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In 46, XY (male pseudohermaphroditism), individuals have ambiguous or female external genitalia but the chromosomal constitution and reproductive organs of a male, though the testes may be malformed or absent.
Treatment of intersex in humans depends upon the age at which the diagnosis is made.
Most often, but not always, the chromosome complement is 46, XX, and in every such individual there also exists evidence of Y chromosomal material on one of the autosomes (any of the 22 pairs of chromosomes other than the sex chromosomes).
Individuals with a 46, XX chromosome complement usually have ambiguous external genitalia with a sizable phallus and are therefore often reared as males.
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In older individuals the accepted gender may be reinforced by the appropriate surgical procedures and by hormonal therapy.
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