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In page Congenital adrenal hyperplasia due to 17α-hydroxylase deficiency:

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Genetic females affected by total 17α-hydroxylase deficiency are born with normal female primary sex characteristics. At the expected time of puberty neither the adrenals nor the ovaries can produce sex hormones, so neither breast development nor pubic hair appear. Testing yields elevated gonadotropins and normal karyotype, while imaging confirms the presence of ovaries and an underdeveloped uterus. Discovery of hypertension and hypokalemic alkalosis usually suggests the presence of one of the proximal forms of CAH, and the characteristic mineralocorticoid elevations confirm the specific diagnosis.[citation needed]