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Old 09-20-2012
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Question A 13-Yr old boy with 17a-hydroxylase deficiency

A 13-year-old boy is brought to the pediatrician by his mother because of increasing body hair. Several months earlier he had been diagnosed with 17α-hydroxylase defi ciency and treated with hydrocortisone. Physical examination reveals
an overweight boy with a moderate amount of both chest and genital hair, and some facial hair growth. His physical examination is otherwise unremarkable. Which of the following is the best treatment for this patient?

(A) Add cosyntropin
(B) Add dexamethasone
(C) Add spironolactone
(D) Increase hydrocortisone
(E) Keep the current dose of hydrocortisone
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C)

You can add spironolactone... her only complaint is that the boy is starting to appear like a gorilla, not anything else.. the treatment he is receiving is ok.. spironolactone inhibits androgen synthesis and androgen receptors.. so i think its ok to give it.
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Quote:
Originally Posted by XpaezX View Post
C)
her only complaint is that the boy is starting to appear like a gorilla
Gorilla
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I would opt for Choice E.
Keep the current treatment as such...

17 alpha hydroxilase is cause of undervirulisation in male as testosterone is not formed...

treatment is by glucocorticoids...

Here on treatment child is showing signs of virulisation so signs of recovery.. hence continue same treatment...
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Answer C . Spironolactone has anti androgen effects
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**** is true ... all patients with 17 hydroxylase deficiency are phenotypically female.. so hair means this little monkey is recovering.. **** i can be stupid sometimes
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C- Add spironolactone.
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ans ........D.........

increase the dose as pt's ACTH is not suppressed thus he's making more androgens , supressing acth with higher dose will solve it ......
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I think this question is wrong, it should be 21a-hydroxylase deficiency.
There is no way he can make androgens with 17a-hydroxylase deficiency.
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Last edited by Novobiocin; 09-20-2012 at 04:29 PM.
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The correct answer is B. In individuals with 17α-hydroxylase deficiency, ACTH secretion is elevated secondary to decreased cortisol levels. The goal of treatment with hydrocortisone is not only to replenish cortisol, but also to suppress ACTH secretion. The reason for this is that if ACTH remains high, it will stimulate androgen production and lead to premature virilization and growth plate ossification. Nightly dexamethasone treatments may be necessary to more completely suppress ACTH secretion in adolescents.
Answer A is incorrect. Cosyntropin stimulates ACTH production, and cosyntropin levels are used to diagnose 1-hydroxylase deficiency. Answer C is incorrect. Spironolactone is a potassium-sparing diuretic and would not affect ACTH levels.
Answer D is incorrect. Increased hydrocortisone alone may not be suffi cient to suppress ACTH secretion in this patient.
Answer E is incorrect. This individual is exhibiting signs of increased virilization and likely needs further suppression of ACTH secretion to prevent premature skeletal maturation.
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Quote:
Originally Posted by Novobiocin View Post
I think this question is wrong, it should be 21a-hydroxylase deficiency.
There is no way he can make androgens with 17a-hydroxylase deficiency.
Now m also thinking that question is wrong ......
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Quote:
Originally Posted by Novobiocin View Post
I think this question is wrong, it should be 21a-hydroxylase deficiency.
There is no way he can make androgens with 17a-hydroxylase deficiency.
absolutely great ! i didnt even bother to think if androgens can be made or not just tried for the ans ............
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Quote:
Originally Posted by K06100 View Post
Now m also thinking that question is wrong ......
It is wrong.

In 17α-hydroxylase deficiency androgens are the treatment. You can't block which is not there by giving more glucocorticoids. This boy needs androgens NOT attempts to block them.

Quote:
At puberty, male patients require sex steroid replacement (ie, testosterone). Female patients require cyclic estrogen-progesterone therapy. These therapies promote development of secondary sexual characteristics in both sexes and cyclic menstrual bleeding in 46,XX females.
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