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

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

  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 ? 6ǂ 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  
  1. 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.
  2. *None of the patients who had female to male gender reassignment had had a history of early orchiectomy.
  3. ǂTwo of the patients were assessed to have masculine psychosexual development, but their parents refused gender change.