Prevalence and predictors of obstructive sleep apnea in young children with Down syndrome

BackgroundChildren with Down syndrome (DS) are vulnerable to obstructive sleep apnoea (OSA) because of their unique craniofacial anatomy and hypotonia. Understanding the predictors of OSA in DS may enable targeted screening.MethodsChildren with DS (n = 202) aged from six months to below six years (110 boys) were recruited from three UK children's hospitals. The clinical assessment included height, weight and tonsillar size. The parents either set up cardiorespiratory polygraphy at home or chose laboratory studies. Studies with less than four hours of interpretable data were repeated where possible. American Academy of Sleep Medicine (AASM) 2012 scoring criteria were used to derive an obstructive apnoea/hypopnoea index (OAHI). Predictors of moderate to severe OSA were examined.ResultsIn total, 188/202 (93%) participants were successfully studied. Of these, 169 studies were completed at home and 19 in a sleep laboratory. Moderate to severe OSA, defined by an OAHI of &gt;5/h, was found in 14% and mild to moderate OSA (1/h≥OAHI &lt;5/h) was found in 59% of the children. Male gender and habitual snoring predicted OSA but did not have independent predictive power in the presence of the other factors. Age in months, body mass index (BMI) centile and tonsillar size did not predict OSA.ConclusionsModerate to severe OSA is common in very young children with DS. Examination of tonsillar size did not predict OSA severity. Population-based screening for OSA is recommended in these children, and domiciliary cardiorespiratory polygraphy is an acceptable screening approach. Further research is required to understand the natural history, associated morbidity, optimal screening methodology and treatment modality for OSA in these children.</p

Epilepsy-specific patient-reported outcome measures of children's health-related quality of life: A systematic review of measurement properties.

This is the final version. Available from the publisher via the DOI in this record.OBJECTIVE: To identify and appraise published evidence of the measurement properties for epilepsy-specific patient-reported outcome measures (PROMs) of children's health-related quality of life (HRQoL). METHODS: We searched multiple databases for studies evaluating the measurement properties of English-language epilepsy-specific PROMs of children's HRQoL. We assessed the methodological quality using the COnsensus-based Standards for the selection of health Measurement INstruments (COSMIN) guidance. We extracted data about the content validity, construct validity, internal consistency, test-retest reliability, proxy reliability, responsiveness, and precision, and assessed the measurement properties with reference to standardized criteria. RESULTS: We identified 27 papers that evaluated 11 PROMs. Methodological quality was variable. Construct validity, test-retest reliability, and internal consistency were more commonly assessed. Quality of Life in Childhood Epilepsy (QoLCE) questionnaires are parent-reported and evaluated more than other PROMs; QoLCE-55 has good and replicated evidence for structural and construct validity and internal consistency. Health-Related Quality of Life Measure for Children with Epilepsy (CHEQoL) has both child and parent-reported versions and good evidence of content, structural, and construct validity. SIGNIFICANCE: This review identified two leading candidate epilepsy-specific PROMs for measuring health-related quality of life in children. Establishing evidence of the responsiveness of PROMs is a priority to help the interpretation of meaningful change scores.National Institute for Health Research (NIHR

Associations between the LEP -2548G/A Promoter and Baseline Weight and between LEPR Gln223Arg and Lys656Asn Variants and Change in BMI z Scores in Arab Children and Adolescents Treated with Risperidone

Data on baseline (antipsychotic-naïve) age, weight, and height and change in these over three subsequent follow up time points up to 313.6 days (CI 303.5-323.7), were collected from 181 risperidone-treated children and adolescents (mean age 12.58 years, SD 4.99, range 2.17-17.7) attending a pediatric neurology clinic in Saudi Arabia. Owing to differences in genotypic distributions in subsamples, results are reported from the white Arabs (N=144). Age and gender-normed BMI-standardised z scores (BMI z) were calculated (lmsgrowth program). Linear regression was performed for baseline weight and BMI z, while change in BMI z was assessed using random effects ordered logistic regression. The following SNPs were analyzed: rs7799039 in the LEP promoter, rs1805094 (previously rs8179183), rs1137100 and rs1137101 in the LEPR, and rs1414334 in HTR2C. We found a nominally significant association between rs7799309 and baseline weight, adjusting for height, age, gender and diagnosis (A/G, P=0.035, β=-3.62, compared to G/G). rs1137101 (G/G, P=0.018, OR=4.13 compared to A/A) and rs1805094 C-allele carriers (P=0.019, OR=0.51) showed nominally significant associations with change in BMI z categories. Our data support and replicate previous relevant associations for these variants including with weight gain on risperidone, whilst being the first to report such associations in those of Arab ethnicity

Core Health Outcomes in Childhood Epilepsy (CHOICE): Development of a core outcome set using systematic review methods and a Delphi survey consensus

OBJECTIVE: Establishing a core set of outcomes to be evaluated and reported in intervention trials aims to improve the usefulness of health research. There is no established core outcome set (COS) for childhood epilepsies. The aim of this study was to select a COS to be used in evaluative research of interventions for children with rolandic epilepsy (RE).METHODS: We followed guidance from the COMET (Core Outcome Measures in Effectiveness Trials) Initiative. First, we identified outcomes that had been measured in research through a systematic review. Second, young people with RE, parents, and professionals were invited to take part in a Delphi survey in which participants rated the importance of candidate outcomes. Last, a face-to-face meeting was convened to seek consensus on which outcomes were critical to include and to ratify the final COS.RESULTS: From 37 eligible papers in the review, we identified and included 48 candidate outcomes in the survey. We sent invitations to 165 people registered to take part in the survey; of these, 102 (62%) completed Round 1, and 80 (78%) completed Round 2 (three young people, 16 parents, 61 professionals). In Round 2 we included four additional outcomes suggested by participants in Round 1. The consensus meeting included two young people, four parents, and nine professionals who were eligible to vote and ratified the COS as 39 outcomes across 10 domains.SIGNIFICANCE: Our methodology was a proportionate and pragmatic approach toward producing a COS for evaluating research on interventions aiming to improve the health of children with RE.</p

Changing Agendas on Sleep, Treatment and Learning in Epilepsy (CASTLE) Sleep-E: A protocol for a randomised controlled trial comparing an online behavioural sleep intervention with standard care in children with Rolandic epilepsy

This is the final version. Available from BMJ Publishing Group via the DOI in this record. INTRODUCTION: Sleep and epilepsy have an established bidirectional relationship yet only one randomised controlled clinical trial has assessed the effectiveness of behavioural sleep interventions for children with epilepsy. The intervention was successful, but was delivered via face-to-face educational sessions with parents, which are costly and non-scalable to population level. The Changing Agendas on Sleep, Treatment and Learning in Epilepsy (CASTLE) Sleep-E trial addresses this problem by comparing clinical and cost-effectiveness in children with Rolandic epilepsy between standard care (SC) and SC augmented with a novel, tailored parent-led CASTLE Online Sleep Intervention (COSI) that incorporates evidence-based behavioural components. METHODS AND ANALYSES: CASTLE Sleep-E is a UK-based, multicentre, open-label, active concurrent control, randomised, parallel-group, pragmatic superiority trial. A total of 110 children with Rolandic epilepsy will be recruited in outpatient clinics and allocated 1:1 to SC or SC augmented with COSI (SC+COSI). Primary clinical outcome is parent-reported sleep problem score (Children's Sleep Habits Questionnaire). Primary health economic outcome is the incremental cost-effectiveness ratio (National Health Service and Personal Social Services perspective, Child Health Utility 9D Instrument). Parents and children (≥7 years) can opt into qualitative interviews and activities to share their experiences and perceptions of trial participation and managing sleep with Rolandic epilepsy. ETHICS AND DISSEMINATION: The CASTLE Sleep-E protocol was approved by the Health Research Authority East Midlands (HRA)-Nottingham 1 Research Ethics Committee (reference: 21/EM/0205). Trial results will be disseminated to scientific audiences, families, professional groups, managers, commissioners and policymakers. Pseudo-anonymised individual patient data will be made available after dissemination on reasonable request. TRIAL REGISTRATION NUMBER: ISRCTN13202325.National Institute for Health and Care Research (NIHR)National Health and Medical Research Council (NHMRC, Australia)Victorian Government’s Operational Infrastructure Support Progra

UK research priority setting for childhood neurological conditions

\ua9 2024 The Author(s). Developmental Medicine &amp; Child Neurology published by John Wiley &amp; Sons Ltd on behalf of Mac Keith Press. Aim: To identify research priorities regarding the effectiveness of interventions for children and young people (CYP) with childhood neurological conditions (CNCs). These include common conditions such as epilepsies and cerebral palsy, as well as many rare conditions. Method: The National Institute for Health and Care Research (NIHR) and the James Lind Alliance (JLA) champion and facilitate priority setting partnerships (PSPs) between patients, caregivers, and clinicians (stakeholders) to identify the most important unanswered questions for research (uncertainties). A NIHR–JLA and British Paediatric Neurology Association collaboration used the JLA PSP methodology. This consisted of two surveys to stakeholders: survey 1 (to identify uncertainties) and survey 2 (a prioritization survey). The final top 10 priorities were agreed by consensus in a stakeholder workshop. Results: One hundred and thirty-two charities and partner organizations were invited to participate. In survey 1, 701 participants (70% non-clinicians, including CYP and parent and caregivers) submitted 1800 uncertainties from which 44 uncertainties were identified for prioritization in survey 2; from these, 1451 participants (83% non-clinicians) selected their top 10 priorities. An unweighted amalgamated score across participant roles was used to select 26. In the final workshop, 14 health care professionals, 11 parent and caregivers, and two CYP ranked the 26 questions to finalize the top 10 priorities. Ten top priority questions were identified regarding interventions to treat CYP with CNCs and their associated comorbidities, for example, sleep, emotional well-being, and distressing symptoms. Interpretation: The results of this study will inform research into the effectiveness of interventions for children with neurological conditions

Pharmacological management of narcolepsy in children and adolescents

Paroxysmal Cerebral Disorder

International consensus on sleep problems in pediatric palliative care: Paving the way

OBJECTIVE: Sleep problems constitute a common and heterogeneous complaint in pediatric palliative care (PPC), where they often contribute to disease morbidity and cause additional distress to children and adolescents and their families already facing the burden of life-threatening and life-limiting conditions. Despite the significant impact of sleep problems, clinical evidence is lacking. The application of general pediatric sleep recommendations appears insufficient to address the unique challenges of the PPC dimension in terms of disease variability, duration, comorbidities, complexity of needs, and particular features of sleep problems related to hospice care. Therefore, we initiated an international project aimed at establishing a multidisciplinary consensus. METHODS: A two-round Delphi approach was adopted to develop recommendations in the areas of Definition, Assessment/Monitoring, and Treatment. After selecting a panel of 72 worldwide experts, consensus (defined as ≥75% agreement) was reached through an online survey. RESULTS: At the end of the two voting sessions, we obtained 53 consensus recommendations based on expert opinion on sleep problems in PPC. CONCLUSIONS: This study addresses the need to personalize sleep medicine's approach to the palliative care setting and its peculiarities. It provides the first international consensus on sleep problems in PPC and highlight the urgent need for global guidance to improve sleep-related distress in this vulnerable population and their caregivers. Our findings represent a crucial milestone that will hopefully enable the development of guidelines in the near future