Pediatric Pulmonology最新文献

Introduction: While chloral hydrate (CH) has been standard for infant pulmonary function testing (iPFT) sedation, CH shortages are necessitating use of different sedation approaches. We aimed to compare the safety, test duration, and test quality of alternative sedation strategies for iPFT.

Methods: We conducted a retrospective chart review of iPFT conducted at our center from January 2019 to December 2021. We manually abstracted patient demographics, sedation medications given, adverse events, and iPFT type (raised volume-rapid thoracic compression, plethysmography, bronchodilator response, and/or multiple breath washout), duration (induction, procedure, recovery, and total times), and quality (satisfactory vs unsatisfactory), then compared features of tests conducted with CH to tests conducted with ketamine and midazolam (KM), dexmedetomidine (DX), or multiple agents (polypharmacy, PP) using bivariate and multivariable analysis.

Results: Sixty-six children had iPFT (CH n = 42, KM n = 10, PP n = 8, and DX n = 6). Testing types and proportion of satisfactory tests did not significantly differ between CH and the other sedation strategies. In the multivariable analysis, compared to CH, we found that procedure time was shorter for KM, induction time was shorter for DX, and recovery time was longer for DX, yet total testing duration did not differ between CH and KM (p = 0.61) or DX (p = 0.22). In adjusted analyses, total testing time was longer for PP compared to CH (β = 12.0 min, p = 0.047). Adverse events, all of which were mild, occurred in three patients (PP n = 2, DX n = 1).

Conclusions: Our findings provide preliminary evidence that KM, DX, and PP may be safe and effective alternatives to CH for iPFT sedation.

The management of cystic fibrosis (CF) has significantly improved with the approval of the CF transmembrane conductance regulator (CFTR) modulators. Elexacaftor/tezacaftor/ivacaftor (ETI) is approved for treatment in people with CF (pwCF) over the age of 2 years with eligible mutations. We report 2 cases highlighting improved clinical outcomes following exposure to ETI in an off-label use, including enhanced nutrition status, decrease in respiratory support, exacerbation rates, and need for antibiotics in one of the infants and resolution of echogenic bowel that was diagnosed in utero in the second.

Objective: To compare the effect of the palatal expansion with Hyrax and Hyrax + Up-Locker on sleep behavior, snoring and sleep architecture in children with Sleep Disordered Breathing (SDB).

Materials and methods: This prospective, single-blind, randomized controlled clinical trial enrolled 34 children aged 6-8 years. Participants were randomly assigned using a computer-generated randomization process to receive treatment with either the Hyrax expansion appliance alone or in combination with the Up-Locker vacuum activator. Sleep architecture was evaluated via polysomnography and sleep behavior through the Children's Sleep Habits Questionnaire (CSHQ). Data were analyzed with Shapiro-Wilks for homogeneity and comparisons were performed with either t-test or Mann-Whitney.

Results: Children in the Hyrax + Up-Locker exhibited superior outcomes than in the Hyrax group, including statistically significant reduction in sleep onset delay (p = 0.04) and night wakings (p = 0.02), as well as a notable decrease in snoring (p < 0.001). Children in the Hyrax + Up-Locker vacuum activator group experienced more significant improvements in sleep latency, total sleep time, REM sleep, AHI in REM and nREM sleep, and arousal indices compared to the Hyrax group (p < 0.05) after treatment.

Conclusion: Combined treatment with Hyrax + Up-Locker vacuum activator yielded superior outcomes in sleep architecture and sleep behavior.

Trial registration: ClinicalTrials.gov identifier: NCT06986343.

Background: Treprostinil for the treatment of bronchopulmonary dysplasia-associated pulmonary hypertension (BPD-PH) has previously been described in small cohort studies, often used later in the course after failure to improve on other therapies.

Objective/methods: We retrospectively describe the clinical course and outcomes of 18 infants (gestational age 26.3 ± 2.6 weeks) from 2012 to 2025 who received parenteral treprostinil to treat BPD-PH, including changes in echocardiographic and cardiac catheterization parameters.

Results: All patients had moderate-to-severe BPD and PH, with a mean pulmonary arterial pressure of 45.6 ± 12.7 mmHg at cardiac catheterization prior to treprostinil. Treprostinil was initiated at a median postmenstrual age of 53.5 (IQR: 45.7, 62.6) weeks. Echocardiograms after 3 months of treatment showed improvement of PH severity. At repeat catheterization, mean pulmonary arterial pressure (delta -16.4 ± 12.2, p < 0.01) and indexed pulmonary vascular resistance (delta -4.2 ± 3.3, p < 0.01) significantly improved. Ten of 18 infants (55.6%) survived to discharge; BNP ≥ 35 pg/mL prior to treprostinil initiation demonstrates potential utility for mortality prediction with area under the receiver operator characteristic curve 0.87 ± 0.10 (95% CI: 0.67-1.00).

Conclusion: Our study shows a potential benefit of treprostinil use in moderate-to-severe BPD-PH; larger studies are needed to validate our findings and guide decision-making around treprostinil initiation and duration.

Objective: Infants hospitalized with bronchiolitis may experience persistent symptoms linked to future chronic lung diseases like bronchiectasis. Identifying phenotypes during hospitalization could guide targeted interventions. As traditional clustering requires large datasets, this study explores whether Unsupervised Feature Extraction Algorithms (UFEAs) and clustering can identify high-risk profiles in a small dataset of Indigenous infants.

Methods: We included 128 Indigenous infants hospitalized with bronchiolitis at the Royal Darwin Hospital, Northern Territory, Australia. Eight UEFAs were applied to reduce the dimensionality of 22 variables across 2-17 dimensions. A support vector machine classifier assessed the effectiveness of each UFEA in classifying bronchiectasis. Kernel Principal Component Analysis with nine dimensions performed best, and these dimensions were used for clustering.

Results: Six clinical profiles were identified. Profile C, the highest-risk group with the most infants with bronchiectasis (45%), preterm birth (95%), low birth weight (86%), weight-for-length z-score < -2 (62%), household smoke exposure (90%), and antibiotics prescribed before hospitalization (100%). Profile D, the second-highest risk, had bronchiectasis (30%), the highest wet/productive cough (45%), crackles/crepitations (36%), and wheeze (18%). Profile F infants included bronchiectasis (22%), oxygen supplementation (91%), and lobar collapse/consolidation on chest X-rays (65%). Profile A included bronchiectasis (5%) and household smoke exposure (30%), and Profile E showed bronchiectasis (9%) and household smoke exposure (36%). Profile B, the lowest-risk group, with no bronchiectasis (0%), preterm birth (15%), low birth weight (10%), and any bacteria (5%).

Conclusion: Using UFEAs and clustering, we reduced dataset dimensionality, effectively identifying six unique, clinically significant risk profiles in Indigenous infants.

Introduction: Respiratory syncytial virus (RSV) bronchiolitis during infancy has the potential to progress to recurrent wheezing and asthma. However, studies evaluating lung function using tidal breath analysis in this age group are limited.

Objective: This study aimed to assess lung function in infants hospitalized during their first episode of acute RSV bronchiolitis using tidal breath analysis, to compare tidal breathing parameters between the acute phase and 2 months after clinical recovery, and to evaluate the impact of bronchiolitis severity on lung function.

Subjects and methods: A total of 78 infants aged 1-12 months were enrolled in this prospective study; 39 diagnosed with RSV bronchiolitis and 39 age-and sex-matched healthy controls. Lung function was measured using tidal breath analysis during hospitalization for the acute episode and re-evaluated 2 months after recovery.

Results: The ratio of time to peak tidal expiratory flow to expiratory time (tPTEF/tE) was significantly lower in the RSV bronchiolitis group than in healthy controls, and remained significantly reduced at the 2-month follow-up [19.2% (14.9-24.2) vs. 31.4% (28.7-35.3), p < 0.01]. No significant changes in tidal breath analysis parameters were observed between the acute phase and two- months after recovery. In the severe subgroup, both tPTEF and tPTEF/tE values were lower than in the mild and moderate subgroups.

Conclusion: Our findings suggest that expiratory airway obstruction may persist despite clinical recovery in infants following RSV bronchiolitis. The lack of improvement in tidal breathing parameters, particularly tPTEF and tPTEF/tE, highlights the need for long-term follow-up. Moreover, greater disease severity was associated with more sustained impairment in lung function. These findings emphasize the importance of early detection, long-term monitoring, and preventive strategies to reduce the long-term respiratory consequences of RSV infection.