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McCune-Albright syndrome - Overview

Alternative Names

Polyostotic fibrous dysplasia

Definition of McCune-Albright syndrome:

McCune-Albright syndrome is a genetic disease that affects the bones and color (pigmentation) of the skin.

Causes, incidence, and risk factors:

McCune-Albright syndrome is caused by mutations in the GNAS1 gene. The abnormal gene is present in a fraction, but not all, of the patient's cells (mosaicism).

This disease is not inherited. It is caused by a new change (mutation) to the DNA that occurs in the womb while the baby is developing. This mutation is not passed on to any of the person's children.

Anterior skeletal anatomy

Reviewed last on: 8/7/2008
Diana Chambers, MS, EdD, Certified Genetics Counselor (ABMG), Charter Member of the ABGC, University of Tennessee, Memphis, TN. Review provided by VeriMed Healthcare Network. Also reviewed by David Zieve, MD, MHA, Medical Director, A.D.A.M., Inc.

References

Garibaldi L. Disorders of Pubertal Development. In: Kliegman RM, Behrman RE, Jenson HB, Stanton BF, eds. Nelson Textbook of Pediatrics. 18th ed. Philadelphia, Pa: Saunders Elsevier; 2007: chap 563.

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