Albright syndrome


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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

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) .

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
References in periodicals archive ?
Obesity, medical conditions like Albright syndrome, congenital adrenal hyperplasia or hypothyroidism, or coming into direct contact with hormones oestrogen or testosterone are some of them.
McCune Albright syndrome (MAS) was diagnosed if patients with mono/ polystotic FD had either cafe-au-lait macules and/or endocrinopathy.
A 34-year-old man with a 20-year history of McCune Albright syndrome presented to the endocrinology department because of increasing multiple hard swellings over his legs and difficulty walking.
Acromegaly and its treatment in the Mc Cune Albright Syndrome.
Characterization of gsp-mediated growth hormone excess in the context of McCune Albright syndrome.
Growth hormone-prolactin-thyrotropin secreting pituitary adenoma in atypical McCune Albright Syndrome with functionally normal Gs alpha protein.
Polyostotic fibrous dysplasia or McCune Albright syndrome when associated with skin hyperpigmentation and endocrine dysfunction is another condition associated with elevated FGF23 concentrations.
Colby was born with Neonatal Cushings McCune Albright syndrome - which only six others in the world are known to have had.