hrp0092p1-164 | Adrenals and HPA Axis (1) | ESPE2019

The Urinary Steroid Signature of Premature Adrenarche

Janner Marco , Sommer Grit , Groessl Michael , Flück Christa

Background: Adrenarche describes the developmental event of the human adrenal cortex when the zona reticularis increases the synthesis of C19 steroids (DHEA/-S) markedly at around 6-8 years of age. Early appearance of this event is called premature adrenarche (PA) and has been associated with adverse outcomes including polycystic ovary syndrome and metabolic syndrome. Recently novel biosynthetic pathways of androgen production have been revealed, but their rol...

hrp0092p2-3 | Adrenals and HPA Axis | ESPE2019

Contraceptives in Female Adolescents with 21-hydroxylase Deficiency (CAH) - a way to Optimize Treatment with Respect to Androgen Excess? A Pilot Study

Boettcher Claudia , Graf Stefanie , Flück Christa E

Background: At present, treatment of „classic" congenital adrenal hyperplasia (21-hydroxylase-deficiency, 21OHD) consists of glucocorticoid and mineralocorticoid replacement. However, often androgen excess and its negative metabolic impact are difficult to control without accepting glucocorticoid overtreatment, especially in adolescence. In healthy subjects oral contraceptives (containing ethinylestradiol) increase cortisol binding capacity and free c...

hrp0092p1-309 | Diabetes and Insulin (2) | ESPE2019

Level of Glycemic Control in Pediatric Patients with Type 1 Diabetes in Bern: A Cross-Sectional Study

Ch. Zingg Tanja , Dennig Michelle , Sommer Grit , Flück Christa E.

Background: Good glycemic control prevents long-term complications of microvascular and macrovascular diseases in type 1 diabetes (T1DM).We aimed to investigate whether our patients had A1c values <7.5% as recommended by ISPAD and how therapy modality, duration of diabetes and pubertal status affected the metabolic control of our patients. We also set out to compare our quality of care with our results of 2008 and with other publ...

hrp0092p3-83 | Diabetes and Insulin | ESPE2019

Diabetes Mellitus in a 16-Year-Old Boy Developing Multiple Neuro-Endocrine Dysfunctions in the Course: Is it Type 1 Diabetes or Wolfram Syndrome, or Both?

Santi Maristella , Emma Flück Christa , Böttcher Claudia

Introduction: Autosomal recessive mutations in the Wolfram syndrome type 1 gene are responsible for the classical Wolfram syndrome (OMIM_ 222300), also known by the acronym "DIDMOAD" (diabetes insipidus, diabetes mellitus (DM), optic atrophy and deafness). The gene encodes wolframin, a membrane glycoprotein, which helps to regulate the calcium homeostasis in the endoplasmic reticulum of many different tissues, including the pancreatic...