Congenital adrenal hyperplasia (CAH) is associated with which of the following?
Correct Answer: Female pseudohermaphroditism
Description: Ref. Guyton. 13th edition. Page. 781
Congenital Adrenal Hyperplasia Types
21 β-Hydroxylase deficiency
Accounts for about 90% of the cases
75% of the cases have mineralocorticoid deficiency
Neonates may present with a salt-wasting crisis.
Salt wasters tend to have hyponatremia, hyperkalemia, and raised plasma renin.
17-hydroxyprogesterone is elevated.
Increased androgens lead to virilization of the female fetus and sexual ambiguity at birth
Males are phenotypically normal at birth but develop precocious pseu- do puberty, growth acceleration, premature epiphyseal plate closure, and diminished final height.
Goal in treatment is to bring glucocorticoid and mineralocorticoid back to the normal range which would also suppress adrenal androgen secretion.
Give hydrocortisone to act as feedback inhibition on pituitary
11 β-Hydroxylase deficiency
Clinical features of increased androgens similar to the preceding form,
including virilization of female fetus.
The principal difference with this form is the hypertension produced by 11-deoxycorticosterone, along with hypokalemia and suppressed renin secretion.
Treatment for all forms of CAH is glucocorticoids such as hydrocortisone and dexamethasone.
17 α-Hydroxytlase deficiency
Extremely rare
Usually diagnosed at the time of puberty when the patient presents with hypertension, hypokalemia, and hypogonadism
Individuals have eunuchoid characteristics.
Category:
Unknown
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