Unilateral true hermaphrodite with 46,XX/46,XY dispermic chimerism.
A 13 year old female presented with ambiguous external genitalia, right inguinal ovotestis, left ovary, apparently normal Mullerian system, and absent Wolffian system. Cultured lymphocytes showed a 46,XX/46,XY karyotype. Histopathology of the gonads confirmed true hermaphroditism. The presence of two genetically different erythrocyte populations was observed. The findings suggested that the patient is a true hermaphrodite dispermic chimera.
Kuwait Medical Genetics Centre.
T I
TI
Farag
S A
SA
Al-Awadi
P
P
Tippett
M
M
el-Sayed
T S
TS
Sundareshan
S A
SA
Al-Othman
M H
MH
el-Badramany
D000293:Adolescent; D002678:Chimera; D012734:Disorders of Sex Development / Q000097:blood / Q000235:genetics*; D005260:Female; D006801:Humans; D007621:Karyotyping