Endocrine Abstracts

Genetic causes of anterior pituitary hormone deficiencies may result in combined or isolated pituitary hormone deficiencies. These disorders represent a heterogeneous group of rare diseases leading to defective function of specific pituitary cell types. Since the first description of POU1F1 human mutations, several other genetic defects of transcription factors have been reported with variable degrees of phenotype-genotype correlations. However to date, despite the identificat...

Aggressive pituitary tumours are particularly challenging to clinicians in terms of diagnosis and treatment. They may first present as typical pituitary adenomas, with a delayed appearance of aggressive signs, or initially as aggressive tumours. Predicting pituitary tumour behaviour remains difficult: increased mitotic, Ki-67, and P53 indexes may be associated with tumour aggressiveness. True pituitary carcinomas are rare, representing about 0.2% of all pituitary tumours. The ...

Using the currently available somatostatin receptor (sst) ligands octreotide and lanreotide, that are mainly sst2 agonists, about 60% of patients with acromegaly are adequately controlled. This prompted the development of new drugs targeting other sst subtypes or other receptors that are also expressed on adenomatous cells. BIM 23244 characterized by a high affinity for sst2 and sst5 had been found in vitro to allow a stronger inhibition of GH secretion than octreotide ...

Somatostatin acts as an inhibitor of hormonal secretion and cell proliferation by acting through 5 somatostatin receptors subtypes (sst1–5). Coupling with Gi/o proteins is associated with effects on various transduction pathways, as adenylate cyclase inhibition or phosphatases activation. Dopamine receptor subtype 2 (D2DR) acts through coupling with similar G-proteins and transduction pathways. Sst, mostly sst2 and sst5, are coexpressed with D2DR in many neuroendocrine no...

Congenital lipoid adrenal hyperplasia (CLAH) is a severe disorder characterized by early impairment of both adrenal and gonadal steroidogenesis, leading to early adrenal failure and male sex reversal. The most common aetiology of CLAH is mutation of Steroidogenic acute regulatory protein (StAR) gene.Objective: We report evolution over 20 years of a 46XX patient harbouring a novel StAR gene mutation.Methods: Clinical, hormonal and i...

Introduction: The management of large non secreting adrenal tumors (at least 4 cm) is still a matter of debate as it is unclear whether imaging, especially 18F-FDG, can be used to characterize their potential malignancy. Moreover, the risk of new hypersecretion in non-operated tumors is uncertain. Our aim was to better characterize these large adrenal incidentalomas.Methods: Patients followed in our Center for a non-secreting large (at least 4 cm) adrena...

The GH deficiency (GHD) may be congenital with or without cause identified or acquired secondary to organic lesion in the hypothalamic region (HH). In all cases a magnetic resonance imaging is necessary.Aim: Investigate the causes of GHD Population and methodology GHD children were followed at the department of endocrinology. In addition to clinical examination, a testing hypophysiogramme is made with glucagon/propranolol/GH testing insulin on GH/cortiso...

Context: Following the recent evolution in therapeutic strategies for GH-secreting pituitary adenomas, determining optimal individualized patient management is now crucial.Objective: To determine whether pre-surgical medical treatment (PSMT) in patients with acromegaly improves surgical outcome and to specify thresholds for such a strategy.Methods and design: This retrospective study included 110 newly diagnosed acromegalic patient...

Aim and methods: Cortisol substitution in adrenal insufficiency (AI) is a matter of debate. We performed a pharmacokinetic analysis of plasma and salivary cortisol following oral hydrocortisone intake in patients with AI, and a pharmacokinetic modelling simulation. Fifty patients with primary (n=20) or secondary (n=30) AI were recruited. After 24-h plasma and salivary cortisol measurements under usual hydrocortisone treatment, a pharmacokinetic modelling with dif...

Objective: To analyze characteristics of patients who had surgery for a GH-secreting adenoma in the past decade in our centre, to evaluate their initial outcome and long-term recurrence rate using stringent criteria and identify potential predictive factors of surgical remission.Methods: This retrospective study included 115 consecutive patients with acromegaly operated at the neurosurgical department of the Timone Hospital Marseille between 1997 and 200...