To the Editor: Congenital adrenal hyperplasia due to 21-hydroxylasedeficiency results in deficient production of cortisol and aldosterone,chronic stimulation of the adrenal cortex by corticotropin,and overproduction of androgens. Cortisol-replacement therapyoften fails to normalize corticotropin and androgen secretion,and high doses may be needed.1 Adrenocortical tumors, includingcancers, are rare in patients with 21-hydroxylase deficiencybut have been reported in patients with large adrenal glandsand presumably inadequate cortisol therapy.2
A 16-year-old girl with 21-hydroxylase deficiency was evaluatedfor hirsutism and primary amenorrhea. She had a muscular habitus,hyperpigmentation, and a low voice. Physical examination revealeda beard, . . . [Full Text of this Article]
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