Endocrine Abstracts

Objective: There is evidence that current replacement regimens fail to restore well-being in patients with adrenal insufficiency (AI). No data is available on the effect of different therapeutical regimes (hydrocortisone, prednisolone, cortisone acetate) on the quality of life in these patients.Methods: About 883 patients with adrenal insufficiency were contacted, 526 patients participated (60%) and received a disease specific questionnaire and three sta...

Objective: There is evidence that current replacement regimens fail to restore well-being in patients with adrenal insufficiency. No data is available on the effect of twice or thrice daily administration of hydrocortisone on the quality of life in these patients.Methods: About 883 patients with adrenal insufficiency were contacted, 526 patients participated (60%) and received a disease specific questionnaire and three standardized questionnaires (SF36, ...

Objective: Pheochromocytoma is a rare disease with an incidence of 2–6 per million, and a prevalence of 0.1–0.6% in patients with hypertension. Pheochromocytoma may occur sporadically or as part of hereditary syndrome. According to the latest studies, among patients with non-syndromic pheochromocytoma, up to 24% of tumors may be inherited. However, genetic testing was not generally performed until recently. There is still a debate which pheochromocytoma patient shoul...

Adrenal crisis (AC) is a rare but life-threatening complication in chronic adrenal failure. Here we evaluated frequency, causes and potential risk factors of AC in a large sample of patients with primary AI (PAI) or secondary AI (SAI).In a cross-sectional study 883 patients with AI were contacted by mail. 526 patients agreed to participate and received a disease specific questionnaire. Diagnoses and co-morbidities were verified by review of medical recor...

Objective: There is evidence that current replacement regimens fail to restore well-being in patients with adrenal insufficiency (AI). No data is available on the effect of different therapeutical regimes (hydrocortisone, prednisolone and cortisone acetate) on the quality of life in these patients.Methods: Eight hundred and eighty three patients with adrenal insufficiency were contacted, 526 patients participated (60%) and received a disease specific que...

Adrenocortical carcinoma (ACC) is a rare and heterogeneous malignancy with incompletely understood pathogenesis and poor prognosis. Recent data from the German ACC Registry (n=377) demonstrate an overall 5-year survival of 46%. Survival is clearly stage-dependent (P<0.01) with a 5-year survival of 85% in stage 1, 56% in stage 2, 42% in stage 3, and 16% in stage 4, respectively.In stages I –III open surgery by an expert surgeon aimi...

Background: Adrenocortical carcinoma (ACC) is a rare and heterogeneous malignancy with incompletely understood pathogenesis and poor prognosis. Computerized tomography (CT) and magnetic resonance imaging (MRI) are routinely performed for imaging of the adrenal mass and for standard staging of chest and abdomen as lung and liver are the primary organs for metastatic spread in ACC. Contrast ultrasound is a non-invasive procedure which has been shown to have a high sensitivity an...

Introduction: Streptozotocin (SZ) is an active drug for the treatment of advanced adrenocortical carcinoma (ACC) in a minority of patients with an objective response rate of <10%. It has been reported that expression of glucose transporter-2 (GLUT-2) is essential for SZ to enter tumor cells and that high activity of O-6-methylguanine-DNA methyltransferase (MGMT) counteracts the alkylating effect of SZ. Therefore, we aimed to clarify the role of GLUT-2 and MGMT in the respo...

Background: Potentially fatal adrenal crises (AC) still occur in educated patients with adrenal insufficiency (AI). Our study investigates clinical and biochemical fingerprints of increased susceptibility to AC.Material and methods: 199 patients with chronic AI (disease duration ≥6 years), classified into tertiles according to the frequency of AC/patient-years (group 1 no AC, group 2 ≤0.07 AC/py, group 3 >0.07 AC/py). Following parameters...

Background: Patients with congenital adrenal hyperplasia due to 21-hydroxylase deficiency (CAH) require lifelong glucocorticoid replacement therapy, including stress dose adjustment to prevent life-threatening adrenal crises (AC). Previous studies indicate a high incidence of inadequate stress dose adjustment and AC in patients with CAH. The aim of this study was to prospectively assess AC incidence, frequency and details of stress dose adjustment as well as knowledge of the d...