Nephrotics (Part3)

Congenital Nephropathy

Rarely, a child may be born with congenital nephropathy, a condition that causes nephrotic syndrome. The most common form of this condition is congenital nephropathy of the Finnish type (CNF), inherited as an autosomal recessive trait-meaning the gene for CNF must be inherited from both parents.

Another condition that causes nephrotic syndrome in the first months of life is diffuse mesangial sclerosis (DMS). The pattern of inheritance for DMS is not as clearly understood as the pattern for CNF, although the condition does appear to be genetic.

Since medicines have little effect on congenital nephropathy, transplantation is usually required by the second or third year of life, when the child has grown enough to receive a kidney. To keep the child healthy, the doctor may recommend infusions of the protein albumin to make up for the protein lost in urine and prescribe a diuretic to help eliminate the extra fluid that causes swelling. The child's immune system may be weakened, so antibiotics should be given at the first sign of infection.

Congenital nephropathy can disturb thyroid activity, so the child may need the substitute hormone thyroxine to promote growth and help bones mature. Some children with congenital nephropathy have excessive blood clotting, or thrombosis, which must be treated with a blood thinner like warfarin.

A child with congenital nephropathy may need tube feedings to ensure proper nutrition. In some cases, the diseased kidneys may need to be removed to eliminate proteinuria. Dialysis will then be required to replace kidney function until the child's body is big enough to receive a transplanted kidney. Peritoneal dialysis is preferable to hemodialysis for young children.

In peritoneal dialysis, a catheter is surgically placed in the abdomen and then used to introduce a solution into the abdominal cavity, or peritoneum. The solution draws wastes and extra fluid from the bloodstream. After a few hours, the solution is drained and replaced with a fresh supply. The drained solution carries the wastes and extra fluid out of the body.

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Points to Remember

• Nephrotic syndrome is a set of signs or symptoms that may point to kidney problems.
• Childhood nephrotic syndrome is most common between the ages of 1½ and 5 years.
• Nephrotic syndrome causes proteinuria, low levels of protein in the blood, less frequent urination, and swelling and weight gain from the buildup of fluid.
• Diagnosis of nephrotic syndrome requires urine and blood samples and may include a kidney biopsy.
• Most cases of childhood nephrotic syndrome result from minimal change disease.
• The two most common diseases that damage the kidneys' tiny blood-filtering units and cause nephrotic syndrome are focal segmental glomerulosclerosis (FSGS) and membranoproliferative glomerulonephritis (MPGN).
• Congenital nephropathy is a rare condition that causes nephrotic syndrome in newborns.

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Hope through Research

The NIDDK conducts and supports research to help people with kidney disease, including children. The NIDDK's Division of Kidney, Urologic, and Hematologic Diseases (DKUHD) maintains the Pediatric Nephrology Program, which supports research into the causes, treatment, and prevention of kidney disease in children. In 2002, the DKUHD initiated the Focal Segmental Glomerulosclerosis Clinical Trial to learn more about the best way to treat FSGS. Then, in 2003, the DKUHD began the Prospective Study of Chronic Kidney Disease in Children to learn more about the negative effects of pediatric kidney disease, including cardiovascular disease and neurocognitive impairment.

People interested in participating in clinical trials of new treatments for nephrotic syndrome can find a list of centers recruiting patients atwww.ClinicalTrials.gov.

The U.S. Government does not endorse or favor any specific commercial product or company. Trade, proprietary, or company names appearing in this document are used only because they are considered necessary in the context of the information provided. If a product is not mentioned, the omission does not mean or imply that the product is unsatisfactory.

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For More Information

American Kidney Fund
6110 Executive Boulevard, Suite 1010
Rockville, MD 20852
Phone: 1-800-638-8299 or 301-881-3052
Email: helpline@kidneyfund.org
Internet: www.kidneyfund.org 

American Society of Pediatric Nephrology
3400 Research Forest Drive, Suite B7
The Woodlands, TX 77381
Phone: 281-419-0052
Fax: 281-419-0082
Email: info@aspneph.com
Internet: www.aspneph.com 

National Kidney Foundation
30 East 33rd Street
New York, NY 10016
Phone: 1-800-622-9010 or 212-889-2210
Fax: 212-689-9261
Internet: www.kidney.org 

You may also find additional information about this topic by visiting MedlinePlus at www.medlineplus.gov.
This publication may contain information about medications. When prepared, this publication included the most current information available. For updates or for questions about any medications, contact the U.S. Food and Drug Administration toll-free at 1-888-INFO-FDA (1-888-463-6332) or visit www.fda.gov. Consult your doctor for more information.

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