Endocrine Abstracts

Background: Congenital Hyperinsulinism (CHI) is characterised by dysregulated and excess secretion of insulin leading to severe hypoglycaemia. Monitoring of glucose levels is essential in this condition as prolonged hypoglycaemia can cause life-threatening complications such as permanent neurological impairment. Interstitial glucose monitoring by continuous glucose monitoring (CGM) devices can identify nocturnal hypoglycaemia retrospectively through data analysis. Analysis can...

Background: For children with congenital hyperinsulinism (HI), detection and avoidance of hypoglycaemia is the cornerstone of clinical management and poses significant demands on families. Standard of care remains intermittent fingerprick monitoring but the lack of predictive information has resulted in continuous glucose monitoring (CGM) increasing in popularity. Accuracy is suboptimal in this group and family feedback identifies various barriers to use. We a...

Background: Congenital hyperinsulinism of infancy (CHI) is the most common cause of severe hypoglycaemia in children. CHI arises from mutations in ion channel genes (ABCC8/KCNJ11), which lead to inappropriate insulin secretion. CHI is also associated with increased cell proliferation and altered islet cell development. The aim of this study was to investigate the composition of the islet capsule in CHI and to relate this to the organisation of islet cells.</p...

Background: Congenital Hyperinsulinism in Infancy (CHI) is characterised by inappropriate insulin release from islet β-cells. We currently attribute hypoglycaemia to β-cell dysfunction because of defects in the ion channel genes ABCC8 or KCNJ11. However, the CHI pancreas is also associated with the inappropriate expression of foetal-like transcription factors and enhanced cell proliferation. We hypothesised that islet cell differentiation and neogen...

Background: The mechanisms responsible for inappropriate insulin release from β-cells in Congenital Hyperinsulinism in Infancy (CHI) have largely focused upon defects in KATP channels. Little is known about insulin biogenesis, the profiles of insulin in insulin-containing secretory granules or whether the impact of KATP channel defects (due to mutations in ABCC8 or KCNJ11) is the same in diffuse- and focal disease.<p class="abst...

Adrenal insufficiency (AI) is characterised by lack of cortisol production from the adrenal glands which is treated with replacement doses of hydrocortisone. At times of physiological stress there is an increased requirement for exogenous glucocorticoids, which if untreated can lead to an adrenal crisis. Currently there are no unified guidelines for those <18 years old in the UK; this can lead to a substantial variation in the management of AI in both an emergency and peri...