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Johannsson G.,Gothenburg University | Falorni A.,University of Perugia | Skrtic S.,Gothenburg University | Lennernas H.,Uppsala University | And 3 more authors.
Clinical Endocrinology | Year: 2015

Glucocorticoid replacement therapy in patients with adrenal insufficiency (AI), whether primary (Addison's disease) or secondary (due to hypopituitarism), has been established for some 50 years. The current standard treatment regimen involves twice- or thrice-daily dosing with a glucocorticoid, most commonly oral hydrocortisone. Based on previous small-scale studies and clinical perception, life expectancy with conventional glucocorticoid replacement therapy has been considered normal, with a low incidence of adverse events. Data from the past 10- 15 years, however, have shown that morbidity remains high and life expectancy is reduced. The increased morbidity and decreased life expectancy appear to be due to both increased exposure to cortisol and insufficient cortisol coverage during infections and other stress-related events. This is thought to reflect a failure of treatment to replicate the natural circadian rhythm of cortisol release, together with a failure to identify and deliver individualized cortisol exposure and to manage patients adequately when increased doses are required. The resulting over- or under-treatment may result in Cushing-like symptoms or adrenal crisis, respectively. This review summarizes the morbidity and mortality seen in patients receiving the current standard of care for AI and suggests areas for improvement in glucocorticoid replacement therapy. © 2014 John Wiley & Sons Ltd.

De Corbiere P.,Ambroise Pare University Hospital | Ritzel K.,Ludwig Maximilians University of Munich | Cazabat L.,Ambroise Pare University Hospital | Cazabat L.,University of Versailles | And 22 more authors.
Journal of Clinical Endocrinology and Metabolism | Year: 2015

Context: Adrenocortical carcinomas (ACCs) are rare, aggressive tumors, of which some express receptors for estradiol, progesterone, and/or human chorionic gonadotoropin. Because this disease is encountered frequently in young women, pregnancy is a relevant issue. Objective: to evaluate the impact of pregnancy on outcome of patients previously treated for ACC. Design/Setting: retrospective observational multicenter study of the European Network for the Study of Adrenal Tumors. Patients: Seventeen ACC patients (21 pregnancies), becoming pregnant at least 3 months after the initial treatment, were compared with 247 nonpregnant ACC patients less than 47 years old. A control group of 34 patients matched for age, sex, and tumor stage was used for survival analysis. Main Outcome Measure(s): Overall survival, tumors characteristics at diagnosis, pregnancy outcome. Results: All 17 patients with pregnancies had localized ACC. The median time between surgery and conception was 4 years (0.3-12 y). Two pregnancies were terminated at 8 weeks. Sixteen women gave birth to 19 live infants. With exception of 1 (presumably unrelated) cardiac malformation, no severe fetal or maternal complication was observed. After a median follow-up time of 8.36 years and 5.26 years after the first conception, 1 of the 17 patients had died and 5 had experienced a recurrence, among whom 3 occurred before conception. Overall survival was not significantly different between the "pregnancy group" and the matched controls. Conclusion: Pregnancy in patients previously treated forACCseems to notbeassociated with worse clinical outcome, although a "healthy mother effect" cannot be excluded. Copyright © 2015 by the Endocrine Society.

Quinkler M.,Endocrinology in Charlottenburg | Quinkler M.,Charite - Medical University of Berlin | Oelkers W.,Endokrinologikum | Remde H.,Charite - Medical University of Berlin | Allolio B.,University Hospital of Wuerzburg
Best Practice and Research: Clinical Endocrinology and Metabolism | Year: 2015

Patients with primary adrenal insufficiency usually show pronounced impairment of aldosterone secretion and, therefore, require also mineralocorticoid replacement for full recovery. Clinical signs of mineralocorticoid deficiency comprise hypotension, weakness, salt craving and electrolyte disturbances (hyperkalemia, hyponatremia). Mineralocorticoid deficiency is confirmed by demonstration of profoundly decreased aldosterone and highly elevated plasma renin activity (PRA). Standard replacement consists of 9α-fluorocortisol (fludrocortisone) given once daily as a single oral dose (0.05-0.2 mg). Monitoring of mineralocorticoid replacement consists of clinical assessment (well-being, physical examination, blood pressure, electrolyte measurements) and measurement of PRA aiming at a PRA level in the upper normal range. Current replacement regimens may often be associated with mild hypovolemia. Dose adjustments are frequently needed in pregnancy to compensate for the anti-mineralocorticoid activity of progesterone and in high ambient temperature to avoid sodium depletion. In arterial hypertension a dose reduction is usually recommended, but monitoring for hyperkalemia is required. © 2014 Elsevier Ltd. All rights reserved.

Remde H.,Clinical Endocrinology | Hanslik G.,Clinical Endocrinology | Rayes N.,Charite - Medical University of Berlin | Quinkler M.,Endocrinology in Charlottenburg
Hormone and Metabolic Research | Year: 2015

In recent years, an increasing number of studies have revealed deleterious effects of aldosterone via the mineralocorticoid receptor (MR). Especially in patients with primary aldosteronism (PA) a significant higher estimated risk of developing cardiovascular comorbidities and comortalities compared to essential hypertensives was reported. As diabetes mellitus and the metabolic syndrome are one of the major contributors to cardiovascular morbidity and mortality their connection to aldosterone excess became a focus of research in PA patients. Several studies assessed the effect of PA on glucose metabolism, the prevalence of diabetes mellitus, and the effect of PA treatment on both revealing different results. Therefore, we performed an extensive literature research. This review focuses on the current knowledge of the connection between aldosterone excess, glucose homeostasis, and diabetes mellitus in patients with PA. We have highlighted this topic from a pro and contra perspective followed by a summarizing concluding remark. Additionally, we have briefly reviewed the data on possible underlying mechanisms and indicated future considerations on the possible impact of cortisol co-secretion in PA. © Georg Thieme Verlag KG.

Remde H.,Charite - Medical University of Berlin | Zopf K.,Charite - Medical University of Berlin | Schwander J.,Endocrinology in Charlottenburg | Quinkler M.,Charite - Medical University of Berlin
Hormone and Metabolic Research | Year: 2015

Autoimmune adrenalitis (AA) and congenital adrenal hyperplasia (CAH) are the most common reasons for acquired and monogenetic primary adrenal insufficiency. Both concern women in their fertile years. The aim of the work was to examine fertility rates, pregnancy outcome, and children’s characteristics in AA and CAH patients in 2 German endocrine centers. One hundred and fifty-eight women were contacted. Thirty-nine patients with CAH due to 21-hydroxlase deficiency and 54 AA patients agreed and were included. Information about course and outcome of pregnancies was obtained by questionnaire and telephone interview. Fertility rates were calculated and compared to expected values from the German general population. Twelve CAH patients (30.8%) had 25 pregnancies, which resulted in 16 children. In AA patients, 93 pregnancies in 42 women (75%) were reported resulting in 73 childbirths. Fertility rates were normal in nonclassic CAH and in AA-only patients, but significantly reduced in classic CAH and autoimmune polyendocrine syndrome type 2 (APS-2). Rates of miscarriages were high in all CAH (36%) and APS-2 (22%) patients. The majority of children in both groups were born at term, but rates of cesarean section were elevated in classic CAH and in AA patients<5 years before or after diagnosis. Children born to CAH patients weighed significantly less than expected and 33.3% of them were born small for gestational age. Fertility seems not to be reduced in general, but specific in classic CAH and APS 2 patients. Nevertheless all CAH and AA patients seem to be at risk of miscarriages and cesarean section. Copyright © 2015, Georg Thieme Verlag KG. All rights reserved.

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