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. 2020 Mar 20;9(4):R81–R92. doi: 10.1530/EC-19-0537

Table 1.

Examples of 46,XY differences/disorders of sex development (DSD).

Condition Incidence Comment Advantage in sports?
5α-reductase deficiency type 2 Extremely rare with geographical regions of higher incidence Individuals may be assigned female sex at birth. Virilization proceeds at puberty. Yes
Partial androgen insensitivity (PAIS) 1/130,000 Ambiguous genitalia at birth. From puberty, high testosterone, but with varying effect depending on androgen receptor sensitivity. Possibly
17β-hydroxysteroid dehydrogenase type 3 (17β- HSD3) deficiency 1/147,000 Individuals are often raised as girls but undergo virilization at puberty. Yes
Ovotesticular DSD 1/100,000 The individual has both ovarian and testicular tissue. Born with ambiguous genitalia. Virilization may proceed at puberty. Possibly