McCune-Albright syndrome


Mc·Cune-Al·bright syn·drome

(măk-kyūn' awl'brīt), [MIM*174800] polyostotic fibrous dysplasia with irregular brown patches of cutaneous pigmentation and endocrine dysfunction, especially precocious puberty in girls. Infrequently, associated with primary hyperthyroidism gigantism or acromegaly, ACTH-independent Cushing syndrome, and chronic active (that is, nonautoimmune or viral) hepatitis.
See also: pseudohypoparathyroidism. Synonym(s): Albright disease, Albright syndrome (1)

McCune-Albright syndrome

(mə-kyo͞on′ôl′brīt)n. Fibrous dysplasia involving multiple bones, caused by a somatic mutation and characterized by irregular brown spots on the skin, endocrine dysfunction, and precocious puberty, especially in girls.

McCune-Albright syndrome

An autosomal dominant condition (OMIM:174800) due to altered regulation of cAMP, endocrinopathy (e.g., hyperthyroidism) and hypophosphatemia.
Clinical findings
Precocious puberty, polyostotic (cystic fibrous dysplasia) spontaneous fractures at young age, café-au-lait spots on skin, ovarian cysts.
 
Lab
Cyclical 4–6-week fluctuations of plasma oestrogen; afflicted young girls have decreased gonadotropins, decreased response to LH-RH; increased testosterone, increased alkaline phosphatase.
 
Molecular pathology
Defects in GNAS, which encodes a G protein that modulates various membrane signalling cascasdes, cause McCune-Albright syndrome.
Management
Aromatase inhibitor testolactone.

Mc·Cune-Al·bright syn·drome

(mik-kyūn' awl'brīt sin'drōm) Polyostotic fibrous dysplasia with irregular brown patches of cutaneous pigmentation and endocrine dysfunction, especially precocious puberty in girls.
See also: pseudohypoparathyroidism
Synonym(s): Albright disease, Albright syndrome (1) .

McCune-Albright syndrome (MCAS)

A genetic syndrome characterized in girls by the development of ovarian cysts and puberty before the age of 8, together with abnormalities of bone structure and skin pigmentation.Mentioned in: Ovarian Cysts

Albright,

Fuller, U.S. physician, 1900-1969. Albright disease - Synonym(s): McCune-Albright syndromeAlbright-Hadorn syndrome - softening and bending of bones associated with abnormally small concentration of potassium in blood.Albright hereditary osteodystrophy - an inherited form of hyperparathyroidism associated with ectopic calcification and ossification and skeletal defects. Synonym(s): Albright syndrome (1)Albright syndrome - (1) Synonym(s): Albright hereditary osteodystrophy - (2) Synonym(s): McCune-Albright syndromeAlbright IV syndrome - Synonym(s): Martin-Albright syndromeAlbright synovectomyForbes-Albright syndrome - see under Forbes, Anne PMartin-Albright syndrome - see under Martin, August EMcCune-Albright syndrome - see under McCune

McCune,

Donovan James, U.S. pediatrician, 1902-1976. McCune-Albright syndrome - polyostotic fibrous dysplasia with irregular brown patches of cutaneous pigmentation and endocrine dysfunction, especially precocious puberty in girls. Synonym(s): Albright disease; Albright syndrome (2)

Mc·Cune-Al·bright syn·drome

(mik-kyūn' awl'brīt sin'drōm) [MIM*174800] Polyostotic fibrous dysplasia with irregular brown patches of cutaneous pigmentation and endocrine dysfunction.
Synonym(s): Albright disease, Albright syndrome (1) .