Early gender assignment in true XXXX .

@entity1 @entity154 @entity12094 @entity4998 @entity1283 @entity2357
True @entity4998 is a rare form of intersex @entity154 where both male and female gonads are present. True hermaphrodites diagnosed in the past have been preferentially reared as males. However, based on the appearance of their external genitalia and the ability to reconstruct a functional genitourinary system, most of these @entity1 should be raised as @entity1 . Between 1968 and 1987, we managed eight @entity1 with true @entity4998 . The karyotypes were as follows: five 46XX, one 46XY, one 47XXY, and one 46XX/mosaic. Gonadal distribution was bilateral (two ovotestes) in two, lateral (testis and ovary) in two, and unilateral (ovotestis and ovary) in four. Seven @entity1 were diagnosed shortly after birth. Six were raised as @entity1 , two as @entity1 . Based on a review of the relationship of external-internal genitalia, gonads, and karyotype of our own @entity1 and those published by others, we believe that the appearance of the external genitalia alone allows a prompt and definitive gender assignment in infancy. This led us to develop a classification of external genitalia based on the following characteristics. Class I represents normal female genitalia, class II an @entity1283 , class III partially fused labioscrotal folds, class IV fused labioscrotal folds, class V a @entity2357 and @entity12094 , and class VI represents normal male genitalia. We had two @entity1 that were class II, two class III, three that were class IV, and one to IV should be raised as females; in class V, male gender assignment is preferable. This classification, based on the appearance of the external genitalia, allows a simple screening and prompt gender assignment in @entity1 identified as true hermaphrodites.
