McCune-Albright Syndrome
Polyostotic Fibrous Dysplasia
Some characteristics of this disease are irregularities in the pigment of the skin causing cafe-au-lait spots; bone diseases which cause bone weakness, fractures, deformities in the legs, arms and skull; and endocrine problems. The endocrine system is the hormone-producing system of the body and this disease can cause early puberty (precocious puberty) and increased growth rage. Precocious puberty is far more common in affected girls than boys but other manifestations of McCune-Albright Syndrome occur equally in both sexes.
The first symptom of characteristic lesions of cafe-au-lait spots is often present at birth. These are large, flat, light brown spots. They are distinguished by the serrated borders which is called "coast of Main" and they have the tendency to stop abruptly at the midline of the body. Many other children have cafe-au-lait spots, but normally these have smooth borders and they are called, "coast of California".
The People with McCune-Albright Syndrome have different levels of severity. Sufferers can be affected by all these characteristics to different degrees and some may have the condition and have no noticeable problems at all.
McCune-Albright Syndrome is listed as a rare disease which means it affects less than 200,000 people in the US population. The following are a list of signs and symptoms for McCune-Albright Syndrome:
Bone disease--fibrous growths in bones:
Fractures
Bone deformity
Leg deformity
Arm deformities
Jaw deformity
Sex Hormone Problems:
Early puberty (most often seen in girls)
Early menstruation (as early as 4 months of age)
Early breast development (as young as 4 months)
Early development of testes
Early public hair
Early underarm hair
Increased rate of growth
Ovarian cysts
Irregular menstrual bleeding
Skin Changes (seen in about 50% of cases and is often present in infancy)
"Cafe-au-lait" spots
Increased pigment
Thyroid Gland Abnormalities (in about 50% of the cases)
Goiter
Thyroid nodules
Pituitary Gland Problems
Acromegaly-like symptoms (tallness)
Coarse facial features
Enlarged hands
Enlarged feet
Arthritis
Adrenal Gland Problems
Cushing's syndrome-like symptoms (growth failure and hypertension in infancy)
Weight gain
Skin fragility
Delayed growth
Phosphorus Problems
Excessive phosphate in urine
Rickets
Apart from a small group of patients that develop malignancies, McCune-Albright Syndrome is not associated with a significantly increased mortality risk.
Properly treated, the prognosis for people with McCune-Albright Syndrome is excellent for longevity and intelligence.
Published by Doreen Bradley Satter, RN
DOREEN BRADLEY SATTER, RN is a mostly-retired Registered Nurse, Artist, Published Author and Freelance Writer and has been writing for the Yahoo! Contributor Network for several years. She has one published... View profile
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- The first symptom is usually 'cafe-au-lait' spots.
- McCune-Albright Syndrome is listed as a rare disease.
- Properly treated, the prognosis for people with McCune-Albright Syndrome is excellent.