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Denys-Drash Syndrome
Abstract
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NORD is very grateful to Agnieszka Swiatecka-Urban, MD, FASN, Assistant Professor of Pediatrics and Cell Biology and Physiology, Children's Hospital of Pittsburgh, University of Pittsburgh School of Medicine, for assistance in the preparation of this report.
Synonyms of Denys-Drash Syndrome
- DDS
- Drash syndrome
- nephropathy and disorders of sexual development
- Wilms tumor, pseudo or true hermaphroditism
Disorder Subdivisions
- No subdivisions found.
General Discussion
Denys-Drash syndrome (DDS) is characterized by abnormal kidney function (congenital nephropathy), a cancerous tumor of the kidney called Wilms tumor, and disorders of sexual development in affected males. Most affected females have normal genitalia. DDS is agenetic disorder caused by mutations in the Wilms tumor suppressor gene, WT1.
Organizations related to Denys-Drash Syndrome
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