Endocrine Abstracts

Introduction: Acromegaly is associated with multiple comorbidities and excess mortality. However, disease burden is reduced by maintaining IGF-1 (and/or GH) levels under safe levels. First generation long-acting SMSa, administered at monthly intervals, represent the first line medical treatment. According to guidelines, its efficacy is evaluated through IGF-I measurements. However, there are no data indicating the optimal time for measuring IGF-I levels after the SMSa injectio...

Background: The benefit to risk ratio of mitotane high starting dose regimen in ACC remains unknown.Methods: To evaluate a high starting dose strategy, we performed a single-center, prospective study with three main objectives: 1) to assess the time taken to reach a mitotane plasma level above 14 mg/l (benefit); 2) to evaluate mitotane tolerance (risk) in the first 3 months of treatment and 3) to analyse the correlation using a regression analysis test b...

Limited data are available about pregnancy in acromegalic women. Forty-three women, 32±1 years, presented GH hypersecretion (GH=44±7 ng/ml, IGF-1=1009±82 ng/ml) due to micro (n=6) or macroadenoma (n=37). Thirty-six women had transsphenoidal incomplete surgical resection and 9 external radiation of the pituitary adenoma performed 2.8±0.5 and 7.1±3.3 years before pregnancy. Women received dopamine agonists (n=6), somatostatin analogu...

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...

Context: Somatostatin receptor ligands (SRLs) are the cornerstone medical treatment of acromegaly, however many patients remain uncontrolled under SRLs. Well-established predictive markers of response are needed.Objective: We aimed to explore the relationship between responsiveness to SRLs, on one hand, and somatostatin (SST)2A and 5 receptor expression, adenoma granulation, and T2-weighted MRI signal intensity (T2WSI), on the other hand.<p class="ab...

Introduction: Paradoxical increase of GH following oral glucose load has been described in ~30% of patients with acromegaly and has been related to the ectopic expression of the glucose-dependent insulinotropic polypeptide (GIP) receptor (GIPR) in somatotropinomas. Recently, we identified germline pathogenic variants of lysine demethylase 1A (KDM1A) in patients with GIP-dependent primary bilateral macronodular adrenal hyperplasia with Cushing’s syndrome. Patients...

Background: Acromegaly is associated with an increased left ventricular mass, as reported in echo-based and more recently in few cardiac MRI studies. One possible explanation of this increased ventricular mass could be water retention and consequently edema of the ventricular wall.Methods: In this prospective, cross-sectional study 26 patients with active acromegaly and 31 control subjects of comparable age and sex were investigated by cardiac MRI. Patie...

Background: Hyperphagia leading to craniopharyngioma-related obesity (CRO) is a common and serious sequel of treatments of craniopharyngiomas. The few therapeutic approaches that have been tested until now for the control of eating behaviour and weight have poor efficacy. Glucagon-like peptide-1 (GLP-1) analogues might be an option. Methods: This multicentre, randomised, double-blind superiority trial was conducted in France. Adults with CRO (BMI > 3...

Introduction: PBMAH is an heterogeneous disease from the clinical, hormonal, and morphological point of view. ARMC5 inactivating mutations have been reported as a cause of PBMAH. PDE11A4 variants have been associated with PBMAH and NR3C1 variants with bilateral adrenal incidentalomas.Aim: To analyse the frequency of ARMC5 pathogenic mutations and PDE11A4 and NR3C1 variants in PBMAH patients.<p cla...

Dopamine agonists (DAs), used as first line therapy in patients with macroprolactinomas, and antipsychotics have opposite effects on dopamine receptors (D2R). In patients with severe psychiatric conditions treated with antipsychotics, the rare occurrence of a macroprolactinoma, particularly with optic chiasm compression, represents a therapeutic challenge. Indeed, on one hand, antipsychotics by their antagonistic effect on D2R, could decrease or even abolish the effects of DAs...