Characteristics of sleep in children with heavy prenatal alcohol exposure.

BACKGROUND: Sleep plays an important role in neurodevelopment. However, the effects of prenatal alcohol exposure on sleep quality have been understudied, despite reports of sleep disturbance in infants prenatally exposed to alcohol and elevated levels of sleep problems reported by caregivers of children with fetal alcohol spectrum disorders. The current study characterizes sleep in children with prenatal alcohol exposure using both objective (actigraphy) and subjective (questionnaires, sleep diaries) methods. METHODS: Participants aged 6-10 years, with and without prenatal alcohol exposure, were included in the study (alcohol-exposed [AE]: n = 35; control [CON]: n = 39). Objective sleep was measured via 24-h actigraphy for 2 weeks. Parents completed sleep diaries and sleep questionnaires (Children's Sleep Habits Questionnaire, Pediatric Sleep Questionnaire). Multivariate analysis of variance was used to characterize the sleep profile (objective, subjective) and examine group differences. RESULTS: There were no group differences on actigraphy metrics averaged across 2 weeks. However, the AE group showed significantly greater intraindividual variability on most actigraphy measures, particularly total sleep time, percent sleep, wake after sleep onset, and number of wake bouts. Parents reported significantly more sleep problems in the AE group than in the CON group, primarily driven by night wakings, parasomnias (e.g., sleepwalking), snoring, and daytime sleepiness. These effects were more severe in children >8.5 years of age. CONCLUSIONS: Despite similar 2-week average sleep outcomes, children with prenatal alcohol exposure showed greater intraindividual sleep variability and parents reported more sleep problems related to sleep behavior and snoring. These difficulties with sleep may be related to other cognitive and behavioral outcomes. Importantly, sleep is a modifiable behavior, and interventions that focus on variability in sleep, particularly in sleep duration, can impact the quality of life in children with prenatal alcohol exposure and their families.

Effectiveness of long-term noninvasive ventilation measured by remote monitoring in neuromuscular disease.

Background and objective: Patients with neuromuscular disease are often treated with home noninvasive ventilation (NIV) with devices capable of remote patient monitoring. We sought to determine whether long-term NIV data could provide insight into the effectiveness of ventilation over time. Methods: We abstracted available longitudinal data for adults with neuromuscular disease in monthly increments from first available to most recent. Generalised linear mixed-effects modelling with subject-level random effects was used to evaluate trajectories over time. Results: 1799 months of data across 85 individuals (median age 61, interquartile range (IQR) 46-71 years; 44% female; 49% amyotrophic lateral sclerosis (ALS)) were analysed, with a median (IQR) of 17 (8-35) months per individual. Over time, tidal volume increased and respiratory rate decreased. Dynamic respiratory system compliance decreased, accompanied by increased pressure support. Compared to volume-assured mode, fixed-pressure modes were associated with lower initial tidal volume, higher respiratory rate and lower pressures, which did not fully equalise with volume-assured mode over time. Compared with non-ALS patients, those with ALS had lower initial pressure support, but faster increases in pressure support over time, and ALS was associated wtih a more robust increase in respiratory rate in response to low tidal volume. Nonsurvivors did not differ from survivors in ventilatory trajectories over time, but did exhibit decreasing NIV use prior to death, in contrast with stable use in survivors. Conclusion: NIV keeps breathing patterns stable over time, but support needs are dynamic and influenced by diagnosis and ventilation mode. Mortality is preceded by decreased NIV use rather than inadequate support during use.

Association of body anthropometry and obstructive sleep apnea in children: Variations observed in Hispanic children.

Objectives: Obesity is a risk factor for obstructive sleep apnea (OSA) in children. Childhood obesity rates vary amongst different ethnic groups. Here the interaction of Hispanic ethnicity and obesity on OSA risk was evaluated. Methods: Retrospective cross-sectional analysis of consecutive children undergoing polysomnography and anthropometry using bioelectrical impedance from 2017 to 2020. Demographics obtained from the medical chart. Children who had also undergone cardiometabolic testing were identified and the relationship of cardiometabolic markers with OSA and anthropometry was assessed. Results: Data from 1217 children revealed Hispanic children were more likely to have moderate-severe OSA (36.0%) compared to Non-Hispanic children (26.5%), p < 0.001. Hispanic children had greater Body mass index (BMI), BMI percentile and percent body fat, p < 0.0001. In children that underwent cardiometabolic testing, Hispanic children had significantly greater serum alanine aminotransferase levels (ALT) levels. Following adjustment of age and sex, Hispanic ethnicity was not found to moderate the association of anthropometry with OSA, anthropometry with cardiometabolic markers, and OSA with cardiometabolic markers. Conclusions: OSA was more likely in Hispanic children; this relationship was likely driven by obesity status rather than ethnicity. Among children undergoing cardiometabolic testing, Hispanic children were observed to have greater ALT concentrations however ethnicity did not impact the association of anthropometry and ALT or other cardiometabolic markers.

Non-invasive ventilation usage and adherence in children and adults with Duchenne muscular dystrophy: A multicenter analysis.

INTRODUCTION/AIMS: Non-invasive ventilation (NIV) is routinely prescribed to support the respiratory system in Duchenne muscular dystrophy (DMD) patients; however, factors improving NIV usage are unclear. We aimed to identify predictors of NIV adherence in DMD patients. METHODS: This was a multicenter retrospective analysis of DMD patients prescribed NIV and followed at (1) The Hospital for Sick Children, Canada; (2) Rady Children's Hospital San Diego, USA; and (3) University of California San Diego Health, USA, between February 2016 and October 2020. The primary and secondary outcomes were 90-day period NIV adherence and clinical and socioeconomic predictors of NIV adherence. RESULTS: We identified 59 DMD patients prescribed NIV (mean ± SD age = 20.1 ± 6.7 y). Overall, percentage of nights used, and average nightly usage, were 79.9 ± 31.1% and 7.23 ± 4.12 h, respectively. Compared with children, adults had higher percentage of nights used (92.9 ± 16.9% vs. 70.4 ± 36.9%; P < .05), and average nightly usage (9.5 ± 4.7 h vs. 5.3 ± 3.7 h; P < .05). Non-English language (P = .01), and absence of deflazacort prescription (P = .02) were significantly associated with higher percentage of nights used while Hispanic ethnicity (P = .01), low household income (P = .02), and absence of deflazacort prescription (P = .02) were significantly associated with higher nightly usage. Based on univariable analysis, older age and declining forced vital capacity were associated with increased percentage of nights used and increased average nightly usage. DISCUSSION: Certain clinical and socioeconomic determinants had a significant impact on NIV adherence in DMD patients, providing insight into those at risk for high versus low compliance with respiratory therapy.

Reducing Mask-Related Pressure Injuries in Pediatric Patients During Noninvasive Ventilation by Targeting Patient Mask Leak: A Quality Improvement Initiative.

PURPOSE: To compare outcomes following implementation of patient mask leak range of 25 to 55 liters per minute (lpm) to guide strap tension of sleep masks during noninvasive ventilation against baseline data with no patient mask leak range on number of noninvasive mask-related pressure injuries (PIs). PARTICIPANTS AND SETTING: All noninvasively ventilated pediatric acute care patients admitted to general wards and intensive care units between February 1, 2018, and February 1, 2019, in a quaternary hospital in the southwest United States. APPROACH: Using the Plan Do Study Act model, we employed an intervention to examine the rate of PIs per noninvasive positive pressure ventilation (NIPPV) days and patient-days before and after implementation of patient mask leak parameters between 25 and 55 lpm to guide mask strap tension. Since patients are at an increased risk of sleep mask-related PIs only when on NIPPV, we sought to describe that as number or PIs per number of days at risk, described as NIPPV days, and patient days which is the traditional denominator for wounds and hospital-acquired conditions. OUTCOMES: Preintervention, 6 out of 115 subjects (5.2 %) incurred PI at a rate of 0.51 per 100 NIPPV days or 0.26 per 1000 patient-days. Of the 1932 NIPPV days since education was completed, only 1 subject out of 87 (1.1%) incurred a high-stage PI (0.05/100 NIPPV days-a 96.79% reduction or 0.05/1000 patient-days-a 92.86% reduction). Upon reaching more than 90% compliance with patient mask leak range in December 2018, 1221 NIPPV days resulted in 0 noninvasive mask-related PIs. Greater than 90% compliance with a patient mask leak of 25 to 55 lpm allowed us to successfully achieve our hospital's operating plan goal of 0.15 of 1000 patient-days within this group. IMPLICATIONS FOR PRACTICE: Maintaining a patient mask leak range between 25 and 55 lpm should be considered as part of proper fit for pediatric patients using NIPPV with a mask. Additional work is needed to assess this leak range in more children and in studies conducted at multiple sites.

Completion of postoperative polysomnography for children with severe obstructive sleep apnea: A quality improvement project.

Objective: Pediatric patients with severe obstructive sleep apnea (OSA) are at risk for residual OSA following tonsillectomy with/without adenoidectomy (T ± A). We initiated a quality improvement (QI) project to increase the percentage of postoperative (postop) polysomnography (PSG) completion to identify residual OSA. Methods: This is a prospective QI project carried out at a tertiary pediatric academic hospital. Children ≤18 years of age who underwent T ± A for severe OSA were included. Our Specific, Measurable, Attainable, Relevant, and Time-based (SMART) aim was to increase the percentage of completed postop PSGs in this cohort from a baseline of 70% to95% by May 31, 2021. We focused on patient education and leveraged both clinical decision support and reporting functionalities of the electronic medical record for project implementation. Results: During the pre-intervention period between January 1, 2019 to June 30, 2020, 472 patients met the inclusion criteria with an average age of 8.6 years (SD 4.6). The rate of postop PSG completion was 69.7% (SD 11.4%) with an average time of 99 days (SD 66) between surgery and the postop PSG. A shift was observed starting in September 2020, and the PSG completion rate improved to 94.9% by September 30, 2021. Post-intervention, there were 178 patients with an average age of 9.3 years (SD 4.9). The average time between surgery and the postop PSG was significantly reduced to 57 days (SD 16; p < .001). Conclusions: Through a multidisciplinary approach, we successfully completed our SMART aim. With the establishment of QI infrastructure, our goal is to deliver better care in a sustainable fashion using QI methodology.

The impact of daytime transoral neuromuscular stimulation on upper airway physiology - A mechanistic clinical investigation.

There is a need for alternatives to positive airway pressure for the treatment of obstructive sleep apnea and snoring. Improving upper airway dilator function might alleviate upper airway obstruction. We hypothesized that transoral neuromuscular stimulation would reduce upper airway collapse in concert with improvement in genioglossal muscle function. Subjects with simple snoring and mild OSA (AHI < 15/h on screening) underwent in-laboratory polysomnography with concurrent genioglossal electromyography (EMGgg) before and after 4-6 weeks of twice-daily transoral neuromuscular stimulation. Twenty patients completed the study: Sixteen males, mean ± SD age 40 ± 13 years, and BMI 26.3 ± 3.8 kg/m2 . Although there was no change in non-rapid eye movement EMGgg phasic (p = 0.66) or tonic activity (p = 0.83), and no decrease in snoring or flow limitation, treatment was associated with improvements in tongue endurance, sleep quality, and sleep efficiency. In this protocol, transoral neurostimulation did not result in changes in genioglossal activity or upper airway collapse, but other beneficial effects were noted suggesting a need for additional mechanistic investigation.

Characterization of sleep-disordered breathing in children with Duchenne muscular dystrophy by the American Academy of Sleep Medicine criteria vs disease-specific criteria: what are the differences?

STUDY OBJECTIVES: Individuals with Duchenne muscular dystrophy (DMD) frequently develop sleep-disordered breathing. Noninvasive ventilation is often prescribed for sleep-disordered breathing treatment based on the American Academy of Sleep Medicine (AASM) criteria. In 2018, DMD disease-specific criteria for sleep-disordered breathing were established. Our study aimed to examine the clinical interpretation differences using these different criteria. METHODS: We performed a multicenter, retrospective chart review of children with DMD followed at The Hospital for Sick Children, Toronto, Canada, and Rady Children's Hospital, San Diego, California, who underwent polysomnography from August 1, 2012, to February 29, 2020. Baseline characteristics and polysomnography data were summarized using descriptive statistics. Agreement for the diagnosis of sleep-disordered breathing evaluated by kappa statistics and sensitivity/specificity analysis was assessed. RESULTS: One hundred five male children with DMD (mean ± SD age: 12.1 ± 3.8 years; body mass index z score: 0.2 ± 2.3) were included. The proportions of children with DMD that met at least 1 AASM criterion and at least 1 DMD criterion were 45.7% and 67.6%, respectively. We found that 32.4% of children met neither AASM nor DMD criteria. Overall agreement between AASM and DMD criteria was moderate (k = 0.57). There was almost perfect agreement in sleep apnea diagnosis (k = 0.90); however, there was only slight agreement in hypoventilation diagnosis (k = 0.12) between AASM and DMD criteria. CONCLUSIONS: There were more children with DMD diagnosed with nocturnal hypoventilation and prescribed noninvasive ventilation using DMD criteria compared with AASM criteria. Future studies should address whether the prescription of noninvasive ventilation for children with DMD based on both criteria is associated with different clinical outcomes. CITATION: Hurvitz MS, Sunkonkit K, Massicotte C, Li R, Bhattacharjee R, Amin R. Characterization of sleep-disordered breathing in children with Duchenne muscular dystrophy by the American Academy of Sleep Medicine criteria vs disease-specific criteria: what are the differences? J Clin Sleep Med. 2022;18(2):609-615.

Role of endothelial cells in pulmonary fibrosis via SREBP2 activation.

Idiopathic pulmonary fibrosis (IPF) is a progressive lung disease with limited treatment options. Despite endothelial cells (ECs) comprising 30% of the lung cellular composition, the role of EC dysfunction in pulmonary fibrosis (PF) remains unclear. We hypothesize that sterol regulatory element-binding protein 2 (SREBP2) plays a critical role in the pathogenesis of PF via EC phenotypic modifications. Transcriptome data demonstrate that SREBP2 overexpression in ECs led to the induction of the TGF, Wnt, and cytoskeleton remodeling gene ontology pathways and the increased expression of mesenchymal genes, such as snail family transcriptional repressor 1 (snai1), α-smooth muscle actin, vimentin, and neural cadherin. Furthermore, SREBP2 directly bound to the promoter regions and transactivated these mesenchymal genes. This transcriptomic change was associated with an epigenetic and phenotypic switch in ECs, leading to increased proliferation, stress fiber formation, and ECM deposition. Mice with endothelial-specific transgenic overexpression of SREBP2 (EC-SREBP2[N]-Tg mice) that were administered bleomycin to induce PF demonstrated exacerbated vascular remodeling and increased mesenchymal transition in the lung. SREBP2 was also found to be markedly increased in lung specimens from patients with IPF. These results suggest that SREBP2, induced by lung injury, can exacerbate PF in rodent models and in human patients with IPF.

Management and outcome of extreme pediatric obstructive sleep apnea.

OBJECTIVES: Classify post-adenotonsillectomy (AT) respiratory support, identify variables that predict these interventions, and evaluate outcomes in children with extreme obstructive sleep apnea (OSA). METHODS: Retrospective chart analysis was performed on patients found to have apnea/hypopnea index (AHI) > 100 events/h. Patients with chronic diseases other than obesity were excluded. RESULTS: Forty-one subjects were studied, average age of 11.4 ± 4.3 years, majority (73.1%) were Hispanic, with a mean total AHI (TAHI) of 128.1 ± 22.9/h. Twenty-eight (68.3%) patients underwent AT. Lower age (P < 0.001), lower BMI Z-score (P < 0.01), higher OAHI (P < 0.05) were associated with having surgery. Eleven out of 28 (39.3%) surgical patients required respiratory support (oxygen or positive airway pressure) postoperatively. Longer % total sleep time SpO2 <90% during PSG (P < 0.05) and lower SpO2 nadir (P < 0.05) were associated with requiring airway support. No patients experienced mortality, reintubation, or hospital readmission following AT, with majority (71.4%) discharged 1 day post-operatively. Eleven (57.9%) of the 19 patients who had a postoperative PSG had residual OSA, defined as AHI >5 events/h, but there was a significant improvement in TAHI (P < 0.01). CONCLUSION: Our findings confirm the need for postoperative observation in a controlled setting for patients with extreme OSA undergoing AT. Although at higher risk of needing respiratory support, those patients undergoing AT for extreme OSA did not require re-intubation post-operatively or suffer serious harm. Barring contraindications to AT, surgery may still be a first-line therapy for some children with extreme OSA.

Determinants of usage and nonadherence to noninvasive ventilation in children and adults with Duchenne muscular dystrophy.

STUDY OBJECTIVES: Duchenne muscular dystrophy (DMD) is a neuromuscular disorder that leads to chronic respiratory insufficiency and failure. Use of home noninvasive ventilation (NIV) has been linked to improved outcomes including reduced mortality. Despite the importance of NIV, factors promoting optimal NIV usage and determinants of nonadherence have not been rigorously examined. Moreover, given that respiratory issues in DMD span between childhood and adulthood, examination across a broad age group is needed. The objectives of this study were to (1) evaluate NIV usage across a broad spectrum of patients with DMD, including both children and adults, and (2) identify biological and socioeconomic determinants of NIV usage and NIV nonadherence. METHODS: We performed a retrospective review of all patients with DMD from February 2016 to February 2020 who underwent evaluation at associated pediatric and adult neuromuscular disease clinics. NIV use was determined objectively from device downloads. A priori, we defined nonadherence as < 4 hours use per night, quantified as the percentage of nights below this threshold across a 30-day period within 6 months of a clinic visit. We also assessed the average hours of NIV usage over this time period. Predictors examined included demographics, social determinants, and pulmonary function. RESULTS: 33 patients with DMD were identified, 29 (87%) of whom were using NIV (13 age < 21 years). Mean age was 22.9 ± 6.6 years (range 13-39 years), body mass index was 23.4 ± 10.4 kg/m2, and seated forced vital capacity was 23% ± 18% predicted. Mean nightly NIV usage was 7.4 ± 3.8 hours and mean percentage of nonadherent nights was 13% ± 30%. In univariable analysis, age did not predict use. Those with lower forced vital capacity had higher NIV usage hours (P = .01) and a trend toward less nonadherence (P = .06). Higher estimated household income demonstrated a trend toward increased usage hours and less nonadherence (both P = .08). Multivariable analysis found increased usage hours were predicted best by higher income, higher inspiratory positive airway pressure, and higher bicarbonate. Nonadherence was higher in those with lower income or higher forced vital capacity. CONCLUSIONS: In this cohort of adult and pediatric patients with DMD, most individuals were using NIV. While usage hours were higher with lower lung function, substantial variability remains unexplained by examined factors. Nonadherence was observed in some individuals, including those with advanced disease. Further investigations should focus on evaluating patient-oriented outcomes to define optimal NIV usage across the spectrum of disease and determine strategies to counteract issues with nonadherence. CITATION: Hurvitz MS, Bhattacharjee R, Lesser DJ, Skalsky AJ, Orr JE. Determinants of usage and nonadherence to noninvasive ventilation in children and adults with Duchenne muscular dystrophy. J Clin Sleep Med. 2021;17(10):1973-1980.

The accuracy of a portable sleep monitor to diagnose obstructive sleep apnea in adolescent patients.

STUDY OBJECTIVES: The efficacy of portable-monitor (PM) sleep testing in children is not well understood. While most studies have evaluated PM in a lab setting, the utility of PM in the home environment is relatively unknown. We sought to determine whether home PM accurately diagnoses obstructive sleep apnea in adolescents and to assess patient satisfaction with home PM sleep testing. METHODS: We evaluated adolescents (age 12-18 years) with suspected obstructive sleep apnea using a PM device. In addition to in-laboratory polysomnography (PSG), all participants had PM testing performed twice, once in their home and once concurrent to in-laboratory PSG. PM was compared to PSG using 2 primary outcomes: the apnea-hypopnea index and oxygen desaturation index. All participants were approached for interview to evaluate their experience with PM sleep testing. RESULTS: Twenty adolescents participated. Bland-Altman analysis comparing the apnea-hypopnea index and oxygen desaturation index determined by home or in-laboratory PM to in-laboratory PSG revealed mostly agreement; however, some deviations were observed when either parameter was markedly increased. While PM testing tended to underestimate the apnea-hypopnea index, the diagnostic agreement between home PM and PSG was 80% (by the White-Westbrook method). Most preferred PM to PSG and found PM easy to very easy to set up. CONCLUSIONS: In a small cohort of adolescents, our study supports the application of home PM in the diagnosis of suspected obstructive sleep apnea. Until studies implementing PM using larger cohorts become readily available, the findings from this preliminary study could contribute to adolescents receiving sleep apnea therapy more promptly. CLINICAL TRIAL REGISTRATION: Registry: ClinicalTrials.gov; Identifier: NCT03748771. At the time of issue publication, this registration is not publicly available because the trial includes a device that is not approved or cleared for use in pediatric populations. Once the device is FDA cleared, the registration will become public.

Adherence to Sleep Therapies in Children and Adolescents.

Improved recognition of obstructive sleep apnea (OSA) in children has led many to identify effective strategies to treat pediatric OSA. Positive airway pressure (PAP) therapy in children, which has been shown to resolve OSA, is highly contingent on adequate adherence. In pediatrics, adherence is complex, related largely to the influence of age. Consequently, reported adherence rates in children are often lower than adults. Notwithstanding, studies have identified significant risk factors, some modifiable, and several intervention strategies that may improve pediatric adherence. Close follow-up, including use of cloud-based monitoring, of children using PAP therapy may optimize adherence further.

Elevated risk of depression among adolescents presenting with sleep disorders.

STUDY OBJECTIVES: Depression is prevalent among patients with sleep disorders, and studies show associations between suicidal ideation and insufficient sleep. Using retrospective clinic records, we examined positive depression screening rates among adolescent sleep clinic patients relative to other subspecialty clinic patients. We also examined relationships between sleep diagnoses and positive depression screening rate in adolescent sleep clinic patients. METHODS: Data were analyzed from patients ages 12-18 (n = 12,520) who were screened for depression using the Patient Health Questionnaire-2 (PHQ-2). Those who screened positive were administered the PHQ-9. Logistic regression was used to examine effects of age, sex, race, ethnicity, and clinic on likelihood of a positive depression screen. Within sleep clinic patients (n = 308), demographic factors, sleep disorder diagnosis, and body mass index percentile were examined using logistic and linear regression. RESULTS: Among all patients screened, older age and female sex predicted positive depression screens. Sleep clinic patients were more likely to screen positive than patients in 9 other clinics [odds ratios 2.03-6.83]. Results were similar even when the PHQ-9 sleep item was excluded [odds ratios 2.18-6.41]. Within sleep clinic patients, sleep disorder diagnosis (eg, insomnia, obstructive sleep apnea) was predictive of a positive depression screen (χ²(1) = 10.88, P = .004): insomnia patients were most likely to be experiencing depression. CONCLUSIONS: Adolescent sleep clinic patients are at increased risk for depressive symptoms. Among insomnia patients, risk was independent of age, sex, and obesity, suggesting a unique relationship between insomnia and affective distress, as has been found in adults. Assessing adolescents for sleep disorders should be prioritized, given the strong association with depression.

Adherence in children using positive airway pressure therapy: a big-data analysis.

BACKGROUND: Positive airway pressure (PAP) has become a prominent treatment for children with sleep-disordered breathing. However, there are no large-scale studies to clarify whether PAP is well tolerated in children, and which factors are associated with better adherence to PAP therapy. In this study, we aimed to clarify adherence patterns of PAP therapy in a large paediatric population. METHODS: We did a cross-sectional big-data analysis in children from Oct 1, 2014, to Aug 1, 2018, using existing data derived from PAP devices uploaded nightly in the AirView cloud database. The AirView database is a usage tracking system available to all patients who are assigned PAP therapy, which requires consent from the patient or parent or guardian. All patients older than 4 years and younger than 18 years who used continuous or automated PAP devices were evaluated. Only patients living in the USA and enrolled with a single insurance company were included. If patients were participating in an engagement programme, programme onset must have been within 7 days of therapy onset. Our primary outcome was the proportion of patients who used PAP continuously over 90 days. The primary outcome was assessed in all patients who met the age inclusion criterion and had reliable age data available. Data on missing PAP use were imputed as zero, but data on other metrics were not imputed and excluded from analysis. FINDINGS: We used data recorded from Oct 1, 2014, to Aug 1, 2018. Of 40 140 children screened, 36 058 (89·8%) were US residents and 20 553 (90·1%) of them met the eligibility criteria and had accessible data (mean age 13·0 years [SD 3·7]). On the basis of 90 days of monitoring data, 12 699 (61·8%) patients continuously used PAP. Factors significantly associated with adherence included age group, residual apnoea-hypopnoea index, use and onset of patient engagement programmes, PAP pressure, and nightly median PAP mask leak, all over the 90-day study period. INTERPRETATION: To our knowledge, our study represents the largest analysis of children using PAP therapy to date. The findings suggest that adherence to PAP therapy is lower than in previous reports from adults. However, numerous actionable factors were associated with improvements in adherence and should be used strategically in clinical decision making to improve PAP adherence in children. FUNDING: ResMed.

Multidisciplinary clinic for care of children with complex obstructive sleep apnea.

OBJECTIVE: While adenotonsillectomy (AT) remains first line therapy for pediatric obstructive sleep apnea (OSA), management of children who are not candidates for AT or who have residual OSA post AT varies and spans across multiple specialties. We aim to report our experience in managing this population through a multidisciplinary sleep clinic composed of specialists in pediatric dentistry, otolaryngology, plastic surgery, and pulmonary/sleep medicine. STUDY DESIGN: Retrospective chart review. METHOD: The medical records of children attending our complex sleep apnea clinic were reviewed. Data pertaining to demographics, underlying diagnoses, prior evaluation and treatment, recommendations, and initial therapy were collected. RESULT: Two-hundred and thirty patients (mean age 10.7 ± 5.1 years, 62.2% male) were assessed. Underlying conditions included Trisomy 21 (n = 65, 28.2%), other genetic syndromes (n = 37, 16.1%), obesity in an otherwise typically developing child (n = 36, 15.2%), cerebral palsy (n = 27, 11.7%), and craniofacial syndromes (n = 7, 3.0%). Mean obstructive apnea hypopnea index (OAHI) was 14.2 events/hour at first clinic visit, and the majority of children had previously undergone at least one upper airway surgery (n = 168, 73.0%), primarily adenotonsillectomy. Recommended initial treatment plans included positive airway pressure (PAP) therapy (n = 108, 47.0%), surgery (n = 75, 32.6%), allergy management (n = 52, 22.6%), and/or weight loss (n = 34, 14.8%). Patients prescribed PAP therapy with follow up data were found to be adherent 43.9% of the time. Surgical patients with post-operative polysomnography had pre-operative OAHI 15.6 ± SD13.4 decrease to 10.7 ± 14.2 events/hour (p = 0.61). CONCLUSION: Genetic conditions and obesity were the most common underlying diagnoses cared for in the complex sleep apnea clinic. Patients presented with severe OSA, most having already had upper airway surgery. Management plans were frequently adjusted, and we observed improvement in SDB in a sub-segment of patients, suggesting benefit to a coordinated, multi-disciplinary approach.

Findings of routine nocturnal polysomnography in children with Down syndrome: a retrospective cohort study.

STUDY OBJECTIVE: Children with Down syndrome (DS) are at risk for sleep disorders including; obstructive sleep apnea (OSA). Although OSA is diagnosed by polysomnography (PSG), the practicality of PSG in DS is questionable. Further, OSA treatment efficacy in DS is largely unknown given the challenges of PSG. Our aims were to review (i) the feasibility of PSG, and (ii) the efficacy (improvement in obstructive apnea hypopnea index (OAHI)) of OSA treatment using follow-up PSG in DS. METHODS: Retrospective review of patients aged <21 years with DS who underwent PSG from October 2016 to June 2019. Successful PSG was determined using total sleep time (TST). PSG following treatment with adenotonsillectomy (AT) or positive airway pressure (PAP) was evaluated and compared to pre-treatment. RESULTS: Among 248 patients with DS, only 11(4.4%) had unsuccessful PSG (TST<1h). Of the 237 successful studies (age: 7.9 ± 0.3y), average TST and sleep efficiency was 5.6 ± 0.1h and 79.5 ± 1.3%. 41 had post-AT PSG and 11(27%) achieved OSA cure (OAHI<2) with all demonstrating improved SE (p = 0.01) and OAHI (p = 0.0003). Multivariate analysis revealed only age was predictive (p = 0.003) of residual OSA post-AT. Of 24 children who underwent PAP titration, 20(83%) tolerated titration with improved OAHI (p = 0.01), however, no significant improvements in SE were observed. CONCLUSIONS: In a large cohort of DS children, PSG was well tolerated. Following AT or PAP therapy, post treatment PSG confirmed efficacy, although residual OSA was identified. PSG is thus both feasible and useful in identifying OSA, OSA treatment response and should guide in decision making in children with DS.