The Enigma That Is ROHHAD Syndrome: Challenges and Future Strategies.

Rapid-onset obesity with hypoventilation, hypothalamic dysfunction, and autonomic dysregulation (ROHHAD) is a rare syndrome presenting in early childhood associated with a high risk of mortality between 50 and 60%. It is characterised by rapid, early onset of obesity between 1.5-7 years, along with central hypoventilation and hypothalamic dysfunction, such as central hypothyroidism, hyperprolactinemia, disorders of sodium and water balance, growth hormone deficiency, adrenocortical insufficiency, or disorders of puberty and features of autonomic dysregulation.

Daily Duration of Eating for Children and Adolescents: A Systematic Review and Meta-Analysis.

Time-limited eating is a dietary intervention whereby eating is limited to a specific window of time during the day. The usual eating windows of adults, and how these can be manipulated for dietary interventions, is well documented. However, there is a paucity of data on eating windows of young people, the manipulation of which may be a useful intervention for reducing obesity.

Development of Momentary Appetite Capture (MAC): A versatile tool for monitoring appetite over long periods.

Understanding how an intervention impacts appetite in real-life settings and over several days remains a challenging and under-explored research question. To this end, we developed Momentary Appetite Capture (MAC), a form of ecological momentary assessment that combines automated text messaging with an online platform. Participants report their appetite using visual analogue scales (hunger, desire to eat, and fullness) and a virtual portion-size selection task.

Integrating COM-B and the person-based approach to develop an ACT based therapy programme to raise self-determination in adolescents with obesity.

Background: This paper details the development of the Adolescent Intrinsic Motivation 'AIM2Change' intervention to support weight-management in young people previously unable to make changes whilst attending a tier 3 weight management service for children and young people. AIM2Change is an acceptance and commitment therapy based intervention that will be delivered one-to-one online over a seven-week period.

Methods: To develop this intervention, we have triangulated results from a qualitative research study, patient and public involvement groups (PPI) and a COM-B (capability, opportunity, motivation, behaviour) analysis, in a method informed by the person-based approach.

Current evidence for designing self-management support for underserved populations: an integrative review using the example of diabetes.

Aims: With numerous and continuing attempts at adapting diabetes self-management support programmes to better account for underserved populations, its important that the lessons being learned are understood and shared. The work we present here reviews the latest evidence and best practice in designing and embedding culturally and socially sensitive, self-management support programmes.

Methods: We explored the literature with regard to four key design considerations of diabetes self-management support programmes: Composition - the design and content of written materials and digital tools and interfaces; Structure - the combination of individual and group sessions, their frequency, and the overall duration of programmes; Facilitators - the combination of individuals used to deliver the programme; and Context - the influence and mitigation of a range of individual, socio-cultural, and environmental factors.

Case report: Molecular characterisation of adipose-tissue derived cells from a patient with ROHHAD syndrome.

There have been over 100 cases of Rapid-onset obesity with hypothalamic dysfunction, hypoventilation, and autonomic dysregulation (ROHHAD) syndrome reported, but there is currently no curative treatment for children with this condition. We aimed to better characterise adipose cells from a child with ROHHAD syndrome. We isolated pre-adipocytes from a 4 year-old female patient with ROHHAD syndrome and assessed proliferation rate of these cells.

The Effect of Intermittent Fasting on Appetite: A Systematic Review and Meta-Analysis.

Previously, narrative reviews have considered the effects of intermittent fasting on appetite. One suggestion is that intermittent fasting attenuates an increase in appetite that typically accompanies weight loss. Here, we conducted the first systematic review and meta-analysis to quantify the effects of intermittent fasting on appetite, when compared to a continuous energy restriction intervention.

Evaluating Eating Behaviour, Energy Homeostasis, and Obesity in Childhood-Onset Craniopharyngioma: A Feasibility Study.

Introduction: Craniopharyngiomas are rare brain tumours (incidence 1.1-1.7 cases/million/year).

Exploring the acute effects of running on cerebral blood flow and food cue reactivity in healthy young men using functional magnetic resonance imaging.

Acute exercise suppresses appetite and alters food-cue reactivity, but the extent exercise-induced changes in cerebral blood flow (CBF) influences the blood-oxygen-level-dependent (BOLD) signal during appetite-related paradigms is not known. This study examined the impact of acute running on visual food-cue reactivity and explored whether such responses are influenced by CBF variability. In a randomised crossover design, 23 men (mean ± SD: 24 ± 4 years, 22.

An App-Based Intervention for Pediatric Weight Management: Pre-Post Acceptability and Feasibility Trial.

Background: A multidisciplinary approach to weight management is offered at tier 3 pediatric weight management services in the United Kingdom. Encouraging dietary change is a major aim, with patients meeting with dieticians, endocrinologists, psychologists, nurse specialists, and social workers on average every other month.

Objective: This research sought to trial an inhibitory control training smartphone app-FoodT-with the clinic population of a pediatric weight management service.

Evaluating the benefit of early patient and public involvement for product development and testing with small companies.

Introduction: There is a growing understanding of the benefits of patient and public involvement (PPI), and its evaluation, in research. An online version of the CUBE PPI evaluation framework has been developed. We sought to use the CUBE to evaluate the value of early PPI with two small healthcare companies during product development.

Rapid-onset obesity, hypothalamic dysfunction, hypoventilation, and autonomic dysregulation syndrome - neuro-endocrine tumours (ROHHAD-NET): case series and learning points.

Objectives: Rapid-onset obesity with hypoventilation, hypothalamic dysfunction, autonomic dysregulation (ROHHAD) is a rare syndrome associated with high morbidity and mortality. Diagnosis is often challenging. We describe three cases of ROHHAD with heterogeneous presentations but some consistent clinical features, including hyperprolactinaemia at diagnosis.

Developments in the design and delivery of self-management support for children and young people with diabetes: A narrative synthesis of systematic reviews.

Aims: Facilitated self-management support programmes have become central to the treatment of chronic diseases including diabetes. For many children and young people with diabetes (CYPD), the impact on glycated haemoglobin (HbA ) and a range of self-management behaviours promised by these programmes remain unrealised. This warrants an appraisal of current thinking and the existing evidence to guide the development of programmes better targeted at this age group.

Preference for high-carbohydrate foods does not change for children and adolescents in insulin-induced hypoglycemia.

Introduction: Hypoglycemia elicits coordinated counter-regulatory neuroendocrine responses. The extent to which this process involves an increased drive to eat, together with greater preference for foods high in carbohydrate content, is unclear. Our objective was to examine this effect in children and adolescents (age 5-19 years) without diabetes and no prior known experience of hypoglycemic episodes.

Evaluating future risk of NAFLD in adolescents: a prediction and decision curve analysis.

Background: Non-alcoholic fatty liver disease (NAFLD) is the commonest liver condition in the western world and is directly linked to obesity and the metabolic syndrome. Elevated body mass index is regarded as a major risk factor of NAFL (steatosis) and NAFLD fibrosis. Using data from the Avon Longitudinal Study of Parents and Children (ALSPAC), we sought to investigate whether other variables from adolescence could improve prediction of future NAFL and NAFLD fibrosis risk at 24 years, above BMI and sex.

Rituximab therapy in ROHHAD(NET) syndrome.

Objectives: Rapid-onset obesity with hypoventilation, hypothalamic dysfunction, autonomic dysregulation, and neural-crest tumour (ROHHAD(NET)) is a rare syndrome presenting in early childhood associated with high morbidity and mortality. There is no specific diagnostic biomarker and diagnosis is based on clinical features. An autoimmune origin has been postulated.

Piloting the objective measurement of eating weight and speed at a population scale: a nested study within the Avon Longitudinal Study of Parents and Children.

Effective measurement and adaption of eating behaviours (e.g., eating speed) may improve weight loss and weight over time.

The feasibility, acceptability, and benefit of interventions that target eating speed in the clinical treatment of children and adolescents with overweight or obesity: A systematic review and meta-analysis.

Eating at a faster speed is positively correlated with having a higher BMI. Modifying eating speed may offer a treatment opportunity for those with overweight and obesity. This review sought to understand the feasibility, acceptability, and benefit to using eating speed interventions in paediatric clinical weight-management settings.

Treatment of Barth Syndrome by Cardiolipin Manipulation (CARDIOMAN) With Bezafibrate: Protocol for a Randomized Placebo-Controlled Pilot Trial Conducted in the Nationally Commissioned Barth Syndrome Service.

Background: Barth syndrome is a rare, life-threatening, X-linked recessive genetic disease that predominantly affects young males and is caused by abnormal mitochondrial lipid metabolism. Currently, there is no definitive treatment for Barth syndrome other than interventions to ameliorate acute symptoms, such as heart failure, cardiac arrhythmias, neutropenia, and severe muscle fatigue. Previous mechanistic studies have identified the lipid-lowering drug bezafibrate as a promising potential treatment; however, to date, no human trials have been performed in this population.

When do children learn how to select a portion size?

The reduction of portion sizes supports weight-loss. This study looks at whether children have a conceptual understanding of portion size, by studying their ability to manually serve a portion size that corresponds to what they eat. In a clinical setting, discussion around portion size is subjective thus a computerised portion size tool is also trialled, with the portion sizes chosen on the screen being compared to amounts served manually.

How full am I? The effect of rating fullness during eating on food intake, eating speed and relationship with satiety responsiveness.

Modifying eating behaviours may be an effective strategy to limit excess food intake, such as eating slower and mindfully. We hypothesized that regularly rating fullness whilst eating a standard meal in one course would increase post-meal satiety and reduce intake in a subsequent course during the same sitting. A between-subjects design was employed (n = 65; 75% female; mean age = 26.