XXXXY Syndrome

The 49,XXXXY Syndrome flag.

49,XXXXY Syndrome, also sometimes known as Fraccaro Syndrome, is an extremely rare intersex variation in AMAB or CTM individuals in which they have three extra X chromosomes. It occurs in approximately 1 out of 85,000 - 100,000 AMAB individuals. It is considered a variation of Klinefelter Syndrome.

Traits of 49,XXXXY syndrome can vary; however, it usually includes learning difficulties/intellectual disabilities, low muscle tone, hypogonadism, delayed growth, dental issues, curved pinky fingers, a variety of congenital disabilities that may affect the heart, bones, brain and/or kidneys, and distinctive facial features such as widely spaced eyes, a flat nose bridge, and epicanthic folds. Some of these symptoms may classify one with this syndrome as physically disabled.

One common symptom is an undergrown penis (micropenis) and/or undescended testicles (cryptorchidism). This often causes them not to produce as much testosterone, leading them not to grow much body hair and not have many masculine traits. As a result, breast growth may occur. This may also cause an overproduction of estrogen, which may cause them to also fall under Aromatase Excess Syndrome and/or AMAB Hypogonadism. All of those with this variation are incapable of sperm production and thus are infertile.

History

This sex was first diagnosed in 1960 and was named Fraccaro syndrome after the individual who researched it.

Flag

FANDOM user ViralSystem created the flag on March 15th, 2021. The greens are for facial features, yellow is for the intersex spectrum, light orange is for chromosomal abnormality, and the rest of the oranges are for community.