Best Practice & Research Clinical Endocrinology & Metabolism
3Therapeutic management of adrenal insufficiency
Section snippets
Glucocorticoid replacement
In the 1930s lipid extracts from adrenal glands were introduced for the treatment of adrenal insufficiency, and the mortality rapidly dropped from 100% to a seemingly normal life expectancy. The synthesis of 11-deoxycortisone (11-DOC) in 1937 and cortisone in 1949 represented major improvements in therapy. However, since the introduction of fludrocortisone for mineralocorticoid replacement in the 1950s, the treatment regimens remained almost unchanged (Table 1).
Adrenocorticotropic hormone
Prevention and management of adrenal crisis
During stressful events adrenal glucocorticoid production rate can rapidly rise to up to six times normal levels. Acute adrenal insufficiency is a rare but life-threatening condition that develops as a result of inadequate adrenal steroid production not matching increased demands during stress (e.g. during infection). Early diagnosis is the key for effective and life-saving treatment of affected patients. In our series of 444 patients with chronic primary or secondary adrenal insufficiency, 42%
Summary
Initiation of hormone replacement therapy in newly diagnosed adrenal insufficiency leads to impressive and rapid improvements. However, current long-term standard mineralocorticoid and glucocorticoid treatment regimens fail to fully restore well-being and life expectancy to normal in many patients. Several studies suggest that the addition of DHEA may further improve well-being, particularly in women. However, large long-term trials with DHEA are still lacking, and DHEA is therefore not yet
References (64)
- et al.
Adrenal insufficiency
Lancet
(2003) - et al.
The risk of affective disorders in patients with adrenocortical insufficiency
Psychoneuroendocrinology
(2006) - et al.
Association between premature mortality and hypopituitarism. West Midlands Prospective Hypopituitary Study Group
Lancet
(2001) - et al.
Cortisol production rates measured by liquid chromatography/mass spectrometry
Steroids
(1990) - et al.
Cortisol production rate measurement by stable isotope dilution using gas chromatography-negative ion chemical ionization mass spectrometry
Steroids
(1999) - et al.
Rapid glucocorticoid effects on immune cells
Steroids
(2002) - et al.
Efficacy of modified-release versus standard prednisone to reduce duration of morning stiffness of the joints in rheumatoid arthritis (CAPRA-1): a double-blind, randomised controlled trial
Lancet
(2008) - et al.
Widespread tissue distribution of steroid sulfatase, 3 beta-hydroxysteroid dehydrogenase/delta 5-delta 4 isomerase (3 beta-HSD), 17 beta-HSD 5 alpha-reductase and aromatase activities in the rhesus monkey
Molecular and Cellular Endocrinology
(1994) Intracrinology
Molecular and Cellular Endocrinology
(1991)- et al.
DHEA treatment: myth or reality?
Trends in Endocrinology and Metabolism: TEM
(2002)
Determination of cDNA, gene structure and chromosomal localization of the novel human 17beta-hydroxysteroid dehydrogenase type 7(1)
FEBS Letters
Influence of oral dehydroepiandrosterone (DHEA) on urinary steroid metabolites in males and females
Steroids
The neurosteroid dehydroepiandrosterone sulfate is an allosteric antagonist of the GABAA receptor
Brain Research
Dehydroepiandrosterone activates endothelial cell nitric-oxide synthase by a specific plasma membrane receptor coupled to Galpha(i2,3)
The Journal of Biological Chemistry
Dehydroepiandrosterone replacement in addison's disease
European Journal of Obstetrics, Gynecology, and Reproductive Biology
Suppression and recovery of adrenal response after short-term, high-dose glucocorticoid treatment
Lancet
Subjective health status in Norwegian patients with Addison's disease
Clinical Endocrinology
Impaired subjective health status in 256 patients with adrenal insufficiency on standard therapy based on cross-sectional analysis
The Journal of Clinical Endocrinology and Metabolism
Premature mortality in patients with Addison's disease: a population-based study
The Journal of Clinical Endocrinology and Metabolism
Estimation of daily cortisol production and clearance rates in normal pubertal males by deconvolution analysis
The Journal of Clinical Endocrinology and Metabolism
The daily cortisol production reinvestigated in healthy men. The serum and urinary cortisol production rates are not significantly different
The Journal of Clinical Endocrinology and Metabolism
Comparison of different regimens of glucocorticoid replacement therapy in patients with hypoadrenalism
Journal of Endocrinological Investigation
Evaluation of two replacement regimens in primary adrenal insufficiency patients. effect on clinical symptoms, health-related quality of life and biochemical parameters
Journal of Endocrinological Investigation
Improvement of treatment of primary adrenal insufficiency by administration of cortisone acetate in three daily doses
Journal of Endocrinological Investigation
Glucocorticoid replacement therapy: are patients over treated and does it matter?
Clinical Endocrinology
An assessment of optimal hydrocortisone replacement therapy
Clinical Endocrinology
Weight-related dosing, timing and monitoring hydrocortisone replacement therapy in patients with adrenal insufficiency
Clinical Endocrinology
Quality of glucocorticoid replacement in adrenal insufficiency: clinical assessment vs. timed serum cortisol measurements
Clinical Endocrinology
Continuous subcutaneous hydrocortisone infusion in Addison's disease
European Journal of Endocrinology
Modified-release hydrocortisone for circadian therapy: a proof-of-principle study in dexamethasone-suppressed normal volunteers
Clinical Endocrinology
Long-acting hydrocortisone for glucocorticoid replacement therapy
Hormone Research
Diagnosis and therapy surveillance in Addison's disease: rapid adrenocorticotropin (ACTH) test and measurement of plasma ACTH, renin activity, and aldosterone
The Journal of Clinical Endocrinology and Metabolism
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Current and novel approaches to children and young people with congenital adrenal hyperplasia and adrenal insufficiency
2015, Best Practice and Research: Clinical Endocrinology and MetabolismCitation Excerpt :Fludrocortisone is usually administered once daily, although twice daily therapy with two equally divided doses can be helpful. For example, in a situation of increased physiological fluid and electrolyte loss, such as a hot climate the patient may benefit from higher more frequent doses of fludrocortisone [32]. At present it remains unclear as to whether additional mineralocorticoid replacement is required during stress and for elective surgical procedures.
Diagnosis and management of secondary adrenal crisis
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