60 



BIOLOGIC BASIS OF SEX 



dominated. The uteri were absent in some 

 cases, even when complete external female 

 genitalia were present. Ovotestes were found 

 on the right side of the body in over half 

 the cases; separate left ovaries or testes 

 were about equally frequent ; in three cases 

 separate testis and ovary were indicated. 

 The left side of the body showed a different 

 distribution of gonad types; about one- 

 fourth had ovotestes, another fourth ova- 

 ries, and one-twelfth testes. Unilateral dis- 

 tribution of gonad types was most frequent. 

 The presence or absence of the prostate 

 seemed to have significance because it is 

 sometimes absent in purely female types. In 

 recent literature similar cases have been 

 called true hermaphrodites. This is an ex- 

 aggeration in terms of long established 

 practice in plants and animals where true 

 hermaphroditism includes fully functioning 

 gametes of each sex. 



Hungerford, Donnelly, Nowell and Beck 

 (1959» have reported on a case of a Negro 

 in which the culture cells had the chromo- 

 some complement of a normal female 46, 

 with XX sex chromosomes. Unfortunately, 

 the possibility that this case may be a chro- 

 mosome mosaic was not tested by karyo- 

 type samples from several parts of the body. 



Harnden and Armstrong (1959) estab- 

 lished separate skin cultures from both sides 

 of the body of another hermaphroditic type. 

 The majority of the cells were apparently of 

 XX constitution with a total of 46 chromo- 

 somes. However, in one of the 4 cultures 

 established, some 7 per cent of cells had an 

 abnormal chromosome present, suggesting 

 that the case might involve a reciprocal 

 translocation between chromosomes 3 and 

 4 when the chromosomes were ordered ac- 

 cording to size. All the other cell nuclei were 

 normal. The fact that the majority of the 

 cells in these two cases were XX and with 

 46 chromosomes seems to predicate against 

 the view that either changes in chromosome 

 number or structure of the fertilized egg are 

 necessary for the initiation of hermaplu'o- 

 dites. 



Ferguson-Smith (1960) describes two 

 cases of gynandromorphic type in which the 

 reproductive organs on the left side were 

 female and on the right side were male. The 

 recognizable organs were Fallopian tube, 

 ovary with primordial follicles only, imma- 



ture uterus in one case, none in the other, 

 rudimentary prostate, small testis and epi- 

 didymis, vas deferens, bifid scrotum, phal- 

 lus, perineal urethra, pubic and axillary 

 hair, breasts enlarging at 14 years. Testicu- 

 lar development with hyperplasia of Leydig 

 cells, germinal aplasia, and hyalinization of 

 the tubules was suggestive of the Klinefelter 

 syndrome. Nuclear-chromatin was positive 

 in both cases. Modal chromosome number 

 was 46. The sex chromosomes were inter- 

 preted as XX. The 119 cell counts on one 

 patient showed a rather wide range; 7 per 

 cent had 44 chromosomes, 13 per cent had 

 45, 62 per cent had 46, and 18 per cent had 

 47 chromosomes. The extra chromosome 

 within the cells containing 47 chromosomes 

 was of medium size with submedian kineto- 

 chore as generally observed for chromo- 

 somes of group 3. 



It is surprising that the male differentia- 

 tion in these four and other hermaphroditic 

 cases (Table 1.5) is as complete as it is. 

 Other observations show that the Y chro- 

 mosome contains factors of strong male 

 potency, yet in its absence the hermaphro- 

 dites develop an easily recognized male 

 system. It is not complete but the degree of 

 gonadal differentiation is as great as that 

 observed in the XXY 4- 2A Klinefelter 

 types. The bilateral sex differentiation in 

 hermaphrodites would seem to require other 

 conditions than those heretofore considered. 



Another case of hermaphroditism is that 

 presented by Hirschhorn, Decker and 

 Cooper (1960). The patient's j:)henotype 

 was intersexual with phallus, hypospadias, 

 vagina, uterus. Fallopian tubes, two slightly 

 differentiated gonads in the position of ova- 

 ries. The child was 4 months old. Culture 

 of bone marrow cells showed that the indi- 

 vidual was a mosaic of two types. About 

 60 per cent of the cells had 45 chromosomes 

 of XO karyotype, and 40 per cent had 46 

 chromosomes with a karyotA'^pe XY. The 

 Y chromosome when present was larger 

 than Y chromosomes of normal individuals. 

 The change in size may be related to the 

 association of the XO and XY cells and 

 be similar etiologically to the case discussed 

 by yivtz (1959) in Sciara triploids. 



There are mosaics in Drosophila formed 

 from the loss by the female in some cells of 

 one of lioi- X chromosomes, as for instance 



