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Usually, it is caused by unequal crossing over between X and Y chromosomes during meiosis in the father, which results in the X chromosome containing the normally-male SRY gene.

When this X combines with a normal X from the mother during fertilization, the result is an XX male.

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Symptoms usually include small testes and subjects are invariably sterile.

Individuals with this condition sometimes have feminine characteristics, with varying degrees of gynecomastia but with no intra-abdominal Müllerian tissue.

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