Congenital nephrotic syndrome

Alternative Names

Nephrotic syndrome – congenital

Definition of Congenital nephrotic syndrome

Congenital nephrotic syndrome is disorder passed down through families in which a baby develops protein in the urine and swelling of the body. Congenital means it is present from birth.

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 inherited, which means it is passed down through families.

Signs and tests

An ultrasound done on the pregnant mother before birth may show a larger-than-normal placenta. The placenta is the organ that develops during pregnancy to feed the developing baby.

Treatment

Early and aggressive treatment is needed to control the disorder.

Expectations (prognosis)

The disorder commonly results in infection, malnutrition, and kidney failure. It can often lead to death by 5 years of age, and many children die within the first year. Congenital nephrotic syndrome may be successfully controlled in some cases with early and aggressive treatment, including early kidney transplantation.

Review

Parul Patel, MD, Private Practice specializing in Nephrology and Kidney and Pancreas Transplantation, Affiliated with California Pacific Medical Center, Department of Transplantation, San Francisco, CA. Review provided by VeriMed Healthcare Network. Also reviewed by David Zieve, MD, MHA, Medical Director, A.D.A.M., Inc. – 8/19/2009

Female urinary tract
Male urinary tract

ADAM Medical Encyclopedia

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