Learn About Denys-Drash Syndrome (DDS)

What is the definition of Denys-Drash Syndrome (DDS)?

Denys-Drash syndrome is a condition that affects the kidneys and genitalia.

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What are the causes of Denys-Drash Syndrome (DDS)?

Mutations in the WT1 gene cause Denys-Drash syndrome. The WT1 gene provides instructions for making a protein that regulates the activity of other genes by attaching (binding) to specific regions of DNA. On the basis of this action, the WT1 protein is called a transcription factor. The WT1 protein plays a role in the development of the kidneys and gonads (ovaries in females and testes in males) before birth.

How prevalent is Denys-Drash Syndrome (DDS)?

The prevalence of Denys-Drash syndrome is unknown; at least 150 affected individuals have been reported in the scientific literature.

Is Denys-Drash Syndrome (DDS) an inherited disorder?

This condition is inherited in an autosomal dominant pattern, which means one copy of the altered gene in each cell is sufficient to cause the disorder.

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What are the latest Denys-Drash Syndrome (DDS) Clinical Trials?
NCI-COG Pediatric MATCH (Molecular Analysis for Therapy Choice) - Phase 2 Subprotocol of LOXO-292 in Patients With Tumors Harboring RET Gene Alterations

Summary: This phase II pediatric MATCH treatment trial studies how well selpercatinib works in treating patients with solid tumors that may have spread from where they first started to nearby tissue, lymph nodes, or distant parts of the body (advanced), lymphomas, or histiocytic disorders that have activating RET gene alterations. Selpercatinib may block the growth of cancer cells that have specific geneti...

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NCI-COG Pediatric MATCH (Molecular Analysis for Therapy Choice)- Phase 2 Subprotocol of Tipifarnib in Patients With Tumors Harboring HRAS Genomic Alterations

Summary: This phase II pediatric MATCH trial studies how well tipifarnib works in treating patients with solid tumors that have recurred or spread to other places in the body (advanced), lymphoma, or histiocytic disorders, that have a genetic alteration in the gene HRAS. Tipifarnib may block the growth of cancer cells that have specific genetic changes in a gene called HRAS and may reduce tumor size.

Who are the sources who wrote this article ?

Published Date: March 01, 2013Published By: National Institutes of Health

What are the Latest Advances for Denys-Drash Syndrome (DDS)?
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Results of Treatment for Patients With Multicentric or Bilaterally Predisposed Unilateral Wilms Tumor (AREN0534): A report from the Children's Oncology Group.