Pediatric Pulmonology最新文献

Background: Mental health was identified as a top research priority of the cystic fibrosis (CF) community. The CF Foundation formed a Mental Health Research Priorities Working Group and sought input into specific mental health topics for research prioritization.

Methods: A survey of adults with CF, caregivers/family members, CF Foundation staff, and multidisciplinary CF healthcare providers elicited feedback regarding prioritization of a range of mental health research topics. We compared quantitative and qualitative responses based on respondent type and demographic characteristics, and analyzed group means using two-sided T-tests with Bonferroni adjustment for multiple comparisons (p < 0.1). We summarized progress to date in addressing the identified research priorities.

Results: Community (n = 693) and provider (n = 352) respondents agreed on the top six research priorities rated as "very important" or "a top priority": (1) anxiety, including procedural anxiety; (2) depression/mood disorders; (3) effects of mental health on physical health; (4) effects of treatment burden on mental health; (5) understanding risk factors and prevalence of mental health conditions in people with CF (PWCF); (6) effects of CF on the family, including family planning. Providers rated substance misuse and disordered eating/body image "very important/top priority" more often than community members. Community members rated mental health side effects of CF medications, PTSD/medical trauma, grief/bereavement, and survivor's guilt "very important/top priority" more often than providers.

Conclusions: The CF Foundation Mental Health Research Prioritization survey yielded a compelling roadmap for CF mental health research. These priorities are shaping initiatives to improve the mental health and well-being of PWCF and their families.

Rationale: The lung is uniquely positioned for chemical inhalation exposures considering its direct communication with the environment. Childhood interstitial lung diseases (chILD) syndrome is a rare and heterogeneous group of pediatric lung diseases. Despite common inhalation exposures, few studies have associated chemical inhalation exposures with chILD. The purpose of this review was to assess for chILD syndromes following toxic chemical inhalation exposures.

Methods: PubMed and Embase databases were searched with the following inclusion criteria: (1) toxic chemical inhalation exposure, (2) pediatric subjects, and (3) chILD syndrome. Studies were excluded due to incorrect (1) study design, (2) patient population, and/or (3) outcome.

Results: Two hundred and one studies were identified, of which 142 articles were retrieved, with 74 articles included after inclusion/exclusion criteria were applied by two independent reviewers. Most of the evidence stemmed from two pandemics: humidifier disinfectant associated lung disease (HD-ILD; n = 27) and e-cigarette, or vaping product associated, lung injury (EVALI; n = 45). Common signs and symptoms included cough, shortness of breath, hypoxemia, and inspiratory crackles. Common radiographic findings included centrilobular nodules and ground-glass opacities with subpleural sparing. Histopathologic features included airway-centric injury/inflammation with foamy macrophages, as well as subpleural sparing. Oxidized lipids were common plasma biomarkers associated with both HD-ILD and EVALI. Long-term pulmonary function testing suggests a restrictive phenotype in HD-ILD but variable phenotypes in EVALI.

Conclusions: ChILD syndromes secondary to toxic chemical inhalation exposures manifest with common radiographic and histopathologic findings of airway-centric disease with subpleural sparing. Long-term monitoring is under-reported in toxic chemical inhalation chILD syndromes but suggests persistent lung function impairment, especially after high-dose, repeated exposures. Additional monitoring, evaluation, and reporting of chILD syndromes secondary to chemical inhalation exposure are needed to better understand its complex pathogenesis and long-term lung function implications.

Introduction: There is need for real-life, long-term and large-scale multicentre studies on remote monitoring (RM) in respiratory care to determine its true potential for daily practice. We aimed to analyze the effect of RM with digital action plans on healthcare utilization in long-term pediatric asthma care.

Methods: This was a cohort study in six Dutch pediatric asthma clinics from 2017 until 2023 using healthcare utilization and RM data. Children aged 6-18 years with ≥ 2 years follow-up were included. Differences in median number of outpatient visits between the RM and regular care groups were assessed. Incidence rate ratios (IRR) were calculated for emergency visits and hospitalizations. Interrupted time series analysis was used to evaluate changes in number of outpatient visits after introduction of RM over time. Asthma control of children with RM was analyzed over time.

Results: We included 2526 children, of which 1372 (54.2%) used RM. The median number of annual outpatient visits was lower in the RM group than in the regular care group (ΔMedian 0.65, p < 0.001). RM was associated with a decreased risk of emergency visits (IRR 0.52; 95% CI 0.44, 0.61) and hospitalizations (IRR 0.43; 95% CI 0.34, 0.55). RM was associated with an annual outpatient visit reduction of -9.4 (95% CI -17.0, -1.9) per 100 children. The proportion of children with controlled asthma increased from 76.7% to 86.0% 3 years after introduction of RM.

Conclusions: RM with digital treatment plans is associated with reductions in both regular and emergency healthcare utilization while maintaining high rates of well-controlled asthma.

Background: In cystic fibrosis (CF), the defect of the CF transmembrane conductance regulator (CFTR) can also affect sensory nerve cell function, as recently demonstrated in animal models. The aim of this prospective cohort study was to investigate whether taste and smell disorders in CF correlate with persistent CFTR dysfunction detectable by iontophoresis or rather with inflammation or lung function. Participants with primary ciliary dyskinesia (PCD) and controls without pulmonary disease served as comparators.

Methods: In 65 participants (age median 19 years IQR [12-26]; CF n = 23, PCD n = 22, controls n = 20) at the University Children´s Hospital Bochum, we measured taste (salty, sweet, sour, bitter) at four concentrations ("Taste-Strips," score 0-16, hypogeusia age-adjusted < 8/< 9/< 9.9/< 10 points) and smell ("U-Sniff"-test, score 0-12, reduced odor identification performance < 8 points), pilocarpine iontophoresis, spirometry, inflammatory markers (e.g., CRP) and subjective chemosensory impairment.

Statistics: Chi²/Fisher's-exact, Mann-Whitney-U, Kruskal-Wallis, linear regression; p < 0.05.

Results: Hypogeusia occurred only in CF (17.4%). Particularly misidentification of the taste "salty" occurred significantly more frequently in CF (34.8% vs. PCD 19.3% and controls 17.5%), especially in the CF subgroup with elevated sweat chloride ≥ 60 mmol/l. Reduced odor identification performance was significantly more common in PCD (30% vs. CF 4%). Chemosensory disorders were not related to current lung function or inflammation.

Conclusion: Taste disorders in CF are mostly attributed to difficulties tasting salty and are associated with elevated sweat chloride, probably caused by increased salivary salt following CFTR dysfunction in salivary glands rather than in the nerve cells. Smell disorders, however, remain a significant issue, particularly in PCD.

Objectives: An estimated 23% of children with grade 3 bronchopulmonary dysplasia (BPD) require tracheostomy for long term positive pressure ventilation. Transition from an intensive care unit (ICU) ventilator to a portable home ventilator (PHV) is one of the key steps in the process of discharging a patient home. The objective of this study was to identify clinical factors and ventilator settings associated with successful transition to a PHV in infants with grade 3 BPD.

Study design: We performed a retrospective record review of 74 ventilator-dependent infants with grade 3 BPD hospitalized at 15 centers within the BPD Collaborative who transitioned to PHV between March 2021 and March 2023. Hierarchical cluster analysis of patient demographics and ICU ventilator settings at the time of transition was identify similar groups and compare factors associated with successful transitions.

Results: Patients with higher mean airway pressure (MAP) on the PHV compared with the MAP on their ICU ventilator were more likely to successfully transition on the first attempt, had fewer days from their first attempt until successful transition, and transitioned at an earlier postmenstrual age than patients with a lower MAP on their PHV. Cluster analysis of baseline ICU ventilator settings did not predict successful transition to PHV.

Conclusions: We conclude that higher PHV pressures may enable patients with severe BPD to transition to a PHV. Premature weaning of ventilator pressures may be associated with prolonged need for an ICU ventilator.

Objective: To compare the efficacy and safety of flow-driver-generated synchronized nasal intermittent positive-pressure ventilation (SNIPPV) and biphasic positive airway pressure (BiPAP) after extubation in preterm infants.

Hypothesis: SNIPPV delivered via a flow-driver device improves post-extubation outcomes compared with non-synchronized BiPAP.

Study design: This single-center, retrospective, observational cohort study was conducted in the neonatal intensive care unit of Nagoya University Hospital, Nagoya, Japan.

Patient selection: Preterm infants born at < 34 weeks of gestation who underwent endotracheal ventilation after birth and were subsequently supported with either BiPAP (from October 2017 to March 2020) or SNIPPV (from April 2020 to September 2022) after extubation. Infants with major anomalies or surgical ventilator dependence were excluded.

Methodology: Data on demographic and clinical characteristics, respiratory outcomes, and complications were collected. The primary outcome was reintubation within 72 h. Secondary outcomes were bronchopulmonary dysplasia (BPD), respiratory support duration, and major complications.

Results: Sixty-seven infants were included (BiPAP n = 37, SNIPPV n = 30). Reintubation within 72 h occurred in 6.7% and 29.7% of infants in the SNIPPV and BiPAP groups, respectively (p = 0.028). The incidence of respiratory distress-related failure was significantly lower in the SNIPPV group (3.3% vs. 24.3%, p = 0.019). There were no significant differences in BPD at 36 or 40 weeks, oxygen therapy duration, or complications such as necrotizing enterocolitis, retinopathy of prematurity, or severe neurological injury.

Conclusion: Flow-driver-generated SNIPPV significantly reduces early extubation failure in preterm infants compared to BiPAP without increasing the rate of adverse outcomes. This is a practical and effective alternative to noninvasive respiratory support.

Introduction: Leucine-rich α-2 glycoprotein (LRG) is an acute-phase reactant protein which reflects inflammation differently from C-reactive protein. We investigated the value of cord blood LRG levels for predicting bronchopulmonary dysplasia (BPD) in preterm infants.

Methods: This prospective cohort study included 64 infants born at 22-31 weeks' gestation (22 and 42 in the BPD and non-BPD arms, respectively), and between-group comparisons of LRG and interleukin (IL)-6 levels in the cord blood, neonatal LRG, and immunoglobulin (Ig)M were undertaken. Accounting for gestational age, inverse probability weighted generalized estimating equation assessed the biomarkers' independent effects on BPD.

Results: In the BPD group, the incidences of histological chorioamnionitis, funisitis, neonatal steroid treatment, and ventilator duration were significantly higher (p = 0.023, 0.043, 0.011, and < 0.001, respectively) whereas the Apgar score 1st minute, 5th minute, birthweight, and gestational age were significantly lower (p = 0.001, 0.011, < 0.001, < 0.001, respectively). After adjustment, umbilical artery (UA)-LRG (odds ratio [OR]: 2.678, 95% confidence interval [CI]: 1.225-0.5.857; p = 0.014) and umbilical vein (UV)-IL-6 (OR: 1.258, 95% CI: 1.020-1.552; p = 0.032) remained independent risk factors for BPD, whereas UV-LRG, UA-IL-6, IgM, and neonatal-LRG showed no significant associations.

Conclusions: UA-LRG may serve as a biomarker for predicting BPD and identify infants who are at a higher risk of BPD and could benefit more from corticosteroids or other therapies. UA-LRG's superior predictive accuracy over UV-LRG is attributable to the fact that cytokines in the amniotic fluid might cause fetal inflammation in utero before the development of histologically chorioamnionitis.