[True hermaphroditism: experience with 36 patients].
OBJECTIVE
To show data on true hermaphrodites (TH) followed in two reference centers in the state of São Paulo.


METHODS
Twenty-six TH patients have been followed from 1975 to 2004 in the Pediatric Endocrinology Unit, São Paulo University Medical School, and the other 10 patients from 1989 to 2004 in the GIEDDS-UNICAMP.


METHODS
Clinical and pathological data were retrospectively analyzed, as well as the decision about the sex of rearing.


RESULTS
Genital ambiguity was the most frequent complaint (34/36 cases) and the age at the first appointment was 20 mo (median). In 55.6% of the patients the female option was taken. The most frequent karyotype was 46,XX (47.2%), followed by mosaicisms (27.8%). SRY was negative in all 46,XX TH patients and the prevalent gonad was ovotestis (OT-47%), followed by ovary (OV-27%) and testis (TT-24%). The prevalent gonadal associations were OV+TT (30.5%), OT+OT (22.2%) and OT+OV (22.2%). The decision about the sex of rearing did not depend on karyotype, phallus size, and testosterone levels. However, the location of the urethral meatus was taken into account in this decision: 20 (80%) patients out of the 25 with perineal urethra were reared as females while all 11 with "non-perineal" urethra were reared as males. In five 46,XX patients reared as females it was possible to maintain the ovarian portion of the ovotestis.


CONCLUSIONS
TH keeps on defying clinicians and investigators since most patients are 46,XX and SRY-negative, and yet they are able to develop testicular and ovarian tissues. The female sex of rearing seems to be the most adequate, and it is important to try to preserve, in these cases, the ovarian portion of the ovotestis, allowing the patient to have normal puberty and fertility.
Unidade de Endocrinologia Pediátrica, Instituto da Criança, Faculdade de Medicina, Universidade de São Paulo, SP. durvald@iconet.com.br
Durval
D
Damiani
Dulce Rondina
DR
Guedes
Daniel
D
Damiani
Nuvarte
N
Setian
Andréa T
AT
Maciel-Guerra
Maricilda Palandi de
MP
Mello
Gil
G
Guerra-Júnior
D000293:Adolescent; D000328:Adult; D002648:Child; D002675:Child, Preschool; D005260:Female; D006801:Humans; D007223:Infant; D008297:Male; D050090:Ovotesticular Disorders of Sex Development / Q000175:diagnosis; D012189:Retrospective Studies