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

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 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

单词	albright syndrome
释义	Albright syndrome Mc·Cune-Al·bright syn·drome (măk-kyūn' awl'brīt), [MIM174800] 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 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 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
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