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17-beta Hydroxysteroid Dehydrogenase 3 Deficiency

(also: 17 beta, 17-beta HSD)

Table of Contents

General Information

People with this intersex variation have XY chromosomes and testes. They do not have one of the enzymes needed to synthesize testosterone. Most people with this variation have a vagina, vulva, and undecended testes. Some have ambiguous genitalia. Some have a micropenis that may have proximal hypospadias.

During puberty, people with 17 beta will often develop secondary sex characteristics associated with a typical testosterone puberty.

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