Pediatric Pulmonology最新文献

Background: Interstitial lung disease (ILD) has a significant impact on morbidity and mortality in juvenile idiopathic inflammatory myopathies (JIIM). Early and noninvasive detection methods are crucial to improve outcomes through timely diagnosis and intervention. Fractional exhaled nitric oxide (FeNO) is commonly used to assess airway inflammation; however, its utility for the diagnosis of ILD in JIIM remains uncertain. This study investigates the role of FeNO as a potential noninvasive biomarker for ILD in pediatric JIIM patients.

Methods: We enrolled 34 pediatric JIIM patients and classified them into ILD (n = 13) and non-ILD (n = 21) groups based on clinical and high-resolution computed tomography (HRCT) findings. Pulmonary function tests (PFTs), diffusing capacity for carbon monoxide (DLCO) and FeNO measurements were analyzed. Statistical analyses included correlations and receiver operating characteristic (ROC) analyses.

Results: The prevalence of ILD in our cohort was 38.2%. ILD patients had significantly reduced lung function parameters including forced expiratory volume in 1 s (FEV1), forced vital capacity (FVC), total lung capacity (TLC), and diffusing capacity for carbon monoxide corrected for alveolar volume (DLCOcSB/VA) compared to the non-ILD group (p < 0.05). FeNO levels were significantly higher in patients with ILD (median 17 ppb vs. 10 ppb; p = 0.011). Furthermore, FeNO was negatively correlated with TLC, and ROC analysis showed discriminatory power in identifying ILD (AUC = 0.797; p = 0.011). Therefore, FeNO could be considered a valuable marker for detecting ILD.

Conclusion: FeNO correlates with impaired lung function in JIIM-associated ILD and thus shows considerable potential as a noninvasive biomarker to differentiate ILD in pediatric JIIM patients. Our results suggest that integrating FeNO measurements into clinical practice could improve early detection, facilitate timely intervention, and enhance clinical management. Importantly, this study is the first to specifically investigate the clinical utility of FeNO in JIIM patients with ILD. Further large-scale, prospective studies are warranted to validate the role of FeNO in predicting disease progression and therapeutic decisions.

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: Recurrent wheeze in infancy is common; although symptoms often resolve, some children develop persistent disease. To better capture clinical heterogeneity, we analyzed wheeze trajectories based on symptom frequency rather than simply recording presence or absence.

Objective: To identify distinct wheeze phenotypes in 1-year-old children with recurrent wheeze by modeling longitudinal wheezing frequency trajectories.

Methods: We conducted a 2-year, multicenter prospective cohort study involving children aged 12-23 months with recurrent wheeze at 27 sites in Japan. Monthly caregiver-reported wheeze frequency was collected, and trajectories were classified using latent class growth analysis. Clinical characteristics, environmental exposures, and biomarkers were assessed at enrollment and age 3. Ordinal and binary logistic regression analyses were performed to identify risk and protective factors.

Results: Among 253 enrolled children, 219 completed follow-up. Four trajectories were identified: Early-Resolving (24.2%), Low-Frequency with Mid-Peak (57.1%), Persistent High-Frequency (12.8%), and Late-Peaking High-Frequency (5.9%). The latter two groups showed greater symptom burden, including more frequent corticosteroid use and interference with daily activities. Ordinal logistic regression showed that parental allergic rhinitis and pet ownership were associated with lower odds of more severe trajectories. Binary logistic regression comparing high- (Clusters 3-4) versus low-frequency (Clusters 1-2) groups revealed parental smoking as a strong risk factor (OR 5.49), while allergic rhinitis (OR 0.12) and pet ownership (OR 0.11) remained protective.

Conclusions: High-frequency wheeze trajectories were linked to greater clinical burden. Early identification of at-risk children and targeted environmental interventions-particularly avoidance of passive smoking-may reduce morbidity in early-onset recurrent wheeze.

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.