Generalized glucocorticoid resistance
| Generalized glucocorticoid resistance | |
|---|---|
| Other names | Chrousos syndrome |
homodimer in the nucleus interacting with DNA. Right. Binding of synthetic glucocorticoid dexamethasone to ligand-binding domain of receptor in cytoplasm. | |
| Specialty | Endocrinology |
Generalized glucocorticoid resistance or Chrousos syndrome is a rare genetic disorder that can run in families or be sporadic. It is characterized by partial or generalized target-tissue insensitivity to glucocorticoids.[1]
The clinical spectrum includes severe, potentially fatal conditions like hypoglycemia, alkalosis, or severe hypokalemia, as well as completely asymptomatic forms. The disease's most prevalent symptom is fatigue.[2]
The elevated 24-hour
The goal of treatment for generalized glucocorticoid resistance is to reduce excessive ACTH secretion, which in turn reduces the production of more adrenal steroids that have androgenic and mineralocorticoid properties.[4] High dosages of synthetic glucocorticoids that spare mineralocorticoids, like dexamethasone, are used as part of the treatment.[5]
Signs and symptoms
Individuals who have generalized glucocorticoid resistance may exhibit biochemical
In rare instances,
Causes
Mechanism
Steroid hormones known as "glucocorticoids" are produced in the zona fasciculata of the adrenal cortex and numerous other extra-adrenal organs, such as the skin, thymus, and gut.[11] These lipophilic molecules are essential for the maintenance of both resting as well as threatened homeostasis[12] and are secreted in the circulatory system in reaction to stressors[13] and also in an ultradian and circadian manner.[14]
Diagnosis
The elevated 24-hour
When diagnosing generalized glucocorticoid resistance, the differential diagnosis consists of additional factors that can lead to
Treatment
The goal of treatment for generalized glucocorticoid resistance is to reduce excessive
See also
References
Further reading
- Molnár, Ágnes; Patócs, Attila; Likó, István; Nyírő, Gábor; Rácz, Károly; Tóth, Miklós; Sármán, Beatrix (2018). "An unexpected, mild phenotype of glucocorticoid resistance associated with glucocorticoid receptor gene mutation case report and review of the literature". BMC Medical Genetics. 19 (1): 37. PMID 29510671.
- van Rossum, Elisabeth F.C.; Lamberts, Steven W.J. (2006). "Glucocorticoid resistance syndrome: a diagnostic and therapeutic approach". Best Practice & Research Clinical Endocrinology & Metabolism. 20 (4). Elsevier BV: 611–626. PMID 17161335.