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partial gonadal dysgenesis with minifascicular neuropathy 46,XY

Genetics: - associated with defects in DHH (desert hedgehog protein) Clinical manifestations: - presence of a testis on one side & a streak or an absent gonad at the other, persistence of Muellerian duct structures, & a variable degree of genital ambiguity

General

gonadal dysgenesis

Database Correlations

OMIM 607080

References

OMIM :accession 607080