Endocrine Abstracts

Growth hormone (GH) excess is extremely rare in children. It can be associated with endocrine syndromes including MEN1, Carney complex and Mc Cune Albright syndrome. We describe two cases of GH excess and their management. Patient A (15yr M) presented with fibrous dysplasia affecting his cranium and a large café-au-lait spot covering his right scapula. His height was 193cm (2.6 SDS) and BMI 29.6kg/m2(2.3SDS). His IGF1 was 84.1nmol/L (22.5 – 65.9) and GH did not suppr...

Background: The cardiovascular and metabolic outcomes of patients with AI are poor, and may be related to the non-physiological cortisol profile achieved with hydrocortisone treatment. Objectives: To describe salivary adrenal biomarkers, glucose and 24 hour ambulatory blood pressure (ABP) profiles, carotid intima media thickness (CIMT) and flow mediated dilatation (FMD) in CYP with primary AI, to improve knowledge of the relationship between non-physiolo...

Background: Congenital hyperinsulinism (CHI) is the result of unregulated insulin secretion from the pancreatic β-cells leading to severe hypoglycaemia.1 Diazoxide is effective in virtually all forms of CHI except in those due to recessive (and some dominant) inactivating mutations in ABCC8 and KCNJ11 and in patients with focal CHI.2 We report a case of CHI with acute kidney injury secondary to diazoxide.Case ...

Background: Cystic Fibrosis related diabetes affects 40-50% of adults with cystic fibrosis (CF). This can significantly affect pulmonary function and life expectancy. Kaftrio (Ivakaftor, tezacaftor and elexacaftor) has recently been licensed for use in CF. Previous data highlight that glucose regulation may be altered on commencing this treatment.Methods: Eight children and young people (CYP), aged 14 (12-15) years, who were diagnosed with CFRD aged 12.8...

Background: Cortisol is inactivated to cortisone in the salivary gland by 11β-HSD type 2. Concentrations of cortisone and cortisone in saliva correlate strongly with serum cortisol concentrations (1). Only free, biologically active hormone is measured in saliva, testing is non-invasive and can be performed at home/school, reducing cost and inconvenience to families and NHS resources. We previously reported pilot data from healthy children and young people (CYP) (1). Here we de...

Background: Hypoglycaemia, possibly due to non-physiological hormone replacement, and poor cardiovascular outcomes are described in patients with adrenal insufficiency (AI), particularly in the adult population. In this study, we describe glucose profiles and risk factors for premature cardiovascular disease (CVD) in children with secondary AI (SAI).Methods: Participants underwent continuous glucose monitoring (CGM), for seven days (blinded Dexcom G6 mon...

Background: Increased hair cortisol concentrations are reported in Turner Syndrome (TS) patients compared to healthy controls (HC).(1) Increased cortisol exposure could contribute to cardiovascular, metabolic and bone morbidity in TS. Hair cortisol concentrations give no information about the circadian profile of cortisol, which is important for cardiovascular health. Cortisol is inactivated to cortisone by 11β hydroxysteroid dehydrogenase (11βHSD) type-2 and regener...

Introduction: Evidence is currently limited regarding the management of abnormal lipid profiles in children and young people (CYP) with Type 1 Diabetes Mellitus (T1DM). ISPAD recommend monitoring every five years and statin use to maintain LDL cholesterol <3.4 mmol/L1. Guidance is specific to LDL. NICE guidelines do no recommend routine screening for CYP with T1DM.Methodology: We reviewed all lipid profiles (including cholesterol, triglyce...

Introduction: Graves’ disease (GD), although the most common cause of hyperthyroidism, is relatively uncommon in children, usually manifesting insidiously. Thyroid storm is a well described but rarely seen phenomenon in childhood GD; thyrotoxic crisis is less often discussed and its management can be challenging. We describe a case of GD with thyrotoxic crisis.Case: A 14-year-old female, known to have inadequately c...

Background: Saliva is ideal for measuring free, biologically active hormones. Measurements of salivary androgens may be valuable in diagnosis and monitoring of adrenal disorders, however the diurnal profile and robust reference ranges in healthy children are currently undefined. We report salivary testosterone, androstenedione (A4), 11-ketotestosterone (11-KT), 11-β hydroxyandrostenedione (11β-OHA4) concentrations measured throughout the day in healthy children.<...