McCune-Albright syndrome

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Related to McCune-Albright syndrome: neurofibromatosis, Carney complex, Silver-Russell 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)

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 syndrome

Albright-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 syndrome

Albright IV syndrome - Synonym(s): Martin-Albright syndrome

Albright synovectomy

Forbes-Albright syndrome - see under Forbes, Anne P

Martin-Albright syndrome - see under Martin, August E

McCune-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) .

References in periodicals archive ?

McCune-Albright syndrome and acromegaly: effects of hypothalamo-pituitary radiotherapy and/or pegvisomant in somatostatin analog-resistant patients.

A rare cause of acromegaly: short review of McCune Albright syndrome/Akromegalinin nadir bir sebebi: McCune Albright sendromu icin kisa literatur ozeti

A case of pituitary adenoma associated with McCune-Albright syndrome. Pituitary 1999: 3; 297-302.

Macroorchidism in an Indian Boy with McCune-Albright Syndrome. Indian J Pediatr 2011: 78; 1145-7.

Clinical presentation of McCune-Albright syndrome in males.

McCune Albright syndrome in association with excessive GH secretion: case report

McCune-Albright syndrome. J Pediatr Endocrinol Metab.

Genetics of McCune-Albright syndrome. J Pediatr Endocrinol Metab.

McCune-Albright syndrome and disorders due to activating mutations of GNAS1.

Genetic and molecular aspects of McCune-Albright syndrome. Pediatr Endocrinol Rev.

Fibrous dysplasia

Fibrous dysplasia of bone in the McCune-Albright syndrome: Abnormalities in bone formation.

Fibrous dysplasia of the frontal bone

[1] There are two primary categories of the disease: monostotic fibrous dysplasia involves a single bone and represents 70% of all cases; polyostotic fibrous dysplasia involves multiple bones and makes up the remainder, [2] Also, approximately 3% of patients with fibrous dysplasia will experience McCune-Albright syndrome, in which the bony involvement is accompanied by skin lesions and endocrinopathy.

McCune-Albright syndrome occurs when fibrous dysplasia is accompanied by the appearance of scattered areas of melanotic skin pigmentation (cafe-au-lait spots) and sexual precocity.

Fibrous dysplasia of the temporal bone: Report of a case and a review of its characteristics

Polyostatic fibrous dysplasia is seen in both Jaffe-Lichtenstein and McCune-Albright syndromes, along with skin hyperpigmentation (cafe au lait tan macules) and sexual precocity (the most common endocrine disturbance).