Endocrine Abstracts

Autoimmune Addison’s disease (AAD) results from the immune mediated selective destruction of adrenal steroid hormone-secreting cells. Autoantibodies (Abs) against 21-hydroxylase (21OH) are diagnostic present in 85–90% newly diagnosed patients. Its genetic susceptibility is conferred by human leukocyte antigen (HLA) DQ2 and DQ8. In many patients autoimmunity extends forming the autoimmune polyglandular syndrome type 2 (APS-2). The aim of this study was to test, whethe...

Introduction: The role of surgery for recurrent ACC is not well defined. Therefore, we used the German ACC Registry to evaluate treatment modalities after first recurrence in patients amenable to surgery.Methods: Patients with recurrence after radical resection and follow-up data were included. Patients with extensive metastasized disease (>2 tumoral organs, peritoneal carcinomatosis) were excluded. Progression-free and overall survival (PFS/OS) were...

Subject: Adrenal tumours (AT) are often detected incidentally and represent a variety of differential diagnoses with variable therapeutic consequences. We have recently developed [123I]Iodometomidate ([123I]IMTO), that specifically binds to adrenal CYP11B enzymes as a SPECT tracer for adrenal scintigraphy. This radiotracer is now evaluated in patients with adrenal tumours in an ongoing clinical trial. The study was approved by the ethical committee and pa...

Objective: Hypokalemic Conn’s syndrome is a rare disease with a prevalence of 0.5% in unselected hypertensive populations. However, recent studies indicate a higher prevalence of a milder variant of Conn’s syndrome, reaching 10% in some studies. Long term outcome and health care costs of hypo- and normokalemic variants are largely unknown. The National Conn’s Registry is an initiative to create a national database of sufficient epidemiological strength to invest...

Objective: Primary aldosteronism (PA) is associated with vascular end organ damage. We evaluated the newly established German Conn’s Register for evidence of renal impairment and compared the data with renal function from hypertensive subjects from an epidemiologic cohort.Methods: The registry was founded in 2006 and has at present 7 participating centres in 5 locations. Data are entered in a central electronic database. Up to July 2007 555 patients...

Objectives: Adrenocortical carcinoma (ACC) is a rare malignancy with incompletely understood pathogenesis and poor prognosis. Overexpression of epidermal growth factor receptor (EGFR) has been demonstrated in several tumors and is partly associated with a more aggressive phenotype and a worse prognosis. In addition, targeting the EGFR tyrosine kinase represents a successful new therapeutic strategy, e.g. in non-small cell lung cancer. Therefore, we investigated the role of EGF...

Objective: The incidence of childhood obesity and type 2 diabetes has reached epidemic proportions. Glucocorticoid excess causes central obesity and diabetes mellitus as seen in Cushing’s syndrome. The 11beta-hydroxysteroid dehydrogenase type 1 enzyme (11beta-HSD1), which is predominantly expressed in liver and adipose tissue, regenerates active cortisol from inactive cortisone. Increased 11beta-HSD1 may cause tissue-specific Cushing syndrome with central obesity and impa...

Objective: The incidence of childhood obesity and type 2 diabetes has reached epidemic proportions. Glucocorticoid excess causes central obesity and diabetes mellitus as seen in Cushing’s syndrome. The 11beta-hydroxysteroid dehydrogenase type 1 enzyme (11beta-HSD1), which is predominantly expressed in liver and adipose tissue, regenerates active cortisol from inactive cortisone. Altered 11beta-HSD1 may cause tissue-specific Cushing syndrome with central obesity and impair...

BackgroundThe ESE-ENSAT guidelines emphasize the role of local therapies and suggest radiotherapy (RT) as an individualized treatment in patients with advanced ACC. However, the evidence for this recommendation is very low. The aim of this study was to retrospectively investigate the efficacy and tolerance of radiation therapy in advanced ACC.MethodsWe screened all patients in five European reference centers ...

Context11-oxygenated C19 steroids have recently gained attention as markers of androgen control in congenital adrenal hyperplasia (CAH) due to 21hydroxylase deficiency (21OHD). However, they have not yet been systematically investigated in the context of different glucocorticoid (GC) replacement regimens and in particular not in patients receiving new modified-release formulations.MethodsCross-sectional singl...