Endocrine Abstracts

Objective: Isolated ACTH-deficiency (IAD) is considered a rare autoimmune endocrinopathy most frequently associated with autoimmune thyroid diseases (ATD). We have previously diagnosed IAD in four patients with primary hypothyroidism and negative TPO antibodies. The aim of this study was to determine the prevalence of undiagnosed IAD in patients with ATD.Methods: We studied 45 patients with ATD on stable L-thyroxine replacement (dose range...

Cranial radiotherapy is widely used in combination with chemotherapy to treat patients with primary brain tumours. Increased survival has lead to the recognition of long term sequelae, including endocrine dysfunction due to radiation-induced hypothalamic–pituitary damage. Here we assessed health-related quality of life and endocrine function in 31 adult long term survivors (median age 45, range 29–65 years; 21M, 10F) of primary brain tumours outside the hypothalamic&...

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 and cortisone acetate) on the quality of life in these patients.Methods: Eight hundred and eighty three patients with adrenal insufficiency were contacted, 526 patients participated (60%) and received a disease specific que...

Adrenocortical carcinoma (ACC) is a rare and heterogeneous malignancy with incompletely understood pathogenesis and poor prognosis. Recent data from the German ACC Registry (n=377) demonstrate an overall 5-year survival of 46%. Survival is clearly stage-dependent (P<0.01) with a 5-year survival of 85% in stage 1, 56% in stage 2, 42% in stage 3, and 16% in stage 4, respectively.In stages I –III open surgery by an expert surgeon aimi...

Androgen excess is a key feature of polycystic ovarian syndrome (PCOS). Pre-receptor regulation contributes to this with increased activation of testosterone (T) to 5α-dihydrotesterone (DHT) by 5α-reductase type 1 (SRD5A1), as we have shown previously in PCOS (Lancet 1990,335:431; JCE&M 2003,88:2760). Peripheral blood mononuclear cells (PBMCs) are easily accessible and a useful model for studying pre-receptor regulation in the immune compartment. We have previous...

Objective: Local tumor recurrence is common in adrenocortical carcinoma (ACC) and the most frequent cause for re-operation. Therefore, a concept for an adjuvant treatment is urgently needed. The role of radiotherapy in ACC has not been well defined and many authors have considered radiotherapy as ineffective. However, two studies with a small number of patients (n=3 and n=4, resp.) have suggested adjuvant radiation as an effective treatment to prevent recurrence ...

A recent study has reported impaired subjective health status (SHS) in 79 patients with primary adrenal insufficiency (AI) despite routine steroid replacement1. Here we have performed a survey in a large cohort of patients with primary and secondary AI. 348 patients (148 outpatients and 200 registered participants of the self-help network for adrenal and pituitary diseases) were contacted by mail. 256 (74%) agreed to participate and received a disease specific and 3...

Treatment of adrenocortical carcinoma (ACC) is still unsatisfactory. As IGF-II and IGF-I receptor are frequently overexpressed in human ACC and inhibitors of the IGF system are currently under preclinical investigation, interference with IGF-signaling might have an additive effect in antitumour treatment. We have therefore analyzed several cytotoxic agents (etoposide, doxorubicine, cisplatin, streptocozin; 0.01–100 μM) on adrenal cell proliferation in vitro an...

To clarify the role of the pituitary in zebrafish interrenal development we investigated wildtype (wt) zebrafish and 3 pituitary mutants: aal (with lactotrophes only), and pit1 (with corticotrophes only), fgf3-lia (lacking all adenohypophyseal cell types) and in MC2R knockdown embryos (MC2R morphants).The interrenal primordium (IP) is detectable at 22hpf (hours post fertilization) as bilateral clusters of ff1b expressing cells which fuse to one domain at...

In severe sepsis circulating DHEA sulfate (DHEAS) has been shown to decrease whilst serum cortisol increases. This has led to the suggestion of an intraadrenal shift from adrenal androgen towards glucocorticoid synthesis in severe stress. Patients with sepsis are therefore assumed to be DHEA deficient and have been suggested to benefit from DHEA replacement. However, only desulfated DHEA is biologically active and DHEAS and DHEA may not freely interconvert as previously though...