Endocrine Abstracts

Introduction: Glucocorticoid (GC) replacement therapy is administered to patients with adrenal insufficiency (AI) in multiple daily doses; however, there are no consensus guidelines on the optimal regimen.Methods: The European Adrenal Insufficiency Registry (EU-AIR) is a multinational, multicentre, observational study sponsored by Shire, initiated to monitor the long-term safety of GC replacement in patients with chronic AI. We analysed baseline data to ...

Introduction: No consensus guidelines currently exist as to the optimal glucocorticoid regimen in adrenal insufficiency (AI). In clinical practice physicians utilise a number of different glucocorticoids, usually administered in several doses throughout the day.Methods: The EU-AIR registry is a European multinational, multicentre, prospective, observational study sponsored by Shire inclusive of all patients with AI irrespective of aetiology. We analysed ...

Introduction: During recent years many authors have advocated lower hydrocortisone doses in patients with primary adrenal insufficiency (PAI) mainly due to worries for non-physiological effects like increased cardiovascular risk and bone resorption, but very little attention has been drawn to fludrocortisone dosing. Our main hypothesis was that that the higher hydrocortisone dose, the lower the fludrocortisone dose and vice versa.Design: The Eur...

Introduction: Current understanding about the frequency of adrenal crisis in patients with adrenal insufficiency (AI) relies exclusively on retrospective data (~6.3 adrenal crisis per 100 patient-years). In addition, no data are available about the frequency of intercurrent illness events in AI patients. For the first time we prospectively used a patient’s diary in recording events of adrenal crisis and intercurrent illness in AI patients.Design: Th...

Introduction: Data concerning patients with adrenal insufficiency (AI) have been limited by small numbers of patients reflecting the relatively low prevalence of this disease. The European Adrenal Insufficiency Registry (EU-AIR) is a multicenter observational study of patients with both primary and secondary AI. Enrollment commenced August 2012. Comprehensive demographic data, aetiology of AI, and details of glucocorticoid replacement therapy are collected at baseline. Safety ...

Background: Women with congenital adrenal hyperplasia (CAH) due to 21-hydroxylase deficiency have a poor Quality of Life (QoL) compared to the general population and are less satisfied with their body appearance. Data indicates that psychosocial determinants such as good professional and general support could be associated with an improved QoL. Since there is only little data available that has been deduced from samples of small size this effect should be further examined....

Introduction: A once-daily modified-release formulation of hydrocortisone (Plenadren®) has been developed to better mimic physiological cortisol secretion in the treatment of patients with adrenal insufficiency (AI). Here, we describe EU-AIR, an ongoing post-authorization observational study (registry) designed to collect information on AI management and to assess the long-term safety of Plenadren® compared with traditional glucocorticoid replac...

Adrenal crisis (AC) is a life threatening condition in chronic adrenal insufficiency (AI). Furthermore, recent data demonstrate increased mortality in this patient group.Incidence and causes of adrenal crisis as well as general morbidity and hospitalization was assessed in 472 patients in a prospective trial. Patients were contacted via mail every 6 months and in case of AC additionally by phone to evaluate the exact circumstances of AC. Criteria for AC ...

Adrenocortical carcinoma (ACC) is part of the Li-Fraumeni tumour syndrome which is due to germline mutations in TP53. Recent studies demonstrate low penetrance mutations leading to later tumour manifestation. Furthermore, in ACC mutations outside the hotspot region have been found. TP53-polymorphisms have also been described to impact on p53 function. We, therefore investigated TP53 sequence alterations in a large cohort of adult patients with ACC.Method...

Introduction: Chemokines and their receptors (CR) have been demonstrated to be involved in tumour growth and site specific metastasis. Furthermore, several cytokines have been described to modulate adrenocortical function. Therefore, we have investigated the expression pattern and functional activity of chemokine receptors and of corresponding chemokines in adrenocortical tissue.Methods: Chemokine and CR expression was assessed by RT-PCR in normal adrena...