- Individuals with this syndrome are genetic males with a 46,XY karyotype, but they have normal female external genitalia and are raised as girls.
- The basic defect is resistance of target tissues to the action of androgens.
- The fetal testes produce testosterone
- Because the Wolffian ducts and genital tissues cannot respond to testosterone, female differentiation of the external genitalia takes place.
- The fetal testes also produce Müllerian duct–inhibiting factor, which acts to inhibit the Müllerian anlage. As a consequence, the fallopian tubes, uterus, and upper vagina do not develop.
- Clinical features.
- The result is a phenotypic woman with a vagina that ends in a blind pouch, hypoplastic male ducts instead of the fallopian tubes and uterus, and testes located in the abdomen, inguinal canal, or labia majora.
- Endogenous estrogen stimulates normal breast development at puberty.
- The condition is suspected when menarche fails to occur or when a testis is felt as an abdominal mass, which is explored.
- The testes are prone to malignant degeneration and should be removed.
- Estrogen treatment is then given to maintain secondary sexual characteristics.
What Is The Definition Of Testicular feminization syndrome
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Testicular feminization syndrome – N. Vlahos
Testicular feminization syndrome (Androgen resistance)
17yo phenotypicalfemale with primary amenorrhea
Normal external genitalia
Normal breast development (Tanner III)
Complete Androgen Insensitivity Syndrome (Testicular Feminization) GENESYS FERTILITY CENTER
this is a case of a 26y old patient of female phenotype with perfectly normal secondary sexual characteristics. she has a mullerian remnant (rudimentary uterine horn) on her left side, along with the round ligament and a underdeveloped fallopian tube with fimbriae. there is only one gonad (testis), also on the left side. on her right side there is no gonadal or mullerian structure. blood caryotype shown a male genotype (46 XY)
How Is Testicular Feminization Inherited?