Kidney disease in children: nephrotic and nephritic syndrome

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What is it about ?

The kidneys are made up of millions of microscopic filters (the glomeruli). Blood flows continuously through these filters. Filters clean the blood permanently: they rid the blood of excess waste and water, and keep the amount of salts within certain limits. What is in excess is evacuated via the urine.

Sometimes these filters are damaged. This destabilizes the water balance in the body. Some wastes can build up in the blood. Filters can leak items that should not normally pass through the filter, such as proteins or red blood cells. The blood then contains too little protein, and these proteins and red blood cells are found in the urine.

If the kidney filters keep getting damaged, they eventually don’t work at all. In this case, the waste remains in the blood, which can be toxic to the body.

A syndrome is a collection of symptoms.

Nephrotic syndrome and nephritic syndrome are syndromes caused by damage to the kidney filters.

In nephrotic syndrome, the kidneys mostly pass protein in the urine (proteinuria). It is characterized by an accumulation of fluid in the tissues (edema) and the presence of protein in the urine (proteinuria). The cause of this disease in children is not always known.

In nephritic syndrome, the kidney filters are damaged by inflammation. There may also be blood in the urine (hematuria) and, to a lesser extent, protein. Often the cause is a viral or bacterial infection. The most common form is a inflammation of the kidney filters (glomerulonephritis) after infection with a particular bacteria, streptococcus. Sometimes it is an abnormal functioning of the immune system that is the cause: the body makes antibodies against its own cells. Finally, it may be a rare birth defect.

In whom and how often do they occur?

Nephrotic syndrome is rare (2 in 10,000 children per year). The first symptoms appear between the ages of 2 and 5 years. The syndrome is more common in boys than in girls.

Since nephritic syndrome is often caused by other disorders, there are no precise figures on the number of cases. We know that 80% of cases of nephritic syndrome in children occur after exposure to group A streptococci during a throat or skin infection.

How to recognize it?

The primary characteristic of nephrotic syndrome is an accumulation of fluid (edema). Fluid can accumulate anywhere on the body, but especially in the legs and face (around the eyes). When you press on an swollen area, a slight indentation is created and persists after the pressure has been stopped. Edema can also develop around the genitals and, in extreme cases, in the belly. In this case, the belly swells quickly and there is weight gain.

In nephritic syndrome, kidney symptoms usually appear 1 to 2 weeks after a throat or skin infection. These symptoms can range from the presence of a little blood in the urine, without other complaints, to the formation of edema, hypertension andkidney failure, so that the child hardly urinates anymore. In the case of systemic disease (rarer), typical kidney symptoms are accompanied by general symptoms such as fever, stomachache, vomiting, fatigue, rash, skin bleeding and joint inflammation. Shortness of breath and heart failure can also occur.

How is the diagnosis made?

Faced with generalized edema, the doctor will always perform a blood and urine test. The presence of large amounts of protein in the urine is a sign of nephrotic syndrome. Hematuria is often (noticeably) present in nephritic syndrome, and usually not in nephrotic syndrome.

What can your doctor do?

Hospitalization is usually necessary to initiate treatment.

In nephrotic syndrome, the amount of fluid and the level of protein in the blood must be carefully regulated by administering serum and plasma. A low salt diet is recommended. Drug treatment consists of the administration of high doses of cortisone, which will be gradually reduced. The child usually responds well to treatment, but relapses are possible.

Streptococcal nephritic syndrome is treated with high doses of antibiotics. Fluid and salt intake should be limited, as well as diuretics and treatment for hypertension. Symptoms usually go away after 2 to 3 weeks.

Want to know more?

Source

Foreign clinical practice guide ‘Kidney disease in children (nephrotic and nephritic syndrome)’ (2015), updated on 12.12.2016 and adapted to the Belgian context on 21.05.2018 – ebpracticenet

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