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 |
? |
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 | ||
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.