Endocrine Abstracts

Introduction: Aggressive ACTH-independent Cushing’s syndrome is rare and frequently related to malignant adrenocortical tumor. However, in exceptional situations, ectopic steroids secretion has been described.Case report: We report the case of an 87 years-old woman referred to our clinic in September 2020 after a low trauma sacral fracture. Her recent medical history revealed the onset, in the previous months, of severe hypokalemia, uncontrolled typ...

Introduction Primary bilateral macronodular adrenal hyperplasia (PBMAH) is potentially responsible for variable degree of cortisol excess. In patients with PBMAH the complete remission of cortisol hypersecretion can be achieved only by performing bilateral adrenalectomy, leading to a persistent hypocortisolism and to a consequent need of a lifelong glucocorticoid replacement therapy. Therefore, bilateral adrenalectomy is worth doing only in patients with severe hypercortisolis...

Pasireotide (Pas) is a multireceptor-targeted somatostatin analogue approved for the treatment of patients with Cushing’s Disease (CD) who fail or are poor candidates to surgery. Pas markedly improves signs and symptoms of the disease, reduces urinary free cortisol (UFC) up to its normalization in 55% of patients and pituitary tumour size in up to 100%. Here we present a patient with severe recurrent CD treated with Pas and showing opposite results between hormonal levels...

Relacorilant is a highly selective glucocorticoid receptor modulator that antagonizes the effects of excess cortisol while showing no significant affinity for the mineralocorticoid and progesterone receptors. In a Phase 2 study in patients with endogenous hypercortisolism, relacorilant demonstrated improvements in glycemic and hypertension control with no treatment-related hypokalemia or antiprogesterone effects. An international, multicenter, Phase 3 clinical trial, using ran...

Background: Early diagnosis of Cushing’s syndrome and initiation of effective treatment are essential to limit long-term morbidity and early mortality. However, diagnosis is often delayed due to the non-specificity of symptoms, or because symptoms are not recognised by physicians, leading to more complex treatment needs and a worsening of patient quality of life. A survey was conducted to better understand the patient experience of Cushing’s syndrome and the true b...

Introduction: Primary aldosteronism (PA) has been described in association with endocrine and non-endocrine neoplasms. Aldosterone-producing adenomas mainly associate with hyperparathyroidism, prolactinomas and pancreatic endocrine tumors, particularly in the context of multiple endocrine neoplasia type 1 MEN1 syndrome. Next-generation sequencing (NGS) studies have shown frequent somatic mutations underlying PA and, rarely, germline mutations of CYP11B1/CYP11B2</e...

Background: The Endocrine Society Guidelines recommend screening for hypercortisolism especially in patients with specific features that best discriminate Cushing’s Syndrome (CS): easy bruising, facial plethora, proximal myopathy and striae. Clinical experience suggests that these features, though suggestive of hypercortisolism, are not enough sensitive. Indeed, patients with hypercortisolism frequently manifest primarily less discriminatory cortisol-related features, suc...

Background: Recent data suggest a direct effect of follicle stimulating hormone (FSH) on the skeletal metabolism. Particularly, it can encourage bone resorption and also inhibit osteoblast differentiation. High FSH levels have been found to correlate with impaired bone health in females, whilst evidence in males remains somewhat poor and conflicting. Intriguingly, men with primary and central hypogonadism might represent a novel study model in this context.<p class="abstex...

Introduction: The classical replacement therapy for hypoadrenalism may expose patients to non-physiologic glucocorticoids levels with negative metabolic consequences. Up to now, one study demonstrated that, as compared to the classical treatment, a modified-release hydrocortisone (MRH), improves weight, blood pressure, glycaemic control and QoL in a 3-month follow-up period. Few data are available on the long-term persistence of these benefits.Design/met...

Background: Radiofrequency Echographic Multi Spectrometry (REMS) is a new ultrasound-based tool for bone mineral density (BMD) measurement, recently approved by FDA for the use in clinical routine to diagnose osteoporosis (OP) and to monitor bone changes. However, data on patients with secondary OP are not available. The aim of our study was to compare REMS and DXA ability in identifying clinical (cFX) and morphometric vertebral fractures (VFx) in patients with primary OP (pOP...