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Repetitive surges in heart rate (HR) at respiratory event termination underpin the altered autonomic HR control associated with sleep disordered breathing (SDB). As children born preterm are at greater risk of adverse cardiovascular outcomes, we aimed to determine whether the HR response to obstructive respiratory events was elevated compared to term-born children.Fifty children (3-12 years) born preterm, were matched for SDB severity, age and gender with term born children. Multilevel modelling determined the effect of preterm birth and arousal on HR changes between a 10s baseline to the latter half of respiratory events and 15s post event during NREM and REM.1203 events were analysed (NREM: term 380; preterm 383; REM: term 207; preterm 233). During NREM fewer events terminated in arousal in the preterm compared with term group (preterm 68%; term 84%; χThe greater magnitude of surges in HR following respiratory events terminating with arousal in preterm born children, although small, occur repeatedly throughout the night and may contribute to adverse cardiovascular outcomes, although further studies are required.

We aimed to investigate the frequency and severity of periodic breathing (PB) in clinically stable very preterm infants and identify infant and maternal factors associated with increased time spent and severity of PB in these infants.Thirty-eight infants (28-32 weeks gestational age) who were ≥3 days off noninvasive respiratory support, were studied for 2-3 h with a daytime sleep study at 31-36 weeks postmenstrual age. Percent total sleep time spent in PB (%TSTPB) and time spent with SpOThe majority of infants (92%) had at least one episode of PB and infants spent a median 9.1 [interquartile range: 1.2, 15.5] %TSTPB. 80%, 37%, and 37% of infants experienced SpOThe majority of very preterm infants exhibited PB when they were off respiratory support and considered clinically stable. The time spent in PB was very variable between infants and was associated with significant hypoxia in some infants. Fewer days spent on respiratory support was associated with both increased frequency and severity of PB. However, the potential contribution of PB to neurocognitive outcomes remains uncertain and warrants further investigations.

Objective Hospital utilisation research is important in pursuing cost-saving healthcare models. Tonsillectomy is one of the most common paediatric surgeries and the most frequent reason for paediatric hospital readmission. This study aimed to report the government-funded costs of paediatric tonsillectomy in the state of Victoria, Australia, extrapolate costs across Australia, and identify the cost determinants. Methods A population-based longitudinal study was conducted with a bottom-up costing approach using linked datasets containing all paediatric tonsillectomy and tonsillectomy with adenoidectomy surgeries performed in the state of Victoria between 2010 and 2015. Results The total average annual cost of tonsillectomy hospitalisation in Victoria was A$21 937 155 with a median admission cost of A$2224 (interquartile range (IQR) 1826–2560). Inflation-adjusted annual tonsillectomy costs increased during 2010–2015 (P < 0.001), not explained by the rising number of surgeries. Hospital readmissions resulted in a total average annual cost of A$1 427 716, with each readmission costing approximately A$2411 (IQR 1936–2732). The most common reason for readmission was haemorrhage, which was associated with the highest total cost. The estimated total annual expenditure of both tonsillectomy and resulting readmissions across Australia was A$126 705 989. Surgical cost in the upper quartile was associated with younger age, male sex, lower socioeconomic status, surgery for reasons other than infection alone, overnight vs day case surgery, public hospitals and metropolitan hospitals. Surgery for obstructed breathing during sleep had the strongest association to high surgical cost. Conclusions This study highlights the cost of paediatric tonsillectomy and associated hospital readmissions. The study findings will inform healthcare reform and serve as a basis for strategies to optimise patient outcomes while reducing both postoperative complications and costs.

Aim: In children with Prader-Willi syndrome (PWS), growth hormone (GH) improves height and body composition; however, may be associated with worsening sleep-disordered breathing (SDB). Some studies have reported less SDB after GH initiation, but follow-up with polysomnography is still advised in most clinical guidelines. Methods: This retrospective, multicentre study, included children with PWS treated with GH at seven PWS treatment centres in Australia over the last 18 years. A paired analysis comparing polysomnographic measures of central and obstructive SDB in the same child, before and after GH initiation was performed with Wilcoxon signed-rank test. The proportion of children who developed moderate/severe obstructive sleep apnoea (OSA) was calculated with their binomial confidence intervals. Results: We included 112 patients with available paired data. The median age at start of GH was 1.9 years (range 0.1–13.5 years). Median obstructive apnoea hypopnoea index (AHI) at baseline was 0.43/h (range 0–32.9); 35% had an obstructive AHI above 1.0/h. Follow-up polysomnography within 2 years after the start of GH was available in 94 children who did not receive OSA treatment. After GH initiation, there was no change in central AHI. The median obstructive AHI did not increase significantly (P = 0.13), but 12 children (13%, CI95% 7–21%) developed moderate/severe OSA, with clinical management implications. Conclusions: Our findings of a worsening of OSA severity in 13% of children with PWS support current advice to perform polysomnography after GH initiation. Early identification of worsening OSA may prevent severe sequelae in a subgroup of children.

Prader-Willi Syndrome (PWS) is a rare genetic disorder associated with emotional/behavioral disturbances. These difficulties are well documented in the literature, but the positive attributes of these individuals are not described. Taking a strengths-based approach, the aim of this study was to describe the emotional/behavioral strengths and difficulties in children and young people with PWS from their parent caregivers' perspectives. Parent caregivers of 52 individuals with PWS aged 4-24 years (median = 12.1 years; including 22 males) completed the parent form of the Developmental Behavior Checklist (DBC-P), including its original two open-ended questions regarding positive traits. Prevalences of emotional/behavioral disturbances were comparable to those reported in previous literature: common behaviors of concern across studies being skin-picking (75%), impulsivity (69%), poor sense of danger (67%), lying (67%), and tantrums (54%). Total DBC-P scores showed that just over half (n = 28, 54%) had scores indicative of clinically significant behavior problems. However, thematic analysis of caregivers' written comments regarding their children's strengths resolved into three themes: warmth (94%), persistence (41%), and skills (41%). Warmth encompassed friendliness, happiness, and empathy. A strength-based approach to behavioral difficulties in PWS provides a more balanced view of the children and a more holistic foundation for interventions.

This paper investigated cortical thickness and volumetric changes in children to better understand the impact of obstructive sleep disordered breathing (SDB) on the neurodevelopment of specific regions of the brain. We also aimed to investigate how these changes were related to the behavioral, cognitive and autonomic deficits observed in the condition. Neuroimaging, behavioral and sleep data were obtained from 30 children (15 non snoring controls, 15 referred for assessment of SDB) aged 7 to 17 years. Gyral-based regions of interest were identified using the Desikan-Killiany Atlas. Student’s T-tests were used to compare regions of interest between the controls and SDB groups. We found cortical thickness was significantly greater in the right caudal anterior cingulate and right cuneus regions and volumetric increases in the left caudal middle frontal, bilateral rostral anterior cingulate, left, right and bilateral caudate brain regions in children with SDB compared to controls. Neither cortical thickness nor volumetric changes were associated with behavioral or cognitive measures. The findings of this study indicate disruptions to neural developmental processes occurring in structural regions of the brain; however, these changes appear unrelated to behavioral or cognitive outcomes.

Improving efficiency of genetic counseling can allow genetic counselors to see more patients, increasing access to this valuable service. However, the patient experience should be carefully considered as changes are made, so that quality is not sacrificed. The primary outcome of this study was time in session with a board-certified genetic counselor at baseline (T0), after the addition of a genetic counseling assistant (GCA) (T1). The secondary outcome was the patient experience, which was collected from an electronic survey sent three days after the genetic counseling session. A total of 689 appointments were evaluated over 12 months; 291 in T0 by two genetic counselors (Jan-June 2019), 398 in T1 by two genetic counselors (August 2019-Jan 2020). The overall genetic counseling median appointment time decreased by 10 min in T1 (p 0.001), and the median amount of time spent on post-session activities by the two genetic counselors decreased by 15 min (p 0.001). There was an increase in the average number of patients seen per FTE per month from 24.3 in T0 to 33.2 in T1. There was no difference in overall patient experience from T0 to T1 (p = 0.3). There was high patient satisfaction, including with the amount of time spent in a session during both time periods (p = 0.63). This study found decreased appointment time with the addition of a GCA in a single clinic without impacting patient experience.

The goal of this position paper on ventilatory support at home for children is to provide expert consensus from Australia and New Zealand on optimal care for children requiring ventilatory support at home, both non-invasive and invasive. It was compiled by members of the Thoracic Society of Australia and New Zealand (TSANZ) and the Australasian Sleep Association (ASA). This document provides recommendations to support the development of improved services for Australian and New Zealand children who require long-term ventilatory support. Issues relevant to providers of equipment and areas of research need are highlighted.

Objectives Adherence to Continuous Positive Airway Pressure (CPAP) in children can be challenging. Advancements in CPAP technology have potential to influence adherence. The aim of this study was to compare adherence rates of children with obstructive sleep apnoea (OSA) initiated on autotitrating CPAP (APAP) with remote modem monitoring compared to a cohort started on fixed pressure CPAP alone. Methods Children aged over 3 years starting APAP at our centre between February 2017 and February 2020 were included. Therapy data was obtained for the initial 90 days. Data was compared to a cohort of children started on CPAP between July 2004 and September 2008. Results A total of 61 patients with a median age of 14.3 years formed the APAP group, and were significantly older than the CPAP group who had a median age of 8.6 years (p = 0.02). Co-morbid conditions were present in 51% compared with 69% in the earlier cohort (p = 0.11). No significant difference was found in any adherence parameters between the groups. The value closest to achieving a significant difference was hours used per day used, with an median of 5.2 h in the CPAP group compared with 7.0 h in the APAP group (p = 0.07). Two-way ANOVA including age group (above or below 13 years) showed that both age group and treatment group (CPAP vs APAP) were significantly associated with a difference in adherence (F = 4.41, p = 0.006), with mean hours used on days used being highest in the APAP group aged under 13 years. However no significant interaction was found between age and treatment group. Conclusion Despite the convenience for patients with outpatient initiation and ability to achieve optimal pressures quickly and remotely, our results show no improvement in adherence using APAP with remote monitoring, with the possible exception of children aged under 13 years. A large randomized controlled trial would be required to confirm these findings.

ObjectiveDiagnosis of obstructive sleep apnoea (OSA) is made on overnight polysomnography (PSG). Given the widespread availability of smartphone video technology, we aimed to develop and test a standardised scoring system for smartphone videos and compare these scores to PSG results.MethodsChildren aged 1–16 years undergoing PSG for suspected OSA were included. Parents were asked to take 1–2 min videos of the breathing they were concerned about. Videos were scored using a newly developed and tested tool on five components: inspiratory obstructive noises (1–4), presence of obstructive events (0–1), increased work of breathing (0–1), mouth breathing (0–1) and neck extension (0–1). Video scores and the Obstructive Apnoea Hypopnoea Index (OAHI) were compared using Spearman correlation. Sensitivity, specificity, positive predictive value and negative predictive value were calculated for different cut-off scores to achieve the best results.ResultsVideos from 43 children (28 men (65.1%), median age 5.7 years (range 2.6–14.0 years), median OAHI 3.8/hour (range 0–82 events/hour) were included. Nine children (20.9%) had a video score of 5 events/hour) .ConclusionWe have developed and validated a simple clinical tool (the Monash Obstructive Sleep Apnoea Video Score) to quantify abnormalities in breathing seen on short video recordings made on a smartphone. A low score rules out moderate–severe OSA and may be valuable in the triage of children with symptoms of OSA.

Obstructive sleep disordered breathing (SDB), has adverse neurocognitive and behavioral sequelae in children, despite conventional measures of sleep disruption being unaffected. There is growing evidence that sleep spindles may serve as a more sensitive marker of sleep quality. We investigated the relationship between sleep spindles and sleep fragmentation and neurocognition across the spectrum of SDB severity in children.Children 3-12 years old referred for clinical assessment of SDB and age matched control children from the community were recruited and underwent polysomnography. Sleep spindles were identified manually during N2 and N3 sleep. Spindle activity was characterised as spindle number, density (number of spindles/h) and intensity (spindle density x average spindle duration). Children completed a battery of tests assessing global intellectual ability, language, attention, visuospatial ability, sensorimotor skills, adaptive behaviors and skills and problem behaviors and emotional difficulties.Children were grouped into control, Primary Snoring, Mild OSA and Moderate/severe OSA, N = 10/group. All measures of spindle activity were lower in the SDB groups compared to the Control children and this reached statistical significance for Mild OSA (p 0.05 for all). Higher spindle indices were associated with better performance on executive function and visual ability assessments but poorer performance on auditory attention and communication skills. Higher spindle indices were associated with better behavior.The reduced spindle activity observed in the children with SDB, particularly Mild OSA, indicates that sleep micro-architecture is disrupted and that this disruption may underpin the negative effects of SDB on attention, learning and memory.

Prader-Willi syndrome (PWS) is a rare genetic disorder characterised by neurodevelopmental delays, hyperphagia, difficulties with social communication and challenging behaviours. Individuals require intensive supervision from caregivers which may negatively affect caregiver quality of life. This study used data collected in the Australasian PWS Registry (n = 50, mean age 11.2 years) to evaluate associations between child behaviours and caregiver mental well-being. Symptoms of sleep-related breathing disorder, child depression and social difficulties were associated with poorer caregiver mental and physical well-being. Growth hormone therapy use was associated with better caregiver mental and physical well-being. Optimising management of problematic behaviours and sleep disturbances have the potential to support caregivers who are the most vital network of support for individuals affected by PWS.

We aimed to investigate the amount of time spent in periodic breathing (PB) and its consequences in preterm infants before and after hospital discharge.Preterm infants born between 28-32 weeks gestational age were studied during daytime sleep in the supine position at 32-36 weeks post menstrual age (PMA), 36-40 weeks PMA, 3 months and 6 months corrected age (CA). The percentage of total sleep time (TST) spent in PB (%TSTPB) was calculated and infants were grouped into below and above the median (8.5% TSTPB) at 32-36 weeks and compared with 36-40 weeks, 3 and 6 months.%TSTPB was not different between 32-36 weeks PMA (8.5%; 1.5, 15.0) (median, IQR) and 36-40 weeks PMA (6.6%, 0.9, 15.1), but decreased at 3 (0.4%; 0.0, 2.0) and 6 months CA (0%, 0.0, 1.1). Infants who spent above the median %TSTPB at 32-36 weeks PMA spent more %TSTPB at 36-40 weeks PMA (18.1%, 7.7, 23.9 vs 2.1%, 0.6, 6.4) and 6 months CA (0.9%, 0.0, 3.3) vs 0.0% (0.0, 0.0).Percentage sleep time spent in PB did not decrease as preterm infants approached term CA, when they were to be discharged home. High amounts of PB at 32-36 weeks PMA was associated with high amounts of PB at term CA (36-40 weeks PMA), and persistence of PB at 6 months CA.

STUDY OBJECTIVES: Studies of sleep-disordered breathing (SDB) in children with Prader-Willi syndrome (PWS) have focused on early childhood and growth hormone (GH)–naïve children, but little is known about older children, including those on long-term GH therapy. This study aimed to describe the nature and prevalence of SDB in school-aged children with PWS in the growth hormone era. METHODS: This retrospective single-center chart review included children aged 6–18 years with PWS who had overnight polysomnography not involving respiratory support over 5 years (2012–2017). The main outcome measures were the presence of obstructive sleep apnea, central sleep apnea, or hypoventilation defined by an elevated transcutaneous partial pressure of carbon dioxide (TcPCO(2)) as per standard pediatric criteria. RESULTS: Seventeen children (8 males; median age 11.6 years, range 6.6–16.1 years) were included. Fifteen demonstrated SDB of different types: central sleep apnea (18%), obstructive sleep apnea (24%), both obstructive and central sleep apnea (29%), or hypoventilation without obstructive or central sleep apnea (18%). Twelve (71%) children had evidence of hypoventilation. Those with hypoventilation had a higher central apnea-hypopnea index but no difference in the obstructive apnea-hypopnea index, age, sex, growth parameters, or the presence of scoliosis or sleep-related symptoms compared with those without hypoventilation. CONCLUSIONS: Sleep-related hypoventilation is common in school-aged children with PWS. The presence of central sleep apnea, including the quantification of central hypopneas, but not obstructive sleep apnea or clinical factors, predicted the presence of hypoventilation. Long-term polysomnography surveillance in children with PWS should include identification of central hypopneas and measurement of continuous pCO(2). CITATION: Schaefer J, Davey MJ, Nixon GM. Sleep-disordered breathing in school-aged children with Prader-Willi syndrome. J Clin Sleep Med. 2022;18(4):1055–1061.

BACKGROUND Sleep disordered breathing (SDB) in typically developing (TD) children is associated with adverse cardiovascular effects. As children with Down syndrome (DS) are at increased risk for SDB, we aimed to compare the cardiovascular effects of SDB in children with DS to those of TD children with and without SDB. METHODS Forty-four children with DS (3-19 years) were age and sex matched with 44 TD children without SDB (TD-) and with 44 TD children with matched severity of SDB (TD+). Power spectral density was calculated from ECG recordings, for low frequency (LF), high frequency (HF), total power and the LF/HF ratio. RESULTS Children with DS had lower HF power, and higher LF/HF during sleep and when awake. There were no differences between groups for LF power. SpO2 nadir, average SpO2 drop and SpO2 > 4% drop were larger in the DS group compared to the TD+ group (p

The concept of personalised medicine is likely to revolutionise the treatment of adult obstructive sleep apnoea as a result of recent advances in the understanding of disease heterogeneity by identifying clinical phenotypes, pathophysiological endotypes, biomarkers and treatable traits. Children with the condition show a similar level of heterogeneity and paediatric obstructive sleep apnoea would also benefit from a more targeted approach to diagnosis and management. This review aims to summarise the adult literature on the phenotypes and endotypes of obstructive sleep apnoea and assess whether a similar approach may also be suitable to guide the development of new diagnostic and management approaches for paediatric obstructive sleep apnoea.

These two companion articles were originally published in June 1960. They are republished here as part of a special issue commemorating the 75th year of the Bulletin.

Chronic neonatal lung disease (CNLD) is defined as continued need for any form of respiratory support (supplemental oxygen and/or assisted ventilation) beyond 36 weeks PMA. Low‐flow supplemental oxygen facilitates discharge from hospital of infants with CNLD who are hypoxic in air and is widely used despite lack of evidence on the most appropriate minimum mean target oxygen saturations. Furthermore, there are minimal data to guide the home monitoring, titration or weaning of supplemental oxygen in these infants. The purpose of this position statement is to provide a guide for the respiratory management of infants with CNLD, with special emphasis on role and logistics of supplemental oxygen therapy beyond the NICU stay. Reflecting a variety of clinical practices and infant comorbidities (presence of pulmonary hypertension, retinopathy of prematurity and adequacy of growth), it is recommended that the minimum mean target range for SpO2 during overnight oximetry to be 93–95% with less than 5% of total recording time to be below 90% SpO2. Safety of short‐term disconnection from supplemental oxygen should be assessed before discharge, with majority of infants with CNLD not ready for discharge until supplemental oxygen requirement is ≤0.5 L/min. Sleep‐time assessment of oxygenation with continuous overnight oximetry is recommended when weaning supplemental oxygen. Palivizumab is considered safe and effective for the reduction of hospital admissions with RSV infection in this group. This statement would be useful for paediatricians, neonatologists, respiratory and sleep physicians and general practitioners managing children with CNLD., Watch the video abstract

This article describes the Purdue University Libraries mandatory co-mentoring program. Surveys with all Purdue University Libraries faculty librarians and select follow-up interviews were conducted. This program supports all faculty librarians and differs from other mentoring programs since it is team-based, includes the supervisor, and advises on promotion. These Faculty Review Committees integrate performance reviews with progress toward promotion and have the responsibility to prepare written annual reviews and evaluations, and represent the candidate at the promotion review. The program is considered very effective at Purdue University Libraries and a unique model. This program is also compared to four other ARL co-mentoring programs using a survey and follow-up interviews.

In adults there is a distinct gender difference in the prevalence and severity of sleep disordered breathing (SDB), however there have been limited studies examining the effects of gender in children with SDB. We aimed to compare the effects of gender on severity of SDB, blood pressure, sleep and respiratory characteristics, quality of life, behavior and executive function.We included 533 children aged 3-18 years, who underwent standard pediatric overnight polysomnography (PSG) between 2004 and 2016. Blood pressure was recorded prior to each study. Quality of life, behavior and executive function were assessed with parental questionnaires. Children were grouped by gender and SDB severity based on their obstructive apnea hypopnea index (OAHI) into non-snoring controls, Primary Snoring (PS) (OAHI≤1 event/h), Mild obstructive sleep apnea (OSA) (OAHI1-≤5 events/h) and moderate/severe (MS) OSA (OAHI5 events/h) and data compared with 2-way ANOVA.A total of 298 boys and 235 girls were studied. There were no differences in age, BMI z-score, SDB severity sleep characteristics or blood pressure between genders. Diastolic blood pressure was elevated in females with MS OSA compared to males (P 0.05). Quality of life, behavior and executive function scores were all elevated in the SDB groups compared to controls. Females with MS OSA exhibited more internalizing behavioral problems compared to males (59.2 ± 2.4 vs. 51.4 ± 2.3, P 0.05).In contrast to studies in adults, we identified no gender differences in the severity or consequences of SDB in children, other than females with moderate-severe OSA exhibiting more internalizing problems and higher diastolic blood pressure.

The purpose of this study was to examine the effects of a single acute dose of yohimbine hydrochloride on repeated anaerobic sprint ability. Physically active females (n = 18) completed two separate repeated supramaximal sprint trials each with a different single-dose treatment: placebo (PL; gluten-free corn starch) or yohimbine hydrochloride (YHM; 2.5 mg). For each trial, participants consumed their respective treatment 20 min before exercise. Following a warm-up, participants completed 3 × 15 s Wingate anaerobic tests (WAnTs) separated by 2 min of active recovery. A capillary blood sample was obtained pre- and immediately post-exercise to measure blood concentrations of lactate (LA), epinephrine (EPI), and norepinephrine (NE). Heart rate (HR) and rate of perceived exertion (RPE) were measured following each WAnT. Findings showed that mean power (p < 0.001; η2 = 0.024), total work (p < 0.001; η2 = 0.061), and HR (p < 0.001; η2 = 0.046), were significantly higher with YHM supplementation versus PL. Fatigue index (p < 0.001; η2 = 0.054) and post-exercise LA (p < 0.001; d = 1.26) were significantly lower with YHM compared to PL. YHM resulted in significantly higher EPI concentrations versus PL (p < 0.001; η2 = 0.225) pre- and post-exercise while NE only increased as a function of time (p < 0.001; η2 = 0.227) and was unaffected by treatment. While RPE increased after each WAnT, no differences between treatments were observed (p = 0.539; η2 < 0.001). Together, these results suggest that acute YHM ingestion imparts ergogenic benefits which may be mediated by lower blood LA and fatigue concomitantly occurring with blood EPI increases. Thus, YHM may improve sprint performance although more mechanistic study is warranted to accentuate underlying processes mediating performance enhancement.

Individuals with Prader-Willi syndrome (PWS) often have excessive daytime sleepiness and emotional/behavioral disturbances. The objective of this study was to examine whether daytime sleepiness was associated with these emotional/behavioral problems, independent of nighttime sleep-disordered breathing, or the duration of sleep. Caregivers of individuals with PWS (aged 3 to 25 years) completed the Pediatric Sleep Questionnaire (PSQ), Epworth Sleepiness Scale for Children and Adolescents (ESS-CHAD), and the parent version of the Developmental Behavior Checklist (DBC-P). Sleep adequacy was adjusted for age by computing sleep duration against age-specific recommendations. The associations between ESS-CHAD and the total DBC and its subscale scores were evaluated by linear regression, adjusted for sleep-related breathing difficulties, sleep adequacy, and body mass index (BMI). There were 54 responses for individuals with PWS (including 22 males) aged 4.4–24.0 (mean 12.5) years. Daytime sleepiness predicted a substantial proportion of the variance in total DBC-P scores in the unadjusted model (28%; β = 0.028; p β = 0.023; p = 0.007). This relationship was not moderated by BMI Z-scores, but the relationship was more prominent for children younger than 12 years than for children older than 12 years.Conclusions: These findings provide preliminary novel evidence that daytime sleepiness may drive the expression of emotional/behavioral disturbances, and should be explored as a potential modifiable risk factor for these disturbances in PWS, particularly pre-adolescent children. What is Known:• Individuals with Prader-Willi syndrome (PWS) commonly experience excessive daytime sleepiness and exhibit emotional/behavioral disturbances.• In the typically developing population, sleepiness is associated with emotional/behavioral disturbances, independently of sleep-disordered breathing.. What is New:• This study found evidence for a direct link between daytime sleepiness and emotional/behavioral disturbances, independent of sleep-related breathing difficulties, sleep adequacy, and body mass index.• Excessive daytime sleepiness may be a modifiable risk factor for emotional/behavioral disturbances in PWS.