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McCune-Albright Syndrome
Affects many body systems

From , former About.com Guide

Created: December 17, 2006

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Fibrous Dysplasia FoundationFibrous Dysplasia Foundation
McCune-Albright syndrome consists of a pattern of bone, endocrine, and skin abnormalities, especially replacement of normal bone with fibrous growth (fibrous dysplasia). Its effects on the body may range from mild to severe.

It is not known how often McCune-Albright syndrome occurs, but it does not seem to be inherited. It affects both males and females of all ethnic backgrounds.

Symptoms
The symptoms of McCune-Albright syndrome are related to the body systems affected by it, and may vary from individual to individual.

    Bone symptoms
  • Normal bone replaced by fibrous tissue (fibrous dysplasia)
  • Broken bones not due to injury (pathological fractures)
  • Arms and/or legs unequal in length
  • Changes in the face and skull due to fibrous dysplasia
    Endocrine (hormone) symptoms
  • Early onset of puberty, before 8 years of age in a girl or 9-1/2 years of age in a boy (called precocious puberty)
  • Thyroid gland enlargement (goiter) or lumps (nodules or cysts)
  • Excess production of growth hormone
    Skin symptoms
  • Large areas of light brown skin with irregular borders (called café-au-lait spots)
  • Café-au-lait spots may be difficult to see in dark-skinned individuals

Diagnosis
Several different types of medical tests are done to confirm a diagnosis of McCune-Albright syndrome. Blood tests are done to check for abnormally high levels of sex hormones (in precocious puberty), and growth hormone. Thyroid hormone levels are also checked. X-rays or bone scans are done to look for fibrous dysplasia and broken bones. A sample (biopsy) may be taken of bone tissue and any lumps in the thyroid gland.

Treatment
Treatment of McCune-Albright syndrome is focused on the symptoms the individual is having. Typical treatments for precocious puberty are generally not effective, so studies are testing other medications which might treat children with McCune-Albright syndrome. There are no effective treatments for fibrous dysplasia. The fractures it causes are treated medically and/or surgically. Excess growth hormone can be inhibited by taking (Sandostatin).

Sources:
- "McCune Albright Syndrome." NORD Rare Diseases Database. 2002. National Organization for Rare Disorders. 17 Dec 2006 <http://www.rarediseases.org/search/rdbdetail_abstract.html?disname=McCune%20Albright%20Syndrome>.
- Boston, Bruce. "McCune-Albright Syndrome." eMedicine. 24 Jul 2006. WebMD. 15 Dec 2006 <http://www.emedicine.com/ped/topic1386.htm>.
- Feuillan, Penelope. "McCune-Albright Syndrome: General Information." The MAGIC Foundation. 25 Sep 2006. The MAGIC Foundation. 17 Dec 2006 <http://www.magicfoundation.org/www/docs/109/mccune_albright_syndrome.html>.

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