eMedicine Specialties > Pediatrics: Genetics and Metabolic Disease > Metabolic Diseases

Denys-Drash Syndrome: Follow-up

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
Coauthor(s): Prasad Devarajan, MD, Louise M Williams Endowed Chair in Pediatrics, Professor of Pediatrics and Developmental Biology, Director of Nephrology and Hypertension, Director of Clinical Nephrology Laboratories, Chief Executive Officer of Dialysis Unit, Department of Pediatrics, Cincinnati Children's Hospital Medical Center, University of Cincinnati College of Medicine
Contributor Information and Disclosures

Updated: Apr 10, 2009

Follow-up

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.4  
  • Arrange for follow-up with a pediatric endocrinologist for management of intersex disorders.

Transfer

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

Complications

  • 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.

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.

Patient Education

  • Provide genetic counseling.
  • Explain role of prophylactic surgery to prevent Wilms tumor and gonadoblastoma.
  • Explain renal replacement therapy options, including renal transplantation.

Miscellaneous

Medicolegal Pitfalls

  • Failure to consider the diagnosis in a patient presenting with abdominal mass, Wilms tumor, early nephrotic syndrome, or intersex disorder
  • Failure to advocate early prophylactic nephrectomy and gonadectomy to prevent malignant transformation
 


More on Denys-Drash Syndrome

Overview: Denys-Drash Syndrome
Differential Diagnoses & Workup: Denys-Drash Syndrome
Treatment & Medication: Denys-Drash Syndrome
Follow-up: Denys-Drash Syndrome
Multimedia: Denys-Drash Syndrome
References
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References

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Further Reading

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Keywords

Denys-Drash syndrome, Drash syndrome, DDS, Wilms tumor, intersex disorder, congenital nephropathy, end-stage renal disorder, ESRD, diffuse mesangial sclerosis, nephrotic syndrome, pseudohermaphroditism, gonadoblastoma, oliguria, generalized edema, ascites, hypertension, anemia, rickets, abdominal mass, penoscrotal hypospadias, cryptorchidism, enlarged clitoris with labial fusion, bifid scrotum with palpable gonads, micropenis

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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, FASN is a member of the following medical societies: American Society of Nephrology, American Society of Pediatric Nephrology, International Society of Nephrology, and Women in Nephrology
Disclosure: Nothing to disclose.

Coauthor(s)

Prasad Devarajan, MD, Louise M Williams Endowed Chair in Pediatrics, Professor of Pediatrics and Developmental Biology, Director of Nephrology and Hypertension, Director of Clinical Nephrology Laboratories, Chief Executive Officer of Dialysis Unit, Department of Pediatrics, Cincinnati Children's Hospital Medical Center, University of Cincinnati College of Medicine
Prasad Devarajan, MD is a member of the following medical societies: American Heart Association, American Society of Nephrology, American Society of Pediatric Nephrology, National Kidney Foundation, and Society for Pediatric Research
Disclosure: Nothing to disclose.

Medical Editor

Ian Krantz, MD, Department of Pediatrics, Assistant Professor, University of Pennsylvania and Children's Hospital of Philadelphia
Ian Krantz, MD is a member of the following medical societies: American Society of Human Genetics
Disclosure: Nothing to disclose.

Pharmacy Editor

Mary L Windle, PharmD, Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy, Pharmacy Editor, eMedicine
Disclosure: Pfizer Inc Stock Investment from financial planner; Avanir Pharma Stock Investment from financial planner ; WebMD Salary and stock Employment and investment from financial planner

Managing Editor

Leonard G Feld, MD, PhD, MMM, FAAP, Sara H Bissell and Howard C Bissell Endowed Chair in Pediatrics, Chief Medical Officer, Levine Children's Hospital, Carolinas Medical Center
Leonard G Feld, MD, PhD, MMM, FAAP is a member of the following medical societies: American Academy of Pediatrics, American College of Physician Executives, American Society of Nephrology, American Society of Pediatric Nephrology, International Society of Nephrology, and Juvenile Diabetes Foundation International
Disclosure: Nothing to disclose.

CME Editor

Paul D Petry, DO, FACOP, FAAP, Consulting Staff, Freeman Pediatric Care, Freeman Health System
Paul D Petry, DO, FACOP, FAAP is a member of the following medical societies: American Academy of Osteopathy, American Academy of Pediatrics, American College of Osteopathic Pediatricians, and American Osteopathic Association
Disclosure: Nothing to disclose.

Chief Editor

Bruce Buehler, MD, Professor, Department of Pediatrics, Pathology and Microbiology, Executive Director, Hattie B Munroe Center for Human Genetics and Rehabilitation, University of Nebraska Medical Center
Bruce Buehler, MD is a member of the following medical societies: American Academy for Cerebral Palsy and Developmental Medicine, American Academy of Pediatrics, American Association on Mental Retardation, American College of Medical Genetics, American College of Physician Executives, American Medical Association, and Nebraska Medical Association
Disclosure: Nothing to disclose.

 
 
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