Something is wrong with your child’s kidneys and nephrotic syndrome is suspected? You may be unsure of what to expect and what to expect of your child. This information sheet gives you an initial overview of this rare clinical picture.
At a glance
In idiopathic nephrotic syndrome (iNS), the affected child loses proteins through the urine. Water retention in the body (edema) occurs. After a few years, the disease heals in most children. Only rarely does a permanent restriction of kidney function develop.
An effective treatment is medication that suppresses the body’s own immune system. Relapses are common. The aim of the treatment is then to reduce the relapses and at the same time to keep the side effects of the medication as low as possible.
The kidneys work like a sieve or filter. They filter out harmful substances and retain substances that are valuable for the body. In nephrotic syndrome, the sieve leaks and valuable substances such as proteins enter the urine. Thus, proteins are missing in the blood. Since the proteins in the blood normally hold the water, more water now gets into other tissues. These water deposits can make themselves felt as swellings. The technical term is edema.
Idiopathic nephrotic syndrome (iNS for short) affects around 250 children in Germany every year. Mostly children between 1 and 10 years are affected. Idiopathic means that the cause is unclear. It is known, however, that the body’s own defense system plays a role in this process.
Signs and symptoms
Typical signs are:
Edema mainly in the face (eyelids), legs and feet, and genital area (scrotum or labia)
darkly discolored and foamy urine
Too few proteins in the blood
Increased fat levels in the blood
Other symptoms may occur, such as nausea, fatigue or high blood pressure. In addition, the children are susceptible to infections, since they lack proteins that otherwise ward off pathogens. The loss of water in the blood can make it easier for vessels to clog. The risk of blood clots (thromboses) is increased.
If left untreated, the kidneys can be so severely damaged that they fail completely (kidney failure). If left untreated, fluid retention can also become a threat, for example in the lungs.
iNS occurs in episodes. This means that there are times without symptoms and times when signs of the disease appear. The typical iNS usually disappears when the affected person becomes an adult.
The doctor will interview you and your child in detail and examine him or her physically. Your child’s urine and blood are tested in the laboratory and the kidneys are examined by ultrasound.
The doctor will also check whether your child has iNS or whether there is a specific cause of nephrotic syndrome, for example, a hereditary disease or inflammation of the blood vessels, as a result of which nephrotic syndrome can develop.
Experts recommend tissue removal from the kidney only in certain situations, for example if the medication is not working or the child is older than 10 years at the onset of the disease.
If iNS occurs for the first time, experts recommend medication that acts like cortisone. In technical language they are called glucocorticoids. These suppress the body’s own defense system. Relapses of the disease and permanent kidney damage are thus to be avoided. In about 9 out of 10 affected children, these cortisone-like medications are effective.
If the condition occurs for the first time, experts currently recommend taking the cortisone-like drug prednisone for a period of 12 weeks. Studies indicate that relapses occur less frequently after 12 weeks of treatment than after 8 weeks. Whether an even longer treatment period can prevent more relapses is unclear according to current studies.
Experts tell children to take prednisone once a day in the morning for the first 6 weeks. For another 6 weeks, the drug is only used every other day. The dose depends on the size and weight of the child.
Despite treatment, about 3 out of 4 affected children relapse. Experts speak of a relapse. Many people experience repeated relapses. Then you take the prednisone for a shorter period than the first time. Usually the remedy is working well again, so the kidneys will recover. The goal is to keep the side effects of the medication down, such as stunted growth, bone loss, weight gain, lens clouding of the eyes, or high blood pressure.
Difficulty arises when the prednisone does not work or no longer works. Affected people often need repeated or permanent use of other medications that suppress the body’s defenses (immunosuppressants).
Very rarely, kidneys may stop working permanently. Kidney weakness (renal insufficiency) develops. Then treatments are needed that take over the work of the kidneys – in technical language they are called: Kidney Replacement Therapies. This includes blood washing (dialysis) and kidney transplants.
Close cooperation between parents and a pediatric team that specializes in kidney disease (pediatric nephrology) can usually prevent permanent consequences of the disease and damage from the medication. Regular check-ups in a nephrology practice or outpatient clinic are also recommended in adulthood.
What you can do yourself
Your child doesn’t need to take it easy physically, they can do anything just like other kids. Even during an episode, your child does not need bed rest.
During a relapse, your child should be on a low-salt diet. It is best to discuss the amount to drink with the medical team.
To detect a relapse in time, check your child’s urine for protein with a test strip in the morning.
Vaccinations protect against certain infections. However, they can trigger a relapse of the iNS. Get advice on this topic.
With support, everyday life can be managed more easily. If needed, you can get psychosocial support as a family.
Find out about self-help organizations and share your experiences with other sufferers, for example at group meetings.