Prednisolone is associated with a worse bone mineral density in primary adrenal insufficiency.

Context: Patients with primary adrenal insufficiency (PAI) or congenital adrenal hyperplasia (CAH) receive life-long glucocorticoid (GC) therapy. Daily GC doses are often above the physiological cortisol production rate and can cause long-term morbidities such as osteoporosis. No prospective trial has investigated the long-term effect of different GC therapies on bone mineral density (BMD) in those patients.

I polymorphism of the glucocorticoid receptor and adrenal crisis in primary adrenal insufficiency.

Context: Patients with primary adrenal insufficiency (PAI) or congenital adrenal hyperplasia (CAH) are at a high risk of adrenal crisis (AC). Glucocorticoid sensitivity is at least partially genetically determined by polymorphisms of the glucocorticoid receptor (GR).

Objectives: To determine if a number of intercurrent illnesses and AC are associated with the GR gene polymorphism I in patients with PAI and CAH.

Reduction in daily hydrocortisone dose improves bone health in primary adrenal insufficiency.

Objective: Individuals with primary adrenal insufficiency (PAI) or congenital adrenal hyperplasia (CAH) receive life-long glucocorticoid (GC) replacement therapy. Current daily GC doses are still higher than the reported adrenal cortisol production rate. This GC excess could result in long-term morbidities such as osteoporosis.

Palliative treatment of uncontrollable hypercalcemia due to parathyrotoxicosis: denosumab as rescue therapy.

Unlabelled: Parathyroid carcinoma is a rare disease leading to severe hypercalcemia due to hyperparathyroidism. Surgery is the primary treatment option. A more progressive form of the disease is characterized by parathyrotoxicosis, and subsequent hypercalcemia is the most common cause of death.

Modified-release hydrocortisone decreases BMI and HbA1c in patients with primary and secondary adrenal insufficiency.

Objective: Patients with adrenal insufficiency (AI) have impaired health-related quality of life (HRQoL), which is thought to be in part due to unphysiological glucocorticoid replacement therapy. The aim was to compare once-daily hydrocortisone (HC) dual-release tablet (modified-release) with conventional HC therapy regarding clinical data and HRQoL.

Design And Methods: We conducted an open, prospective trial at one endocrine center.

Sexual well-being in adult male patients with congenital adrenal hyperplasia.

Introduction. Men with congenital adrenal hyperplasia (CAH) due to 21-hydroxylase deficiency show impaired fecundity due to testicular adrenal rest tumors and/or suppression of the gonadal axis. Sexual well-being might be an additional factor; however, no data exists.

BclI polymorphism of the glucocorticoid receptor gene is associated with increased bone resorption in patients on glucocorticoid replacement therapy.

Context: Patients with primary adrenal insufficiency (PAI) and patients with congenital adrenal hyperplasia (CAH) receive weight-adapted standard glucocorticoid replacement therapy. Clinically, some patients appear more sensitive to therapeutic administration of glucocorticoids than others. Glucocorticoid sensitivity is at least partially genetically determined by polymorphisms of the glucocorticoid receptor (GR) and might influence bone mineral density (BMD).

Improvement of health-related quality of life in adult women with 21-hydroxylase deficiency over a seven-year period.

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. It is not known if HRQoL can be improved in adult 21-OHD patients.

Quality of life is less impaired in adults with congenital adrenal hyperplasia because of 21-hydroxylase deficiency than in patients with primary adrenal insufficiency.

Objective: Current replacement regimens fail to restore well-being in patients with primary adrenal insufficiency (PAI). Data on health-related quality of life (HRQoL) in patients with congenital adrenal hyperplasia (CAH) are scarce, inconsistent and largely restricted to women. The objective of the study therefore was to study HRQoL in CAH because of 21-hydroxylase deficiency in comparison with PAI and healthy controls.

Spinal cord atrophy in triple A syndrome associated with a novel compound heterozygous mutation.

A 38-year-old male patient was admitted with slowly progressive spastic gait disturbance. Imaging revealed general spinal cord atrophy. Because of adrenal insufficiency, alacrima and achalasia, triple A syndrome was suspected.

Delayed diagnosis of adrenal insufficiency is common: a cross-sectional study in 216 patients.

Introduction: Little information is available on patients with adrenal insufficiency (AI) in regard to complaints before diagnosis, time until correct diagnosis, false diagnosis, and professional changes due to the diagnosis.

Objective: We retrospectively evaluated circumstances before and at diagnosis of AI in patients with primary and secondary AI by using established Hospital Anxiety and Depression Scale, Short Form-36 and Giessen Complaint List (GBB-24) questionnaires, and a self-established general registration form.

Methods: In this cross-sectional study, questionnaire sets were available from 216 patients (primary AI, n = 99; secondary AI, n = 117).

Epidemiology of adrenal crisis in chronic adrenal insufficiency: the need for new prevention strategies.

Objective: Adrenal crisis (AC) is a life-threatening complication of adrenal insufficiency (AI). Here, we evaluated frequency, causes and risk factors of AC in patients with chronic AI.

Methods: In a cross-sectional study, 883 patients with AI were contacted by mail.

Influence of hydrocortisone dosage scheme on health-related quality of life in patients with adrenal insufficiency.

Context: Recent studies suggest that current glucocorticoid replacement therapies fail to completely restore well-being in patients with adrenal insufficiency (AI).

Objective: The objective of this study was to investigate health-related quality of life (QoL) in patients with AI depending on dose and frequency of daily intake of hydrocortisone (HC).

Design And Patients: In a cross-sectional study, primary and secondary AI patients were contacted and asked to complete three validated self-assessment questionnaires [Short Form-36 (SF-36), Giessen Complaint List (GBB-24), Hospital Anxiety and Depression Scale (HADS)].

Frequent incidental discovery of phaeochromocytoma: data from a German cohort of 201 phaeochromocytoma.

Context: Adrenal and extra-adrenal phaeochromocytoma are chromaffin cell-derived tumours that are discovered due to classical symptom triad with headache, sweating and palpitations combined with persistent or paroxysmal hypertension. However, an increasing proportion of phaeochromocytoma seems to be discovered incidentally upon abdominal imaging.

Objective: To specify the exact circumstances of discovery of adrenal and extra-adrenal phaeochromocytoma.

Impaired subjective health status in chronic adrenal insufficiency: impact of different glucocorticoid replacement regimens.

Context: Recent studies have suggested that current glucocorticoid replacement therapies fail to fully restore well-being in patients with adrenal insufficiency (AI).

Objective: To investigate the effect of different glucocorticoid preparations used for replacement therapy on subjective health status (SHS) in AI.

Design And Patients: In a cross-sectional study, primary and secondary AI patients were contacted by mail.

Cushing's syndrome due to ACTH-secreting pheochromocytoma.

Ectopic secretion of adrenocorticotropic hormone (ACTH) is an infrequent cause of Cushing's syndrome. We report a case of ectopic ACTH syndrome caused by a pheochromocytoma. A 53-year-old female with clinical features of Cushing s syndrome presented with serious recurrent hypertensive crisis.

[Long-term follow-up of patients with suprasellar germinomas].

Background: Suprasellar germinomas are rare intracranial neoplasms, which mainly occur in children and adolescents and manifest with endocrine symptoms and/or compression syndromes.

Patients And Methods: The clinical, hormonal and morphological findings as well as treatment and complications were investigated in seven patients (six male, one female) with germinomas.

Results: Mean age at diagnosis was 19.

Long-term management in five cases of TSH-secreting pituitary adenomas: a single center study and review of the literature.

Objective: TSH-secreting pituitary tumors (TSH-omas) are a rare cause of hyperthyroidism and account for <1% of all pituitary adenomas. Failure to recognize the presence of a TSH-oma may result in dramatic consequences such as thyroid ablation that may cause further growth in pituitary tumor. The primary goal of the treatment of TSH-omas is to remove the pituitary tumor.

Loss of body cell mass in Cushing's syndrome: effect of treatment.

Cushing's syndrome (CS) is associated with low fat-free mass, but it is unclear whether hypercortisolism causes a loss of whole body protein. Body composition was studied prospectively in 15 patients with untreated CS (n = 14 pituitary adenoma; n = 1 adrenal adenoma), in 15 nonobese healthy controls, and in 15 weight-matched obese controls by 3 different methods: total body potassium counting (TBP), bioelectrical impedance analysis (BIA), and anthropometry. In 6 patients, body composition was studied before and within 6 months after pituitary surgery.

Endocrinological and genetic studies in patients with Polycystic Ovary Syndrome (PCOS).

Our studies involved 21 women of Caucasian descent with PCOS, as diagnosed by ultrasound, clinical and hormonal findings. We determined 17alpha-OHP, 21-DOF and cortisol levels both just before and one hour after intravenous ACTH administration (0.25 mg Synacthen) in addition to the basal plasma levels of DHEA-S and free testosterone.