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KMID : 0358419960390010231
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Korean Journal of Obstetrics and Gynecology
1996 Volume.39 No. 1 p.231 ~ p.244
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이재관
이경호/고희경/허준용/서호석/박용균/조수용/주갑순
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Abstract
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Congenital adrenal hyperplasia is an inborn error transmitted by an autosomal recessive gene and may be due to a defect in any of the enzymatic steps in the biosynthesis of cortisol.
Most affected individuals have a failure of 21-hydroxylation which prevents the conversion of 17 a-hydroxyprogesterone to 11-deoxy cortisol. A defect in 21-hydroxylase leads not only to inefficient formation of cortisol but also to excessive
production
of adrenal androgens causing virilization.
Female pseudohermaphroditism with congenital adrenal hyperplasia is the most common type of intersex problem seen in children.
In females with this disorder, the internal genital organs are usually normal, but variable degrees of virilization can be observed externally.
This is a case report of a 10-year-old girl with attenuated congenital adrenal hyperplasia who had clitorial hypertrophy.
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KEYWORD
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