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female.why?

Supposed to be male, but looks like a

incomplete androgenic exposure of a fetus predestined to be male. Karyotype: still 46,XY, Will either have testes or no gonads.

Inadequate production of testosterone by the fetal testis. Diminished responsiveness of the genital anlagen to normal quantities of androgen failure of 5-DHT formation

Uterus, fallopian tubes, and upper vagina do not develop because: testes were present for at least some time in embryonic life, so mllerian-inhibiting substance is produced.

Fetal testicular testosterone production may fail if there is: An enzymatic defect of steroidogenesis Abnormality in the LH-hCG receptor Leydig cell hypoplasia.

Formerly testicular feminization most extreme form of androgen resistance syndrome No tissue responsiveness to androgen female phenotype with short, blind- ending vagina, no uterus or fallopian tubes and no wolffian duct structures.

Incomplete androgen insensitivity slightly responsive to androgen with modest clitoral hypertrophy at birth; Develop pubic and axillary hair at puberty but no virilization

Partial Androgen Insensitivity Syndrome- XY Female (Male Pseudohermaphroditism)

24 years old unmarried female presented with complaints of failure to menstruate.

After orchidectomy and vaginoplasty to establish coitally functional vagina was done, patient was kept on long term estrogen replacement therapy along with psychological support and follow up

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