What is included in the long-term monitoring 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

After treatment of infantile Cushing syndrome with bilateral adrenalectomy, monitoring is warranted to ensure adequate adrenal steroid replacement. Attention to growth rate is important: Both undertreatment with glucocorticoids and overtreatment can result in decreased growth velocity. Slight undertreatment (hydrocortisone 10-12 mg/m2/day) should provide adequate maintenance replacement without growth suppression.

Mineralocorticoid treatment should be carefully monitored; overtreatment with fludrocortisone can result in hypertension. Adequate mineralocorticoid replacement is confirmed by monitoring blood pressure and periodically assessing plasma renin activity.

Increased doses of steroids are required during times of stress. During febrile illnesses, both hydrocortisone and fludrocortisone doses should be doubled. During times of severe stress (eg, trauma or surgery), hydrocortisone doses should be administered at approximately 10 times maintenance levels.


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