What is the role of pharmacologic therapy in the treatment of infantile Cushing syndrome in McCune-Albright syndrome (MAS)?

Updated: Jan 17, 2019
  • Author: Gabriel I Uwaifo, MD; Chief Editor: George T Griffing, MD  more...
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Answer

No effective medical treatment for adrenocorticotropic hormone (ACTH)-independent Cushing syndrome is available, and the currently recommended treatment is bilateral adrenalectomy.

During the procedure and afterwards, the patient needs replacement of both glucocorticoids and mineralocorticoids in appropriate amounts. Stress doses of glucocorticoid (approximately 10 times maintenance) should be administered perioperatively and slowly reduced to maintenance levels (eg, hydrocortisone 12-16 mg/m2/day in 3 divided doses). Mineralocorticoid replacement (eg, fludrocortisone 0.05-0.1 mg/day) should be started soon after surgery as the hydrocortisone dose is weaned toward maintenance levels.


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