Denys-Drash syndrome , DDS (Abkürzung)

Having a geneticsyndrome that increases the risk of kidney problems, such as Denys-Drash syndrome.

He was one of the original describers of the Denys-Drash syndrome.

Denys-Drash syndrome is a syndrome characterized by the following conditions:

Denys-Drash syndrome.

Most nephroblastomas are unilateral, being bilateral in less than 5% of cases, although patients with Denys-Drash syndrome mostly have bilateral or multiple tumors.

Denys-Drash syndrome (DDS) is a very rare disorder.

WAGR syndrome, Denys-Drash syndrome, or sporadic aniridia.

However, non-inactivating mutations can result in altered WT1 protein function that also results in Wilms tumor, such as in the Denys-Drash syndrome.

Inactivation of WT1 is causes Wilm's tumour, and Denys-Drash syndrome (DDS), leading to nephropathy and genital abnormalities.

The most common etiologies of renal failure were bilateral nephrectomy for persistent or recurrent tumor, progressive tumor in the remaining kidney without nephrectomy, Denys-Drash syndrome (DDS), and radiation nephritis.

Pelletier J, Bruening W, Kashtan CE, et al.: Germline mutations in the Wilms' tumor suppressor gene are associated with abnormal urogenital development in Denys-Drash syndrome.

The glomerulonephropathy presents later than in Denys-Drash syndrome, and the tumour risk phenotype is different - whilst DDS is associated with Wilms' tumour, Frasier syndrome is associated with gonadoblastoma.

Indeed, at least two examples of inherited syndromes with developmental defects are known to involve mutations in ZNF genes: GLI3 in Greig cephalopolydactyly syndrome (11) and WT1 in Denys-Drash syndrome (12).

Kist-van Holthe JE, Ho PL, Stablein D, et al.: Outcome of renal transplantation for Wilms' tumor and Denys-Drash syndrome: a report of the North American Pediatric Renal Transplant Cooperative Study.

Children with a significantly increased predisposition to develop Wilms tumor (e.g., most children with Beckwith-Wiedemann syndrome, WAGR syndrome, Denys-Drash syndrome, hemihypertrophy, or sporadic aniridia) should be screened with ultrasound every 3 months at least until they reach age 8 years.