Childhood Nephrotic Syndrome (Minimal Change Disease; Nephrosis)

Overview

Childhood nephrotic syndrome is a condition marked by the loss of large amounts of protein through the urine. It most commonly occurs for the first time between the ages of 1½ and 5 years. It affects boys about twice as often as girls.

Causes

Eighty percent of cases of childhood nephrotic syndrome are caused by a kidney disorder known as minimal change disease. The condition is called minimal change disease because children with this form of nephrotic syndrome have normal or nearly normal biopsies. Researchers don't know what exactly triggers minimal change disease although it appears to be preceded by viral infection allergic reactions or recent immunizations.

In the other 20 percent of children with nephrotic syndrome the kidney biopsy reveals scarring or deposits in the blood filtering units of the kidney. The two most common diseases that cause this damage are the almost unpronounceable focal segmental glomerulosclerosis (FSGS) and membranoproliferative glomerulonephritis (MPGN).

Very rarely a child may be born with a condition that causes nephrotic syndrome (congenital nephropathy).

Symptoms

Childhood nephrotic syndrome is characterized by high levels of protein in the urine low levels of protein in the blood and swelling caused by a buildup of salt and water. The first sign is often swelling around the eyes particularly in the morning. Later the swelling may extend to the abdomen and the ankles and feet. You may notice it is more difficult to put on the child's shoes. Your child may also look pale be more tired and irritable than usual and be eating less than usual.

Treatment

If your child is diagnosed with minimal change disease the doctor will probably prescribe prednisone which belongs to a class of drugs called corticosteroids. Prednisone stops the flow of protein from the blood into the urine but it does have side effects which your doctor will explain. He or she may also prescribe a diuretic such as furosemide which reduces the swelling by helping your child urinate. When protein is no longer present in the urine the doctor will begin to reduce the dosage of prednisone. This process takes several weeks.

While some children never get sick again others will experience a relapse usually following a viral illness such as a cold or the flu. The good news is that most children "outgrow" this disease by their late teens with no permanent damage to their kidneys.

Prednisone is less effective in children whose nephritic syndrome is caused by something other than minimal change disease. In such cases the doctor may use additional drugs. However if your child has rare congenital nephropathy medicines will have little effect and transplantation is usually required by the second or third year of life when the child has grown sufficiently to receive a kidney.