Pediatric Nephrotic Syndrome

Nephrotic syndrome is a problem where too much protein called albumin is released from the body into the urine. It means that one or both kidneys are damaged.

The kidneys contain many coils of tiny blood vessels. Each of these is called a glomerulus. Glomeruli filter substances from the blood into the urine. Nephrotic syndrome occurs when the glomeruli stop working normally.

The most common type is called minimal change nephrotic syndrome (MCNS). With MCNS, a child has times when symptoms get worse (relapses). But the condition can be managed over time. In rare cases, a child may develop kidney failure and need dialysis.

Most children with this problem have idiopathic nephrotic syndrome. Idiopathic means that it occurs with no known cause.

In some cases, a rare nephrotic syndrome may occur in the first week of a child’s life. This is called congenital nephrotic syndrome. It is inherited by an autosomal recessive gene. This means that boys and girls are equally affected. A child inherits one copy of the gene from each parent, who are carriers. Carrier parents have a one in four chance of having a child with this syndrome with each pregnancy. The outcome for this type of nephrotic syndrome is very poor.

Symptoms can occur a bit differently in each child. They can include:

• Extreme tiredness (fatigue)
• A general feeling of discomfort (malaise) 
• Decreased appetite
• Weight gain and facial swelling
• Belly swelling or pain
• Foamy urine
• Fluid buildup in the body (edema)
• Fluid buildup in the belly area (ascites)
• Pale fingernail beds
• Dull hair 
• Ear cartilage that feels less firm
• Food intolerance or allergies

The symptoms of nephrotic syndrome can be like other health conditions. Make sure your child sees his or her health care provider for a diagnosis.

Your health care provider will ask about your child’s symptoms and health history. He or she may also ask about your family’s health history. The provider will give your child a physical exam. Your child may also have tests performed, such as:

• Urine test. This is done to check the level of protein
• Blood tests. These check your child’s levels of cholesterol, a blood protein called albumin and other blood chemicals
• Renal ultrasound (sonography). This is a painless test that uses sound waves and a computer to create images of body tissues. During the test, a health care provider moves a device called a transducer over the belly in the kidney area. This sends a picture of the kidney to a video screen. The health care provider can see the size and shape of the kidney. He or she can also see a growth, kidney stone, cyst or other problems
• Renal biopsy. The health care provider takes a small sample of kidney tissue. This is done through the skin with a needle or during surgery. The sample is looked at under a microscope

Treatment will depend on your child’s symptoms, age and general health. It will also depend on how severe the condition is.

During the first episode of nephrotic syndrome, your child may need to stay in the hospital and be observed if the swelling is severe or if she or he has blood pressure or breathing problems.

Medicines may be used to treat initial symptoms and relapses. These may include:

• Corticosteroid medicine
• Immunosuppressive medicine
• Diuretic medicine to reduce the swelling
• IV (intravenous) albumin to replace low levels in the blood
• A diet that restricts salt and fluid (see below)

The medicines used to treat nephrotic syndrome weaken the immune system. Because of this, your child should not be given live vaccines. If your child has not had the chickenpox vaccine and is exposed to the virus, he or she may need a vaccine.

Talk with your child’s health care providers about the risks, benefits and possible side effects of all medicines.

Children with nephrotic syndrome may have trouble regulating their body's water balance. This can cause swelling from fluid retention (edema). The diet for a child with nephrotic syndrome may include limiting salt and fluids. This may help to regulate your child's fluid balance. Fluids include any food that is liquid at room temperature, such as popsicles and ice cream. Salt affects body swelling. Don't add salt at the table and avoid feeding your child salty foods. Your child's health care provider will talk with you about the amount of salt and fluids your child should have each day.

In rare cases, a child may develop kidney failure and need dialysis. Dialysis is a procedure that filters waste and extra fluid from the blood. This is normally be done by the kidneys.

You can help your child stay healthy in these ways:

• Help prevent your child from having contact with people who have colds or other respiratory illnesses. A viral respiratory illness could cause a relapse
• Test your child’s urine for protein every day. This will help to find the early signs of a relapse
• Postpone routine vaccines until your child is in remission and off steroids for at least three months. Talk with your child's health care provider about the right time to vaccinate

You can get more resources and support from these organizations:

• American Association of Kidney Patients, 800-749-2257
• National Kidney Foundation, 800-622-9010

Nephrotic syndrome can get better on its own and with treatment. It may also get worse despite treatment. A child may have periods of improvement as well as relapses. Family support is important for a child’s well-being.

Once a child reaches his or her teen years, the syndrome usually stays in remission. This means that symptoms decrease or go away. In most cases, symptoms don’t return in adulthood.

Call your child’s health care provider if your child has:

• Symptoms that don’t get better, or get worse
• New symptoms
• Nausea or vomiting, or abdominal pain
• Unexpected weight gain or swelling in the legs, ankles or around the eyes
• Dark colored urine
• Less or no urine

Call 911 if your child has:

• Severe weakness, dizziness, headache, fainting, sleepiness or confusion
• Chest pain or shortness of breath