Table 1

Description of the six cases of 46,XX males and 46,XX true hermaphrodites

Patient and ageDiagnosisExternal genitaliaInternal genitaliaGonadsTestosterone
JW (control) 15 yXX maleMaleMaleTestes with no signs of spermatogenesis3.9 ng/ml
MB 7 yXX maleMale, penile hypospadiasMaleTestesND
KM 1 yXX maleMale, penile hypospadiasMaleTestes, normal tissue adequate for age1–2.7 ng/ml1-150
AK 17 yXX true hermaphroditeAmbiguousAmbiguousLeft ovary, right testis with no signs of  spermatogenesisND
RZ 14 yXX true hermaphroditeAmbiguousAmbiguousLeft ovotestis with no signs of spermatogenesis  in the testicular part, right ovary0.6–5.5 ng/ml1-150
PG 6 mthXX true hermaphroditeAmbiguousAmbiguousLeft ovotestis with signs of dysgenesis in the testicular part, right ovary<0.1–0.2 ng/ml1-150
  • ND - not determined.

  • 1-150 Testosterone before and after stimulation with human chorionic gonadotrophin (hCG).