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Alternative titles; symbols. Other entities represented in this entry:. A disorder of sex development DSD is a 'congenital condition in which development of chromosomal, gonadal, or anatomic sex is atypical. Sex-reversed 46,XX individuals can present as phenotypically normal males, as men with genital ambiguities, or as true hermaphrodites Ahmad et al. A 'true hermaphrodite' must have both mature ovarian and mature testicular tissue with histologic evidence of follicles and tubules, respectively van Niekerk and Retief, It is a genetically heterogeneous condition. Van Niekerk and Retief found that the ovotestis was the most common gonad of the true hermaphrodite, found in

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