SUMMARY :
Three family cases
of pseudohermaphroditism probably due to alpha 5 reductase deficiency, and another
case due to enzymopathy in the synthesis
of testosterone, were corrected surgically
and hormonally to a feminine direction.
This type of intervention was selected
because the cases in question had been assigned and raised as girls, and all of
them wished to maintain their female identity, refusing any other type of intervention.
The
psychological-assessment interview confirmed the feminine comportment in sexual
identity. On the date of the first medical examination, two of the family cases
and the enzymopathy one had already gone through puberty (13, 15 and 19 years
of age) ; the fourth case had still not reached puberty (7 years old). Presently
(22 years after surgical correction in the three family cases and 20 years in
the one from enzymopathy), somatic feminization is perfect in the case started
at pre-puberty, very good in two of the cases (one of the family ones – started
at 13, and the one from enzymopathy), and another considered unsatisfactory (the
remaining family case, started at 15 years of age).
None of the four has
called into question their feminine identity. All of them have or have had heterosexual
sexual relations, and they deny having homosexual feelings. They have also experienced
good professional adjustment. Two of the cases have received psychiatric support
for problems of a depressive nature. We discuss upbringing, genetic and hormonal
aspects in establishing sexual identity.