Congenital nephrotic syndrome

Alternative names:

nephrotic syndrome - congenital

Definition:

An inherited disorder characterized by protein in the urine and swelling of the body (see also Nephrotic syndrome).

Causes, incidence, and risk factors:

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 exact cause is unknown. 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 (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). Loss of proteins includes insufficient amounts of immune system antibodies. The disorder commonly results in infection, malnutrition, and kidney failure. It usually causes death within the first year.