ESPE Abstracts

Background: Congenital hyperinsulinism (HI) results in hyperinsulinemic hypoglycaemia in infants; a significant risk factor for brain injury due to neuroglycopenia and the suppression of alternative neuroprotective mechanisms such as ketogenesis. Neuronal damage can occur in both transient and persistent HI, with worse neurodevelopmental outcomes reported in those who receive a delayed diagnosis. There is currently no method for screening for HI and neuronal d...

Background and Aims: Hypoglycaemia is a life-threatening risk for many patients and prevention is individualised and complex. Continuous Glucose Monitoring (CGM) shows promise but current accuracy is insufficient for acute hypoglycaemia detection and data review services are complex and generic. Machine Learning is increasingly used but ignores weekly hypoglycaemia patterns and behaviour change and thus has demonstrated no real-world reduction in hypoglycaemia...

Introduction: The neonatal threshold for hypoglycaemia is debatable and makes its treatment challenging. Neonatal hypoglycaemia can be transient and yet pose a significant risk of neuroglycopaenia, especially with severe and recurrent hypoglycaemia in Congenital Hyperinsulinism. Untreated hypoglycaemia induced cerebral injury can be identified by magnetic resonance (MR) brain scan changes affecting cerebral white matter, occipital lobes and posterior parietote...

Importance: Current practice of doubling or trebling the basal glucocorticoid (GC) replacement dose during acute illnesses in children and young people (CYP) with GC deficiency is extrapolated from research in adults and not based on robust evidence. Endogenous cortisol levels during acute illnesses in CYP without GC deficiency can provide insight for optimal GC stress dosing for CYP with GC deficiency.Objective: To summ...

Background and Aims: In patients with congenital hyperinsulinism (CHI), recurrent hypoglycaemia can lead to longstanding neurological impairments. At present, glycaemic monitoring is with infrequent fingerprick tests; a practice which can miss hypoglycaemic episodes between tests. Continuous glucose monitoring (CGM) is a promising alternative method which has the utility to identify risk and patterns of hypoglycaemia. Although CGM is well established in type 1...

Congenital Hyperinsulinism (CHI) is primarily associated with defects in the regulated release of insulin from ß-cells but little information is available about the role of other islet cell types. Pancreatic polypeptide (PP) cells represent a minor component of the islet endocrine cell population. PP causes satiety, decreases gastrointestinal tract motility and suppresses glucagon release. Since CHI is associated with feeding problems and loss of glucagon-mediated counter...

Background: Long-term treatment with recombinant human growth hormone (r-hGH) is often challenging for children and adolescents, their caregivers and healthcare professionals (HCPs), as treatment requires daily injections over several years. Connected digital health devices facilitate this by automating the injection process to improve comfort, reduce anxiety and collect GH treatment data so that accurate adherence information is available to HCPs in real-time...

Introduction: Easypod-connect™ for childhood growth disorders is a unique connected system that enables the transmission of injection adherence information for recombinant human growth hormone (r-hGH). Although this system has the potential to facilitate greater adherence, observational studies have shown declining adherence over prolonged periods when used without additional support. Supplemental nurse practitioner support has been envisaged but not inv...

Background: Congenital hyperinsulinism (CHI) is a rare disease affecting neonates, infants, and children caused by dysregulated insulin secretion resulting in severe recurrent hypoglycemia. Early treatment is necessary to limit the risk of neurologic and developmental sequelae. Dasiglucagon is a glucagon analog (stable liquid formulation) suitable for continuous subcutaneous infusion shown to raise blood glucose in a dose-dependent manner. Result...

Introduction: Patients with hyperinsulinism suffer recurrent and severe hypoglycaemia. Inpatient glucose monitoring is currently via intermittent, infrequent fingerprick self-monitoring blood glucose (SMBG), and risks missing hypoglycaemia between tests. Continuous glucose monitoring (CGM) offers a supplementary therapy which may identify unexpected hypoglycaemia and ultimately reduce total exposure. However, CGM has never been evaluated for its efficacy in th...