Endocrine Abstracts

Background: Many patients require IV insulin during their critical illness. Maintenance of insulin in St James’s Hospital ICU is governed by a local protocol. At the time of ICU discharge, IV insulin therapy is often stopped. Transitioning from IV to subcutaneous insulin is often done with endocrinology input. If this is unavailable inappropriate insulin dosing may increase the risk of hypo-/hyperglycaemia.Aims: To reduce the number of episodes of h...

Objective: To describe the Carney Complex (CNC) manifestations presented by patients harboring the PRKAR1A mutation c.709(-7-2)del (one of the three hotspots) in a large cohort of patients.Methods: Multicenter retrospective study. Age at the diagnosis or at the screening of the different CNC manifestations is described by mean ± standard deviation.Results: Forty patients [12 index cases, 27 females, 46±15 years o...

Introduction: Sirolimus, a mTOR (mechanistic Target of Rapamycin) inhibitor, is well known for its impact on glucides and lipids metabolism. These effects vary according to factors such as dose and treatment duration, species, cell types and environmental factors. In vitro and in vivo, sirolimus inhibits adipogenesis by decreasing adipocytes number and size, as well as pre-adipocytes differentiation, leading to subcutaneous and visceral fat mass decrease in m...

Introduction: Resistance to thyroid hormone alpha (RTHα) is characterised by tissue-specific hypothyroidism associated with barely normal thyroid function tests. Clinical features include dysmorphic facies, skeletal dysplasia, growth retardation, constipation, dyspraxia and intellectual deficit. Hormonal assessment often shows decreased/low-normal free thyroxine (fT4) and increased/high-normal free triiodothyronine (fT3) concentrations, resulting in a low fT4/fT3 ratio, w...

Background: In a phase 3b, open-label study (NCT02354508) assessing efficacy and safety of long-acting pasireotide in patients with uncontrolled acromegaly after ≥3 months of treatment with first-generation SSAs, 18/123 (15%) patients achieved the primary endpoint of mean growth hormone (mGH) <1.0 μg/l and insulin-like growth factor-1 (IGF-1) <ULN (upper limit of normal) at week 36. Here, we present the results from the extension phase of this study.<p c...

Introduction: Dopamine agonists (DA), reference treatment for microprolactinomas, rarely allow long term remission. The performance of endoscopic pituitary surgery should be examined as first-line treatment.Objective: To evaluate the remission rate of microprolactinoma operated by endoscopic transphenoidal approachMethods: We retrospectively studied the surgical results of the 35 microprolactinomas operated between 2010 to 2017 in ...

Lipodystrophy is a rare disorder characterised by loss of adipose tissue, severe insulin resistance, diabetes, hypertriglyceridemia, hepatic steatosis with hypoleptinemia. Renal complications are also present but end stage renal disease is less frequent in partial lipodystrophy. We report efficacy and pharmacokinetics of metreleptine during hemodialysis in one case of partial lipodystrophy. A caucasian female presented with acute pancreatitis, major hypertriglyceridemia (25 g/...

Introduction: Testosterone circulates under different forms in blood, mainly bound to proteins i.e. Sex Hormone Binding Globulin (SHBG) and albumin. Free testosterone (FT), the biologically active form, represents 2% of total testosterone (TT). FT measurement is mainly indicated when TT level is discordant with clinical picture but remains technically challenging. Indeed, as for all free hormones, gold standard method relies on equilibrium dialysis, unusable in routine. Direct...

Introduction: Steroid profiling by mass spectrometry approaches consists in the simultaneous measurement of several steroid molecules in a biological sample, allowing an optimal characterization of steroidogenesis alterations, particularly in the context of adrenal tumors. Twenty-four hours urine samples have the advantage of being non-invasive and of giving an integrated view of steroidogenesis. Urinary steroid profiling has thus been shown to be particularly useful in the di...

Somatostatinomas are rare neuroendocrine tumors diagnosed at a rather young age (mean age at diagnosis from 40 to 60 years). Non-auto-immune diabetes may associate with somatostatinoma as a result of abnormal somatostatin secretion, which inhibits insulin release. Steatorrhea and cholelithiasis are two other classical symptoms of these tumors. Here we report a case of somatostatinoma discovered while exploring a recently diagnosed diabetes, whose clinical and biological featur...