robin082006
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A 16-year-old girl is brought to your office by her mother with a chief complaint of primary amenorrhea. She denies any particular symptoms. She is not sexually active and has never had a boyfriend. Her personal and family medical histories are normal. On examination, you note fully developed breasts and absent axillary and pubic hair; external genitalia have a normal appearance, but no vagina is noted. Initial work-up reveals no uterus on ultrasound and an FSH level of 95 U/L (Normal is 4-30). Chromosomal study reveals a 46, XY karyotype. Which of the following events is most likely to have caused the absence of in utero development of the internal reproductive organs?
A. Absence of Mullerian Inhibiting Factor secretion
B. Presence of Mullerian Inhibiting Factor secretion
C. Agenesis of Wolffian ducts
D. Agenesis of Mullerian ducts
E. Testosterone surge
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| sarika
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B. Presence of Mullerian Inhibiting Factor secretion
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| prathapdoctor
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the history and findings are suggestive of klinifelters syn except karyotype findings,i will go with option B.
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| mazinger
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I go for C, this is an androgen insensitivity syndrome, klinefelters are phenotypically males... Wolf ducts develope thanks to the action of testosterone... Thats what I remember from step 1 of course comments are welcome...
Edited part ahead ---> now that I get to think of it I made dumb conclusions it is an androgen insensitivity but there is no uterus thanks to an involution of mullerian ducts thanks to the presence of antimullerian factor... Wolfian ducts form male internal genitalia..
The answer is B
Sorry....
Edited by mazinger on 01/04/07 - 09:58 PM
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original mazinger z
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| r_albayunen
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B
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| robin082006
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yes, answer is B
This is androgen insensitivity syndrome.
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