Figure 16.

Figure 16.

Recommended management for hypercortisolism in individuals with fibrous dysplasia / McCune-Albright syndrome

b.i.d. = twice daily; MAS = McCune-Albright syndrome; m2 = meters squared (referring to body surface area)

1. Affected individuals are often critically ill at presentation, which may impact treatment options.

2. Hepatotoxicity is an important consideration due to frequent comorbid liver disease [Brown et al 2010].

3. Spontaneous resolution may occur due to involution of the adrenal fetal zone, which is the source of hypercortisolism in MAS [Carney et al 2011].

4. Children with a current or remote history of MAS-associated hypercortisolism are at increased risk for neurodevelopmental delays, and should be considered for early interventional services [Brown et al 2010].

From: Fibrous Dysplasia / McCune-Albright Syndrome

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