Endocrine Abstracts

The first line treatment of somatotroph adenomas is pituitary surgery, that aims at removing the tumor, but which is frequently incomplete, as invasion into cavernous sinuses often limits the surgical resection. Multimodal medical treatment, which is burdensome, very expensive and has variable therapeutic efficacy, is currently required in more than 60% of the patients. New therapeutic approaches are therefore necessary for controlling hormone secretion, tumor development and ...

Background: Pasireotide is a second line treatment for acromegaly. Besides the growth hormone (GH) lowering efficacy, clinical use is limited by side effects on glycemic control. The aim of this study was to evaluate longitudinal changes in beta-cell function and insulin sensitivity induced by pasireotide therapy in patients with acromegaly.Methods: We performed a retrospective study in 33 patients. Efficacy (GH and IGF-I concentrations; tumor size) and ...

Background: Cabergoline (CAB) is the most effective dopamine agonist (DA) used for the treatment of macroprolactinoma. Few data on the dose of CAB necessary for achieving and maintaining normal prolactin (PRL) levels are available. We aimed to study these parameters in a large series of patients with macroprolactinomasMethods: We retrospectively analysed the clinical, biochemical and imaging features at diagnosis and the treatment response to CAB (dose n...

Context: Mitotane due to its adrenolytic action is highly effective in long-term management of ACTH-dependent Cushing’s syndrome (CS). However, the slow onset of its anticortisolic effect makes its use problematic in severe CS, when very rapid therapeutic response is required. Association with metyrapone and ketoconazole, rapidly acting steroidogenesis inhibitors, could warrant CS control while waiting for the full efficiency of mitotane and thus avoid urgently performed ...

Objectives: X-linked hypophosphatemic Rickets (XLHR) is characterized by phosphate wasting and decreased production of 1,25OH-vitamin D, due, in most patients, to elevated FGF23 and PHEX mutation. In children, the disease has been extensively studied because of the devastating presentation of rickets, teeth abcesses, and growth retardation. In adults, however, metabolic complications, such as hyperparathyroidism or consequences on glucose and lipid metabolism of FGF23 excess, ...

Context: Surgical removal is the primary treatment option for pituitary adenomas. However, pituitary surgery is frequently incomplete because of invasion of extrasellar cerebral structures, notably, of the cavernous sinus. Our objective was to study the molecular basis of the cavernous sinus invasion by pituitary adenomas.Methods: We analyzed a tissue collection of 19 invasive pituitary adenomas with a sample from the intrasellar portion and a sample fro...

Background/aim: X-linked hypophosphatemia (XLH) is a rare disease characterized by low phosphate level. Scientific evidence points to link between hypophosphatemia and obesity in general population. The aim of our longitudinal observational study was to investigate the prevalence of obesity and associated factors in a large cohort of children with XLH.Patients/methods: We selected 172 XLH-children of 5-20 years (113 girls/59 boys). Anthropometric paramet...

Background: X-linked hypophosphatemia (XLH) is a rachitic disorder characterized by renal tubular phosphate wasting resulting from increased circulating activity of the fibroblast growth factor FGF23. Secondary and tertiary hyperparathyroidism have been reported in XLH patients in small retrospective studies, however this complication has never been systematically evaluated in a large cohort.Aim of the study: To compare parathyroid function of adult XLH ...

Context: In patients with hypoparathyroidism refractory to conventional treatment, recombinant human (rh)PTH(1-84) or rhPTH(1-34) can be used as second-line therapy and effectively control hypocalcaemia. However, whether rhPTH replacement therapy is safe in the long term is unclear. Our objective was to assess bone effects of long-term therapy of chronic hypoparathyroidism with rhPTH(1-34). Methods: We conducted a monocenter retrospective cross-sectional...

Background/aim: Burosumab is a monoclonal antibody against anti-FGF23, which has been recently approved for the treatment of X-linked hypophosphatemia (XLH). Beyond clinical trials, little is known about its efficacy/safety in clinical practice which is the aim of the present study.Patients/methods: Thirty-nine children with XLH were switched from conventional therapy to burosumab (starting dose 0.4 mg/kg), on the basis of following indications: non-resp...