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Xx male syndrome, also known as de la chapelle syndrome or 46,xx testicular disorder of sex development (or 46,xx dsd) is a rare intersex condition in which an individual with a 46,xx karyotype develops a male phenotype [2][3][4][5][6] in 90 percent of these individuals, the syndrome is caused by the father's y chromosome 's sry gene being atypically included in the crossing over of genetic. A deviation from the norm what is xx male syndrome Xx male syndrome, also known as de la chapelle syndrome, is a rare genetic condition in which an individual who is genetically female (with two x chromosomes) develops male physical characteristics. Sex chromosomes are the genetic elements that define a person's biological sex Learn about the xx and xy chromosomes, the sex determination system, and other chromosome combinations. Understanding xx male syndrome xx male syndrome, also known as de la chapelle syndrome, is a condition where an individual has two x chromosomes, which are typically associated with female genetic sex, but exhibits male external genitalia and internal reproductive structures. A rare disorder where individuals with two x chromosomes develop male characteristics due to sry gene translocation. Xx male syndromedefinitionxx male syndrome occurs when the affected individual appears as a normal male, but has female chromosomes Two types of xx male syndrome can occur Those with detectable sry gene and those without detectable sry (sex determining region y) Sry is the main genetic switch for determining that a developing embryo will become male Source for information on xx male. What is the prognosis of xx male syndrome With hormonal replacement, therapy, surgery and multidisciplinary follow up the prognosis of xx male syndrome is good The patients can live a fairly normal life but like all disorders, they require a strong social support system, in the presence of which the quality of life is greatly improved.