Endocrine Abstracts

Introduction: Optimizing glucocorticoid (GC) therapy in patients with congenital adrenal hyperplasia (CAH) remains a challenge. While overdosing may result in Cushing’s syndrome, underdosing is associated with female virilization and adrenal insufficiency. This study evaluated day-profiling of salivary 17-hydroxyprogesterone (17OHP) for the biochemical control of adolescents with CAH due to 21-hydroxylase deficiency.Methods: Twelve patients (4 males...

Introduction: Pasireotide (SOM230) is a novel multi-ligand somatostatin analogue with high binding affinity for four of the five somatostatin receptor subtypes (sst1,2,3 and sst5). A randomized study of 59 patients showed that pasireotide effectively controls GH and IGF-I levels in patients with acromegaly and reduces pituitary tumor size. The impact of pasireotide on GH levels during glucose suppression and glucose metabolism in 12 patients enrolled in t...

Introduction: Treatment of poorly differentiated neuroendocrine tumors (NET) usually includes chemotherapeutic intervention. However, both the rarity and the heterogeneity of the disease have led to relatively few clinical trials. This study evaluated the outcome of two chemotherapy regimens in patients suffering from undifferentiated and histologically confirmed NET.Methods: Eighteen patients (11 males; age 56.7±2.5) with proven progressive disease...

Adrenal pheochromocytomas are neoplasms characterized by catecholamine excess. We recently reported on the diagnostic value of plasma metanephrines measured by RIA for the diagnosis of pheochromocytoma. However, RIA may not be used in many laboratories.This study evaluated plasma and urinary metanephrines determined by a newly available ELISA as well as serum chromogranin A (CgA) for the diagnosis of pheochromocytoma. Spontaneous blood samples and 24h-ur...

Background: The diagnosis of hyperaldosteronism is hindered by the absence of a well-defined gold-standard. CYP11B2 (aldosterone synthase) belongs to the steroid-metabolizing enzymes catalyzing the last step of the aldosterone synthesis. To establish a molecular marker for aldosterone-producing adenomas, we compared the expression in various adrenal tumors.Methods: Tissue from 20 aldosterone-producing adenomas (APA), 12 hyperplasias associated with hyper...

Introduction: The aim of the present study was to explore pain prevalence, pain locations and its impact on everyday activities and health-related quality of life (QoL) in a large cohort of patients with acromegaly.Methods: We conducted a survey at three major centers specialized on the treatment of pituitary diseases. 124 patients with acromegaly (mean age 56.6y ± 13.5) filled in a self-constructed questionnaire on acromegaly symptoms, pain includi...

Plasma aldosterone concentration (PAC) to active renin concentration (ARC) ratio (ARR) is an established screening tool for primary aldosteronism. Previously we provided reliable cut-offs measuring PAC by a radioimmunoassay (RIA; Byk&DiaSorin) und ARC by an established immunoluminometric assay (Nichols Institute Diagnostics). Since the latter one is not longer available, we aimed to establish cut-offs for currently available assays.One hundred seven ...

Metyrapone treatment in endogenous Cushing’s syndrome. Results from a prospective multicenter, open-label, phase III/IV study: PROMPTBackground: Metyrapone is a steroidogenesis inhibitor approved in Europe for the treatment of endogenous Cushing’s syndrome (CS) based on observational retrospective studies published over more than 50 years. We present data from the first prospective study designed to confirm metyrapone efficacy and good tolera...

BackgroundRetrospective studies led to European approval of the steroidogenesis inhibitor Metyrapone for the treatment of endogenous Cushing’s syndrome (CS). We prospectively showed good efficacy and safety of Metyrapone after 12 weeks (Wk12) treatment in the phase III/IV PROMPT study and now report results of an extension study (EXT) sponsored by HRA Pharma Rare Diseases.DesignThis was a single arm, ope...

BackgroundPrenatal dexamethasone treatment (Pdex) has been used since the 1980s to prevent virilization in female offspring suspected to have congenital adrenal hyperplasia (CAH). However, due to lack of strong evidence for its best practice as well as limited data regarding longterm adverse effects, use of dex is highly controversial. This study reveals the current medical practice regarding Pdex in female fetuses at risk of CAH due to 21hydroxylase def...