congenital Finnish nephrosis

What is Congenital Finnish Nephrosis?

Congenital Finnish nephrosis is an inherited disease in which the kidneys are unable to properly filter protein from the urine due to an abnormality in a protein called nephrin. The disease occurs mainly in people of Finnish origin. It is often fatal by the age of five and many cases are fatal within the first year. If the child survives to the age of two or three, kidney transplantation may allow for a more normal lifespan.

Children with congenital Finnish nephrosis are often born prematurely with a low birth weight. High levels of protein in the blood, combined with kidney failure, cause the whole body to swell with excess fluids. These children have a poor appetite and urinate less frequently than chidren without the disease. Children with congenital Finnish nephrosis have difficulty getting needed nutrients and may not grow as large as they would otherwise.

People with congenital Finnish nephrosis cannot retain sufficient amounts of antibodies that help the body fight infection. As a result, they are more prone to infection. They are also prone to inappropriate and potentially harmful blood clots.

Symptoms of the disease begin in the first days or weeks after birth, but always before the age of three months.

How common is Congenital Finnish Nephrosis?

As indicated by its name, congenital Finnish nephrosis is fairly common in Finland, where it affects 1 in 8,000 births. In the United States, it is rare, but more common in people of Finnish ancestry. The disease is extremely common among Old Order Mennonites in Lancaster County, Pennsylvania. It is estimated that 1 in 500 children born in this population are affected by the disease.

How is Congenital Finnish Nephrosis treated?

Because congenital Finnish nephrosis is often fatal in infancy, early and vigilant treatment is necessary to allow the child to live until the age of two or three, at which time he or she may receive a kidney transplant. This is the only hope for a normal lifespan. The disease does not affect the new kidney.

If the disease is too severe, the child's kidneys may need to be removed before he or she is old enough for a transplant. Dialysis machines can be used as a stopgap measure to filter wastes from the child's blood until a transplant can be completed.

A physician may recommend infusions of protein for these children to help replace what is lost in the urine. Diuretic drugs may be prescribed to help eliminate excess water and thus eliminate some swelling. Antibiotics will be necessary to control infection.

Some children with the disease have abnormal thyroid activity and may require hormone replacement. Others have a tendency towards blood clots and may benefit from a blood thinner.

Good nutrition is key to growth. Those who cannot eat sufficient quantities may need a feeding tube.

What is the prognosis for a person with Congenital Finnish Nephrosis?

Many cases of congenital Finnish nephrosis are fatal within five years. If the child lives until the age of two or three, an early kidney transplant may help him or her to live a more normal lifespan.