Nephrotic syndrome: what is it, symptoms, treatment, prognosis

What is nephrotic syndrome?

Nephrotic syndrome is not a disease in itself; quicker this is a group of symptoms, which:

• Indicate kidney damage - especially damage to the glomeruli, tiny units in the kidney where blood is filtered
• cause too much protein to be excreted from the body into the urine.

When the kidneys are damaged albumin proteinusually found in the blood will pass into the urine. Proteins are large, complex molecules that perform a number of important functions in the body.

There are 2 types of nephrotic syndrome:

1. primary - the most common type of nephrotic syndrome that begins in the kidneys and affects only the kidneys;
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2. secondary - the syndrome is caused by other diseases, affecting, etc. organs.

Nephrotic syndrome can develop gradually or it can come on suddenly. The condition can occur at any age. It is most common in children between the ages of 18 months and 4 years, more often in boys than in girls. In adults and the elderly, both sexes are equally affected.

What are kidneys and what do they do?

The kidneys have two bean-shaped organs, each about the size of a fist. They are located just below the ribcage, one on each side of the spine. Each day, the kidneys filter 120 to 150 liters of blood to produce 1 to 2 liters of urine, which is made up of waste and excess fluid.

Children produce less urine than adults, and the amount of food they produce depends on their age. Urine flows from the kidneys into the bladder through tubes called ureters. The bladder stores urine. When the bladder empties, urine flows out of the body through a tube called the urethra, located at the bottom of the bladder.

The kidneys work at a microscopic level. The kidney is not one big filter. Each kidney is made up of about a million filtering units called nephrons. Each nephron filters a small amount of blood.

The nephron includes a filter called a glomerulus and a tubule. Nephrons work in two stages. The glomerulus allows fluid and waste to pass through it; however, it prevents the passage of blood cells and large molecules, mainly proteins. The filtered fluid is then passed through a tube, which sends essential minerals back into the bloodstream and removes waste.

What Causes Nephrotic Syndrome?

Although idiopathic or unknown diseases are the most common cause of primary nephrotic syndrome in patients, researchers associate certain diseases and some specific genetic changes that damage the kidney with primary nephrotic syndrome.

Secondary nephrotic syndrome is caused by an underlying disease or infection. It is the underlying disease or infection that causes changes in kidney function that can lead to a secondary form of the condition.

Congenital diseases - diseases that are present at birth - can also cause nephrotic syndrome.

Primary nephrotic syndrome

The following diseases represent different types of idiopathic nephrotic syndrome:

• Minimal Change Disease includes damage to the glomeruli, which can only be detected with an electron microscope. This type of microscope shows the smallest details better than any other microscope. Scientists do not know the exact cause of minimal change disease.
  Minimal change disease is the most common cause of idiopathic nephrotic syndrome.
• Focal segmental glomerulosclerosis is the formation of scars in scattered areas of the kidney:
  • Focal means that only some of the glomeruli become scarred.
  • "Segmental" means that the damage affects only part of an individual glomerulus.
• Membranoproliferative glomerulonephritis is a group of disorders involving antibody deposits that accumulate in the glomeruli, causing thickening and damage. Antibodies are proteins made by the immune system to protect the body from foreign substances such as bacteria or viruses.

Secondary nephrotic syndrome

Some common conditions that can cause secondary nephrotic syndrome include

• diabetes, a condition that occurs when the body is unable to use glucose (a type of sugar) entering the body;
• purple Shenlein-Genoch (hemorrhagic vasculitis), a disease that causes inflammation of the small blood vessels in the body;
• viral hepatitis, inflammation of the liver caused by a virus;
• human immunodeficiency virus (HIV), a virus that alters the immune system;
• lupus erythematosus, an autoimmune disease that occurs when the body attacks its own immune system;
• malaria, a blood disease that is spread by mosquitoes;
• streptococcal infection, an infection that occurs when bacteria that cause an acute pharyngitis or a skin infection is left untreated.

Other causes of secondary nephrotic syndrome may include taking certain medications, such as aspirin, ibuprofen, or others non-steroidal anti-inflammatory drugs, and exposure to chemicals such as mercury and lithium.

Congenital diseases and nephrotic syndrome

Congenital nephrotic syndrome is rare and affects children in the first 3 months of life. This type of nephrotic syndrome, sometimes called infantile nephrotic syndrome, can be caused by:

• hereditary genetic defects that are passed from parent to child through genes;
• infections during birth.

Who is most likely to suffer from nephrotic syndrome?

In cases of primary nephrotic syndrome, which is caused by idiopathy (an independent disease), researchers cannot accurately determine which patients are more likely to develop the syndrome. However, as researchers continue to study the link between genetics and nephrotic syndrome, it may be possible to predict the occurrence of the syndrome in some children in the future.

The secondary type of the syndrome develops more often if:

• there are diseases that can damage the kidneys of a person;
• take certain medications (such as NSAIDs);
• develop certain types of infections.

Signs and symptoms of nephrotic syndrome

Signs and symptoms of nephrotic syndrome may include

• swelling, most commonly in the legs, feet, or ankles, and less commonly in the hands or face
• albuminuria - when there is a high level of albumin in the urine
• hypoalbuminemia - when there is a low level of albumin in the blood
• hyperlipidemia - when the level of cholesterol and fat in the blood exceeds the norm

In addition, some people with the disease may experience:

• blood in urine (hematuria);
• symptoms of infection such as fever, lethargy, irritability, or abdominal pain;
• loss of appetite;
• diarrhea (diarrhea);
• high blood pressure (arterial hypertension).

Complications

Complications of nephrotic syndrome in a child or adult may include:

• Infections. When the kidneys are damaged, infections are more likely to develop as the body loses proteins that normally protect against infection. Health care providers will prescribe medications to treat infections. Children with nephrotic syndrome should receive the pneumococcal vaccine and annual vaccinations against flu to prevent these infections.
• Blood clots (thrombi). Blood clots can block the flow of blood and oxygen through blood vessels anywhere in the body. Blood clots are more likely to occur when proteins are lost through the urine. Blood clots will be treated with blood thinners.
• High blood cholesterol. When albumin enters the urine, the level of albumin in the blood drops. The liver makes more albumin to compensate for their low blood levels. At the same time, the liver produces more cholesterol. Sometimes people may need treatment with medications to lower blood cholesterol levels.
• Malnutrition. Loss of protein in the blood can lead to weight loss, which can be masked by edema.
• Chronic kidney disease. Over time, the kidneys may lose their function, requiring dialysis or a kidney transplant.
• Acute renal failure. Kidney damage can cause the kidneys to stop filtering waste, requiring emergency dialysis intervention.

Diagnostics

To diagnose nephrotic syndrome, your doctor will first look at your medical history. Asks about symptoms, medications that have been taken, and if there are any health problems. The doctor will also conduct a physical examination. This may include procedures such as measuring your blood pressure and listening to your heartbeat.

Further, the doctor may order studies of biological fluids, which include a blood and urine test, ultrasound (ultrasound) of the kidneys and a biopsy.

Kidney biopsy Is a procedure that involves taking a small piece of kidney tissue for examination under a microscope. A healthcare professional performs a biopsy at an outpatient center or hospital. Usually, a specialist will perform local anesthesia, but in some cases general anesthesia may be required. A pathologist - a doctor who specializes in the diagnosis of diseases - examines the tissue pieces taken in the laboratory.

Treatment of nephrotic syndrome

Health care providers will decide how to treat nephrotic syndrome based on its type:

• primary type: medicines;
• secondary nephrotic syndrome: treat the underlying disease;
• congenital type of syndrome: medications, surgery to remove one or both kidneys, and transplant.

Primary nephrotic syndrome

Idiopathic nephrotic syndrome is treated with several types of medications that control the immune system, remove excess fluid, and lower blood pressure.

​Drugs that control the functioning of the immune system. Corticosteroids are a group of drugs that decrease the activity of the immune system, decrease the amount of albumin lost in urine, and reduce swelling. Usually, prednisone or a related corticosteroid is used to treat idiopathic nephrotic syndrome. About 90 percent of patients achieve remission with daily use of corticosteroids for 6 weeks and then a few days later at slightly lower doses for the same 6 weeks. Remission is the period when the patient is asymptomatic.

Many patients relapse after initial therapy and are treated by health care providers with a short course of corticosteroids until the disease returns to remission. Children and the elderly may have multiple relapses; however, they most often recover without long-term kidney damage.

When a child has frequent relapses or does not respond to treatment, the doctor may prescribe other medications that decrease the activity of the immune system. These medications prevent the body from producing antibodies that can damage kidney tissue. They include:

• Cyclophosphamide;
• Tacrolimus;
• Cyclosporine;
• Mycophenolate.

Preparations that remove excess fluid. A healthcare professional may prescribe a diuretic to help the kidneys remove excess fluid from the blood. Removing extra fluid often helps lower blood pressure.

Blood pressure lowering drugs. Some people with nephrotic syndrome develop hypertension (high blood blood pressure), and they may need to take additional medications to lower their blood pressure. Two types of drugs that lower blood pressure, angiotensin-converting enzyme inhibitors and receptor blockers angiotensin have the added benefit of slowing down the progression of blood pressure in addition to lowering blood pressure kidney disease. Many with nephrotic syndrome need two or more medications to control their blood pressure.

Secondary nephrotic syndrome

The secondary type of syndrome is treated by treating the primary disease that triggered the condition. For example, a doctor may:

• prescribe antibiotics if the cause is a bacterial infection;
• prescribe medications to treat lupus, HIV, or diabetes;
• or stop taking medications known to be causing secondary nephrotic syndrome.

When treating the underlying cause, the healthcare professional will also treat to improve or restoring kidney function with the same drugs that are used to treat primary forms of the disease.

The patient must take all prescribed medications and follow the treatment plan recommended by the treating physician.

Congenital nephrotic syndrome

Researchers have found that drugs are not effective in treating congenital nephrotic syndrome. and that most children will need a kidney transplant by the time they are 2 or 3 of the year. A kidney transplant is an operation to transplant a healthy kidney from a person who has just died, or from a living donor, most often a family member, into the body of a sick person. To keep the patient healthy before the transplant, the doctor may recommend the following:

• albumin injections to replace the loss of albumin in the urine;
• diuretics to help remove excess fluid that causes swelling
• antibiotics to treat the first signs of infection;
• growth hormones to promote growth and help mature bones;
• removal of one or both kidneys to reduce the loss of albumin in the urine;
• dialysis to artificially filter waste products from the blood if the kidneys fail.

Prevention

Specific prevention methods have not been developed. It is necessary to seek medical help in a timely manner in order to identify and begin treatment of diseases that can provoke this syndrome in the early stages. It is forbidden to take pharmaceutical preparations without a doctor's prescription.

Forecast

The prognosis for nephrotic syndrome can vary. It depends on what is the underlying cause as well as your overall health.

Some diseases that cause nephrotic syndrome go away on their own or with treatment. After treatment of the underlying disorder, nephrotic syndrome should improve. However, other medical conditions can eventually lead to renal failure, even with treatment. When this happens, dialysis and possibly a kidney transplant will be required.

If you have worrying symptoms or suspect nephrotic syndrome is possible, make an appointment with your doctor to discuss your concerns.