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Adam MP, Feldman J, Mirzaa GM, et al., editors. GeneReviews® [Internet]. Seattle (WA): University of Washington, Seattle; 1993-2024.

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Figure 5.

Figure 5.

Recommended evaluations for growth hormone excess in individuals with fibrous dysplasia / McCune-Albright syndrome

GH = growth hormone; H&P = history and physical examination; IGF-1 = insulin-like growth factor 1; MAS = McCune-Albright syndrome; OGTT = oral glucose tolerance test; PP = precocious puberty; PRL = prolactin

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

2. The majority of individuals with MAS-associated GH excess will have increased prolactin secretion [Salenave et al 2014].

3. Practitioners may consider pituitary MRI in individuals suspected of having MAS-associated GH excess; however, findings may be nonspecific and rarely change management [Salenave et al 2014].

4. There are a variety of techniques for frequent GH sampling. Collecting GH samples every 20 minutes for 12 hours from 8 PM to 8 AM, with a lack of nadir below 1.0 ng/mL, is considered consistent with GH excess.

5. In those with craniofacial FD it is prudent to have a low threshold for initiating treatment, as uncontrolled GH excess is associated with increased craniofacial morbidity [Boyce et al 2012b].

6. MAS-associated GH excess may rarely present as late as young adulthood; therefore, ongoing monitoring with periodic IGF-1 levels is prudent in those with significant craniofacial FD.

From: Fibrous Dysplasia / McCune-Albright Syndrome

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