Human pathology

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pure gonadal dysgenesis

Definition: bilateral streak gonads that fail to differenciate.

Etiology

- mutations in the SRY gene on the distal short arm of the Y chromosome in 46XY sex-reversed females with pure gonadal dysgenesis
- translocation in chromosome 9p near the SRY-related SOX9 gene induces campomelic dysplasia (46XY sex-reversal, sreak gonads, severe skeletal malformation)

See also

- intersex
- hermaphrodisms