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KMID : 0358219950220010023
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Korean Journal of Fertility and Sterility
1995 Volume.22 No. 1 p.23 ~ p.28
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A Case of Male Pseudohermaphroditism due to 17B-Hydroxysteroid Dehydrogenas Deficiency
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Abstract
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A deficiency of 17β-hydroxysteroid dehydrogenase is a rare cause of male pseudohermaphroditism and involves the final step in testosterone biosynthesis; reduction of the 17-keto group of androstenedione to form testosterone.
A 35-year-old female was admitted for evaluation of primary amenorrhea. Past history had showed no pubertal virilization. Physical examination showed female breast and normal female external genitalia with short blind-ending vagina. The
chromosome
karyotype of the patient was 46, XY. The plasma levels of estradiol and testosterone were decreased and FSH, LH increased. Androstenedione was increased and revealed elevated androstenedione/testosterone ratio. Bilateral orchiectomy performed and
the
patient is medicated with cyclic oral estrogen/progesterone.
We report a case of male pseudohermaphroditism due to 17β-hydroxysteroid dehydrogenase deficiency who showed no postpubertal virilization.
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