Endocrine Abstracts

Introduction: Patients with primary adrenal insufficiency (PAI) and patients with congenital adrenal hyperplasia (CAH) receive life-long glucocorticoid (GC) replacement therapy. Today daily GC doses are still higher than the reported adrenal cortisol production rate, and are not able to reproduce the physiological secretion pattern. This might result in long-term morbidities such as osteoporosis. Until now no prospective trial was performed investigating the long-term effect o...

Introduction: Patients with primary adrenal insufficiency (PAI) and patients with congenital adrenal hyperplasia (CAH) receive glucocorticoid replacement therapy, which might cause osteoporosis.Objective: i) Is bone mineral density (BMD) depending on the height of the daily glucocorticoid dose? ii) Is BMD decreasing over a 2-year period of glucocorticoid replacement therapy?Methods: Prospective, longitudinal study including 56 pati...

Introduction: Health related quality of life (HRQoL) is impaired in adult patients with 21-hydroxylase deficiency (21-OHD). Up to now, only cross-sectional and no longitudinal studies are available, and it is not known if HRQoL can be improved in adult 21-OHD patients. Objective: To investigate HRQoL in adult female 21-OHD patients over a longer time span.Methods: Longitudinal, single centre, follow-up study over seven years with three visits including 1...

Context: Patients with adrenal insufficiency (AI) receive glucocorticoid replacement therapy, which tries to imitate physiological adrenal secretion. However, the first glucocorticoid dose is usually given after waking in the morning resulting in a 3–5 h delay compared to physiological secretion. Impaired quality of life (QoL) might be, in part, due to this delayed dose scheme. Therefore, modified-release glucocorticoid preparations might have therapeutical advantages.</p...

Introduction: Patients with primary adrenal insufficiency (Addison’s disease) and patients with congenital adrenal hyperplasia (CAH) still tend to receive more glucococorticoids than the normal endogenous production in healthy subjects. CAH patients start glucocorticoid treatment usually with diagnosis in their early childhood, whereas Addison’s patients have a later onset of their disease and start of their treatment.Objective: To compare pati...

Introduction: X-linked hypophosphatemic rickets is the most common form of familial hypophosphatemic rickets. It is caused by a defect in renal phosphate transport leading to phosphate wasting and hypophosphatemia. Furthermore 1,25-dihydroxyvitamin D concentrations are inappropriately normal in regard to hypophosphatemia. Clinical manifestation of the disease are skeletal deformities, short stature, osteomalacia, dental abscesses, bone pain, and loss of hearing. PHEX is locate...

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...

Objective: There is evidence that current replacement regimens fail to restore well-being in patients with adrenal insufficiency (AI). No data is available on the effect of different therapeutical regimes (hydrocortisone, prednisolone, cortisone acetate) on the quality of life in these patients.Methods: About 883 patients with adrenal insufficiency were contacted, 526 patients participated (60%) and received a disease specific questionnaire and three sta...

Objective: There is evidence that current replacement regimens fail to restore well-being in patients with adrenal insufficiency. No data is available on the effect of twice or thrice daily administration of hydrocortisone on the quality of life in these patients.Methods: About 883 patients with adrenal insufficiency were contacted, 526 patients participated (60%) and received a disease specific questionnaire and three standardized questionnaires (SF36, ...

Objective: Pheochromocytoma is a rare disease with an incidence of 2–6 per million, and a prevalence of 0.1–0.6% in patients with hypertension. Pheochromocytoma may occur sporadically or as part of hereditary syndrome. According to the latest studies, among patients with non-syndromic pheochromocytoma, up to 24% of tumors may be inherited. However, genetic testing was not generally performed until recently. There is still a debate which pheochromocytoma patient shoul...