The Denys-Drash Syndrome (DDS) is a genetic disorder associated with a spectrum of disorders affecting the urogenital tract. It is in a continuum that also involves Frasier Syndrome (see next section).
Gene affected: WT1 (Wilm tumor suppressor gene), which is important for normal development of the urogenital system
Chromosome: 11p13
Clinical features:
(1) female external genitalia with female or male karyotype (XY males show gonadal dysgenesis with streak gonads, male pseudohermaphroditism, hypergonadotropic hypogonadism)
(2) glomerulopathy with diffuse mesangial sclerosis leading to renal failure
(3) high risk of Wilm’s tumor
The patient may present with early-onset nephrotic syndrome, hematuria and hypertension (from birth to 24 months of age).
Prophylactic nephrectomy (with renal transplant) and gonadectomy may be performed due to the risk for malignancy.
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