Endocrine Abstracts

Multiple endocrine neoplasia 1 (MEN-1) is an inherited autosomal dominant tumour syndrome affecting mainly the parathyroid gland, pituitary and pancreas. Genetic defect appears to be deletion mutation of MEN1 gene coding for tumour suppression. We describe a case of MEN1 mosaic mutation never reported in the literature.The index case presented aged 52 in 1985 with headaches and dizziness when hypercalcemia of 3.2 mmol/l was noted. Past medical history in...

The ERCUSYN (European Registry on Cushing’s syndrome (CS)) study is a project funded by the European Commission Public Health Program (PHP 800200), of which the ESE is one of the 41 Partners from 25 countries. The aims include obtaining prospective and follow-up data at EU level on epidemiology, mortality, outcome of therapies, co-morbidities, assessment of diagnostic and therapeutic strategies, quality of life evaluation using a disease-generated questionnaire (CushingQo...

Background: Adult hypopituitarism is associated with premature vascular mortality for which the underlying mechanisms are unknown but untreated GH deficiency is proposed as a potential contributor.Objectives: To characterise vascular risk, including paraoxonase-1 (PON1; an antioxidant enzyme which preserves LDL against oxidation), in adults with treated panhypopituitarism.Study subjects: The study had full ethics approval. Data are...

Adrenocortical carcinoma are rare, highly vascular tumours with a generally poor prognosis. We have studied the use of the antiangiogenic drug, thalidomide (TH), in 6 patients with metastatic adrenocortical carcinoma (5 patients had adrenalectomy and one patient was inoperable#). The response to thalidomide treatment is summarized in the following Table. Assuming the residual/metastatic tumours to have a spheroidal form, the tumour volume (V) was calculated using the eq...

Background: Adult hypopituitarism is associated with premature vascular mortality for which the underlying mechanisms are unknown but untreated GH deficiency is proposed as a potential contributor.Objectives: To characterise vascular risk, including paraoxonase-1 (PON1; an antioxidant enzyme which preserves LDL against oxidation), in adults with treated panhypopituitarism.Study subjects: The study had full ethics approval. Data are...

Background: The short synacthen test (SST) is the most commonly performed investigation to assess for suspected adrenocortical dysfunction. We investigated how random cortisol levels may relate to pass/fail on the STT.Methods: We analysed the relation between random cortisol measurements taken between 04.40 and 20.52 in the day and results of SST baseline and 30/60 min cortisol performed over a 12 month period (338 SSTs) at Salford Royal Hospital. Serum ...

Introduction: Hydrocortisone therapy in adrenal insufficiency and medical management of Cushing’s syndrome requires accurate monitoring of glucocorticoid status. Currently, this necessitates admitting patients to hospital for serial measurements of serum cortisol. From previous studies in Cushing’s, the goal of medical therapy is a mean (based on five samples) serum cortisol of 150–300 nmol/l, which is known to equate to a normal cortisol production rate. Saliva...

Introduction: Patients with pituitary deficiencies suffer from impaired quality of life regardless of substitution therapy with hydrocortisone, thyroxine (T4), sex hormones or GH. Central hypothyroidism (CH) is difficult to diagnose and treat because symptoms are non-specific and TSH-levels cannot be used for assessment. There is no consensus for the fT4-goal of thyroxine-replacement in patients with CH.Aim: To determine the impact ...

Objective: Our study evaluates the contribution of untreated GHD to the phenotype in controlled CD, by comparing patients with GHD due to CD (n=322) and those with non-functioning pituitary adenoma (NFPA n=748) before and after 3 years of GH treatment.Methods: The patient cohorts were obtained from KIMS (Pfizer International Metabolic Database) and matched for age and gender. Duration between pituitary disease onset and GH start was 9.7 (CD...

CRN04894 is a potent, orally bioavailable nonpeptide that is a highly selective antagonist for melanocortin type 2 receptor (MC2R). This receptor is found exclusively in the adrenal cortex and is the primary mediator of adrenal activation. We report results from a randomized, double-blinded, placebo-controlled (6 active:3 placebo/cohort), multiple ascending dose (40, 60, and 80 mg) study in health volunteers evaluating safety, pharmacokinetics, and pharmacodynamics of oral, on...