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

Denys-Drash Syndrome: Treatment & Medication

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

Treatment

Medical Care

Cornerstones of Denys-Drash syndrome (DDS) medical therapy include management of fluid and electrolyte balance, treatment of hypertension, renal replacement therapy for patients with end-stage renal disease (ESRD) or after bilateral nephrectomy, and chemotherapy for patients with Wilms tumor.

Surgical Care

Surgical treatment of patients with Denys-Drash syndrome and Wilms tumor should follow the guidelines of the National Wilms Tumor Study-4 Protocols.3 Prophylactic total bilateral nephrectomy has been advocated because the risk of Wilms tumor is very high in any residual renal tissue.

  • For patients without Wilms tumor at the time of initial presentation with Denys-Drash syndrome, early prophylactic bilateral nephrectomy, followed by renal replacement therapy, is recommended to avoid the risk of Wilms tumor development and consequent delay of kidney transplantation. As the risk of gonadal malignancy is also high in patients with gonadal dysgenesis, performing a gonadectomy at the time of nephrectomy is best. Institute appropriate hormonal therapy in these patients at puberty.
  • Kidney transplantation is the treatment of choice after bilateral nephrectomy. For patients with Wilms tumor, a 2-year period free of chemotherapy and tumor is recommended prior to renal transplantation.

Consultations

  • Pediatric nephrologist - For managing the nephropathy, ESRD, hypertension, and medical aspects of transplantation
  • Pediatric oncologist - For diagnosis and treatment of Wilms tumor and gonadoblastoma
  • Pediatric surgeon - For managing Wilms tumor, access in renal replacement therapy and surgical aspects of renal transplantation, and evaluation and possible removal of abnormal gonads
  • Pediatric endocrinologist - For evaluation and management of intersex disorders
  • Geneticist - For chromosomal analysis, molecular diagnosis, and genetic counseling

Diet

  • A low-sodium diet is important to manage hypertension and edema.
  • Patients with renal insufficiency require a low-potassium, low-phosphate diet.

Medication

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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