Searchable abstracts of presentations at key conferences in endocrinology

ea0094cc1 | Section | SFEBES2023

Primary Unilateral Macronodular Adrenal Hyperplasia (PUMAH) With Concomitant Glucocorticoid And Androgen Excess due to KDM1A inactivation and constitute MC2R activation

Elhassan Yasir , Appenzeller Silke , Landwehr Laura , Popat Dillon , Gilligan Lorna , Goh Edwina , Diaz-Cano Salvador , Kircher Stefan , Gramlich Susanne , Sutcliffe Robert , Thangaratinam Shakila , Chan Li , Fassnacht Martin , Arlt Wiebke , Ronchi Cristina

Clinical vignette: We investigated a 33-year-old woman diagnosed during pregnancy with a 7cm unilateral adrenal mass associated with severe ACTH-independent glucocorticoid and androgen excess, a steroid phenotype usually indicative of adrenocortical carcinoma. Pregnancy had been achieved with in-vitro fertilisation on the assumption of underlying PCOS. Neonatal death occurred soon after emergency delivery due to foetal growth arrest at 26 weeks gestation. Hist...

ea0099rc3.1 | Rapid Communications 3: Adrenal and Cardiovascular Endocrinology | Part I | ECE2024

Biochemical control with dose reduction in chronic glucocorticoid therapy over 4 years: A phase III extension study of Chronocort (Efmody) in the treatment of Congenital Adrenal Hyperplasia (CAH)

John M. Ross Richard , Merke Deborah P. , Arlt Wiebke , De La Perriere Aude Brac , Hirschberg Angelica , Newell-Price John D.C. , Prete Alessandro , Rees Aled , Reisch Nicole , Quinkler Marcus , Touraine Philippe A. , Maltby Kerry , Quirke Jo , Aslam Naila , Coope Helen , Porter John

Background: Management of CAH involves replacing cortisol deficiency and reducing raised adrenal androgens, however the supraphysiological glucocorticoid doses often required to treat hyperandrogenism are associated with poor long-term health outcomes. Modified-release hydrocortisone (MRHC) capsules, Efmody, replicate cortisol diurnal rhythm and improve control of CAH compared to standard glucocorticoid therapy. Here we report changes in glucocorticoid daily dose and 9am 17-hy...

ea0099rc3.4 | Rapid Communications 3: Adrenal and Cardiovascular Endocrinology | Part I | ECE2024

CHAMPAIN study: Initial results from a phase II study of efficacy, safety and tolerability of modified-release hydrocortisones: Chronocort® (Efmody®) versus Plenadren®, in primary adrenal insufficiency

Prete Alessandro , Theiler-Schwetz Verena , Arlt Wiebke , Chifu Irina-Oana , Harbeck Birgit , Napier Catherine , Newell-Price John D.C. , Rees Aled , Reisch Nicole , Stalla Gunter K. , Aslam Naila , Coope Helen , Maltby Kerry , Porter John , Quirke Jo , John M. Ross Richard

Background: Current glucocorticoid replacement regimens for patients with primary adrenal insufficiency (PAI) mean patients wake with either low or undetectable cortisol levels1, associated with fatigue and a reduced quality of life (QoL)2. Plenadren® (Takeda, UK) is a once-daily modified-release formulation of hydrocortisone that replaces daytime cortisol levels whereas Chronocort® (modified-release hydrocortisone hard capsules, Diurnal, UK) wh...

ea0099p13 | Adrenal and Cardiovascular Endocrinology | ECE2024

Morning cortisol levels in patients with established primary adrenal insufficiency

Prete Alessandro , Theiler-Schwetz Verena , Arlt Wiebke , Chifu Irina-Oana , Harbeck Birgit , Napier Catherine , Newell-Price John D.C. , Rees Aled , Reisch Nicole , Stalla Gunter K. , Aslam Naila , Coope Helen , Maltby Kerry , Porter John , Quirke Jo , Ross Richard John M.

Background: Primary adrenal insufficiency (PAI) is rare: prevalence ~100–140/million and incidence 4:1 000 000/year in Western societies 1. The diagnosis of PAI is suggested by an early-morning cortisol <140 nmol/l (5 μg/dl) 1. The commonest cause in adults is autoimmunity (~90% in Western countries) and it is generally considered progressive once the diagnosis is made, although it has been reported that residual cortis...

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...

ea0099ep919 | Adrenal and Cardiovascular Endocrinology | ECE2024

Primary unilateral macronodular adrenal hyperplasia with concomitant glucocorticoid and androgen excess and KDM1A inactivation

Elhassan Yasir , Appenzeller Silke , Landwehr Laura-Sophie , Lippert Juliane , Popat Dillon , C. Gilligan Lorna , Abdi Lida , Goh Edwina , Diaz-Cano Salvador , Kircher Stefan , Gramlich Susanne , Sutcliffe Robert , Thangaratinam Shakila , Chan Li , Fassnacht Martin , Arlt Wiebke , L Ronchi Cristina

Background: Primary bilateral macronodular adrenal hyperplasia (PBMAH) is a rare cause of Cushing’s syndrome. Primary unilateral macronodular adrenal hyperplasia with concomitant glucocorticoid and androgen excess has never been studied before.Methods: We investigated a woman with a large, heterogeneous 7 cm adrenal mass (with a radiologically normal contralateral adrenal) and adrenocorticotropic hormone (ACTH)-independent glucocorticoid and androge...

ea0077ec1.3 | Clinical Endocrinology Trust Best Abstract Clinical | SFEBES2021

Phase 3 and extension study of modified-release hydrocortisone in the treatment of congenital adrenal hyperplasia

Merke Deborah P , Mallappa Ashwini , Arlt Wiebke , De La Perriere Aude Brac , Hirschberg Angelica Linden , Juul Anders , Newell-Price John D C , Perry Colin Graham , Prete Alessandro , Rees Aled , Reisch Nicole , Stikkelbroeck Monica , Touraine Philippe A , Lewis Alexander , Maltby Kerry , Treasure Peter , Porter John , Ross Richard John M

Background: Patients with congenital adrenal hyperplasia (CAH) due to classic 21-hydroxylase deficiency have poor health outcomes. We compared disease control in CAH adults treated with modified release hydrocortisone (MRHC, Chronocort®, Diurnal Ltd) versus standard glucocorticoid (GC).Methods: 6-month, Phase 3 study in 122 patients randomised to either MRHC twice daily or standard GC followed by safety extension study on MRHC. Patients had 24-hr 17...

ea0077p7 | Adrenal and Cardiovascular | SFEBES2021

Simulation via Instant Messaging – Birmingham Advance (SIMBA): Impact of online simulation-based learning on doctors’ confidence in managing cases during the COVID-19 pandemic

Zhou Dengyi , Ali Anisah , Warmington Emily , Abdi Zakee , Nirmal Rachel , Sakthivel Pavithra , Soran Vina , Elhariry Maiar , Ooi Emma , Ying Cai , Evans Nia , Arlt Wiebke , Boelart Kristien , Karavitaki Niki , Tait Karen , Narendran Parth , Papanikolaou Nikoleta , Jayasena Channa , Davitadze Meri , Melson Eka , Kempegowda Punith

Background: SIMBA is a simulation-based learning platform designed to increase clinicians’ confidence in managing various clinical scenarios. The restriction of face-to-face learning during the COVID-19 pandemic led to switching Simulation via Instant Messaging – Birmingham Advance (SIMBA) to entirely virtual sessions.Objective: To explore SIMBA’s effectiveness to sustain medical education in endocrinology during the pandemic.<p class=...

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...

ea0081rc2.7 | Rapid Communications 2: Adrenal and Cardiovascular Endocrinology 1 | ECE2022

Improved biochemical control with modified-release hydrocortisone overturns the impaired fludrocortisone effect in salt-wasting CAH patients

Tschaidse Lea , Reisch Nicole , 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 Phillippe A , Coope Helen , Porter John , Ross Richard John M , Quinkler Marcus

Background: Patients with salt-wasting congenital adrenal hyperplasia (CAH) due to classic 21-hydroxylase deficiency require glucocorticoid (GC) and mineralocorticoid (MC) replacement therapy. Recently, it was shown that twice daily modified-release hydrocortisone hard capsules (MRHC, Efmody®, Diurnal Ltd) improved control of CAH with most patients showing good disease control versus standard GC therapy. However, no data has been reported on the renin-angiotens...