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NBME Q Answer

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structure
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Topics: 26
Posts: 28

08/15/06 - 05:15 PM

a 5 day old girl has been feeding poorly since birth . She weighed 2900 g at bit=rth and now weighs 2600 g, Physical examination shows an enlarged clitoris and labial fusion. A sibling born 5 yrs ago died at 1 week of age. A life-threatening complication of this syndrome results from failure to produce which of the following?

A. Aldosterone
B Cholesterol
C. Estrogen
D. Indulin
E. Testosterone

I will pick C

Perhaps
Forum Elite

Topics: 61
Posts: 162

08/15/06 - 06:00 PM

I picked A
I think it is 21 hydroxylase defiency which leads to aldosterone deficiency and hypotesion

frank100
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Topics: 48
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08/15/06 - 07:52 PM

yes, it is (a), at least for me.

study_ing
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08/15/06 - 11:56 PM

me thinks A too. smiling face

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study_ing
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08/15/06 - 11:57 PM

structure u r right about the estrogen deficiency but here they have asked about the LIFE THREATENING deficiency ( in their typical sneaky style!!)

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achilles
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08/16/06 - 06:20 AM

a-aldosterone deficiency

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shumaila
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08/16/06 - 11:34 AM

but 21 hydroxylase def leads to congenital adrenal hyperplasia which lead to excessive production os aldosterone???????

correct me Iam having onfusion regarding this

Perhaps
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08/16/06 - 11:49 AM

shumaila wrote:
but 21 hydroxylase def leads to congenital adrenal hyperplasia which lead to excessive production os aldosterone???????

correct me Iam having onfusion regarding this

The hyperplasia is because of low corstisol -> increase ACTH. 21 hydroxylase is required to make aldosteron. Even with hypertrophy, no aldosterone can be made due to lacking of this enzyme. Hope this is correct.

usmlecracker
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Topics: 37
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08/16/06 - 02:06 PM

a is ans
estrogen def is not life threatening
shumaila..
read the chart in page 58 kaplan

AAAAA
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08/16/06 - 03:11 PM

#10

A

This baby with 21-hydroxylase deficiency have a shortage of the 21-hydroxylase enzyme, which impairs the conversion of cholesterol to cortisol and aldosterone. When the precursors of cortisol and aldosterone build up in the adrenal glands, they are converted to male sex hormones called androgens.

I think "lack of cortisol and aldosterone" are life-threatening to this baby. Correct me if I am wrong !

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AAAAA
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08/16/06 - 03:15 PM

#11

structure wrote:
a 5 day old girl has been feeding poorly since birth . She weighed 2900 g at bit=rth and now weighs 2600 g, Physical examination shows an enlarged clitoris and labial fusion. A sibling born 5 yrs ago died at 1 week of age. A life-threatening complication of this syndrome results from failure to produce which of the following?

A. Aldosterone
B Cholesterol
C. Estrogen
D. Indulin
E. Testosterone

I will pick C

Others have said estrogen deficiency would not cause life-threatening situation but lack of cortisol due to 21 hydroxylase enzyme deficiency is life-threatening !

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