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Nephrotic syndrome is a group of symptoms that indicate the kidneys are not working properly. These symptoms include
The kidneys are made up of about a million filtering units called nephrons. Each nephron includes a filter, called the glomerulus, and a tubule. The glomerulus filters the blood, and the tubule returns needed substances to the blood and removes wastes and extra water, which become urine. Nephrotic syndrome usually happens when the glomeruli are damaged, allowing too much protein to leak from the blood into the urine.View full-sized image
Health care professionals use different terms to refer to nephrotic syndrome in children, depending on
Nephrotic syndrome is not very common in children. On average, fewer than 5 in 100,000 children worldwide develop nephrotic syndrome each year.1
Children of all ages can develop nephrotic syndrome. But the condition most often affects children who are 2 to 7 years old, particularly boys.2
Losing too much protein in the urine can lead to many complications, including3
Swelling around the eyes is the most common sign of nephrotic syndrome in children.3 The swelling is usually greater in the morning and, when mild, may be confused with seasonal allergies.
Other common symptoms include2,3
Some children with nephrotic syndrome may also have
Kidney disease that affects a kidney’s filtering system is the most common cause of nephrotic syndrome in children. Other causes can include diseases that affect other parts of the body, infections, some medicines, and genetics.
Four types of kidney disease can cause primary nephrotic syndrome in children and adolescents.2
Other causes of primary nephrotic syndrome are uncommon.
Causes of secondary nephrotic syndrome in children include3
Among newborns and infants younger than 12 months old, the two most common causes of nephrotic syndrome are4
Nephrotic syndrome in children is diagnosed with
Additional tests to identify the cause of nephrotic syndrome may include3
Many children with nephrotic syndrome will not need a kidney biopsy. The test is usually reserved for children who have complex disease, who have low kidney function, or who are 12 years old or older.3
Nephrotic syndrome in children is most often treated with medicines.
Corticosteroids. Corticosteroids, or steroids, are the medicines most often used to treat children with primary nephrotic syndrome. These medicines suppress the immune system, reduce the amount of protein passed into the urine, and decrease swelling.
In most children, treatment with corticosteroids will make nephrotic syndrome improve—also called “remission.” If symptoms return, called a “relapse,” the health care professional may prescribe a shorter course of corticosteroids until the disease goes into remission again. Although children may have multiple relapses, they often recover without long-term kidney damage. In most cases, relapses happen less often as children get older.3
Although corticosteroids effectively treat nephrotic syndrome in many children, using these medicines for long periods of time can cause side effects, such as impaired growth, obesity, high blood pressure, eye problems, and bone loss.3 Other common side effects include anxiety, depression, and aggressive behavior. These problems are more likely to develop with larger doses and longer use.
In some cases, nephrotic syndrome may not improve with corticosteroids. Cases of nephrotic syndrome that don’t respond to corticosteroids are more difficult to treat than those that do. They are also more likely to progress to end-stage kidney disease.3
Other medicines that suppress the immune system. If corticosteroids are not working or are causing harmful side effects, your child’s health care professional may prescribe other medicines that reduce the activity of the immune system. In some cases, your child may take these medicines together with low-dose corticosteroids.
Medicines for managing symptoms and complications. Health care professionals may also prescribe other medicines to help your child manage the symptoms and complications of nephrotic syndrome. Examples include
Children with nephrotic syndrome should get the pneumococcal vaccine and yearly flu shots to prevent viral and bacterial infections. They should also get age-appropriate vaccinations. But the health care professional may delay certain “live” vaccines—vaccines that use weakened forms of a virus—while your child is taking certain medicines.
Treatment focuses on the cause of nephrotic syndrome. For example, the health care professional may
Your child’s health care professional may also prescribe the same medicines used to manage the symptoms and complications of primary nephrotic syndrome.
Treatment varies depending on whether the cause is genetic or an infection.
Genetic. Your child’s treatment will depend on the type of genetic mutation that is causing nephrotic syndrome and how bad the symptoms and complications are. Many children will lose kidney function over time and ultimately need a kidney transplant. To keep your child healthy until the transplant, the health care professional may recommend4,5
Infection. When nephrotic syndrome is caused by a congenital infection, such as syphilis or toxoplasmosis, it will usually go away when the infection is treated.4
Researchers have not found a way to prevent nephrotic syndrome in children. Knowing the symptoms can help you get your child treated early and reduce the risk of complications.
Children who have nephrotic syndrome may need to change what they eat and drink, such as
In some cases, the child’s health care professional may recommend other dietary changes. Parents or other caregivers should talk with their child’s health care professional before making any changes to the child’s diet.
The NIDDK conducts and supports clinical trials in many diseases and conditions, including kidney diseases. The trials look to find new ways to prevent, detect, or treat disease and improve quality of life.
Children respond to medicines and treatments differently than adults. The way to get the best treatments for children is through research designed specifically for them.
We have already made great strides in improving children's health outcomes through clinical trials—and other types of clinical studies. Vaccines, treatments for children with cancer, and interventions for premature babies are just a few examples of how this targeted research can help. However, we still have many questions to answer and more children waiting to benefit.
The data gathered from trials and studies involving children help doctors and researchers
We understand you have many questions, want to weigh the pros and cons, and need to learn as much as possible. Deciding to enroll in a study can be life changing for you and for your child. Depending on the outcome of the study, your child may find relief from their condition, see no benefit, or help to improve the health of future generations.
Talk with your child and consider what would be expected. What could be the potential benefit or harm? Would you need to travel? Is my child well enough to participate? While parents or guardians must give their permission, or consent, for their children to join a study, the children must also agree to participate, if they are capable (verbal). In the end, no choice is right or wrong. Your decision is about what is best for your child.
The National Institutes of Health (NIH) is committed to ensuring you get all the information you need to feel comfortable and make informed decisions. The safety of children remains the utmost priority for all NIH research studies. For more resources to help decide if clinical trials are right for your child, visit Clinical Trials and You: For Parents and Children.
Researchers study many aspects of nephrotic syndrome in children, such as the kidney diseases that can cause nephrotic syndrome in children and the genetic factors that can cause congenital nephrotic syndrome.
Watch a video of NIDDK Director Dr. Griffin P. Rodgers explaining the importance of participating in clinical trials.
You can view a filtered list of clinical studies on nephrotic syndrome in children that are federally funded, open, and recruiting at www.ClinicalTrials.gov. You can expand or narrow the list to include clinical studies from industry, universities, and individuals; however, the NIH does not review these studies and cannot ensure they are safe. If you find a trial you think may be right for your child, talk with your child’s doctor about how to enroll.
This content is provided as a service of the National Institute of Diabetes and Digestive and Kidney Diseases
(NIDDK), part of the National Institutes of Health. The NIDDK translates and disseminates research findings to increase knowledge and understanding about health and disease among patients, health professionals, and the public. Content produced by the NIDDK is carefully reviewed by NIDDK scientists and other experts.
The NIDDK would like to thank:
Debbie Gipson, M.D., University of Michigan Medical School