hrp0095p1-267 | Fat, Metabolism and Obesity | ESPE2022

Feasibility of integrating an mHealth intervention to increase exercise and physical activity adherence into a weight management service for adolescents with severe obesity: MOTIVATE-WMA

Davies Andrew , Hesketh Katie , Clarke Ellie , Apperley Louise , Sprung Victoria , Jones Helen , Senniappan Senthil , Cocks Matthew

Introduction: The Royal College of Paediatrics and Child Health state that adolescents with significant obesity require access to evidence-based weight management services (WMS). Due to the known benefits of exercise and physical activity (PA) on physiological and psychological outcomes increasing exercise and PA should form a cornerstone of any WMS for adolescents living with obesity. However, supervised exercise interventions face challenges within a clinica...

hrp0095p2-149 | GH and IGFs | ESPE2022

Advancing personalised medicine for growth hormone delivery: mixed-methods participatory study of a next generation, smart auto-injector device

I Labarta José , Rivera-Romero Octavio , Fernández-Luque Luis , Keiser Matthew , Koledova Ekaterina

Background: Treatment of growth hormone deficiency (GHD) requires daily injections over multiple years. Novel technologies facilitate this by automating the injection process – thereby adding comfort and reducing anxiety. An always-connected device, enabled by mobile technologies, also facilitates the collection of injection data such that adherence information is available to healthcare professionals (HCPs) in real-time. In developing new solutions, it ...

hrp0082p3-d1-983 | Thyroid | ESPE2014

Resistance to Thyroid Hormone Syndrome from Childhood to Adulthood: Variation in Symptoms and Thyroid Function

Garrahy Aoife , Grace Mariana , Stapleton Mary , Moran Carla , Chatterjee Krishna , Murphy Matthew , O'Connell Susan

Introduction: Resistance to thyroid hormone (RTH) is a rare autosomal dominant condition characterised by tissue-specific insensitivity to thyroid hormone. In 85% of cases the disorder is associated with thyroid hormone receptor β (TRβ) gene mutations.Case report: A 2.6-year-old boy was referred to the Paediatric service with abnormal thyroid function tests (TFTs) (fT4 30.4 pmol/l; RR 12–26, fT3 10.2 pmol/l; RR 3.7&#1...

hrp0084p2-360 | Fat | ESPE2015

Increasing Waist/Height Ratio and BMI Z-Score are Associated with Increased Comorbidities in Obese Youth, although Neither Accurately Identifies those with Abnormal Glucose Metabolism

Kao Kung-Ting , Alexander Erin , Harcourt Brooke E , McCallum Zoe , Wake Melissa , Werther George , Sabin Matthew

Background: At a population level, increasing age/sex corrected BMI Z-score (BMI-Z) is associated with increased risk of complications. It is unclear whether severity of obesity is a good predictor of comorbidities within groups of obese children. This knowledge is required to inform clinical management and pathways of care.Objective and hypotheses: In obese youth, to identify whether 1 increasing BMI-Z+/− waist/height ratio(WHtR) is associated wit...

hrp0094p2-369 | Pituitary, neuroendocrinology and puberty | ESPE2021

6-Month GnRHa Formulations Are a Good Choice During the COVID-19 Pandemic and Beyond

R. Benson Matthew , N. Atkinson Stuart , M. Boldt-Houle Deborah , Miller Bradley S. ,

Introduction: Achieving/maintaining effective hormone suppression is fundamental in treating Central Precocious Puberty(CPP). CPP patients are vulnerable to late dosing as they cannot self-administer and require clinic/hospital visits for injections, currently exacerbated by COVID-19. In children, the hypothalamic-pituitary-gonadal axis may rebound faster than elderly oncology patients, so hormone escapes are possible with late dosing. Additionally, the stimul...

hrp0089fc1.1 | Adrenals & HPA Axis | ESPE2018

A Novel Non-invasive Short Synacthen Test Validated in a Healthy Paediatric Population

Elder Charlotte , Vilela Ruben , Johnson Trevor , Kemp E Helen , Keevil Brian , Newell-Price John , Ross Richard , Wright Neil

Introduction: Worldwide the Short Synacthen Test (SST) is the most popular diagnostic investigation for adrenal insufficiency (AI) amongst both paediatric and adult endocrinologists. Cannulation and blood sampling are required making it invasive, time-consuming and resource-intensive. We have previously validated a reliably absorbed and well tolerated formulation of nasal synacthen (Nasacthin003) in healthy adult males, measuring the glucocorticoid response in salivary cortiso...

hrp0094p2-9 | Adrenals and HPA Axis | ESPE2021

Variability of advice and education for steroid sick day dosing in Duchenne Muscular Dystrophy and the impact of the COVID-19 pandemic: Results of a UK wide patient survey

Thakrar S , Cheung MS , Johnson A , Katugampola H , Manzur A , Muntoni F , Mushtaq T , Turner C , Wood C , Guglieri M , Wong SC ,

Introduction: The use of long-term oral corticosteroid in DMD is associated with a range of side effects including secondary adrenal insufficiency. The updated international care consensus (2018) recognizes this important issue and recommends emergency plans to be in place.Aim: This online UK-wide patient survey aims to determine the advice and education given for sick day dosing plans and the impact of the COVID-19 pand...

hrp0094wg8.3 | ESPE Working Group on Turner Syndrome Symposium | ESPE2021

Blood Pressure in Turner Syndrome - a practical approach

Matthews Debbie ,

Females with Turner syndrome (TS) have a reduced life expectancy of about 13 years and a substantial excess mortality; about 50% of this is the result of cardiovascular pathology. Hypertension affects 20-60% of females with TS and is the single most modifiable risk factor for cardiovascular disease. However, hypertension in TS is both under-diagnosed and under-treated. The presentation details a practical approach to monitoring blood pressure and managing hypertension in TS. T...

hrp0092p1-139 | Sex Differentiation, Gonads and Gynaecology or Sex Endocrinology | ESPE2019

Gonadal Function of Female Patients with Noonan Syndrome

Moniez Sophie , Capri Yline , Pienkowski Catherine , Lepage Benoit , Hamdi Safouane , Cartault Audrey , Oliver Isabelle , Jouret Béatrice , Diene Gwenaelle , Salles Jean-Pierre , Cavé Hélène , Verloes Alain , Tauber Maithé , Yart Armelle , Edouard Thomas

Background: Abnormalities in the hypothalamo-pituitary-gonadal axis have been reported in Noonan syndrome (NS) males but few data are available in female patients. Objective: The aim of this retrospective study was to evaluate the gonadal function of female patients with NS and to look for genotype-phenotype correlations. Patients and Methods: The study population included 19 girls/adolescents with a genetically confirmed diagnosis of NS and with available cli...

hrp0084fc5.3 | Endocrine Oncology/Turner | ESPE2015

Screening in Children with Succinate Dehydrogenase B (SDHB) Mutations: a Single Centre’s Family Clinic Experience

Shapiro Lucy , Srirangalingam Umasuthan , McAndrew Lorraine , Martin Lee , Tufton Nicola , Kumar Ajith , Drake William , Akker Scott , Storr Helen

Background: Germline mutations in SDHB gene are associated with the familial paraganglioma (PGL) syndrome that carries the highest malignant potential. Although penetrance is lower than initially described, lack of effective treatments for metastatic PGLs makes screening essential for early tumour detection, surgical removal and improved outcome. However, no consensus exists in relation to timing and mode of screening.Objective: To assess publis...