Congenital nephrotic syndrome

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Female urinary tract

Male urinary tract

Nephrotic syndrome - congenital

Early and aggressive treatment is required to control the disorder. Diuretic medications help rid the body of excess fluid. ACE inhibitor medications (like Captopril and others) and non-steroidal anti-inflammatory drugs (like indomethacin) are used to slow the spilling of protein in the urine. Antibiotics may be needed to control infections.

Dietary modifications may include the restriction of sodium and use of dietary supplements as appropriate for the nature and extent of malnutrition. Fluids may be restricted to help control swelling.

Removal of the kidneys, dialysis, and kidney transplant may be recommended.

Many cases are fatal within the first year. Congenital nephrotic syndrome may be successfully controlled in some cases with early and aggressive treatment, including early kidney transplantation.

• Frequent, severe infections
• Malnutrition and related diseases
• Blood clots
• Acute kidney failure
• Chronic kidney failure
• End-stage kidney disease

Call your health care provider if symptoms suggestive of congenital nephrotic syndrome develop.

Review Date: 07/25/2006
Reviewed By: David M. Charytan, M.D., M.Sc., Department of Medicine, Division of Nephrology, Brigham and Women's Hospital, Boston, MA. Review provided by VeriMed Healthcare Network.