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Denys-Drash Syndrome Follow-up

  • Author: Agnieszka Swiatecka-Urban, MD; Chief Editor: Maria Descartes, MD  more...
 
Updated: May 27, 2014
 

Further Outpatient Care

In patients with Denys-Drash syndrome (DDS), arrange for follow-up with a pediatric nephrologist for renal transplantation and for ongoing management of nephrotic syndrome, hypertension, and renal insufficiency.

Arrange for follow-up with a pediatric oncologist for ongoing management of Wilms tumor. The Children's Oncology Group has released guidelines for long-term follow-up.[7]

Arrange for follow-up with a pediatric endocrinologist for management of intersex disorders.

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Transfer

Children with Denys-Drash syndrome require integrated interdisciplinary care, including such pediatric subspecialty services as nephrology, surgery, oncology, endocrinology, and genetics.

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Complications

Potential complications for Denys-Drash syndrome include the following:

  • Patients with nephrotic syndrome may encounter recurrent infections, nutritional deficiencies, and, occasionally, venous thrombosis. Progression to end-stage renal disease (ESRD) is inevitable.
  • Complications of chemotherapy include life-threatening infections and secondary malignancies.
  • The risk for Wilms tumor development in any residual renal tissue and for gonadoblastoma in dysgenetic gonads is high.
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Prognosis

All patients develop nephropathy and progress to ESRD within 2 years from the diagnosis or before age 3 years.

Virtually all patients with Denys-Drash syndrome who have their native kidneys develop Wilms tumor. Patients with unilateral Wilms tumor are at risk for contralateral tumor. Staging and histologic criteria are prognostic in patients with Wilms tumor.

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Patient Education

Provide genetic counseling.

Explain role of prophylactic surgery to prevent Wilms tumor and gonadoblastoma.

Explain renal replacement therapy options, including renal transplantation.

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Contributor Information and Disclosures
Author

Agnieszka Swiatecka-Urban, MD FASN, Assistant Professor, Department of Pediatrics, Cell Biology and Physiology, University of Pittsburgh School of Medicine; Assistant Professor, Department of Nephrology, Children's Hospital of Pittsburgh

Agnieszka Swiatecka-Urban, MD is a member of the following medical societies: American Society of Nephrology, American Society of Pediatric Nephrology, International Society of Nephrology, Women in Nephrology

Disclosure: Received consulting fee from Mallinckrodt Pharmaceuticals for consulting.

Coauthor(s)

Prasad Devarajan, MD, FAAP Louise M Williams Endowed Chair in Pediatrics, Professor of Pediatrics and Developmental Biology, Director of Nephrology and Hypertension, Director of the Nephrology Fellowship Program, Medical Director of the Kidney Stone Center, Co-Director of the Institutional Office of Pediatric Clinical Fellowships, Director of Clinical Nephrology Laboratory, CEO of Dialysis Unit, Department of Pediatrics, Cincinnati Children's Hospital Medical Center, University of Cincinnati College of Medicine

Prasad Devarajan, MD, FAAP is a member of the following medical societies: American Heart Association, American Society of Nephrology, American Society of Pediatric Nephrology, National Kidney Foundation, Society for Pediatric Research

Disclosure: Received none from Coinventor on patents submitted for the use of NGAL as a biomarker of kidney injury for none.

Specialty Editor Board

Mary L Windle, PharmD Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Nothing to disclose.

Chief Editor

Maria Descartes, MD Professor, Department of Human Genetics and Department of Pediatrics, University of Alabama at Birmingham School of Medicine

Maria Descartes, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Medical Genetics and Genomics, American Medical Association, American Society of Human Genetics, Society for Inherited Metabolic Disorders, International Skeletal Dysplasia Society, Southeastern Regional Genetics Group

Disclosure: Nothing to disclose.

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Gross nephrectomy specimen shows a Wilms tumor pushing the normal renal parenchyma to the side.
 
 
 
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