Congenital lipoid adrenal hyperplasia

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Congenital adrenal hyperplasia resulting from the deposition of lipid in the adrenal glands due to a defect of intracellular cholesterol transport or metabolism. The condition is characterized by deficiencies of glucocorticoids, mineralocorticoids, and sex steroids: 46,xy infants are undervirilized, whereas 46,xx infants have no genital manifestations.

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