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XX testicular DSD (Disorder of Sexual Development)

General

Disease Name
XX testicular DSD (Disorder of Sexual Development)
Mutation
unknown
Test Type
Genetic Disease/Disorder
Details
Canine XX sex reversal is a type of XX disorder of sexual development characterized by presence of testicular tissue in the gonads and varying degrees of masculinization in dogs that have a female karyotype. Carrier dogs are fertile. To prevent production of affected dogs, breeding of affected or known carrier dogs should be avoided.
Details 2
Affected dogs (XX male, testicular XX DSD) are most appear as bilaterally cryptorchid male dogs, but have a caudally displaced and pendulous prepuce and hypoplastic penis. Mild hypospadias may also be present. These dogs are sterile (Meyers-Wallen et al., 1988). Variable degrees of masculinization are found in affected dogs with ovotestes (XX true hermaphrodite, ovotesticular DSD). Those most masculinized have an enlarged clitoris containing a bone and/or a misshapen vulva that resembles a prepuce. Clitoral enlargement may be noted within a few months of age or at puberty. Many XX true hermaphrodites in the ACS studies had an apparently normal female external phenotype (Meyers-Wallen and Patterson 1988). Affected dogs with ovotestes may exhibit estrous cycles and rarely, produce offspring. Both XX males and XX true hermaphrodites can occur within the same litter or in the same pedigree. (From OMIA)
Published
Selden, J.R., Wachtel, S.S., Koo, G.C., Haskins, M.E., Patterson, D.F. : Genetic basis of XX male syndrome and XX true hermaphroditism: evidence in the dog. Science 201:644-6, 1978. Pubmed reference: 675252.
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