释义 |
DictionarySeefeminizationtesticular feminization
testicular feminization[tes¦tik·yə·lər ‚fem·ə·nə′zā·shən] (medicine) A hereditary disorder in which affected individuals are chromosomally XY but have a feminine phenotype and are sterile. The X-linked testicular feminization (Tfm) gene results in absent or defective testosterone receptors, leaving cells unable to bind androgens and respond even though normal male levels of androgen are present. Also known as androgen insensitivity syndrome. testicular feminization
feminization [fem″ĭ-nĭ-za´shun] 1. the normal induction or development of female characters" >secondary sex characters.2. the induction or development of female characters" >secondary sex characters in the male.testicular feminization complete resistance" >androgen resistance.tes·tic·u·lar fem·i·ni·za·tion syn·drome [MIM*313700] a type of male pseudohermaphroditism characterized by female external genitalia (may be ambiguous if the syndrome is incomplete), incompletely developed vagina often with rudimentary uterus and fallopian tubes, female habitus at puberty with normal size breasts but with scanty or absent axillary and pubic hair and amenorrhea, and testes present within the abdomen or in the inguinal canals or labia majora; epididymis and vas deferens are usually present; androgens and estrogens are formed, but target tissues are largely unresponsive to androgens; individuals have a normal male karyotype; X-linked recessive inheritance, caused by mutation in the androgen receptor gene (AR) on chromosome Xq. See also: complete androgen insensitivity syndrome. tes·tic·u·lar fem·i·ni·za·tion syn·drome [MIM*313700] a type of male pseudohermaphroditism characterized by female external genitalia (may be ambiguous if the syndrome is incomplete), incompletely developed vagina often with rudimentary uterus and fallopian tubes, female habitus at puberty with normal size breasts but with scanty or absent axillary and pubic hair and amenorrhea, and testes present within the abdomen or in the inguinal canals or labia majora; epididymis and vas deferens are usually present; androgens and estrogens are formed, but target tissues are largely unresponsive to androgens; individuals have a normal male karyotype; X-linked recessive inheritance, caused by mutation in the androgen receptor gene (AR) on chromosome Xq. See also: complete androgen insensitivity syndrome. testicular feminization Androgen-insensitivity syndrome, feminization; Morris syndrome Endocrinology An X-linked pseudohermaphroditic state occurring in a genotypic XY ♂ with a ♀ phenotype; there is normal secretion of and response to müllerian inhibiting hormone, resulting in a phenotypic ♀ Clinical ♀ habitus, sexual behavior and 2º sex characteristics, associated with a blind vaginal pouch without uterine tissue, scanty pubic and axillary hair, and internally, undescended testicles; although the gonads often harbor Sertoli cell adenomas, malignancy occurs in 4% of TF Lab Normal testosterone levels. See Male pseudohermaphroditism. AcronymsSeeTFM |