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ACTH deficiency (central adrenal insufficiency)

A form of hypopituitarism. Etiology: - see hypopituitarism - exogenous glucocorticoids is the most common cause [2] - may occur in association with other forms of hypopituitarism Pathology: - isolated glucocortoicoid deficiency - no mineralocorticoid deficiency - thus partial adrenal insufficiency - ACTH & pro-opiomelanocortin not hypersecreted [2] Genetics: - associated with defects in TBX19 Clinical manifestations: - weight loss - anorexia - weakness - nausea/vomiting - hypotension - no hyperpigmentation or bronizng of skin [2] Laboratory: - serum ACTH is decreased or absent - serum cortisol is low - serum DHEA is low - serum DHEA-sulfate is low - serum aldosterone is low Management: - hydrocortisone 10-30 mg/day divided BID - glucocorticoids prescribed in > physiologic doses for >= 3 weeks (hydrocortisone 15-20 mg/day) should be tapered to allow recovery of the pituitary-adrenal axis [2] - a cosyntropin-stimulation test should then be performed to assess recovery of the pituitary-adrenal axis [2]

Related

adrenal insufficiency

General

hypopituitarism

Database Correlations

OMIM 201400

References

  1. OMIM :accession 201400
  2. Medical Knowledge Self Assessment Program (MKSAP) 16, 17, 18, 19. American College of Physicians, Philadelphia 2012, 2015, 2018, 2022
  3. Grossman AB. Clinical Review#: The diagnosis and management of central hypoadrenalism. J Clin Endocrinol Metab. 2010 Nov;95(11):4855-63. PMID: 20719838