Searchable abstracts of presentations at key conferences in endocrinology

ea0090p77 | Diabetes, Obesity, Metabolism and Nutrition | ECE2023

En route to sarcopenia biomarkers: dp-ucMGP in relation to muscle mass and function

Tandl Veronika , Schweighofer Natascha , Trummer Olivia , Haudum Christoph W. , Lind Alice F. , Kolesnik Ewald , Mursic Ines , Scherr Daniel , Zirlik Andreas , Pieber Thomas R. , Verheyen Nicolas D. , Obermayer-Pietsch Barbara

Sarcopenia is characterized by progressive loss of both mass and function of skeletal muscle when ageing. It poses a serious issue for healthcare systems, as it is associated with increased risk of falls, osteoporosis, and mortality. To date, no ideal serum biomarkers for the diagnosis of this complex condition have been identified. Matrix-GLA-protein (MGP), primarily known as a vitamin K dependent calcification inhibitor, is present and active in bone, muscle, and adipose tis...

ea0070ep190 | Diabetes, Obesity, Metabolism and Nutrition | ECE2020

Matrix–GLA–protein and cardiovascular parameters: uc–dp MGP vs total MGP

Schweighofer Natascha , Strasser Moritz , Haudum Christoph , Schmidt Albrecht , Kolesnik Ewald , Pieske Burkert , Pieber Thomas , Obermayer–Pietsch Barbara

Matrix–GLA–protein ( MGP), independent of its posttranslational modification(s), has been associated with bone parameters and cardiovascular risk in patients with and without type 2 diabetes mellitus (T2DM). We investigated whether uncarboxylated, dephosphorylated MGP (uc–dp MGP), total MGP or both are associated with anthropometric and cardiovascular parameters such as carotid to femoral pulse wave velocity (PWV), intima–media thickness (IMT) and relat...

ea0039p1 | (1) | BSPED2015

Mutations in HS6ST1 cause self-limited delayed puberty (DP) in addition to idiopathic hypogonadotropic hypogonadism (IHH)

Howard Sasha , Poliandre Ariel , Storr Helen L , Metherell Louise A , Cabrera Claudia , Warren Helen , Barnes Michael , Wehkalampi Karoliina , Guasti Leonardo , Dunkel Leo

Background: Self-limited DP often segregates in an autosomal dominant pattern, but in the majority of patients the neuroendocrine pathophysiology and its genetic regulation remain unclear. By comparison, many genes have been identified where loss-of-function mutations lead to IHH. Despite likely overlap between the pathophysiology of DP and conditions of GnRH deficiency, few studies have examined the contribution of mutations in IHH genes to the phenotype of DP.<p class="a...

ea0004dp26 | Diabetes, metabolism and cardiovascular | SFE2002

Diol-ginsenosides delayed the onset of diabetes possibly by the increase in beta-cell resistance in DP-BB rats

Moon C , Ju C , Kim M , Chae S , Kim G , Chung A

The present study was designed to investigate the effects of Diol-ginsenosides, major active components of Panax Ginseng, on the development of diabetes in the Diabetes Prone-BB rats(DP-BB rats). Administration of Diol-ginsenosides to DP-BB rats from age 3-4 weeks to age 11-12 weeks (daily, 1mg/g body weight) resulted in a markedly delayed the onset of diabetes. Islets of the treated rats showed no insulitis or only early/intermediate-stage insulitis and preserved higher pancr...

ea0086oc4.5 | Adrenal and Cardiovascular | SFEBES2022

Comparison of prednisolone and modified-release hydrocortisone capsules in the treatment of congenital adrenal hyperplasia: dose and disease control

Rees Aled , Merke Deborah , Arlt Wiebke , Pierriere Aude , Hirschberg Angelica , Juul Anders , Newell-Price John , Perry Colin , Prete Alessandro , Reisch Nicole , Stikkelbroeck Monica , Touraine Philippe , Coope Helen , Lewis Alexander , Porter John , Ross Richard

Introduction: First-line treatment for congenital adrenal hyperplasia (CAH) is hydrocortisone1. When adequate control is not achieved, prednisolone (or its prodrug prednisone) are often used. However, there has been no formal comparison of disease control in CAH comparing prednis(ol)one vs hydrocortisone and patients are often on a glucocorticoid dose that exceeds the guideline recommended dose of hydrocortisone (≤25 mg/day)1,2. We report an interim...

ea0099p215 | Adrenal and Cardiovascular Endocrinology | ECE2024

Incidence of adrenal crisis in Congenital Adrenal Hyperplasia (CAH) patients during a prospective monitored long-term study of modified-release hydrocortisone (MRHC) capsules, (Efmody)

John M. Ross Richard , Merke Deborah P , Mallappa Ashwini , Arlt Wiebke , DeLaPerriere AudeBrac , Hirschberg Angelica , Newell-Price John DC , Prete Alessandro , Rees Aled , Reisch Nicole , Quinkler Marcus , Touraine Philippe A , Maltby Kerry , Quirke Jo , Aslam Naila , Coope Helen , Porter John

Background: Adrenal crisis is the leading cause of excess mortality in patients with CAH1. Retrospective studies report an adrenal crisis incidence of 5-10/100 patient years (PY), with mortality 0.5/100 PY2. Modified-release hydrocortisone (MRHC) capsules, (Efmody), replicate cortisol diurnal rhythm and improve androgen control in CAH compared to standard glucocorticoid therapy2. Here, we report the incidence of adrenal crisis in CAH patients f...

ea0019p304 | Steroids | SFEBES2009

Modified-release hydrocortisone to provide circadian cortisol profiles

Debono M , Ghobadi C , Rostami-Hodjegan A , Huatan H , Campbell MJ , Newell-Price J , Darzy K , Merke DP , Arlt W , Ross RJ

Background: A basic tenet for hormone replacement is to replicate physiology but this is rarely if ever achieved. The adrenal glucocorticoid, cortisol, has a distinct circadian rhythm regulated by the brain’s central pacemaker. Loss of the cortisol circadian rhythm is associated with metabolic abnormalities, depression, fatigue and a poor health-related quality of life. Based on pharmacokinetic modelling we have developed a modified-release hydrocortisone (MR-HC) and test...

ea0081rc2.6 | Rapid Communications 2: Adrenal and Cardiovascular Endocrinology 1 | ECE2022

Modified release hydrocortisone capsules (MRHC, Efmody) improve control of congenital adrenal hyperplasia (CAH) on a lower glucocorticoid dose than standard treatment

Rees Aled , Merke Deborah P , Arlt Wiebke , Brac De La Periere Aude , Linden Hirschberg Angelica , Juul Anders , Mallappa Ashwini , Newell-Price John D. C. , Graham Perry Colin , Prete Alessandro , Reisch Nicole , Stikkelbroeck Monica , Touraine Philippe A , Coope Helen , Porter John , Ross Richard John M

Background: The therapeutic goal in CAH is androgen control on the lowest achievable glucocorticoid dose, preferably an adrenal replacement dose (15-25 mg hydrocortisone a day)1. However, the glucocorticoid dose required to control androgens frequently exceeds that required for adrenal replacement2. Modified-release hydrocortisone (MRHC) capsules, (Efmody, Diurnal Ltd, Cardiff, UK), replicate cortisol diurnal rhythm and improve CAH control compared to sta...

ea0081rc2.8 | Rapid Communications 2: Adrenal and Cardiovascular Endocrinology 1 | ECE2022

Fertility in congenital adrenal hyperplasia (CAH) patients on modified release hydrocortisone capsules (MRHC, Efmody)

Tschaidse Lea , Quinkler Marcus , Arlt Wiebke , Brac De La Perriere Aude , Linden Hirschberg Angelica , Juul Anders , Mallappa Ashwini , Merke Deborah P , Newell-Price John DC , Perry Colin Graham , Prete Alessandro , Rees Aled , Stikkelbroeck Monica , Touraine Philippe A , Coope Helen , Porter John , Ross Richard John M , Reisch Nicole

Background: Fertility in CAH women is impaired: 0.25 live births vs 1.8 in the UK population and 45% have irregular menses vs 13.6% in healthy women1. Male fertility is also impaired in CAH with oligospermia reported in 48%2. Treatment of infertility usually involves increasing the glucocorticoid dose to normalise adrenal androgens and progesterone to facilitate ovulation and implantation, respectively. Modified-release hydrocortisone (MRHC) capsules, (Ef...

ea0090oc5.3 | Oral Communications 5: Adrenal and Cardiovascular Endocrinology 1 | ECE2023

Switching patients with Congenital Adrenal Hyperplasia to Modified release hydrocortisone capsules: relative bioavailability and disease control

Ross Richard John M , Rees Aled , P. Merke Deborah , Arlt Wiebke , Brac De La Perriere Aude , Linden Hirschberg Angelica , Juul Anders , D. C. Newell-Price John , Perry Colin Graham , Prete Alessandro , Reisch Nicole , Stikkelbroeck Monica , A. Touraine Philippe , Mallappa Ashwini , Aslam Naila , Coope Helen , Porter John

Background: Modified-release hydrocortisone (MRHC) capsules (Efmody, Diurnal Ltd, Cardiff, UK), have been developed to replicate the cortisol diurnal rhythm and shown to improve CAH disease control1. We have examined relative bioavailability of MRHC and disease control of congenital adrenal hyperplasia (CAH) patients switched from standard therapy to MRHC.Methods: An open label, randomised, 2 period, crossover study comparing the relative bioavailability...