BackgroundTracheobronchomalacia (TBM) is characterized by excessive dynamic airway collapse. Severe TBM can be associated with substantial morbidity. Children with secondary TBM associated with esophageal atresia/tracheoesophageal fistula (EA/TEF) and vascular-related airway compression (VRAC) demonstrate clinical improvement following airway pexy surgery. It is unclear if children with severe primary TBM, without secondary etiologies (EA/TEF, vascular ring, intrinsic pulmonary pathology, or complex cardiac disease) demonstrate clinical improvement following airway pexy surgery.Materials and MethodsThe study cohort consisted of 73 children with severe primary TBM who underwent airway pexy surgery between 2013 and 2020 at Boston Children's Hospital. Pre- and postoperative symptoms as well as bronchoscopic findings were compared with Fisher exact test for categorical data and Student's t-test for continuous data.ResultsStatistically significant improvements in clinical symptoms were observed, including cough, noisy breathing, prolonged respiratory infections, pneumonias, exercise intolerance, cyanotic spells, brief resolved unexplained events (BRUE), and noninvasive positive pressure ventilation (NIPPV) dependence. No significant differences were seen regarding oxygen dependence, ventilator dependence, or respiratory distress requiring NIPPV. Comparison of pre- and postoperative dynamic bronchoscopy findings revealed statistically significant improvement in the percent of airway collapse in all anatomic locations except at the level of the upper trachea (usually not malacic). Despite some initial improvements, 21 (29%) patients remained symptomatic and underwent additional airway pexies with improvement in symptoms.ConclusionAirway pexy surgery resulted in significant improvement in clinical symptoms and bronchoscopic findings for children with severe primary TBM; however, future prospective and long-term studies are needed to confirm this benefit.

Abstract Introduction Epstein-Barr virus (EBV) infects more than 90% of humans worldwide, with no evidence of active infection and persists for life. In pediatric renal transplant recipients, EBV primary infection or reactivation occur more frequently (63% and 44%, respectively). There is limited data to suggest duration of symptoms secondary to EBV and treatment options for pediatric hypersomnia secondary to EBV infection. Report of case(s) We describe a case of hypersomnia in a 7-year-old male with ADHD. He had received a renal transplant three years prior, secondary to renal dysplasia from a deceased donor who was EBV positive. He had a baseline polysomnogram (PSG) completed for symptoms of snoring, witnessed apneas and nocturnal enuresis, and was diagnosed with severe obstructive sleep apnea. He was started on CPAP and demonstrated excellent adherence to positive airway pressure (PAP). At age 9, he developed hypersomnolence, where he would sleep for 12-16 hours a day, fall asleep in school, during meals and vacation trips. The parents reported he was diagnosed with EBV a few months prior (EBV IgG and EBV nuclear antigen positive). Completed diagnostic work up included PAP device interrogation with adherence data, an actigraphy, a diagnostic PSG while on PAP and a multiple sleep latency test (MSLT). All the work up was reassuring, except for the MSLT where the patient had a sleep onset latency of 11 minutes and 3 out of 5 naps with sleep onset REM periods. Over the course of his evaluation, his pediatric daytime sleepiness scale ranged between 20-28 (>15 is significant). He was started on atomoxetine, with some reported improvement in hypersomnolence. Repeat PSG revealed primary snoring; therefore, PAP was discontinued. At age 15, his hypersomnolence had resolved, but conversely, exhibited difficulty initiating and maintaining sleep with higher doses of atomoxetine, therefore it was discontinued after 5 years of treatment. Conclusion In pediatric solid-organ transplant recipients with EBV infection, it is imperative to screen for excessive daytime sleepiness in order to facilitate therapies to improve overall quality of life, as well as recognize that the excessive daytime sleepiness may be a short-term symptom. Support (if any)

Background: Radon may have a role in obstructive lung disease outside its known carcinogenicity. Little is known about radon’s effects on asthma morbidity. Objective: To determine the effect of radon on fractional exhaled nitric oxide (F NO), asthma symptom-days, and lung function in inner-city asthmatic school-children. Methods: Two hundred ninety-nine school-aged asthmatic children enrolled in the School Inner-City Asthma Study (SICAS-1) were followed. One and two-month averaged radon was assessed using a spatiotemporal model predicting zip code-specific monthly exposures. F NO and spirometry were measured twice during the academic year. Asthma symptoms were assessed four times during the academic year. The interaction between indoor radon exposure (Bq/m ) and seasonality predicting log-transformed F NO, FEV % predicted, FVC% predicted, FEV /FVC, and asthma symptom-days was evaluated. Results: Participants with high radon exposure had greater change in F NO from warm to cold periods compared to low radon exposure (interaction p=0.0013). Participants with >50 percentile radon exposure experience significant F NO increase from warm to cold weather ( β =0.29 [95% CI: 0.04,0.54], p=0.0240). We report a positive association between radon 1-month moving average (IRR=1.01, p=0.0273) and 2-month moving average (IRR=1.01, p=0.0286) with maximum asthma symptom-days (n=299, obs=1,167). Conclusions: In asthmatic children, radon may be associated with increased asthma morbidity, suggesting radon may be a modifiable environmental risk factor for airway inflammation.

Introduction: Data on the use of remote spirometry are limited in the pediatric population. We sought to assess the feasibility and accuracy of a digital turbine spirometer, Medical International Research (MIR) Spirobank Smart (MIR, New Berlin, WI, USA), compared with a pneumotachography spirometer, Pneumotrac (Vitalograph Inc., Lenexa, KS, USA), in field-based clinical research.Methods: This is a cross-sectional study of a subgroup of school-aged participants enrolled in the Air quality, Environment, and Respiratory Outcomes in Bronchopulmonary Dysplasia (BPD) study, who performed same-day paired coached baseline spirometry measurements from the Pneumotrac and MIR devices. Proportion of successful tests was estimated for each device and compared using McNemar's test. Correlation between devices forced expiratory volume in 1 second (FEV1) and forced vital capacity (FVC) was analyzed by Lin's concordance correlation, and Bland-Altman plots were generated.Results: Twenty-one participants with history of BPD completed home spirometry maneuvers on both devices. The mean age of participants was 8.7 years. The mean FEV1 and FVC measurement was 81% predicted and 90.4% predicted, respectively. The proportion of acceptable tests appeared higher using Pneumotrac (81%) than when using MIR (67%), although without evidence of discordance (P = 0.317). Among subjects with successful tests on both devices, Lin's concordance correlation demonstrated moderate agreement (FEV1 r = 0.955, 95% confidence interval [CI]: 0.87-0.98; FVC r = 0.971, CI: 0.91-0.99). The mean difference in FEV1 between Pneumotrac and MIR was 0.079 L (95% limits of agreement were -0.141 to 0.298 L) and FVC was 0.075 L (95% limits of agreement were -0.171 to 0.322 L). These were relatively small and without evidence of systematic or volume-dependent bias.Conclusions: Utilizing turbine spirometers may be a promising and feasible way to perform pulmonary function testing for field research in children.

Purpose of review Asthma is the most common chronic disease of childhood. Environmental exposures, such as allergens and pollutants, are ubiquitous factors associated with asthma development and asthma morbidity. In this review, we highlight the most recent studies relevant to childhood asthma risk, onset, and exacerbation related to air pollution exposure. Recent findings In this article, we review current research that has been published between 2021 and 2022, demonstrating the effects of early-life exposure to key air pollutants (e.g., particulate matter (PM), nitrogen dioxide (NO 2 ), sulfur dioxide (SO 2 ) and ground-level ozone (O 3 ), environmental tobacco smoke, radon, and volatile organic compounds (VOC) on respiratory health. Summary Air pollution continues to be a global burden with serious consequences related to respiratory health. Interventions aimed at reducing air pollution in the environment must be achieved in an effort to improve asthma outcomes and pediatric health.

Pediatric PulmonologyVolume 57, Issue 12 p. 3165-3168 LETTER Environmental radon and childhood asthma Lana Mukharesh MD, Lana Mukharesh MD orcid.org/0000-0002-7062-2257 Division of Pulmonary Medicine, Boston Children's Hospital, Boston, Massachusetts, USA Harvard Medical School, Boston, Massachusetts, USA Contribution: Writing - original draft, Methodology, Writing - review & editing, Conceptualization, Formal analysis, ​Investigation, Data curationSearch for more papers by this authorKimberly F. Greco MPH, Kimberly F. Greco MPH Institutional Centers for Clinical and Translational Research (ICCTR), Boston Children's Hospital, Boston, Massachusetts, USA Contribution: Formal analysis, Data curation, Methodology, Conceptualization, ​Investigation, Writing - review & editingSearch for more papers by this authorTina Banzon MD, Tina Banzon MD Harvard Medical School, Boston, Massachusetts, USA Division of Allergy and Immunology, Boston Children's Hospital, Boston, Massachusetts, USA Contribution: Writing - review & editing, ​InvestigationSearch for more papers by this authorPetros Koutrakis PhD, Petros Koutrakis PhD Department of Environmental Health, Harvard T.H. Chan School of Public Health, Boston, Massachusetts, USA Contribution: Funding acquisition, Data curation, Methodology, ​Investigation, Conceptualization, Writing - review & editing, Formal analysisSearch for more papers by this authorLongxiang Li ScD, Longxiang Li ScD Department of Environmental Health, Harvard T.H. Chan School of Public Health, Boston, Massachusetts, USA Contribution: Formal analysis, Data curationSearch for more papers by this authorMarissa Hauptman MD, MPH, Marissa Hauptman MD, MPH Harvard Medical School, Boston, Massachusetts, USA Department of General Pediatrics, Boston Children's Hospital, Boston, Massachusetts, USA Contribution: Writing - review & editing, ​Investigation, MethodologySearch for more papers by this authorWanda Phipatanakul MD, MS, Wanda Phipatanakul MD, MS Harvard Medical School, Boston, Massachusetts, USA Division of Allergy and Immunology, Boston Children's Hospital, Boston, Massachusetts, USA Contribution: Funding acquisition, Writing - review & editing, ​Investigation, Methodology, Data curation, Supervision, ConceptualizationSearch for more papers by this authorJonathan M. Gaffin MD, MMSc, Corresponding Author Jonathan M. Gaffin MD, MMSc [email protected] orcid.org/0000-0003-2213-4504 Division of Pulmonary Medicine, Boston Children's Hospital, Boston, Massachusetts, USA Harvard Medical School, Boston, Massachusetts, USA Correspondence Jonathan M. Gaffin, MD, MMSc, Division of Pulmonary Medicine, Boston Children's Hospital, 300 Longwood Ave, Farley 4, Boston, MA 02215, USA. Email: [email protected] Contribution: Conceptualization, Writing - review & editing, Methodology, Supervision, Funding acquisition, ​Investigation, Data curation, Formal analysisSearch for more papers by this author Lana Mukharesh MD, Lana Mukharesh MD orcid.org/0000-0002-7062-2257 Division of Pulmonary Medicine, Boston Children's Hospital, Boston, Massachusetts, USA Harvard Medical School, Boston, Massachusetts, USA Contribution: Writing - original draft, Methodology, Writing - review & editing, Conceptualization, Formal analysis, ​Investigation, Data curationSearch for more papers by this authorKimberly F. Greco MPH, Kimberly F. Greco MPH Institutional Centers for Clinical and Translational Research (ICCTR), Boston Children's Hospital, Boston, Massachusetts, USA Contribution: Formal analysis, Data curation, Methodology, Conceptualization, ​Investigation, Writing - review & editingSearch for more papers by this authorTina Banzon MD, Tina Banzon MD Harvard Medical School, Boston, Massachusetts, USA Division of Allergy and Immunology, Boston Children's Hospital, Boston, Massachusetts, USA Contribution: Writing - review & editing, ​InvestigationSearch for more papers by this authorPetros Koutrakis PhD, Petros Koutrakis PhD Department of Environmental Health, Harvard T.H. Chan School of Public Health, Boston, Massachusetts, USA Contribution: Funding acquisition, Data curation, Methodology, ​Investigation, Conceptualization, Writing - review & editing, Formal analysisSearch for more papers by this authorLongxiang Li ScD, Longxiang Li ScD Department of Environmental Health, Harvard T.H. Chan School of Public Health, Boston, Massachusetts, USA Contribution: Formal analysis, Data curationSearch for more papers by this authorMarissa Hauptman MD, MPH, Marissa Hauptman MD, MPH Harvard Medical School, Boston, Massachusetts, USA Department of General Pediatrics, Boston Children's Hospital, Boston, Massachusetts, USA Contribution: Writing - review & editing, ​Investigation, MethodologySearch for more papers by this authorWanda Phipatanakul MD, MS, Wanda Phipatanakul MD, MS Harvard Medical School, Boston, Massachusetts, USA Division of Allergy and Immunology, Boston Children's Hospital, Boston, Massachusetts, USA Contribution: Funding acquisition, Writing - review & editing, ​Investigation, Methodology, Data curation, Supervision, ConceptualizationSearch for more papers by this authorJonathan M. Gaffin MD, MMSc, Corresponding Author Jonathan M. Gaffin MD, MMSc [email protected] orcid.org/0000-0003-2213-4504 Division of Pulmonary Medicine, Boston Children's Hospital, Boston, Massachusetts, USA Harvard Medical School, Boston, Massachusetts, USA Correspondence Jonathan M. Gaffin, MD, MMSc, Division of Pulmonary Medicine, Boston Children's Hospital, 300 Longwood Ave, Farley 4, Boston, MA 02215, USA. Email: [email protected] Contribution: Conceptualization, Writing - review & editing, Methodology, Supervision, Funding acquisition, ​Investigation, Data curation, Formal analysisSearch for more papers by this author First published: 13 September 2022 https://doi.org/10.1002/ppul.26143Citations: 4Read the full textAboutPDF ToolsRequest permissionExport citationAdd to favoritesTrack citation ShareShare Give accessShare full text accessShare full-text accessPlease review our Terms and Conditions of Use and check box below to share full-text version of article.I have read and accept the Wiley Online Library Terms and Conditions of UseShareable LinkUse the link below to share a full-text version of this article with your friends and colleagues. Learn more.Copy URL Citing Literature Volume57, Issue12December 2022Pages 3165-3168 RelatedInformation

Abstract Introduction Sleep-disordered breathing (SDB) and asthma have a bidirectional relationship. Almost half of former premature school age children with bronchopulmonary dysplasia (BPD) have asthma or asthma-like symptoms. Prematurity is a known risk factor for sleep-disordered breathing. However, little is known about the association of SDB and asthma symptoms in former premature school age children with BPD. Methods Study sample comprised of participants in an ongoing cohort study, the AERO-BPD study. Participants were 6-12 years old, were born < 32 weeks gestational age and had been diagnosed with BPD. We administered the Pediatric Sleep Questionnaire (PSQ) and asthma symptom questionnaires. The prevalence of SDB was determined based on a PSQ > 0.33. The relationship between SDB and daytime asthma symptoms was assessed by multivariate logistic regression, adjusting for sex, race, BMI, rhinitis, asthma controller medication and days on respiratory support in the NICU. Results There were 33 subjects with a mean age of 8.8 years, roughly half were male and one third were overweight/obese. About half (48.5%) had daytime asthma symptoms and 39.4% had SDB. About half were on controller medication (inhaled corticosteroid or leukotriene receptor antagonist) and 39.4% had rhinitis. Among the 13 subjects with SDB, 11 reported daytime asthma symptoms and 5 of the 20 subjects without SDB reported daytime asthma symptoms. Subjects with SDB had over 17 times the odds of daytime asthma symptoms (OR 17.61, 95% CI [1.23, 251.76], p=0.035) compared to subjects without SDB, while adjusting for sex, race, BMI, rhinitis, controller medications and days on respiratory support in the NICU. Conclusion Our findings suggest that SDB is highly prevalent among former premature school age children with BPD. The findings suggest a relationship between SDB and daytime respiratory symptom burden among these patients rather than mere co-existence. Support (If Any)

Purpose: Although few previous studies suggested an increased prevalence of obstructive sleep apnea (OSA) among children with sickle cell disease (SCD) compared to others, there are little data regarding this association in adults. In this study, we aimed to investigate the prevalence of OSA among the patients with SCD and its risk factors. Materials And Methods: This cross-sectional, observational study was conducted at a specialty hematology clinic. From December 2013 to July 2014, all the patients with SCD and chronic anemia were approached. Data were collected on the risk of OSA using the Berlin questionnaire, and the data on daytime sleepiness were collected using the Epworth sleepiness scale (ESS); medical history of the patients was also obtained. The objective diagnosis of OSA using polysomnography was performed in a subsample of the study population. Results: The study included 106 patients, 44.3% with SCD and 55.7% with other types of anemia being used as a comparison group. Among the patients with SCD, 14.9% were at a high risk of OSA, and 23% were confirmed to have OSA, whereas 10.1% and 16.7% of those in the comparison group did, respectively. However, there were no statistically significant differences between the two groups. Mean scores of risk factors for OSA among the patients with SCD with OSA were 28 years for age, 22.1 kg/m2for body mass index, 14.5 inches for neck circumference, 122 mmHg for systolic blood pressure, and 6.67 for ESS score. Conclusion: Although not different from chronic anemia, the OSA is a common, coexisting disease in the patients with SCD. Furthermore, it is suspected that the traditional screening tools may miss potential cases of OSA in the SCD population.

SESSION TITLE: Sleep Disorders Posters II: Consequences of OSA and TreatmentSESSION TYPE: Original Investigation PosterPRESENTED ON: Wednesday, October 28, 2015 at 01:30 PM - 02:30 PMPURPOSE: The purpose of this study is to determine the prevalence of restless legs syndrome (RLS) among adult patients with sickle cell disease (SCD) and compare it to non-SCD anemia patients.METHODS: This is a cross sectional study that was conducted from December 2013 - July 2014. Data was collected from patients attending the Hematology Clinics at King Abdulaziz University Hospital. Patients were of two groups; SCD and non-SCD anemia. Patients with secondary RLS, including that resulting from iron deficiency anemia, were excluded. All patients were individually interviewed and data was collected on the following: demographic features, clinical features, laboratory tests, the International Restless Leg Syndrome Study Group Criteria (IRLSG), International Restless Leg Syndrome Rating Scale, and daytime sleepiness using Epworth Sleepiness Scale (ESS).RESULTS: Eighty-nine patients were recruited, 44 of which were patients with SCD and 45 with other types of anemia. The two groups were comparable in age, gender, body mass index, smoking habit, and co-morbidities. The prevalence of RLS, severity of RLS and levels of daytime sleepiness were similar in both groups of patients, with no statistically significant differences. The prevalence of RLS among SCD patients and non-SCD anemia patients was 13.6% (6/44) and 8.8% (4/45) respectively, and the majority of patients had moderate to severe RLS. Daytime sleepiness was similar in both groups with the rate of excessive daytime sleepiness being 20.5 % and 17.8 % in SCD and non-SCD anemia groups respectively .CONCLUSIONS: RLS is common in adult SCD patients, but as common as in other anemia patients. Moreover, its severity is similar in both groups. Based on previous epidemiological studies, the prevalence of RLS in SCD patients is similar to that in the general population.CLINICAL IMPLICATIONS: SCD patients are not at a higher risk of RLS than other anemia patients. However, expanded research is needed to compare the prevalence of RLS between SCD patients and the general population, and to understand the implications of RLS on SCD patients' health and quality of sleep.DISCLOSURE: The following authors have nothing to disclose: Rajaa Alsaggaf, Siraj Wali, Faten Alsayis, Sara Batawi, Lana Mukharesh, Ghadah BatawiNo Product/Research Disclosure Information SESSION TITLE: Sleep Disorders Posters II: Consequences of OSA and Treatment SESSION TYPE: Original Investigation Poster PRESENTED ON: Wednesday, October 28, 2015 at 01:30 PM - 02:30 PM PURPOSE: The purpose of this study is to determine the prevalence of restless legs syndrome (RLS) among adult patients with sickle cell disease (SCD) and compare it to non-SCD anemia patients. METHODS: This is a cross sectional study that was conducted from December 2013 - July 2014. Data was collected from patients attending the Hematology Clinics at King Abdulaziz University Hospital. Patients were of two groups; SCD and non-SCD anemia. Patients with secondary RLS, including that resulting from iron deficiency anemia, were excluded. All patients were individually interviewed and data was collected on the following: demographic features, clinical features, laboratory tests, the International Restless Leg Syndrome Study Group Criteria (IRLSG), International Restless Leg Syndrome Rating Scale, and daytime sleepiness using Epworth Sleepiness Scale (ESS). RESULTS: Eighty-nine patients were recruited, 44 of which were patients with SCD and 45 with other types of anemia. The two groups were comparable in age, gender, body mass index, smoking habit, and co-morbidities. The prevalence of RLS, severity of RLS and levels of daytime sleepiness were similar in both groups of patients, with no statistically significant differences. The prevalence of RLS among SCD patients and non-SCD anemia patients was 13.6% (6/44) and 8.8% (4/45) respectively, and the majority of patients had moderate to severe RLS. Daytime sleepiness was similar in both groups with the rate of excessive daytime sleepiness being 20.5 % and 17.8 % in SCD and non-SCD anemia groups respectively . CONCLUSIONS: RLS is common in adult SCD patients, but as common as in other anemia patients. Moreover, its severity is similar in both groups. Based on previous epidemiological studies, the prevalence of RLS in SCD patients is similar to that in the general population. CLINICAL IMPLICATIONS: SCD patients are not at a higher risk of RLS than other anemia patients. However, expanded research is needed to compare the prevalence of RLS between SCD patients and the general population, and to understand the implications of RLS on SCD patients' health and quality of sleep. DISCLOSURE: The following authors have nothing to disclose: Rajaa Alsaggaf, Siraj Wali, Faten Alsayis, Sara Batawi, Lana Mukharesh, Ghadah Batawi No Product/Research Disclosure Information