McCune-Albright syndrome

(redirected from 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.

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 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 ?
Gastrointestinal polyps in McCune Albright syndrome. J Med Genet 2011 ;48:458-461.
Detailed history was taken to rule out any history of acne, precocious puberty, pigmented cutaneous lesions and endocrine abnormalities to rule out McCune Albright syndrome. She was referred to Rehabilitation department for further management of her limb length shortening, to improve joint mobility and to correct bone deformities.
McCune Albright syndrome may lead to pituitary micro or macroadenomas and related excessive secretion of GH and/or prolactin, acromegaly due to severe FD, unilateral or bilateral macroorchidism and rarely a clinical picture of precocious puberty secondary to sulphatase activity.
McCune Albright Syndrome. Orphanet J Rare Dis 2008: 3; 12.
Usually mandibular lesions are truly monostotic, where maxillary lesions often involves adjacent bones like zygoma, sphenoid and occipital, hence forth they are not strictly monostotic but they fall into designation of craniofacial forms.1 The other subtypes of polystotic fibrous dysplasia are cafe-u-lait pigmented skin lesions with endocrinopathies which is called Jaffe Lichtenstein syndrome5 and McCune Albright syndrome.2,4 Accordingly our case was craniofacial type with no associated endocrinopathy.
Polyostotic fibrous dysplasia or McCune Albright syndrome when associated with skin hyperpigmentation and endocrine dysfunction is another condition associated with elevated FGF23 concentrations.
Characterization of gsp-mediated growth hormone excess in the context of McCune Albright syndrome. J Clin Endocrinol Metab 2002; 87: 5104-12.
Growth hormone-prolactin-thyrotropin secreting pituitary adenoma in atypical McCune Albright Syndrome with functionally normal Gs alpha protein.
Colby was born with Neonatal Cushings McCune Albright syndrome - which only six others in the world are known to have had.
Bone graphies which were taken to investigate fibrous dysplasia observed in McCune Albright syndrome because of presence of Cafeau-lait spot and to investigate craniosynostosis related with hyperthyroidism revealed no pathological finding.
The spectrum of McCune Albright syndrome. Pediatr Endocrinol Rev.
Dental abnormalities17 and McCune Albright syndrome (18) have also been described by others.