Endocrine Abstracts

To clarify the role of the pituitary in zebrafish interrenal development we investigated wildtype (wt) zebrafish and 3 pituitary mutants: aal (with lactotrophes only), and pit1 (with corticotrophes only), fgf3-lia (lacking all adenohypophyseal cell types) and in MC2R knockdown embryos (MC2R morphants).The interrenal primordium (IP) is detectable at 22hpf (hours post fertilization) as bilateral clusters of ff1b expressing cells which fuse to one domain at...

Introduction Retrospective analyses suggest that patients with adrenal insufficiency (AI) have an increased risk for cardiovascular diseases which was mainly attributed to non-physiological cortisol profiles and/or supraphysiological replacement doses.Material and methodsWe analyzed the 24-hour blood pressure (BP) profiles in patients with primary (PAI) and secondary (SAI) AI. BP threshold criteria for hypert...

Introduction: Retrospective analyses suggest an increased risk for cardiovascular diseases in patients with adrenal insufficiency (AI), mainly due to supraphysiological replacement doses.Objective: To evaluate the 24-hour blood pressure (BP) profile in patients with primary (PAI) and secondary (SAI) AI.Material and methods: BP threshold criteria for hypertension and dipping status of the 2018 ESC/ESH guidelines were used (24-h: &#8...

Introduction: Glucocorticoid (GC) replacement regimens in adrenal insufficiency (AI) only roughly correspond to physiological steroid profiles. Moreover, control of substitution quality is difficult and signs of clinically relevant mild chronic over- or under-replacement might be omitted. FKBP5 regulates GC receptor sensitivity by reducing its affinity to cortisol when bound to the receptor complex. FKBP5 methylation has been inversely correlated with cortisol levels both in h...

Objective: We have developed (123/131I)iodometomidate (IMTO) for specific adrenal imaging and radiotherapy of adrenocortical carcinoma. IMTO binds with high affinity and specificity to the two adrenocortical enzymes aldosterone synthase (CYP11B2) and 11β-hydroxylase (CYP11B1). Metabolic analysis has revealed very rapid metabolization of IMTO both in vitro and in vivo by hepatic esterases. Due to the low metabolic stability of (123/131</su...

Objective: The saline infusion test (SIT) is a standard confirmatory test for primary aldosteronism (PA) and based on impaired aldosterone suppression in PA compared to essential hypertension (EH). In the past, aldosterone was quantified using immunoassays (IA). Due to the more widespread use of liquid chromatography tandem mass spectrometry (LC-MS/MS) in clinical routine, we aimed at a method-specific aldosterone threshold for the diagnosis of PA during SIT and explored the d...

Introduction: Even though it is well known that long-term complications, such as renal insufficiency and brain calcifications can occur in patients with hypoparathyroidism (HPT), the risk of cardiovascular diseases still remains unclear.Objective: To perform a systematic assessment of cardiovascular function in a well-characterized cohort of patients with HPT compared to population-based controls.Methods: 133 patients with chronic ...

Background: Adrenal crisis (AC) is a life-threatening complication in patients with congenital adrenal hyperplasia due to classical 21-hydroxylase deficiency (21-OHD). AC was defined as an acute state of health impairment which required i.v. glucocorticoid administration and hospital admission. No data on AC over life-time in 21-OHD is available.Study design: In a retrospective study AC was studied following two approaches: a) questionnaire-based: 122 ad...

Objectives: No immunohistochemical marker has yet been established to reliably differentiate adrenocortical tumors from other adrenal masses (e.g. metastases). Thus, a panel of several markers like melan A and inhibin is currently used for this purpose, but suffers from limited diagnostic accuracy. We hypothesized that expression of steroidogenic factor 1 (SF-1), a nuclear transcription factor involved in adrenal development and steroidogenesis, might hold significant diagnost...

Congenital adrenal hyperplasia (CAH) represents the most common inborn metabolic error and is associated with significant morbidity and mortality. There is a paucity of clinical data in adult patients, a lack addressed by congenital adrenal hyperplasia adult study executive (CaHASE), a multicentre cross-sectional study of the health status of adults with CAH that recruited 203 patients from 17 centres (54% of patients contacted). Psychometric evaluation was assessed by validat...