Endocrine Abstracts

Gonadal dysgenesis (GD) is characterised by maldevelopment of the gonads and is classified as complete (CGD) or partial (PGD) depending on gonadal morphology and function. The phenotype of PGD is variable and diagnosis is based on clinical and biochemical features, coupled with gonadal histology and genetic findings. “46,XY Gonadal Dysgenesis: diagnosis and long-term outcome” has recently been approved as an I-DSD Registry-based study. The aim is to characterise the ...

Aromatase inhibitors (AIs) have been proposed as a modality in the treatment of short stature in boys. Improvement in final height and predicted final height has been observed in children with constitutional delay of growth and puberty and idiopathic short stature. AIs block the conversion of androgens to estrogens and thus may delay epiphyseal fusion and thereby prolong linear growth. We report our experience with the use of letrozole, a third generation AI, in a boy with gen...

Introduction: Hydrocortisone is the main treatment for congenital adrenal hyperplasia (CAH) in children. The optimal biochemical monitoring and replacement regimen of these children continues to be debated. We explored variations in blood spot 17α-hydroxyprogesterone (17-OHP) levels.Methods: Single centre retrospective cross-sectional study of children with 21-hydroxylase deficiency aged <18 years. Patients treated with hydrocortisone who had dr...

Introduction: Continuous subcutaneous insulin infusion (CSII) can improve glycaemic control and quality of life in Type 1 Diabetes patients. It is uncommon for paediatric patients to discontinue CSII and return to multiple daily insulin injections (MDI) often due to concerns regarding worsening metabolic control. However a trial off CSII is a requirement before transition to the adult clinic. We review the outcomes of those who discontinued CSII in our centre (total 248 patien...

Introduction: Recently a serious incident due to failure in rechecking blood glucose (BG) after hypoglycaemia treatment was reported in an adult inpatient. We evaluated adherence to network guidance on hypoglycaemia management in inpatients and changes in practice following an intervention based on education and system changes.Methods: In an audit (audit-1), hypoglycaemia episodes (BG level <4 mmol/l) were identified in paediatric inpatients (age &#6...

Introduction: The benefits of growth hormone (rGH) replacement during the transition from childhood to adulthood are well established. In adulthood rGH dose is optimized by titrating to serum IGF-I concentration. The use of non-specific IGF-I reference ranges may result in erroneous assessment of IGF-I concentration and inappropriate rGH dosing.Methods: IGF-I methods and reference ranges across 9 UK centres were reviewed. Immulite IGF-1 results for a 12 ...

Background: The Insulin Tolerance Test (ITT) is the gold standard for assessing pituitary function in adults, but used variably in paediatrics due to concerns of serious adverse events. Our aim was to assess the safety of ITT and identify factors associated with the hypoglycaemic response.Methods: We retrospectively collected the following data from patients who underwent ITT (n=122) under Paediatric Endocrinology from 2019-2021: demography, ant...

Background: A substantially reduced fluid replacement regimen was introduced in the New’ BSPED (2015) compared to ‘Old’ BSPED (2009) guideline for DKA management in children. However, effects of varying fluid replacement regimens are limited and we explored this by comparing outcomes of the two guidelines on the resolution of DKA.Methods: In a retrospective audit of consecutively admitted patients (age <18 years) to t...

Introduction: The management of type 1 diabetes (T1D) in young children can be extremely challenging due to high insulin sensitivity, unpredictable eating and activity and difficulty recognizing symptoms of hypoglycaemia. Continuous subcutaneous insulin infusion (CSII) therapy is beneficial in managing young children, however the small insulin doses required challenge the accuracy of standard concentration (100 IU/ml) CSII.Case report and literature revi...

Background: GH replacement during the childhood-adult transition period is important for somatic maturation. The aim of the study was to explore the factors influencing IGF1 response to GH during transition.Methods: KIMS (Pfizer International Metabolic Study) database in UK was interrogated, and 98 patients (55 male, median age 20.7 years (15.7–25.8)) with childhood-onset GH deficiency (Co-GHD), who were started on adult dose of GH during transition...