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  • A classic form of congenital adrenal hyperplasia characterized by complete absence of 21-hydroxylase activity, resulting in deficiency of glucocorticoids and mineralocorticoids accompanied by androgen excess, causing virilization in female infants. Mineralocorticoid deficiency results in renal salt-wasting, and, if untreated, hyponatremia, hyperkalemia, and shock.
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