hrp0092p3-299 | Late Breaking Abstracts | ESPE2019

Review of Neonatal Cortisol Evaluation between 2012–2018 in a Single Centre: Trends, Outcomes and Associations

Makaya Taffy , Sarvasiddhi Satish , Menon Smrithi , van Boxel Elizabeth-Jane , Shine Brian

Background: Neonatal cortisol assessment is indicated in suspected adrenal insufficiency. Clinical presentation includes: low blood glucose, low blood pressure, ambiguous genitalia, electrolyte abnormalities, midline abnormalities.Aims: Review the trends, outcomes and associations of cortisol assessment in neonates within our Trust between 2012-2018.Objectives: Review:<p class=...

hrp0089fc6.2 | Fat, Metabolism and obesity | ESPE2018

Impaired Brain Satiety Responses to a Meal in Children with Obesity

Roth Christian , Melhorn Susan , Elfers Clinton , Scholz Kelley , De Leon Mary Rosalynn , Saelens Brian , Schur Ellen

Behavioral studies suggest that brain satiety responses to food consumption are altered in children with obesity. We studied brain regions involved in satiety processing using functional magnetic resonance imaging (fMRI) before and after a test meal. Satiety-related hormonal changes were assessed. Fifty-four 9-11 year-old children with obesity (OB) and 22 children with healthy weight (HW) were studied. Subjects underwent two fMRI scans, one before and one after a test meal, an...

hrp0089rfc1.1 | Adrenals &amp; HPA Axis | ESPE2018

The Relative Contributions of Genetic and Environmental Factors on Cortisol Metabolism at Pre-, Mid- and Post-Pubertal Ages

van Keulen Britt , Dolan Conor , Andrew Ruth , Walker Brian , Boomsma Dorret , Rotteveel Joost , Finken Martijn

Background: Inter-individual differences in the metabolism of cortisol have been postulated to emerge during puberty, and might be explained by a complex interplay of genetic and environmental factors. The aim of the current study was to estimate the relative contributions of genetic, shared environmental, and unshared environmental factors on cortisol metabolism in a longitudinal twin cohort assessed at pre-pubertal, mid-pubertal and post-pubertal ages....

hrp0082fc1.4 | Adrenal | ESPE2014

A Novel Non-Invasive Short Synacthen Test

Elder Charlotte , Johnson Trevor , Loxley Martin , Keevil Brian , Wales Jerry , Wright Neil

Background: The short synacthen test (SST) is a popular diagnostic investigation for adrenal insufficiency (AI). Cannulation and blood sampling are required making it invasive, time-consuming and resource-intensive. Salivary cortisol is a well-established alternative to serum sampling.Objective and Hypotheses: To develop a non-invasive alternative to the 1 μg SST, using a novel formulation of Synacthen (with a nasal drug enhancer, chitosan) given na...

hrp0082p2-d3-481 | Hypoglycaemia | ESPE2014

Presentation, Clinical and Genetic Outcomes in a Series of Infants With Congenital Hyperinsulinism

Carroll Aoife , Gibney Brian , McDonnell Ciara , Moloney Sinead , Monavari Ardeshir , Murphy Nuala

Background: Congenital hyperinsulinism (CHI) is a rare condition but a significant cause of recurrent hypoglycaemia in infancy and childhood. Prompt recognition and appropriate management is important to avoid long-term neurological sequelae.Objective and hypotheses: To describe the presentation, clinical and genetic outcomes in a series of infants with CHI.Method: Retrospective case series of 35 patients diagnosed with CHI between...

hrp0094fc6.2 | Bone and Mineral Metabolism | ESPE2021

Long-term effectiveness of PTH(1-34) infusion therapy for autosomal dominant hypocalcaemia type 1.

Sastre Ana , Valentino Kevin , Hannan Fadil M , Lines Kate E , Gluck Anna K , Stevenson Mark , Ryalls Michael , Gorrigan Rebecca , Pullen Debbie , Buck Jackie , Sankar Sailesh , Allgrove Jeremy , Thakker Rajesh V , Gevers Evelien F ,

Background: Patients with autosomal dominant hypocalcemia type 1 (ADH1), due to germline gain-of-function calcium-sensing receptor (CASR) mutations, have hypocalcemia and seizures, hyperphosphatemia, hypercalciuria and inappropriately low parathyroid hormone (PTH) concentrations. Treatment for ADH1 comprises calcium and vitamin D analogs, however, their use predisposes to nephrocalcinosis and renal impairment. In contrast, recombinant human PTH(1-34) may incre...

hrp0092fc4.6 | Fat Metabolism and Obesity Session | ESPE2019

Brain Satiety Responses to a Meal in Children Before and After Weight Management Intervention

Roth Christian L. , Melhorn Susan , Elfers Clinton , Rosalynn Mary , Rowland Maya , Grabowski Thomas , Saelens Brian , Schur Ellen A.

Family-based behavioral treatment (FBT) is the recommended intervention for children with obesity (OB). However, there is a large variability in short- and long-term treatment response and mechanisms for unsuccessful treatment outcomes are not understood. We studied brain regions involved in satiety processing in 9-11-year-old children with obesity (OB, n=54) and children with healthy weight (HW, n=22). Subjects underwent a functional magnetic resonan...

hrp0089fc1.1 | Adrenals &amp; 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...

hrp0094p2-375 | Pituitary, neuroendocrinology and puberty | ESPE2021

Salivary sex steroids as markers of puberty in boys during late childhood and adolescence

Patjamontri Supitcha , Spiers Alexander , Smith Rachel B , Shen Chen , Adaway Jo , G Keevil Brian , Toledano Mireille B , Ahmed S Faisal ,

Introduction: Salivary androgens represent a non-invasive marker of puberty that may have utility in population studies as well as in the clinical arena.Objectives: To establish normal reference values of salivary androgens using LC-MS/MS and demonstrate the correlations between salivary androgens and pubertal development in boys.Methods: School-based adolescent cohort study with t...

hrp0095fc3.2 | Early Life and Multisystem Endocrinology | ESPE2022

Results from a Global, Multi-Center, Phase 2b Study (RIZE) in Congenital Hyperinsulinism: Characterization of a High Unmet Treatment Need and Glycemic Response to RZ358

Demirbilek Huseyin , Melikyan Maria , Galcheva Sonya , Dastamani Antonia , Thornton Paul , De Leon Diva , Raskin Julie , Roberts Brian , Hood Davelyn , O'Boyle Erin , Christesen Henrik

Background: Congenital Hyperinsulinism (CHI) is the most frequent cause of severe, persistent hypoglycemia in children. Persistent hypoglycemia places patients at risk for adverse clinical outcomes, and current guidelines recommend maintaining plasma glucose >3.9mmol/l. CHI patients often have substantial, residual hypoglycemia and fail to meet treatment goals with currently available standard of care (SOC) therapies. In this study, we aimed to characterize...