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Men with XX syndrome have a male phenotype (physical appearance), but they have changes typical of Klinefelter syndrome. Although normal in intelligence, some affected men have difficulties making social adjustments. Although about 40 percent of men affected by Klinefelter syndrome have a normal XY pattern, others possess a chromosome variant known as XX syndrome, in which Y chromosome material is transferred to an X chromosome or a nonsex chromosome (autosome).
Men with Klinefelter syndrome have small, firm testes, and they often have breast enlargement (gynecomastia) and inordinately long legs and arms (eunuchoidism) and are infertile. Affected men have decreased serum testosterone concentrations, with urinary excretion of 17-ketosteroids (components of certain male hormones, or androgens) in the normal or low-normal range. Diabetes mellitus, goitre (enlargement of the thyroid gland), and various cancers may be more prevalent among Klinefelter syndrome patients.
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