Childhood Nephrotic Syndrome

This has been written to give you information about a kidney problem called the nephrotic syndrome. This illness is called other names including nephrosis and minimal change disease (the specific name for the most common form of childhood nephrotic syndrome.)

What is nephrotic syndrome?
The kidneys are two fist-sized organs located in the lower back. They have many functions including cleaning the blood to get rid of waste products and excess salt and water. When the kidney filters are diseased, things may leak out into the urine, which should not normally be there, such as protein and blood cells. The loss of too much protein in the urine can lead to nephrotic syndrome. Although the nephrotic syndrome can occur in several different kidney diseases, most commonly in children it is due to minimal change disease.

Who is affected by nephrotic syndrome?
Although people of any age can get nephrotic syndrome, it is most common in children between the ages of six months to five years. Boys are affected almost twice as often as girls. A particular type of nephrotic syndrome can occur in infants.

How do you get nephrotic syndrome?
Most often, the cause of childhood nephrotic syndrome is not known. It often starts after a cold or infection. Many research programs are being conducted to understand more about the causes and treatments of nephrotic syndrome.

How can you tell that a child has nephrotic syndrome? What are the symptoms?
When the protein level in the blood drops due to protein loss in the urine, water leaks out of the blood vessels into the body tissues. This results in swelling (edema) around the eyes (usually worse in the morning), in the abdomen (belly) and in the legs.

A child with nephrotic syndrome gains weight due to the excess water and may urinate less often.

The child with nephrotic syndrome may also:

• be more tired
• be more irritable
• have less appetite
• look pale
• have nausea and vomiting
• have diarrhea

How is nephrotic syndrome diagnosed?
Your doctor will diagnose a child with nephrotic syndrome based on information he or she obtains about the child's symptoms, testing the urine to measure protein, and also by performing certain blood tests.

How is nephrotic syndrome treated?
Most children will improve when treated with a medication called prednisone, which is used to decrease the loss of protein in the urine. The dosage will be adjusted, and it is very important to take this medication exactly as it is prescribed. Other medications may be needed to help the kidneys get rid of the excess water. These medications cause the child to urinate more, reducing the swelling. Your doctor may also recommend changes in diet, like lowering the amount of salt intake.

Does prednisone have side effects?
Although prednisone is a very effective medication, it can cause many potential side effects. The type of side effects mainly depend on the disease and the length of time one is taking prednisone. The side effects go away when the prednisone is stopped. Stopping the medication abruptly can cause serious reactions, so the medication should always be taken exactly as prescribed. Side effects may include:

• increased appetite
• weight gain
• mood swings (happy/sad)
• being overactive
• increased chance of infection
• cataracts
• acne
• decreased growth rate
• high blood pressure
• heartburn, ulcers

Does every child with nephrotic syndrome improve with prednisone treatment?
Almost all children will improve with prednisone treatment. However, some do not, and your doctor may recommend doing a kidney biopsy and treating the child with other kinds of medications. Although these other medications, which are called immunosuppressive medications, are often helpful in decreasing urine protein losses, there are side effects that you should discuss with your doctor. The main side effects include an increased risk of infection, a decrease in blood cell count, and hair loss. Children treated with these medications will be monitored closely by their doctors and will require periodic blood tests.

Are there many complications in children with nephrotic syndrome?
The most common problem is swelling due to the accumulation of water in the body. However, more serious problems may occur, such as an infection in the abdomen or the formation of blood clots. If a child with nephrotic syndrome has a fever and severe pain in the abdomen, or pain and swelling of an arm or leg, the doctor should be called right away.

What is the outlook for children with nephrotic syndrome?
Although most children will respond to treatment (the swelling and protein in the urine will go away), a majority of children will have a relapse. This occurs usually after the child has a cold. Children with nephrotic syndrome should be monitored at home for any indications that a relapse is occurring. The doctor may recommend that the child's urine be tested at home to measure for protein and that his or her weight be checked. It is very helpful to the doctor if the family keeps written records of these results. Although there is not a specific cure for nephrotic syndrome, there are several treatments. Most children do have relapses, but a majority of children eventually "outgrow" this disease when they are teenagers. The longer a child goes without a relapse, the less likely he or she will have one. Even if a child has many recurring attacks of nephrotic syndrome, as long as the response to treatment each time is good, there generally will not be permanent kidney damage, and the long-term outlook is excellent.

Where can I get more information about nephrotic syndrome?
If you have additional questions, you should speak to your doctor. You may also be interested in the following publications available from the National Kidney Foundation:

• Causes of Kidney Disease in Children
• Nutrition for Children With Chronic Kidney Disease
• Sodium and Renal Diets: How to Spice Up Your Cooking
• When Your Child Has Glomerulonephritis
• Kidney Biopsy
• Your Kidneys: Master Chemists of the Body.

More than 20 million Americans-one in nine adults-has chronic kidney disease, and most don't even know it. More than 20 million others are at increased risk. the National Kidney Foundation, a major voluntary health organization, seeks to prevent kidney and urinary tract diseases, improve the health and well-being of individuals and families affected by these diseases, and increase the availability of all organs for transplantation. Through its 51 affiliates nationwide, the foundation conducts programs in research, professional education, patient and community services, public education and organ donation. The work of the National Kidney Foundation is funded by public donations.