Figure 9.

Figure 9.

Recommended evaluations for adrenal gland dysfunction in individuals with fibrous dysplasia / McCune-Albright syndrome

CT = computerized tomography; H&P = history and physical examination; SGA = small for gestational age; US = ultrasound

1. To be performed at initial presentation in all individuals with MAS, regardless of clinical symptoms.

2. Liver disease is highly correlated with MAS-associated hypercortisolism.

3. Prognosis of hypercortisolism is negatively correlated with the presence of comorbid heart disease [Brown et al 2010]. Since hypercortisolism may lead to heart disease, the presence of hypercortisolism makes the prognosis for heart disease worse.

4. Hypercortisolism in MAS results from autonomous activity of the adrenal fetal zone, which involutes rapidly after birth and is typically gone by age one year [Carney et al 2011]. MAS-associated hypercortisolism is unlikely after age one year, and the risk is effectively gone after age three years [Brown et al 2010].

From: Fibrous Dysplasia / McCune-Albright Syndrome

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