Endocrine Abstracts

Background: Nelson syndrome is a rare complication of Cushing disease (CD), which occurs either in patients with very aggressive CD or as a result of misdiagnosis. Clinical characterization of patients with Nelson syndrome could provide important insights in management of the disorder.Methods: We analyzed medical records of patients with CD between 2015 and 2021 to identify those who developed Nelson syndrome.Results: Nine patients...

Introduction: Aggressive corticotropinomas are more invasive than other pituitary tumors. Recent reports have documented the efficacy of temozolomide alone or in combination with an anti-angiogenic agent –bevacizumab in invasive pituitary adenomas treatment.Objective: Presentation a case of 56-year-old woman initially diagnosed as Cushing’s disease and the analysis of treatment procedures with special concentration on temozolomide and bevacizum...

The Nelson’s syndrome (NS) is characterized by hyperpigmentation, sellar growing mass, and increased plasma ACTH concentrations The management of NS remains difficult: neurosurgery represent the first choice therapy. Many drugs have been used with variable results: cyproeptadine, GABA-agonists, somatostatin analogues, chemiotherapy, PPAR-γ-agonists, and dopamin-agonists.Aim of this study was to evaluate the efficacy of cabergoline administratio...

IntroductionWe have previously shown that patients with pituitary Cushing's have impaired quality of life vs. other pituitary tumour groups. Whether this is related to direct neural effects of excess ACTH or cortisol is uncertain. In order to address this, we compared patients who had undergone definitive treatment for pituitary vs adrenal Cushings.DesignWe assessed 14 patients with biochemically cured pituit...

B-type natriuretic peptide (BNP) is a hormone secreted from the heart in response to volume load and serves clinically as a reliable biomarker in the diagnosis of cardiac dysfunction and heart failure. As patients with heart failure present an increased risk for developing diabetes, we aimed to investigate the role of BNP on parameters of glucose metabolism in a placebo-controlled crossover study performed in 10 healthy volunteers (25±1 years; BMI 23±1 kg/m2</su...

Mitogenic signaling by receptor tyrosine kinases that involve increased activity of phosphatidylinositol-3-kinase (PI3K) and over-activation of protein kinase B (PKB/Akt) triggers a cascade of responses that drive tumour progression in a variety of human cancers. Some of these events have been associated with diminished expression of the cell cycle inhibitor p27 through inhibition of a Forkhead transcription factor (FKHR-L1) by Akt, while some others have recently been reporte...

Nelson’s syndrome (NS) is a potentially severe complication following bilateral adrenalectomy for Cushing’s disease (CD). Series assessing outcomes of treatments for NS are limited by small sample size, often short follow-up and variability of success criteria. We performed a UK multi-centre study aiming to review outcomes of primary treatment for NS. Clinical, laboratory, imaging data were collected. Kaplan-Meier method, log-rank test, Cox regression analysis were u...

Background: Phaeochromocytomas and paragangliomas are familial in up to 25% of cases and can result from succinate dehydrogenase (SDH) gene mutations.Objective: To describe the clinical manifestations of subjects with SDH-B gene mutations.Design: Retrospective case series.Patients: Thirty-two subjects with SDH-B gene mutations followed-up between 1975 and 2007. Mean follow-up of 5.8 years (S.D....

Introduction: Family hypobetalipoproteinemia (HBF) is a rare genetic disorder, in 50% of cases are due to mutations APOB gene, which leads to decreased values of total cholesterol, low density lipoprotein (LDL-cholesterol) and apo-B. It is inherited as an autosomal dominant and heterozygous carriers are usually asymptomatic.Clinical case: Thirty-two-year-old male with abdominal pain and diarrhea associated with food, no relevant history and physical exam...

Pituitary carcinomas are extremely rare accounting for only 0.1%–0.2% of all pituitary tumours. The diagnosis is primarily dependent on aggressive imaging characteristics and high tumour mitotic activity on histology. A 47 year old gentleman with Type 1 Diabetes presented with an apparent non-functioning pituitary macro adenoma which was resected transsphenoidally and followed by EBRT. Initial histology was negative for ACTH. He presented 2 years later with florid Cushing...