Endocrine Abstracts

The prevalence of primary hyperparthyroidism (PT) in the general population is 0.15%. The incidence in pregnancy is unknown; however primary hyperparathyroidism during pregnancy poses significant maternal and fetal complications. Complications to the mother include nephrolithiasis, bone disease and muscle weakness. Fetal complications include fetal loss, intrauterine growth retardation, postpartum neonatal tetany and permanent hypoparathyroidism. The symptoms of PT are similar...

The definition of early cure post transphenoidal surgery (TSS) for Cushing’s disease continues to be debated. The low dose dexamethasone suppression test with corticotrophin releasing hormone (LDDST-CRH) is a test for diagnosis of Cushing’s; however it has not been validated in early post TSS to determine remission. The aim of this study was to determine whether the combined LDDST-CRH test could be used in post TSS patients.The combined LDDST-C...

The rising prevalence of obesity and type II diabetes makes the diagnosis of Cushing’s syndrome increasingly difficult. The dexamethasone-suppressed corticotrophin-releasing hormone (CRH) test, which involves CRH administration immediately following a standard low dose dexamethasone suppression test (LDDST), has been reported to diagnose Cushing’s syndrome with 100% sensitivity and specificity. We adapted our LDDST protocol to include CRH administration post-dexameth...

Background: The 3 day human chorionic gonadotrophin (HCG) test is commonly performed to investigate male undervirilisation. However, the utility of routine HCG testing for male undervirilisation in early childhood and correlation with pubertal progress is unclear.Aims: To review performance of the 3 day HCG test for diagnosis and outcomes.Methods: Standard 3 day HCG test data were analysed in 130 boys of age <3 years with under...

Introduction: Congenital hyperinsulinism (CHI) is a disesase of severe hypoglycaemia, often due to in mutations in ABCC8/KCNJ11. Sirolimus, an mTOR inhibitor, has been reported to be successful in CHI patients, but the evidence is limited. We have aimed (i) to review the efficacy and safety profile of sirolimus, (ii) to assess the role of mTOR signalling pathways in CHI, (iii) to assess the impact of sirolimus in CHI pancreatic tissue.Methods: P...

Introduction: Parents of children with complex diseases require easily understandable information about their disease to improve health outcomes. Improved disease understanding will also aid shared decision making between clinicians and families. Congenital hyperinsulinism (CHI) is a rare and complex disease of hypoglycaemia associated with significant neurodevelopmental morbidity for which online video-sharing information resources are available. The utility of such informati...

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 and objective: Neonatal hypoglycemia is common and frequently self-resolving, although rare due to congenital hyperinsulinism are associated with high risk of brain injury. The time period for neonatal hypoglycemia has been described in several studies. It is unknown if low hypoglycemia thresholds (<2.0 mmol/l) lead to missed cases of persistent hypoglycaemia. We aimed to ascertain if lower hypoglycemia threshold risked missing persistent forms of hypoglycemia i...

Introduction: Paediatric obesity is a growing concern for the health service. There is currently no consensus for routine screening of metabolic profiles and medical treatment in obese paediatric patients.Aims/methods: We aimed to determine medium-term outcomes of Metformin treatment on BMI, glucose and insulin levels in obese paediatric patients. In a retrospective review, data were collected from obese paediatric patients on Metformin for insulin resis...

Background: Congenital hyperinsulinism (CHI) can cause severe hypoglycaemia with consequent adverse neurodevelopment. Continuous glucagon infusion (CGI) through intravenous and subcutaneous routes has been utilised to achieve glycaemic stability, but the efficacy has not been reported systematically in a CHI cohort.Aim: We aimed to investigate the efficacy and safety profile of CGI in the management of hypoglycaemia due to CHI.Meth...