Denys-Drash Syndrome

Denys-Drash Syndrome (DDS) is an intersex variation that effects both CTF and CTM individuals. It has only around 150-200 reports, however it may be more frequent than this, just without notice (especially for CTF individuals.)

Those with this variation have diffuse glomerulosclerosis, which is a variation that effects ones kidneys abilities to filter waste. Because of this, they have a 90% chance at gaining Wilms tumor, a rare kidney cancer.

CTM individuals with this variation may have a penis, however they often have ambiguous genitals, undescended testicles (cryptorchidism), or a vagina. Because of this, they typically are infertile and unable to bare children. They also typically have gonadal dysgenesis and/or AMAB hypogonadism as well.

CTF individuals with this variation have a vagina, while the only noticeable trait of DDS is their kidneys. Because of this, they are often diagnosed with other kidney-related variations, rather than with DDS.

Frasier Syndrome
Not to be confused with Fraser Syndrome.

Frasier Syndrome is an intersex variation that falls under DDS. It has the same cause (improper function of the WT1 gene) however it is less severe. The symptoms are the same as DDS, just less intense. It is seemingly rare, with only 50 reported cases, however CTF individuals with this variation may be overlooked.

Causes
This variation is caused when one WT1 gene- a gene designed for connecting regions of DNA together- fails to properly function. Because of this, proteins that effect genitals and kidneys are effected during development in the womb.

History
P. Denys and Allan L. Drash first described the syndrome in 1967.