ICD (International Classification of Diseases) nephrotic syndrome is not an independent kidney disease, but a group of symptoms, the totality of which shows that the kidneys are not working as well as they should.
Small blood vessels (venules, arterioles and capillaries) in the kidneys function as microfilters, removing toxins, waste products and excess water from the blood. These wastes and water enter the bladder and leave our body with urine. Normally, there should be no protein in the urine.
Renal vessels are part of the glomerular network that filters the kidneys. When the filtration network is damaged, too much protein passes through the filters into the urine. The consequence of which is the nephropathic syndrome, i.e. the progressive destruction of the working tissue of the kidneys (nephrons).
This kidney disease affects both adults and children.
Signs of kidney disease
Most people who have been diagnosed with this pathology were not aware of it until they underwent routine clinical examinations at a routine medical examination.
Symptoms of nephropathology include:
- Excessive excretion of protein in the urine (proteinuria).
- Low plasma protein. The medical record may say "hypoalbuminemia".
- High blood cholesterol. The medical term for this is hyperlipidemia.
- High levels of neutral fats in the blood, called triglycerides.
- Swelling of the face, hands, feet and ankles.
- Weight gain.
- Permanent feeling of tiredness.
- Foamy urine.
- Reduced hunger.
If you have clinical nephrotic syndrome on general tests, your he althcare provider will need to find out the cause of the problem. This may require additional tests and diagnostic procedures to find the underlying cause.
Kidney disease often has no clinical symptoms until the working tissue of the kidney is severely damaged (70-80%).
Everyone needs protein
There are many types of proteins, our bodies use proteins in a variety of ways, including building bones, muscles and other tissues that make up organs, and fighting infections.
When kidney tissue suffers, the kidneys stop functioning normally, thereby allowing a protein called albumin to pass through theirfiltration system into the urine.
Albumin helps the body get rid of excess fluid. With a lack of albumin in the blood, fluid accumulates in the body, causing swelling in the face and lower parts of the body.
Cholesterol as an important component of the body
Our body needs cholesterol, which is produced in it on its own. In addition, cholesterol is also ingested from food. Excessive intake of cholesterol into the blood harms blood vessels, since droplets of this substance stick together on the walls of veins and arteries and can form blood clots (complete or partial blockage of the lumen of the vessel). Blood clots in the vessels impede the work of the heart and the flow of blood to organs and tissues, which can subsequently result in a myocardial infarction or stroke.
Triglycerides are a type of "energy" fat in the blood
When we eat food, our body burns calories from the incoming food to generate energy. If we consume more calories than we expend, the extra calories are converted into triglycerides.
Triglycerides are stored in adipose tissue, in case of emergency they are used as energy to maintain normal cell activity. The presence of high levels of triglycerides in the blood indicates a high predisposition to heart disease.
Who is prone to nephrotic syndrome?
People of all ages, genders and ethnic groups may be prone to this pathology, but according to the Russian Ministry of He alth(MoH), more common in men than women.
Nephrotic syndrome in children usually appears between the ages of 2 and 6.
Some factors increase the likelihood of progressive kidney disease, these include:
- Nephropathology (glomerulonephritis, nephrolithiasis, etc.).
- Urolithiasis - urolithiasis.
- Long-term use of medications such as non-steroidal anti-inflammatory drugs (NSAIDs) and antibiotics.
- Infections: HIV, viral hepatitis, malaria.
- Diabetes, lupus and amyloidosis.
Etiological factors (causes)
The syndrome can be caused by various kidney diseases or other factors.
If the disease affects only the kidneys, then they are called the primary causes of nephrotic syndrome. Other factors that affect the entire body, including the kidneys, are called secondary causes.
Most people suffer from advanced kidney disease due to secondary causes.
The most common primary cause in adults is a condition called focal segmental glomerulosclerosis (FSGS). FSGS causes microscopic scarring on the kidney filters called glomeruli.
Various autoimmune diseases and chronic immune diseases can seriously damage the kidneys.
Amyloidosis is a genetically determined disease in which there is an accumulation of a protein substance called amyloid in the blood. It is deposited on the walls of blood vesselsvarious organs, including the kidneys.
The most common secondary factor in adults is diabetes. The pathology is accompanied by a kidney disease known as renal (renal) diabetes.
The most common primary cause of renal syndrome in children is minimal change disease (MCD). The minimally altered disease causes hidden damage to the kidneys that can only be seen with a very powerful microscope.
The most common secondary factor in children is diabetes.
In all forms, the hallmark unifying feature of this disease is the progressive destruction of the glomeruli.
Diseases of the kidneys that affect the tubules and interstitium, such as interstitial nephritis, do not cause nephrotic syndrome.
Diagnostic capabilities
- The Estimated Glomerular Filtration (eGFR) blood test is a rapid test to assess kidney function. Your eGFR is a number based on an analysis of your serum creatinine and urea levels. Primary urine is formed by filtering blood plasma across the glomerular barrier; in humans, the glomerular filtration rate (GFR) is 125 ml/min.
- Clinical urine test. With severe kidney damage, a large amount of protein passes into the urine. This may be one of the earliest signs of nephrotic kidney syndrome. To check for the presence of protein in the urine (so-called proteinuria), it is necessary to pass a general urinalysis withsediment microscopy. The physiological value of plasma albumin is 0.1%, which normally can pass through the glomerular filtration barrier.
- Ultrasound examination of the kidneys and bladder for the diagnosis of nephrotic syndrome. Allows you to assess the morphological (structural) state of the kidneys and blood circulation. Ultrasound will also help identify concomitant pathologies of the urinary system.
You can suspect kidney pathology after an express urine test with a test strip. A high proteinuria reference value will cause the test strip to change color.
A clinical blood test showing low levels of a serum protein called albumin will confirm the diagnosis.
In some cases, when the prescribed treatment is ineffective, a kidney biopsy will be ordered. To do this, a very small sample of kidney tissue is removed with a needle and viewed under a microscope.
Renal proteinuria is the loss of three or more grams of protein per day through the urine or, in a single urine collection, the presence of 2 grams of protein per gram of urine creatinine.
Nephrotic syndrome is characterized by a combination of nephrotic range proteinuria with serum hypoalbuminemia and edema of the facial space and lower parts of the body.
Complicating factors of renal syndrome
Proteins perform many different functions. When serum (blood) protein levels are low, the body becomes predisposed to problems with blood clotting and to developinginfections (considering that the protein fraction of the blood includes immunoglobulins - the main cells of the immune system).
The most common bacterial and viral complications are acute sepsis, pneumonia and peritonitis.
Venous thrombosis and pulmonary embolism (PE) are well-known sequelae of acute nephrotic syndrome.
Other complications include:
- Anemia (anemia).
- Cardiomyopathy, including ischemia.
- High blood pressure - systemic hypertension.
- Chronic edema.
- Acute and chronic renal failure (ARF, CRF).
Therapeutic options for kidney disease
There is no specific treatment for nephrotic syndrome, all treatments are usually only symptomatic (relieve symptoms and complications) and preventive (prevent further destruction of kidney tissue).
It is important to know that complete kidney failure (end-stage renal disease) requires dialysis and a further kidney transplant to save life.
The attending physician prescribes medicines to relieve certain symptoms. These may include drugs to control hypertension and cholesterol to reduce the risk of heart disease.
Hypertension-lowering drugs called ACE (angiotensin-converting enzyme) inhibitors and ARBs (angiotensin II receptor blockers), which lower capillary pressure andprevent the release of protein into the urine.
Diuretics are prescribed to help the body get rid of excess water, as well as to control blood pressure and reduce swelling.
Blood-thinning medicines (anticoagulants) are recommended in cases of risk of blood clots to prevent heart attack (myocardial infarction) and stroke.
Change in diet plays a very important role in treatment; A low-fat diet helps control blood cholesterol levels. Choose fish or lean meats.
Limit your s alt (sodium chloride) intake to reduce swelling and keep your blood pressure at a he althy level.
Immunosuppressive drugs suppress the excessive response of the immune system, with glomerulonephritis and systemic lupus erythematosus, such as glucocorticosteroids (Prednisolone, Decortin, Medopred, etc.).
How to prevent progressive kidney damage?
The only way to prevent this syndrome is to prevent diseases that can cause it.
If you have a disease that can harm your kidneys, consult your doctor to develop clinical guidelines for nephrotic syndrome to control your underlying disease and prevent kidney damage.
Also talk to your doctor about specific kidney function tests.
This is very importantfor people with diabetes, high blood pressure, or a family history of kidney disease. Damage to the kidneys is always irreversible, their cells do not recover after death. But if you have an underlying disease detected at an early stage and timely treatment is prescribed, then there is a chance to prevent the condition from worsening.
Kidney pathology in children
Although nephrotic syndrome can affect people of any age, it is usually first diagnosed in children between the ages of 2 and 5.
Pathology affects more boys than girls. Every year, about 50,000 children are diagnosed with glomerulonephritis with nephrotic syndrome. It tends to be more common in families with a history of kidney or autoimmune disease, or in the Asian diaspora, although it is not yet clear why.
Symptoms of kidney disease in children
Like adults, swelling is first seen around the eyes, then in the lower legs and the rest of the body.
Immunoglobulins are antibodies that are a specialized group of proteins in the blood that fight infections. When the body loses proteins, children are much more likely to develop an infectious disease.
There are changes in the urine - sometimes high levels of protein in the urine cause it to become frothy.
Most children with nephrotic syndrome have "minimal change disease." This means that their kidneys appear to be normal or near normal on tests until a tissue sample obtained from a biopsynot examined under a microscope. The cause of the disease with minimal change is unknown.
In Finnish-type hereditary nephrotic syndrome, the gene for nephrin, a filter gap protein, mutates, leading to kidney disease in infancy.
It also occurs as a result of kidney problems or other conditions such as:
- glomerulosclerosis - when the internal structure of the kidneys becomes damaged;
- glomerulonephritis - inflammation in the filtration system of the kidneys;
- infections such as HIV or hepatitis B and C;
- systemic lupus erythematosus;
- diabetes;
- sickle cell anemia;
- very rare cases of some cancers such as leukemia, multiple myeloma or lymphoma.
But these problems are more common in adults than in children.
Symptoms of nephrotic syndrome in children can be controlled with steroid medications.
Most children respond well to steroids and the risk of kidney failure is minimized. However, a small number of children have (inherited) congenital nephrotic syndrome, and it tends to be more difficult to treat. Ultimately, their syndrome ends in chronic renal failure, and such children require a kidney transplant.
In most children who respond positively to therapy, the symptoms are controlled, there is a remission - a temporary suspension of the development of the disease, then after a while the symptoms return again - a relapse occurs.
BIn most cases, relapses become less frequent as children get older, and nephrotic syndrome often resolves during adolescence.
Pathological Condition Control
Parents should take their child to a specialist (pediatric nephrologist) for advice on nephrotic syndrome, testing and special treatment.
The main treatment is steroids (glucocorticosteroids), but additional treatments can also be used if the child develops significant side effects.
Most children relapse before late adolescence and require steroids during these periods.
Children with congenital nephrotic syndrome are usually given at least a 4-week course of prednisolone followed by a reduced dose every other day for an additional four weeks. This prevents proteinuria.
When Prednisolone is given for short periods of time, there are usually no serious or long-term side effects, although some children experience:
- increased appetite;
- weight gain;
- facial redness;
- frequent mood swings.
Most children respond well to the treatment of nephrotic syndrome with Prednisolone, with protein often disappearing from their urine and swelling disappearing within a few weeks. During this period, remission occurs.
Diuretics, or diuretics, can also be used to reduce the buildupliquids. They work by increasing the amount of urine produced.
Penicillin is an antibiotic and can be given during relapses to reduce the chance of getting an infection.
Diet food is important. Reduce the amount of s alt in your child's diet to prevent further water retention and edema.
