Nephrotic Syndrome

The main function of the kidneys is to clean blood by filtering out excess water, salt and waste products from food. However when the kidneys have damaged filters protein can leak into the urine. As a result there is not enough protein in the blood to soak up the water and therefore the water moves into the body tissues causing swelling and a greater chance of catching infections.

It can affect people of any age, but is often first seen in children aged between two and five years.

Children with nephrotic syndrome have times when their symptoms are under control (remission), followed by times when symptoms return (relapses). Most will relapse less frequently as they get older, eventually "growing out of it" by their late teens.

Symptoms can usually be controlled by medication. Most children with nephrotic syndrome respond well to steroids and aren't at risk of kidney failure.

However, a small number of children have congenital (inherited) nephrotic syndrome and usually do less well.

What problems can it cause?

Some of the main symptoms associated with nephrotic syndrome are:

Swelling

The low level of protein in the blood reduces the movement of water from surrounding tissues back into the blood vessels, leading to swelling (oedema). Swelling is usually first noticed around the eyes, then around the lower legs and rest of the body.

Infections

Antibodies are a specialised group of proteins in the blood that help fight infection. When these are lost, children are much more likely to get infections and frequently experience fatigue, poor appetite and weakness.

Urine changes

Occasionally, the high levels of protein being passed into the urine can cause it to become "frothy". Some children with nephrotic syndrome may also pass less urine than usual during relapses.

Blood clots

Important proteins that help prevent the blood from clotting can be passed out in the urine of children with nephrotic syndrome. This can increase their risk of potentially serious blood clots.

Diagnosis

Diagnosis is made with a simple urine test and blood test. In very few cases a kidney biopsy is required.

Symptoms:

• High levels of protein in urine.
• Low levels of protein in the blood.
• Less frequent urination.
• Weight gain from excess water.
• Oedema (swelling) resulting from build-up of salt and water.
• Vomiting and diarrhoea due to abdomen swelling.
• Skin breakdown during periods of oedema – very pale skin. 

Managing nephrotic syndrome

The main treatment for nephrotic syndrome is steroids, but additional treatments may also be used if a child develops significant side effects.

Steroids

When they are first diagnosed, your child will normally be prescribed at least a four week course of the steroid medicine prednisolone. This will need to be taken every day, followed by another four weeks where the medication is taken every other day. This stops protein leaking from your child's kidneys into their urine.

When prednisolone is prescribed for short periods, there are usually no serious or long-lasting side effects, although some children may experience increased appetite, weight gain, swollen cheeks and mood changes.

Most children respond well to treatment with prednisolone, with the protein often disappearing from their urine and the swelling going down within a few weeks. This period is known as remission.

Diuretics

Diuretics, or "water tablets", may also be given to help reduce the fluid build-up. They work by increasing the amount of urine produced.

Penicillin

Penicillin is an antibiotic and may be prescribed during relapses to reduce the chances of an infection.

Dietary changes

To prevent further water retention and oedema, you may be advised to reduce the amount of salt in your child's diet. This means avoiding processed foods and not adding salt to what you eat.

Vaccinations

Children with nephrotic syndrome are advised to have the pneumococcal vaccine. Some children may also be recommended the varicella (chickenpox) vaccination between relapses.

Live vaccines (such as MMR, chickenpox and BCG) should not be given while your child is taking medication to control their symptoms.

Caring for your child at home

If your child has been diagnosed with nephrotic syndrome, you will need to monitor their condition on a daily basis to check for signs of relapses.

You will need to use a dipstick to test your child's urine for protein the first time they urinate each day. The result for each day will need to be written down in a diary for your doctor to review during your outpatient appointments.

You should also note down the dose of any medication they are taking and any other comments, such as whether your child is feeling unwell.

If the dipstick shows +++ or more of protein in the urine for three days in a row, this means your child is having a relapse. If this happens, you either need to follow the advice given about starting steroids or contact your doctor.

You should seek immediate medical advice if:

• Your child has come into contact with someone who has chickenpox or measles
• Your child is puffy, unwell or has a fever
• Your child has diarrhoea and is vomiting

Support

There is help and support available from various medical professionals involved in your child’s care and treatment.