechanisms.

Children with asthma account for a large share of pediatric pulmonology patients¹ In this review, we highlight pediatric asthma studies published in 2024. After reviewing the collected articles for their potential clinical impact, the authors organized the final selections into thematic groups for reader orientation: Diagnosis and Monitoring, Treatment and Management, Environmental and Social Factors, Genetics and Biomarkers, and Innovative Programs and Health Policy. Given the extensive number of studies published on pediatric asthma and limitations of space, we acknowledge that not every study can be included in this review. We hope this review will spark discussions on current clinical practices, as well as inspire future research in pediatric asthma.

Background: The Global Initiative for Asthma (GINA) and National Asthma Education and Prevention Program (NAEPP) both recommend a combination inhaled corticosteroid (ICS)/formoterol inhaler to replace short-acting beta-agonists (SABA) as the preferred reliever therapy for Steps 3 and 4 of their asthma treatment strategies (known as Single Maintenance and Reliever Therapy or SMART). The extent of adoption and factors influencing use among pediatric asthma specialists remain unclear.

Objective: To gain a better understanding of SMART prescribing practices and factors that facilitate or impede its use in the United States among pediatric asthma specialists.

Methods: An electronic survey on SMART knowledge and practices was distributed to pediatric pulmonology division directors for dissemination to pediatric asthma specialists in allergy and pulmonology divisions.

Results: Sixty-eight completed survey responses were received between March and October of 2024. All asthma specialists were familiar with SMART, and over 90% were comfortable with its implementation in pediatric asthma. However, there was wide variability in the percentage of patients advised to use their prescribed maintenance ICS/formoterol inhaler as a reliever that did not correlate with the comfort level of the clinician or years in clinical practice, but did correlate with the region of practice.

Conclusions: Despite GINA/NAEPP recommending SMART, there is substantial variation in its implementation among pediatric asthma specialists. Survey results indicate that pediatric asthma specialists are comfortable with its use, but that improvement in EMR efficiency and consistent insurance coverage are needed to increase SMART adoption.

Background: The Eastern Mediterranean has climatic conditions that increase exposure to aeroallergens due to higher pollen counts and dust storms. The association of atopy with measures of respiratory health, including fractional exhaled nitric oxide (FeNO) and pulmonary function among asthmatic children during dust and pollen periods has not been assessed.

Objective: Examine the association between allergic sensitization and respiratory health in children with asthma during dust and pollen seasons in the Eastern Mediterranean.

Methods: We conducted an analysis of prospectively collected respiratory health data from schoolchildren with asthma in Cyprus and Crete-Greece during spring and fall of 2019 and 2021. Children were assessed 3 to 4 times over 4 months, including evaluation of allergic sensitization by skin prick tests (SPTs). Mixed effect models were applied, adjusting for potential confounders, including study year, location, weeks on study, age and sex.

Results: There were 131 children included (77 atopic and 54 non-atopic; mean (SD) age = 9.5 (1.6) years; 65% boys). Over the study period, atopic children exhibited 113.71% (95% CI: 68.08%, 171.73%) higher FeNO concentrations and reduced FEV₁/FVC% (-2.62, 95% CI: -5.00, -0.24) compared to non-atopic children. FeNO concentrations were positively associated with the degree of sensitization, as reflected by sensitization to perennial aeroallergens and the number of positive SPT results.

Conclusion: Atopic children with asthma may experience greater morbidity compared to non-atopic children during dust and pollen periods. Given the long-range transport of desert dust, clinicians both within and beyond the Eastern Mediterranean should be aware of this health concern.