Complexities of gender assignment in 17β-hydroxysteroid dehydrogenase type 3 deficiency: is there a role for early orchiectomy?

Table 2 Gender outcomes in patients with 17βHSD-3 deficiency initially raised female

		Female gender maintained			Female to male reassignment
Study	Subjects (n)	Early orchiectomy (n)	No early orchiectomy (n)	Age of late orchiectomy (years)	n*	Age of reassignment (years)
Omrani [13]	3		3	?	0
Ismail [10]	13	5	2	?	6^ǂ	12,14,14,19
Bertelloni [11]	2	1	1	12	0
Mains [14]	1		1	15	0
Lee [15]	4		4	12,14,14	0
Mendonca [12]	10	2	5	15–34	3	15,15, 26
Rosler [16]	9		2	?	7	10, 12–18
Imperato [17]	1				1	>15
Millan [18]	1		1	?	0
Lanes [19]	3				3	11–13
Imperato [20]	1				1	14
Akesode [21]	1				1
Total	49	8	19		22

Only subjects followed until at least 12 or more years of age, with explicitly stated gender identity, were reported. Early orchiectomy was defined as orchiectomy occurring by age 10 years.
*None of the patients who had female to male gender reassignment had had a history of early orchiectomy.
^ǂTwo of the patients were assessed to have masculine psychosexual development, but their parents refused gender change.

ISSN: 1687-9856