Endocrine Abstracts

In overt or subclinical ACTH-independant Cushing’s syndrome (CS), aberrant adrenal receptors may control cortisol secretion.We systematically studied the 20 patients with adrenal incidentaloma screened in our department for illegitimate adrenal receptors between 2000 and 2006. We investigated plasma cortisol level during successive stimulation tests performed while dexamethasone was given orally every 8 h (upright posture, meal, hypothalamic hormone...

The characteristics of adrenal involvement in Multiple Endocrine Neoplasia type 1 (MEN1) have been defined from studies involving a limited number of patients. We have assessed retrospectively the prevalence, characteristics and evolution of adrenal involvement from the French group for the study of endocrine tumours (GTE) registry, involving 688 patients with MEN1. In our series, adrenal tumours identified at abdominal imaging occurred in 130 patients (18.9%). The mean age of...

Within the group of gonosomal aneuploidy, the 47,XXY Klinefelter syndrome is a well-known chromosomal anomaly with a clearly delineated phenotype. Since the 48,XXYY polysomy is rather rare and associated with hypogenitalism, it has often been considered as a variant of the Klinefelter syndrome. Nevertheless, several differences have been reported, in particular the greater severity and prevalence of mental retardation and psychiatric illness in patients with a 48,XXYY syndrome...

Ectopic adrenocorticotropin secretion (EAS) accounts for 10–15% of cases of Cushing’s syndrome and comprises a spectrum of lesions from highly malignant tumours to a variety of less aggressive neuroendocrine tumours. Selective removal of the primary lesion is the optimal management. It is therefore mandatory to localize the source of ectopic ACTH.As no single test is accurate enough to distinguish the ectopic from the pituitary sources of ACTH,...