Endocrine Abstracts

Background: Besson A et al. described a patient with homozygous for C53S-hGH suffering from IGHD (JCEM2005 90:2493-9). They observed reduced ability of the mutant to bind and activate GHR in vitro. C53A-hGH lacks the disulfide bond between C53 and C165, which is conserved in GH/prolactin family.Methods: Mouse pituitary AtT-20 cells, HEK-293 and CHO-K1 cells were transfected with plasmids containing C53S, C53A or C53S/C165A cDNA. hGH from cell supernatant...

There is a high heterogeneity of human growth hormone (hGH), variation rising from the different genes in pituitary and placenta, alternative splicing, post-translational modifications, oligomerization and binding to the growth hormone binding protein (GHBP). Distinguishing between different variants remains problematic and there is still quite little information about the proportion of these variants in circulation of both healthy individuals and of patients with growth hormo...

Introduction: Two heterozygous missense mutations R77C and D112G have been identified in GH-1 gene of patients with short stature by Takahashi et al. These patients had high serum immunoreactive GH concentrations but low IGF-1 concentrations, indicating bioinactivity of their GH. Separation of GH in patients’ serum by isoelectric focusing revealed the coexistence of mutant and wild type (wt) GH. In order to understand the molecular mechanism of the isolated GH defi...

Objective: Patients from FRG and West-Berlin with primary (1AI) or secondary (2AI) adrenal insufficiency were treated with hydrocortisone (HC) while in contrast patients from the former GDR received prednisolone (PR). Investigation of our study is to detect any differences in quality of life (QoL) in patients on HC or PR medication.Methods: 350 patients of our outpatient clinic were contacted by mail. Up to now 131 patients took part in the survey. 2 sta...

Background: Sex differences in clinical picture of ACTH-dependent Cushing’s syndrome are controversial, except for the known higher prevalence in females. We compared a broad range of potential differences to enable a more accurate understanding of the clinical picture of sex-specific ACTH-dependent Cushing’s syndrome.Design: Cohort study.Methods: We included consecutive patients with ACTH-dependent Cushing’s syndrom...

Background: Adrenal crisis, the most feared complication of adrenal insufficiency, is a potentially life-threatening situation of acute glucocorticoid deficiency. After successful surgery, many patients with Cushing’s syndrome develop (transient) adrenal insufficiency. The incidence of adrenal crisis in patients treated for hypercortisolism is unknown.Methods: Cohort study including consecutive patients with Cushing’s syndrome with adrenal insu...

Background: Long-term safety data (1998 to mid-2016) are reported for adult patients with GHD treated with GH (Norditropin® (somatropin), Novo Nordisk) as prescribed by treating physicians in the real-life clinical setting and enrolled in NordiNet® International Outcome Study (IOS) (NCT00960128), a non-interventional, multicentre study.Objective and hypotheses: To describe and report safety data and incidence rates (IRs) (...

Introduction: Although biochemical markers of acromegaly disease activity, including GH and IGF1, may fluctuate from day-to-day, biochemical treatment response in clinical trials is generally monitored using single-point analyses. Accordingly, longitudinal evaluations may assess patient status more accurately. In a phase 3 trial, oral octreotide capsules (OOC) demonstrated sustained composite endpoint GH and IGF1 response for ≤13 months in 151 patients with acromegaly pr...

Background: Adult GH deficiency (GHD) is diagnosed by provocative testing of GH secretion. To improve diagnostic accuracy of GHRH plus arginine (GARG) testing, we evaluated the influence of age, BMI, and sex. Furthermore, we aimed to established normative data for an automatic immunoassay specifically measuring 22kD human GH.Methods: Eighty-seven patients with hypothalamic-pituitary disease and 200 healthy controls were enrolled. Controls were prospectiv...

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...