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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.

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., Author(s): Jessica Mackay [sup.1] [sup.2] [sup.31] , Gillian M. Nixon [sup.3] [sup.4] , Antony R. Lafferty [sup.5] [sup.6] , Geoff Ambler [sup.7] [sup.8] , Nitin Kapur [sup.9] [sup.10] , Philip [...]

Study objectivesChildren with sleep-disordered breathing (SDB) exhibit behavioral, cognitive, and autonomic deficits, suggestive of neural injury. We assessed whether the tissue alterations resulted from acute or chronic processes, and whether alterations correlated with disease severity.MethodsBrain tissue integrity was examined with mean diffusivity (MD) (3.0 T scanner) in 20 nonsnoring controls (mean age ± SEM, 12.2 ± 0.6 years; 10 males) and 18 children with SDB (12.3 ± 0.7 years; 11 males). Sleep, cognitive, and behavioral measures were compared between groups following overnight polysomnography using Student's t tests. Whole-brain MD maps were realigned and averaged, normalized, smoothed, and compared between groups using ANCOVA (covariates: age, gender, and socioeconomic status). Partial correlations were calculated between whole-brain smoothed MD maps and obstructive apnea-hypopnea indices (OAHIs).ResultsAge, gender, and sleep variables did not differ between groups. The SDB group showed higher OAHIs, body mass indices, and systolic blood pressure. Significantly reduced MD values (acute changes) appeared in the hippocampus, insula, thalamus, temporal and occipital cortices, and cerebellum, but were increased (chronic damage) in the frontal and prefrontal cortices in the SDB group over controls. Both positive and negative correlations appeared with extent of tissue changes and disease severity. Externalizing and Total Problem Behaviors were significantly higher in children with SDB. Verbal, performance, and total IQ scores trended lower, and behavioral scores trended higher.ConclusionsPediatric SDB is accompanied by predominantly acute brain changes in areas that regulate autonomic, cognitive, and mood functions, and chronic changes in frontal cortices essential for behavioral control. Interventions need to be keyed to address acute vs chronic injury.

Summary: 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 and cognitive 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 that the cortical thickness was significantly greater in the right caudal anterior cingulate and right cuneus regions and there were volumetric increases in the left caudal middle frontal, bilateral rostral anterior cingulate, left, right, and bilateral caudate brain regions in children with SDB compared with 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 behavioural or cognitive outcomes. [ABSTRACT FROM AUTHOR]

A review published in the Journal of Paediatrics & Child Health suggests that anti-IL-5 treatments, such as mepolizumab, reslizumab, and benralizumab, can be beneficial for adults with chronic severe asthma. These treatments target specific receptors in the body, reducing the frequency of asthma attacks. However, there is limited evidence regarding improvements in lung function or overall quality of life. Safety concerns were identified for benralizumab, with some patients experiencing adverse events. The review primarily focuses on adults, and more research is needed to determine the efficacy and safety of these treatments for children. Another document discusses the use of biologic agents, specifically mepolizumab, for managing severe asthma in children and adolescents. It highlights the effectiveness of mepolizumab in reducing asthma attacks and mentions its long-term safety in children with severe eosinophilic asthma. The document also provides information on funding and eligibility criteria for mepolizumab and other biologic agents in Australia and New Zealand. It emphasizes the importance of these treatments in reducing the need for steroids and improving the quality of life for patients with severe asthma. [Extracted from the article]

Summary: Children with Down syndrome are at increased risk of obstructive sleep disordered breathing, which has deleterious effects on daytime functioning. We aimed to examine the effects of treatment of sleep disordered breathing on sleep quality and daytime functioning in children with Down syndrome, and hypothesised that these would be improved. Thirty‐four children completed a baseline study and a follow‐up 2 years later. Measures at both time points included 7 days of actigraphy and parents completed a number of questionnaires assessing sleep, behaviour, daytime functioning, and quality of life. All children had overnight polysomnography at baseline; 15 children (44%) were treated. At baseline the treated group had more severe sleep disordered breathing compared with the untreated group: obstructive apneoa–hypopnoea index 29.3 ± 38.2 events/h versus 3.3 ± 5.2 events/h (p < 0.01). Actigraphy showed no significant differences in total sleep time, sleep efficiency, sleep schedules from baseline to follow up in either group. The sleep disturbance (p < 0.01) and total problems (p < 0.05) scales on the OSA‐18 and the sleep disordered breathing subscale on the Paediatric Sleep Problem Survey Instrument (p < 0.01) improved in the treated children. There were no changes in any measure in the untreated children. Treatment of sleep disordered breathing improves symptoms, sleep disturbance and quality of life in children with Down syndrome, but has no demonstrable impact on actigraphic sleep measures or daytime behaviour or function. In contrast, children who were not treated, despite having less severe disease at baseline, had increased sleep disruption and no change in quality of life. [ABSTRACT FROM AUTHOR]