ESPE Abstracts

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: Congenital hyperinsulinism (CHI) is a rare disease affecting neonates, infants, and children. CHI is characterized by dysregulated insulin secretion resulting in severe recurrent hypoglycemia. Early treatment is necessary to limit the risk of neurologic and developmental sequelae. Current treatment options are limited and inadequate. Dasiglucagon (DASI) is a glucagon analog suitable for continuous subcutaneous infusion which has been shown to raise...

Background and Objective: Continuous Glucose Monitoring (CGM) is gaining in popularity for patients with paediatric hypoglycaemia disorders such as Congenital Hyperinsulinism (CHI), but no standard measures of accuracy or associated clinical risk are available. A small number of studies have shown suboptimal accuracy of CGM in CHI but assessments have been inconsistent, incomplete and offer no measure of clinical application. Error grids that categorise clinic...

Background & Aims: Hypoglycaemia is a constant threat for all patients with congenital hyperinsulinism (CHI) and, left untreated, can lead to neurological damage and impaired development. To improve glycaemic monitoring, self-monitoring-blood-glucose (SMBG) is increasingly being replaced by Continuous Glucose Monitoring (CGM) with potential to identify illness patterns and treatment responses although with unproven benefit for patients and families. Explor...

Background: Adrenal Insufficiency (AI) is a heterogeneous diagnosis comprising multiple central and peripheral causes. The unifying feature is the requirement for glucocorticoid replacement and subsequent risk of life-threatening Adrenal Crisis (AC) and Adrenal Death (AD). Very few studies have investigated rates of AD in children. Multiple studies have reported symptoms of children with AC as interpreted by medical staff but no studies have looked at parental...

Background: Congenital Hyperinsulinism (CHI) is the most common cause of recurrent and severe hypoglycaemia in childhood and can be broadly categorised into two subtypes. Diffuse CHI (CHI-D) involving all pancreatic cells is usually treated with medications and rarely subtotal pancreatectomy. Focal CHI (CHI-F) involves a solitary insulin hypersecreting pancreatic lesion and can be cured following surgical lesionectomy. Many patients with CHI-F and CHI-D underg...