intercourse
Androgen insensitivity. In these genetically male (46,XY) individuals
with complete lack of androgen receptor function, their bodies do not
respond to the high levels of androgens present.
Without androgen stimulation, internal Wolffian duct structures atrophy.
With testicular Müllerian inhibitory factor present, the Müllerian duct
derivatives involute.
Without body recognition of dihydrotestosterone, external genitalia
differentiate in a female direction. Patients function psychologically and
physically as females and are brought up as girls. At puberty, when
primary amenorrhea is noted, the diagnosis is made.
Female secondary sexual characteristics are present because the testes
do secrete estrogens without competition from androgens. No pubic or
axillary hair is noted. Testosterone levels are normal male.
Management. Testes removal at age 20 because the higher temperatures
associated with the intra-abdominal position of the testes may lead to
testicular cancer. Estrogen replacement is then needed.
Breasts absent, uterus present. Differential diagnosis is gonadal dysgenesis
(Turner syndrome) and hypothalamic–pituitary failure. FSH level and
karyotype help make the diagnosis.
Gonadal dysgenesis. Turner syndrome (45,X) is caused by the lack of
one X chromosome, essential for the presence of normal ovarian follicles.
Instead of developing ovaries, patients develop streak gonads. FSH is
elevated because of lack of estrogen feedback to the hypothalamus and
pituitary. No secondary sexual characteristics are noted.
Management. Estrogen and progesterone replacement for development
of the secondary sexual characteristics.
Hypothalamic–pituitary failure. In the patient without secondary
sexual characteristic but uterus present by ultrasound, another possibility is
the hypothalamic causes of amenorrhea (stress, anxiety, anorexia nervosa,
kiana
(Kiana)
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