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The syndrome of Mixed Gonadal Dysgenesis is characterized by a unilateral testis, usually intra-
abdominal a streak gonad on contralateral side, persistent Mullerian structures and ambiguous
genitalia.
These patients are chromatin negative and exhibit XO/XY mosaicism, probably resulting from a
cytogenetic error very early in embryogenesis.
The testis and the streak gonad should be removed because of the potential devlopement of a
gonadoblastoma and the virilization... |