Denys-Drash Syndrome

Not yet reviewed

This information is AI generated and has not yet been reviewed by a specialist physician. AI can make mistakes.

Disease overview

Disorder characterized by abnormal kidney function, Wilms tumor, and disorders of sexual developmentent in affected males (more info)

Common symptoms

Edemaabdominal distention and recurrent infections sometimes present at birth but often developing between 1-2hypertensionrenal failure within the first three years of lifeWilms tumor (abdominal swellingblood in the urinelow-grade feverloss of appetitepalenessweight losslethargy)males have genitals that are incompletely formedambiguousor visibly female

When to suspect

  • Recommendation 1

    Molecular genetic testing for the WT1 gene

How to test

  • Recommendation 1

    Molecular genetic testing for the WT1 gene

ZebraMD is partnering with Ambry Genetics for clinical-grade whole genome testing for rare diseases. Ambry accepts insurance. If you don’t have a physician to order this test, join our referral list and our team will reach out.

Treatment

  • Recommendation 1

    Supportive

Primary care

  • Recommendation 1

    Nephrectomy if DDS is diagnosed prior to development of Wilms tumor, hypertension and electrolyte management, renal replacement therapy including dialysis or kidney transplant following end-stage renal failure, gonadectomy due to high risk of gonadal malignancy

Further support

  • Recommendation 1

    Pediatric nephrologist, pediatric oncologist, pediatric surgeon, pediatric endocrinologist and geneticist. Referral to Medical Genetics Department, if available. Initial virtual care is also available through organizations like TeleRare Health.

Clinical trials

Clinical Trials

Sources

No data available

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