Mutations in the WT1 gene (coding for a protein involved in the renal and gonadal development). Association of a nephropathy (Focal Segmental Glomerulosclerosis) with a male pseudohermaphrodism. Proteinuria (nephrotic syndrome). Primary amenorrhea. Evolving to end-stage renal failure near the end of adolescence. Low risk of nephroblastoma (after 10 years) but high risk  of gonadoblastoma.

Anesthetic implications: 

Check kidney function, blood pressure.

References:

Updated: April 2019