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)

Farlex Partner Medical Dictionary © Farlex 2012

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.

Albright syndrome

McCune-Albright syndrome, see there.

Al·bright he·red·i·tar·y os·te·o·dys·tro·phy

(awl'brīt hĕr-ed'i-tar-ē os'tē-ō-dis'trŏ-fē)

An inherited form of hyperparathyroidism associated with ectopic calcification and ossification and skeletal defects, notably small fourth metacarpals; intelligence may be normal or subnormal. Inheritance is heterogeneous; the autosomal form is caused by mutation in the guanine nucleotide-binding protein gene (GNAS1) on 20q. There are the recessive and X-linked forms.
See also: pseudohypoparathyroidism
Synonym(s): Albright syndrome (2) .

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) .

Medical Dictionary for the Health Professions and Nursing © Farlex 2012

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

Medical Eponyms © Farlex 2012

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References in periodicals archive

Gastrointestinal polyps in McCune Albright syndrome. J Med Genet 2011 ;48:458-461.

Severe Neonatal Cholestasis as an Early Presentation of McCune-Albright Syndrome

Polyostotic fibrous dysplasia may be associated with Mc-Cune Albright syndrome and Jaffe-Lichtenstein syndrome.

Maxillary Fibrous Dysplasia with Cone Beam Computed Tomography Findings: A Case Report/Konik Isinli Bilgisayarli Tomografi Bulgulari Olan Maksiller Fibroz Displazi: Bir Olgu Sunumu

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.

POLYOSTOTIC FIBROUS DYSPLASIA

McCune Albright syndrome (MAS) was diagnosed if patients with mono/ polystotic FD had either cafe-au-lait macules and/or endocrinopathy.

Fibrous dysplasia & McCune-Albright syndrome: an experience from a tertiary care centre in north India

Acromegaly and its treatment in the Mc Cune Albright Syndrome. Clin Endocrinol 1992; 36: 605-8.

Characterization of gsp-mediated growth hormone excess in the context of McCune Albright syndrome. J Clin Endocrinol Metab 2002; 87: 5104-12.

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

Colby was born with Neonatal Cushings McCune Albright syndrome - which only six others in the world are known to have had.

Sick toddler takes steps to stun docs; SUCCESS

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 in association with excessive GH secretion: case report

The spectrum of McCune Albright syndrome. Pediatr Endocrinol Rev.

Fibrous dysplasia

Polyostotic fibrous dysplasia or McCune Albright syndrome when associated with skin hyperpigmentation and endocrine dysfunction is another condition associated with elevated FGF23 concentrations.

Hypophosphataemic rickets/osteomalacia--is there light at the end of the tunnel?

To distinguish Graves' disease and Mc Cune Albright syndrome, TSH receptor antibody (TRAb) level and bone survey (X-ray) were evaluated.

A case of infantile-onset Graves