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XX male syndrome

XX male syndrome (also called de la Chapelle syndrome) is a rare sex chromosomal disorder in man. Usually it is caused by unequal crossing over between X and Y chromosomes during meiosis. Symptoms include small testes, gynecomastia and sterility. more...

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Men typically have one X chromosome and one Y chromosome in each cell of their bodies, other than sperm cells. Women typically have two X chromosomes. XX males have two X chromosomes, but otherwise appear to be male.

There are a number of other syndromes related to the karyotype of the sex chromosomes, such as XYY syndrome, Turner syndrome (patients have one X and no Y chromsome), Klinefelter_syndrome (patients have two X chromosomes and one Y), XXX syndrome (patients have three X chromosomes), etc. See the list of genetic disorders

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Klinefelter's Syndrome
From Gale Encyclopedia of Childhood and Adolescence, 4/6/01

Klinefelter's syndrome, also known as primary micro-orchidism, affects males only. A normal male has one pair of sex chromosomes made up of one X and one Y. In Klinefelter's syndrome, one or more extra X chromosomes are present. (The extra chromosomes identified as Klinefelter's syndrome fall into one of the following categories: 47XXY, 48XXYY, 48XXXY, 49XXXY, or 49 XX/XXY.

Although individuals with Klinefelter's syndrome appear to be more likely to experience learning difficulties, the syndrome often remains undiagnosed. At puberty , a boy with more than one extra X chromosome may tend to develop enlarged breasts, a condition that can be treated with medication. In addition, the testes remain very small, and most males with Klinefelter's syndrome produce little or no sperm. In fact, it is during tests for infertility that Klinefelter's is most frequently diagnosed.

Further Reading

For Your Information

Books

  • Berch, Daniel B., and Bruce G. Bender. Sex Chromosome Abnormalities and Human Behavior: Psychological Studies. Boulder: Westview Press, 1990.
  • Strom, Charles. Heredity and Ability: How Genetics Affects Your Child and What You Can Do About It. New York: Plenum Press, 1990.
  • Therman, Eeva. Human Chromosomes: Structure, Behavior, and Effects. New York: Springer-Verlag, 1993.

Gale Encyclopedia of Childhood & Adolescence. Gale Research, 1998.

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