Congenital nephrotic syndrome

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Female urinary tract

Male urinary tract

Congenital nephrotic syndrome is an inherited disorder characterized by protein in the urine and swelling of the body (see also Nephrotic syndrome).

Nephrotic syndrome - congenital

Congenital nephrotic syndrome is a very rare form of nephrotic syndrome. It occurs primarily in families of Finnish origin and develops shortly after birth. It is an inherited disorder. The condition is caused by a protein called nephrin which is found in the kidney and is abnormal in children with the disorder.

Proteins and fats are excreted in the urine, and there is an abnormally high fat level in the blood. Swelling occurs from the effects of kidney failure , combined with the loss of blood protein. This is because proteins in the blood normally keep fluids in the blood stream, and when protein level is low, the fluid can leak into the body tissues.

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Some of the proteins lost in the urine are immune system antibodies that fight infections. The disorder commonly results in infection, malnutrition, and kidney failure. It can often lead to death by 5 years of age.

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.