hrp0094p1-9 | Adrenal A | ESPE2021

Backdoor Pathway hormones and 11-oxygenated Androgens are elevated in Patients with 21-hydroxylase deficiency

Kulle Alexandra , Lamprecht Tabea , Pinto Francisca , Wulf Kristina , Hornig Nadine , Reinehr Thomas , Holterhus Paul-Martin ,

Background: Patients with 21-Hydroxylase deficiency (21OHD) present increased levels of cytochrome P450 21-hydroxylase substrates, like progesterone and 17α-hydroxyprogesterone. Previous studies could show that these hormones are involved in the production of androgens via the backdoor pathway. As a second source of androgens, 11-oxyandrogens derived from the human adrenal glands are recognized as being major androgens. Specifically, 11-oxyandrogens ...

hrp0094p1-91 | Thyroid A | ESPE2021

The effect of preoperative calcitriol prophylaxis on post-thyroidectomy hypocalcaemia in children

Vendrig Lisanne , Mooij Christiaan , Derikx Joep , Trotsenburg Paul van , Zwaveling-Soonawala Nitash ,

Background: Transient or permanent hypoparathyroidism is one of the most well-known complications of total thyroidectomy and may lead to symptomatic hypocalcaemia. Treatment of post-thyroidectomy hypocalcaemia usually consists of postoperative calcium and/or vitamin D supplementation. In 2013 we implemented prophylactic pre-thyroidectomy calcitriol supplementation for all children undergoing total thyroidectomy at Amsterdam UMC. The objective of this study was...

hrp0094p1-147 | Sex Endocrinology and Gonads B | ESPE2021

A pre-analytical challenge to determine estradiol in children: A monovette systematically causing increased estradiol-concentrations in LC-MS/MS analysis

Lamprecht Tabea , Kleber Michaela , Rothermel Juliane , Holterhus Paul-Martin , Kulle Alexandra ,

Introduction: In children, estradiol (E2) concentrations are 1000-times lower in comparison to their precursors, the androgens. Depending on sex, age, and pathology, plasma concentrations vary in a broad range. The sensitive and specific determination of E2 is a particular challenge in pediatric endocrinology. Here we report a female patient aged 12 6/12 years and Tanner stage B1 with a presumed diagnosis of ovarian insufficiency. She showed a very high E2 con...

hrp0094p2-446 | Thyroid | ESPE2021

The efficacy and short- and long-term side effects of radioactive iodine treatment in pediatric Graves’ disease: a systematic review

Lutterman Sarah , Zwaveling-Soonawala Nitash , Verberne Hein , Verburg Frederik , van Trotsenburg Paul , Mooij Christiaan

Background: Graves’s disease (GD) is the most common cause of hyperthyroidism. Maximal 30% of pediatric GD patients achieve remission with anti-thyroid drugs. The majority of patients therefore require definitive treatment. Both thyroidectomy and radioactive iodine (RAI) are often used as definitive treatment for GD. However, data on efficacy, and short- and long-term side effects of RAI treatment for pediatric GD are relatively scarce.Methods: A ...

hrp0094p2-448 | Thyroid | ESPE2021

Methimazole-induced remission rates in pediatric graves’ disease: A systematic review

van Lieshout Jelmer , Mooij Christiaan , van Trotsenburg Paul , Zwaveling-Soonawala Nitash

Objective: Comparison of studies on remission rates in pediatric Graves’ disease is complicated by lack of uniformity in treatment protocols, remission definition, and follow-up duration. We performed a systematic review on remission rates in pediatric Graves’ disease and attempted to create uniformity by recalculating remission rates based on an intention-to-treat analysis. Results: Of 1,890 articles, 29 articles consist...

hrp0094p2-230 | Fetal, neonatal endocrinology and metabolism (to include hypoglycaemia) | ESPE2021

The utility of continuous glucose monitoring systems in the management of children with persistent hypoglycaemia

Vijayanand Sathyakala , Stevenson Paul G , Grant Maree , Choong Catherine S , Davis Elizabeth A , Abraham Mary B ,

Background: Glucose monitoring is vital in children with persistent hypoglycaemia to reduce the risk of adverse neuro-behavioural outcomes1. Continuous glucose monitoring (CGM) systems provide real-time glucose levels however; information on its usefulness in monitoring glucose levels in this cohort is limited2, 3.Objective: To ascertain the effectiveness of CGM and to evaluate parents’ experie...

hrp0094p2-246 | Growth hormone and IGFs | ESPE2021

Massive Open Online Learning – accelerating knowledge in digital health in the management of children with growth disorders

Dimitri Paul , Fernandez-Luque Luis , Koledova Ekaterina , Bagha Merat , Shabbir Syed Abdul ,

Background: Over the last decade, and in light of the COVID-19 pandemic, there has been a substantial increase in the use of digital health tools to track growth and manage growth disorders in children. Paediatric endocrinologists acknowledge the usefulness of these tools in clinical decision making but lack confidence and skills to use them. Atique et al. designed a Massive Open Online Learning Course (MOOC) to increase digital health literacy, and identified...

hrp0097t16 | Section | ESPE2023

Dose dependent risks of glucocorticoid treatment in classic CAH

Saragolou Kyriakie , Casteràs Anna , Will Charlton R , Barnes Chris , Thornton Paul

Introduction: In congenital adrenal hyperplasia (CAH), glucocorticoid (GC) treatment must perform two functions – to replace cortisol deficiency and to suppress the excess production of adrenal androgens. Unfortunately, androgen suppression usually requires supraphysiologic GC doses, which are associated with serious comorbidities. Our study examined the exposure or dose-dependent relationships between GCs and GC-related adverse events (GCRAEs) and comor...

hrp0097p1-91 | Fetal, Neonatal Endocrinology and Metabolism | ESPE2023

Short- and Long-term Outcomes of Diazoxide Unresponsive Infants with Diffuse Hyperinsulinism

Rafferty Deborah , Truong Lisa , Nedrelow Jonathan , Uffman Chip , Pugenent Burton , Sanchez Irene , Thornton Paul

Background: Severe diazoxide unresponsive hyperinsulinism (DUHI) is most often caused by autosomal recessive variants in the KATP channel genes. Because of the limited medical treatments available, many patients are treated with 98% pancreatectomy. This results in a high rate of diabetes by the age of 15 years. Many centers now try to avoid surgery to prevent the inevitable transition to post-surgical diabetes.Objectives:</strong...

hrp0097p1-367 | Sex Differentiation, Gonads and Gynaecology, and Sex Endocrinology | ESPE2023

Changes in body composition in transgender adolescents during puberty suppression and hormone treatment

Boogers Lidewij , Reijtenbagh Sterre , Wiepjes Chantal , van Trotsenburg Paul , den Heijer Martin , Hannema Sabine

Context: Transgender adolescents can be treated with puberty suppression (PS) using GnRH agonists (GnRHa), and subsequent hormone therapy (HT). Up to this date, it has not been described at what rate body composition in transgender adolescents changes during the first years of treatment. Also, it is unknown whether Tanner stage at which treatment is initiated, might affect this treatment outcome.Methods: Transgender adol...