Congenital adrenal hyperplasia

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Beta Switch Program by Sue Heintze

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Congenital adrenal hyperplasia is the commonest form of virilisation and is due to a genetic enzyme defect which results in deficient secretion of cortisol. This results in reduced negative feedback to the pituitary resulting in a very high ACTH. This produces adrenal hyperplasia and excess androgen production, especially androsterone which is converted to testosterone peripherally. Androsterone is a 17-ketosteroid and testosterone is not. Therefore, virilisation with an elevated urinary 17-keto steroid level suggest adrenal origin, while a normal or only slightly elevated 17-ketosteroid suggests excess testosterone from ovary or testis.

Congenital adrenal hyperplasia is treated by glucocorti-coids which supply cortisol requirements and suppress the ACTH. Cosmetic surgery may be required.

Virilism due to an adrenal tumour is usually secondary to a large malignant tumour. Surgical resection is still indicated despite the size, to control symptoms and to treat the tumour. Occasionally a good long-term remission may be obtained.

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