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Old 05-14-2012
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Question Primary Ammenorrhea with Clitoromegaly

An 18-year-old woman arrives in your clinic with primary amenorrhea, sexual infantilism, and clitoromegaly.
She has a history of ambiguous external genitalia noted at birth. Reviewing her records, you see that
laparotomy performed at 17 months of age revealed normal internal female genitalia and ovarian biopsy
performed at that time revealed normal-appearing primordial follicles. Laboratory studies today reveal a
normal female karyotype and high serum testosterone and androstenedione concentrations. Estradiol and
estrone are undetectable in the serum. Serum FSH and LH concentrations are high. Pelvic imaging shows
multiple ovarian cysts What is the most likely diagnosis?
A. Congenital adrenal hyperplasia
B. Aromatase deficiency
C. McCune-Albright syndrome
D. Kallmann's syndrome
E. Galactosemia
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Old 05-14-2012
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this aromatase deficiency pt is not able to convert androgens into estrogens and will accumulate the first leading to the presentation described above
the Ovrian cyst, ? i think follicular; unruptured;
high FSH,LH due lack of inhibition from Est & Pro
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Old 05-14-2012
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A. Congenital adrenal hyperplasia --

most common is 21 hydroxylase defficiency. would have symptoms of decreased ALDOSTERONE. (low BP, high K, low Na, metabolic acidosis) and decreased CORTISOL (extreme hypoglycemia)

B. Aromatase deficiency
increased amount of testosterone. no conversion to estrogens.

remember from step 1-- formation of estrogens in the ovaries is a two step process, occurring in the granulosa and theca cells separately.

(check out image 3 on this page http://herkules.oulu.fi/isbn95142684...l/i231654.html)

so a decrease in aromatase wud cause increased FSH/LH and the testosterone resulting in clitoromegally.


ovarian cysts might be due to excessive action of LH/FSH--(not sure about this)



C. McCune-Albright syndrome
precocious puberty and bone lesions

D. Kallmann's syndrome
anosmia and defficient GnrH

E. Galactosemia
doesnt fit here
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Old 05-14-2012
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Quote:
Originally Posted by mbbs2010 View Post

A. Congenital adrenal hyperplasia --

most common is 21 hydroxylase defficiency. would have symptoms of decreased ALDOSTERONE. (low BP, high K, low Na, metabolic acidosis) and decreased CORTISOL (extreme hypoglycemia)

B. Aromatase deficiency
increased amount of testosterone. no conversion to estrogens.

remember from step 1-- formation of estrogens in the ovaries is a two step process, occurring in the granulosa and theca cells separately.

(check out image 3 on this page http://herkules.oulu.fi/isbn95142684...l/i231654.html)

so a decrease in aromatase wud cause increased FSH/LH and the testosterone resulting in clitoromegally.


ovarian cysts might be due to excessive action of LH/FSH--(not sure about this)



C. McCune-Albright syndrome
precocious puberty and bone lesions

D. Kallmann's syndrome
anosmia and defficient GnrH

E. Galactosemia
doesnt fit here
thanks...I want to add that in congenital adrenal hyperplasia there are low levels of FSH and LH because of the high levels of Androgens...and yes it is right that the cause of polycystic ovary in Aromatase deficiency is the high levels of FSH....
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