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adrenogenital syndrome

Adrenogenital syndrome is the autosomal recessive form of congenital adrenal hyperplasia due to 21-hydroxylase deficiency. 21-hydroxylase is an enzyme needed in the synthesis of cortisol. Diminished levels of cortisol results in excessive secretion of ACTH which stimulates adrenal hyperplasia which secretes excessive androgen. Affected genetic females under the influence of androgen would display masculine characteristics such as hirsutism (excessive body hair), small breasts, heavy arms and legs and enlarged clitoris. Affected genetic males display enlargement of penis with small testes, and increased pigmentation. With early treatment, prognosis is good.

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