Endocrine Abstracts

Background: Medical treatment with cortisol-lowering drugs is commonly used following pituitary surgical failure or recurrence of hypercortisolism in patients with Cushing’s disease (CD). Studies using late-night salivary cortisol (LNSF) measurement have shown persistent disruption of the circadian secretion of cortisol despite normalization of UFC in a subset of medically treated CD patients. Study aim Our objective was to assess the long-term cortisol exposure in CD pat...

In the human adrenal gland, serotonin (5-HT), released by subcapsular mast cells stimulates corticosteroid secretion through activation of type 4 serotonin receptors (5-HT4R) positively coupled to cAMP/proteine kinase A (PKA) signaling pathway and calcium influx. The 5-HT4R is principally expressed in zona glomerulosa cells explaining why 5-HT strongly stimulates aldosterone production but only exerts a modest stimulatory action on cortisol. Interestingly, in primary pigmented...

In human adrenals, serotonin (5-HT), released by subcapsular mast cells, increases aldosterone secretion through activation of type 4 serotonin receptors (5-HT4R) but only exerts a modest control on cortisol production. Interestingly, illicit synthesis of 5-HT in adrenocortical cells as well as overexpression of the 5-HT4R and ectopic expression of the type 7 receptor (5-HT7R) have been observed in bilateral macronodular adrenal hyperplasia res...

Autoimmune polyendocrine syndrome type 1 (APS1) is an autosomic recessive disease due to AIRE gene mutations, inducing central immune tolerance breakdown. It was poorly known in France.Objectives: To describe clinical and immunological phenotypes, to determine main genotypes in West Northern France (9 millions inhabitants), to identify factors that could influence phenotypes, and to analyse immunosuppressive therapies indications in APS1.<p cl...

Objective: Adrenal venous sampling (AVS) is the gold standard method to assess lateralization of aldosterone secretion in patients with primary aldosteronism (PA). The selectivity index (SIcortisol, the adrenal to peripheral vein ratio of cortisol concentrations) determines correct catheter positioning during AVS. The lateralization index (LIcortisol, the aldosterone to cortisol ratios between adrenal veins) distinguishes unilateral aldosterone producing ...

Context: Successful used of temozolomide (TMZ) treatment has been described in 40–50% of aggressive pituitary tumour (PT) or rare pituitary carcinoma. These results are based on 50 case-reports and small series, data on long-term follow-up being rare.Objectives: To describe initial results and long-term follow-up of a large French cohort of patients presenting PT treated with temozolomide.Design: Members of the French Society ...

Context: Paragangliomas and pheochromocytomas (PPGL) are rare neuroendocrine tumors, characterized by a strong genetic component. Indeed, up to 40% of patients carry a germline mutation in a PPGL susceptibility gene. In accordance with the international recommendations, genotyping of PPGL susceptibility genes is therefore proposed to all patients with PPGL, but it has actually never been shown whether the identification of a germline mutation in one PPGL susceptibility gene ch...