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Study Objectives: Sleep-disordered breathing (SDB) is prevalent in children with sickle cell disease (SCD) and is associated with worse outcomes. This study aims to compare the outcomes of polysomnography (PSG) performed for pediatric patients with SCD at three United States centers., Methods: We included children with SCD aged 0-21 who underwent PSG at three American Academy of Sleep Medicine accredited centers: the University of Alabama at Birmingham (UAB), the University of Florida (UF), and Duke University Hospital (DUH), between 2012 and 2022. Descriptive statistics were used as appropriate to compare the baseline characters and PSG outcomes among the different centers., Results: A total of 210 children with SCD from the three centers were included, with comparable sex, SCD genotypes, hemoglobin, hematocrit levels, and chronic transfusion. Children from the different centers exhibited variations in age ( P < .001), BMI ( P < .05), mean corpuscular volume ( P < .05), and hydroxyurea use ( P < .05) at the time of the PSG. Overall, the three centers showed significantly different PSG outcomes. Patients from UF had worse obstructive sleep apnea, oxygenation, and periodic leg movement events, together with lower hydroxyurea usage. While those from DUH showed higher hypoventilation and arousal indices., Conclusions: This multicenter study underscores variations in PSG outcomes among pediatric SCD patients at different centers in the Southeast United States. These findings emphasize the need for standardized approaches to screen for SDB, refer to PSG, and interpret the results in children with SCD. These conclusions may apply to other genetic disorders associated with an increased risk of sleep-disordered breathing., (© 2024 American Academy of Sleep Medicine.)

The American Thoracic Society Core Curriculum updates clinicians annually in pediatric pulmonary disease. This is a summary of the Pediatric Pulmonary Medicine Core Curriculum presented at the 2023 American Thoracic Society International Conference. The respiratory disorders of infancy discussed in this year's review include: the care of the patient with bronchopulmonary dysplasia in the neonatal intensive care unit, clinical phenotypes and comorbidities; diffuse lung disease; pulmonary hypertension; central and obstructive sleep apnea. The care of infants with respiratory disorders often poses significant challenges to the general pediatric pulmonologist, sleep clinician, and neonatologist. This review aims to highlight the most clinically relevant aspects of the evaluation, management, and outcomes of infants with these key respiratory disorders, while emphasizing the importance of multidisciplinary care. Furthermore, this document summarizes essential aspects of genetic testing, novel imaging and treatment modalities, and includes multiple resources for clinical practice., (© 2024 Wiley Periodicals LLC.)

Objectives: The COVID-19 pandemic resulted in daily functioning changes for many families. Adjustments in daily functioning may have impacted asthma management and subsequent morbidity. The current study seeks to build upon extant literature by exploring differences in youth asthma exacerbations and control, as well as youth and caregiver asthma-related quality of life (ArQOL) throughout COVID-19 transitional points., Methods: Ninety-three youth (9-17 years old) with asthma and their caregivers completed measures of demographic/medical information, asthma control, and ArQOL. Participants were recruited between January 2020 and October 2021 via their medical appointments and a hospital registry. We conducted Kruskal-Wallis H -tests to examine differences in youth asthma exacerbations (measured by short-acting beta agonist use), asthma control, and ArQOL, as well as caregiver ArQOL, across phases of the COVID-19 pandemic., Results: Asthma exacerbations were higher prior to the onset of the pandemic compared to "during lockdown" and "post-lockdown," H(2) = 7.31, p  < .05. Youth's asthma control was lower prior to the onset of the pandemic compared to youth enrolled "post-lockdown," H(2) = 7.04, p  < .05. There were no differences in youth ArQOL across the duration of the pandemic. Caregiver ArQOL was significantly higher in the "post-lockdown," period, compared to caregivers enrolled prior to the pandemic onset, H(2) = 9.86, p  < .01., Conclusion: Youth and caregiver asthma functioning improved following the onset of the pandemic. These findings build upon existing literature to highlight higher ArQOL in caregivers following the pandemic onset, likely related to improvements in youth asthma control and morbidity. Future research should explore trajectories of asthma and psychosocial functioning throughout the pandemic for families., Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (© 2023 Sinisterra, Sweenie, Ezmigna and Fedele.)

Background Hypersensitivity pneumonitis (HP) infrequently presents in childhood. Asthma or a pneumonia-like clinical presentation may lead to diagnostic delay, especially in children. Case Report: We present two cases of HP, a 6-year-old (Case 1) male and a 5-year-old (Case 2) female. Both cases had a negative infectious work-up and patchy ground glass lung opacities on chest computed tomography. Lung biopsies demonstrated lymphocytic bronchiolitis with granulomatous interstitial and peribronchial inflammation. Serology demonstrated elevated immunoglobulin precipitins toward Thermoactinomyces and Aspergillus species in Case 1 and Aspergillus fumigatus in Case 2. Both patients received steroid therapy and had symptom resolution. Conclusions: A diagnosis of HP should be considered in pediatric lung biopsies with granulomatous interstitial and peribronchial inflammation, if infectious etiologies are excluded. Integration of clinical, radiological, and laboratory findings can facilitate a timely diagnosis.

Objective: The asthma predictive index (API) predicts later asthma in preschoolers with frequent wheeze. We hypothesized that airway cytology differs between API positive (API+)/negative (API-) children with uncontrolled/recurrent wheezing with dominance of eosinophils in API+ and neutrophils in API- groups respectively. The main objective of this study is to compare bronchoalveolar lavage (BAL) cell profiles in API+/API- children with recurrent wheezing unresponsive to inhaled corticosteroids (ICS)., Design: Retrospective analysis of BAL in 43 children, 3-36 months (median: 14 months) receiving ICS (31 API+, 12 API-). BAL cell differential counts, bacterial/viral cultures, and lipid-laden macrophage percentages were analyzed. Cell counts presented as median (range)., Results: Neutrophil percentages were increased in both groups (API- 16% [1%-76%]; API+ 42% [1%-95%]; p = NS). Cell percentages were similar for lymphocytes (API- 12% [1%-30%]; API+ 7% [1%-37%]), and macrophages (API- 67.5% [12%-97%]; API+ 41% [2%-94%]). Eosinophil percentages were low in both groups (API- 1% [1%-2%]; API+ 1% [1%-11%]). There was no difference in cellular distributions using absolute cell counts comparing API groups. Bacterial cultures were positive in 18 (60%) API+ and 5 (41%) API- patients (p = 0.10)., Conclusion: Cell profiles do not differ between API groups in children ≤36 months with recurrent wheezing unresponsive to ICS. Neutrophil percentages and total granulocyte count correlate with positive bacterial cultures independent of API status. Persistent bacterial bronchitis likely plays an important role in the persistence of symptoms unresponsive to ICS therapy regardless of API status with a trend to more positive cultures in API positive children., (© 2021 Wiley Periodicals LLC.)

Background: It is necessary to find a non-invasive and accurate procedure to predict persistent bacterial bronchitis (PBB) causative organisms and guide antibiotic therapy. The study objective was to compare the diagnostic accuracy of nasopharyngeal swab cultures with bronchoalveolar lavage (BAL) cultures in children with PBB., Methods: Nasopharyngeal swab and BAL fluid specimens were collected and cultured for bacterial pathogens prospectively from less than five-year-old children undergoing flexible bronchoscopy for chronic wet cough., Results: Of the 59 children included in the study, 26 (44.1%) patients had a positive BAL bacterial culture with neutrophilic inflammation. Prevalence of positive cultures for any of the four common respiratory pathogens implicated in PBB ( Moraxella catarrhalis , Streptococcus pneumoniae , Staphylococcus aureus , and Haemophilus influenzae ) was significantly higher ( p = 0.001) in NP swabs compared to BAL fluids (86.4% and 44.1% of PBB cases, respectively). NP swab cultures for any of the four main bacterial pathogens had 85% (95% CI: 65-96%) and 48% (95% CI: 31-66%) sensitivity and specificity of detecting PBB, respectively. Positive and negative predictive values were 56% (95% CI: 47-65%) and 80% (95% CI: 60-91%), respectively. In conclusion, in children less than 5 years of age with chronic wet cough (PBB-clinical), a negative NP swab result reduces the likelihood of lower airway infection; however, a positive NP swab does not accurately predict the presence of lower airway pathogens. Flexible bronchoscopy should be considered in those with recurrent PBB-clinical or with clinical pointers of central airway anomalies.

Background: In children with sickle cell disease (SCD), comorbid asthma is associated with increased disease severity and morbidity, but it remains underdiagnosed and optimal management paradigms are not well defined. The purpose of this study was to determine the feasibility and preliminary outcomes of an integrated pediatric SCD and pulmonary care clinic in children with SCD., Methods: We implemented a pre-post quality improvement (QI) project in our pediatric hematology clinic between 2017 and 2019. Guided by the chronic care model, patients who were ages 2-18 years, diagnosed with SCD and suspected pulmonary comorbidities, received care in an interdisciplinary clinic. We examined feasibility and compared clinical outcomes to 24 months prior (2015-2017) to the implementation of the integrated care model., Results: Twenty-four patients were included in the QI project: 88% (n = 21) received pulmonary function testing, 92% (n = 22) were diagnosed with asthma, and 33% (n = 8) with obstructive sleep apnea. Adherence to pulmonary appointments was increased by 81% (mean difference [MD] = 1.3, 95% confidence interval [CI] = 0.71-1.92; P < .001). Unplanned acute health care utilization was reduced by 59% (MD = 2.9, 95% CI = 1.14-4.69; P < .01) and packed red blood cell transfusion was reduced by 81% (MD = 1.38, 95% CI = 0.71-2.04; P < .001)., Conclusion: Asthma is prevalent in children with SCD, and interdisciplinary clinics can improve access to subspecialty pulmonary care and reduce unplanned acute care. Additional patients and a longer follow-up period are required to determine the true treatment effect., (© 2020 Wiley Periodicals LLC.)

A de-identified data repository of electronic medical record (EMR) data, i2b2 (Informatics for Integrating Biology and the Bedside), including 4 geographically diverse academic medical centers, was queried to determine the use of diagnostic spirometry testing in African American children and young adults 5-34 years old with sickle cell disease (SCD) with or without a documented history of asthma and/or acute chest syndrome (ACS). A total of 2,749 patients were identified with SCD, of these 577 had asthma and 409 had ACS. Cross-referencing the CPT code for diagnostic spirometry showed that for patients identified as having SCD, a history or ACS, and a diagnosis of asthma, only 31% across all 4 centers had spirometry. Having an asthma diagnosis was associated with ACS. Among SCD patients with asthma, the proportion with ACS for the four centers was 47%, 75%, 38%, and 36% respectively. The bivariate association between asthma and ACS for each Center was significant for each (p<.001). To summarize, only one third of patients with co-morbid SCD, ACS, and asthma received the spirometry procedure as recommended in evidence-based guidelines, suggesting limited testing for changes in pulmonary function. Future studies to determine barriers and facilitators to implementation of pulmonary testing in SCD are warranted., Competing Interests: Conflicts of Interest Disclosure: The authors declare no competing financial interests.