Article
46,XY disorder of sex development (DSD) due to 17β-hydroxysteroid dehydrogenase type 3 deficiency.
The Journal of steroid biochemistry and molecular biology - 1 Jan 2017
Mendonca Berenice B, Gomes Nathalia Lisboa, Costa Elaine M F, Inacio Marlene, Martin Regina M, Nishi Mirian Y, Carvalho Filomena Marino, Tibor Francisco Denes, Domenice Sorahia
Abstract excerpt
17β-hydroxysteroid dehydrogenase 3 deficiency consists of a defect in the last phase of steroidogenesis, in which androstenedione is converted into testosterone and estrone into estradiol. External genitalia range from female-like to atypical genitalia and most affected males are raised as females. Virilization in subjects with 17β-HSD3 deficiency occurs at the time of puberty and several of them change to male...
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