ACTH deficiency (central adrenal insufficiency)
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Introduction
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]
More general terms
Additional terms
References
- ↑ OMIM https://mirror.omim.org/entry/201400
- ↑ Jump up to: 2.0 2.1 2.2 2.3 2.4 2.5 Medical Knowledge Self Assessment Program (MKSAP) 16, 17, 18, 19. American College of Physicians, Philadelphia 2012, 2015, 2018, 2022
- ↑ Grossman AB. Clinical Review#: The diagnosis and management of central hypoadrenalism. J Clin Endocrinol Metab. 2010 Nov;95(11):4855-63. PMID: https://www.ncbi.nlm.nih.gov/pubmed/20719838