Archives de pediatrie : organe officiel de la Societe francaise de pediatrie · 1994
[Disorder of gender identity in a child with asymmetric gonadal dysgenesis with complex karyotype abnormality].
Клинический случайАннотация на английскомСтатьяНазвание и аннотация: Перевод в очереди
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Hourtane M, Leheuzey M F
Аннотация
BACKGROUND: Gender identity is influenced by genetic and hormonal factors and also by the sex of rearing.
CASE REPORT: A child was born with ambiguous genitalia. Male sex assignment was made. Hypospadias and left inguinal hernia were present. The hernial sac contained an ovarian tissue. Plasma testosterone, 17-OH progesterone, cortisone levels were normal as were basal FSH and LH levels. There was a vagina but no uterus. The karyotype showed 45X/46XY mosaicism. The testis in the right scrotum was removed and vulvoplasty was performed at the age of 10 months; the patient was than raised as a female. Several courses of reconstructive genital surgery were necessary between 1 and 8 years of age. The girl suffered from trichotillomania and sleep disorders from the age of 4 years and learning difficulties and social phobia from the age of 8. When 11.5 years-old, she had clinical features of Turner's syndrome, was depressive but appeared to prefer male playmates.
CONCLUSION: Two mechanisms may explain alterations in gender identity in this patient with asymmetric gonadal dysgenesis; 1) early and prolonged androgen exposure as seen in patients with congenital adrenal hyperplasia; 2) insufficient counselling with parents regarding questions of gender identity.
CASE REPORT: A child was born with ambiguous genitalia. Male sex assignment was made. Hypospadias and left inguinal hernia were present. The hernial sac contained an ovarian tissue. Plasma testosterone, 17-OH progesterone, cortisone levels were normal as were basal FSH and LH levels. There was a vagina but no uterus. The karyotype showed 45X/46XY mosaicism. The testis in the right scrotum was removed and vulvoplasty was performed at the age of 10 months; the patient was than raised as a female. Several courses of reconstructive genital surgery were necessary between 1 and 8 years of age. The girl suffered from trichotillomania and sleep disorders from the age of 4 years and learning difficulties and social phobia from the age of 8. When 11.5 years-old, she had clinical features of Turner's syndrome, was depressive but appeared to prefer male playmates.
CONCLUSION: Two mechanisms may explain alterations in gender identity in this patient with asymmetric gonadal dysgenesis; 1) early and prolonged androgen exposure as seen in patients with congenital adrenal hyperplasia; 2) insufficient counselling with parents regarding questions of gender identity.