Believe
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A 14-year-old girl is brought to the physician's office by her mother who is concerned that the girl has not begun to menstruate. Physical examination shows normal breast development, no pubic hair, normal female external genitalia, bilateral inguinal hernias, and a vagina that ends in a blind pouch, and no palpable uterus or ovaries. Ultrasound examination confirms the absence of internal female genitalia. The underlying problem in this patient is related to which of the following?
A) Defective androgen receptors
B) Excess production of fetal estrogen
C) Excess production of fetal progesterone
D) Insufficient fetal testosterone production
E) Insufficient production of paramesonephric (müllerian) inhibitory factor during fetal development
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| cyra
Moderator
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A
I am pathetic when it comes to these syndromes...but i think it is testicular feminization syndrome/androgen insensitivity syndrome.
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| reet
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yeah,, i think A too
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| roopashri
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its a case of defective androgen receptor protein in which they r insensitive to testosterone. the genotype is usually 46 XY, meaning genotypically male ....but becoz of the defective receptors (during fetal life)male sexual development is impaired n female external genitalia are formed but internally u wont find a uterus.......as in this case. the bilateral inguinal hernias r the male gonads.(undescended). ....so there u go the answer is A
read carefully....it says "absence of internal female genitalia"
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| Believe
Forum Senior
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The syndrome of complete androgen resistance and its "variant" form.
A, A 17-year-old patient with the complete syndrome. This phenotypic female was chromatin negative, had a 46,XY karyotype, and had total absence of sexual hair with female secondary sexual characteristics. A small vagina ended blindly.
B, The testes exhibited Leydig cell hyperplasia and seminiferous tubules that lacked germinal elements.
C, At laparotomy, abdominal testes, rudimentary wolffian structures, and no müllerian structures were found.
D, The variant form of syndrome in a 25-year-old female. Sexual hair was present, although sparse.
E, The testes exhibited Leydig cell hyperplasia.
F, The clitoris was hypertrophied, but there was no labial fusion. A shallow vagina ended blindly. At laparotomy, hypoplastic wolffian structures and absent müllerian structures were noted.
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Focus on the one step you can take right now. Let go all regrets and resentment about what has already happened.
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| drk1980
Forum Guru
Topics: 147
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Ur efforts are highly appreciated!!
Wasnt aware these patients had a broad ligament...
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| Musuq
Forum Guru
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A) Defective androgen receptors
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| ManuNastai
Transilvanian..
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A
very nice pictures
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