Adrenocorticotropic hormone deficiency
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Adrenocorticotropic hormone deficiency is a rare disorder characterized by secondary adrenal insufficiency with minimal or no cortisol production and normal pituitary hormone secretion apart from ACTH.[3] ACTH deficiency may be congenital or acquired, and its symptoms are clinically similar to those of glucocorticoid deficiency. Symptoms consist of weight loss, diminished appetite, muscle weakness, nausea, vomiting, and hypotension. Low blood sugar and hyponatremia are possible; however, blood potassium levels typically remain normal because affected patients are deficient in glucocorticoids rather than mineralocorticoids because of their intact renin-angiotensin-aldosterone system. ACTH may be undetectable in blood tests, and cortisol is abnormally low.[1] Glucocorticoid replacement therapy is required. With the exception of stressful situations, some patients with mild or nearly asymptomatic disease may not require glucocorticoid replacement therapy.[2] As of 2008 about two hundred cases have been described in the literature.[5]
Adrenocorticotropic hormone deficiency | |
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Other names | ACTH Deficiency, Isolated adrenocorticotropic hormone deficiency, Isolated ACTH Deficiency. |
Adrenocorticotropic hormone (ACTH), structure | |
Specialty | Endocrinology |
Symptoms | Fatigue, anorexia, weight loss, hypoglycemia, muscle weakness, nausea, vomiting, and hypotension.[1] |
Complications | Adrenal crisis[2] |
Causes | Autoimmune processes, congenital etiologies, and physical trauma.[3] |
Diagnostic method | Morning serum cortisol levels,[4] Insulin tolerance testing, and ACTH stimulation test[2] |
Differential diagnosis | Congenital adrenal hyperplasia, Addison's disease, and Secondary adrenal insufficiency.[1] |
Treatment | Glucocorticoids.[2] |