The world is flooded with a huge number of viruses, bacteria and a variety of diseases. It is simply impossible to know about everyone, however, when making a certain diagnosis, you want to know about your disease in more detail than the doctor can tell at a 15-minute reception. Today we will talk about amyloidosis of the kidneys.
What is kidney amyloidosis?
Amyloidosis of the kidneys is considered a frequent case of a systemic disease - amyloid dystrophy. This pathology begins with metabolic disorders, which leads to excessive formation and accumulation in the organs and tissues of a certain substance, not characteristic of a healthy organism, amyloid.
Amyloid - a product of protein origin, an insoluble glycoprotein, the accumulation of which occurs as a result of a violation of protein metabolism. Amyloid can accumulate in the kidneys, and in any other organs - the stomach, liver, spleen, even in blood vessels and leads to disruption and gradual atrophy of the organ. Quite often amyloidosis affects the kidneys. Postponed in the renal glomeruli, interstitial tissue, vascular and peritubular zone, amyloidosis gradually leads to a complete failure of the kidneys.
According to the statistical data of amyloidosis, middle-aged and elderly people are more likely to suffer. Reveals amyloidosis of the kidneys in approximately 1-2 patients per 100,000 inhabitants.
Types
Depending on the onset of the disease and its structure, several types of amyloidosis of the kidneys are distinguished:
- Primary species( AL amyloidosis).This type of pathology develops without cause, it can affect any organs, including the kidneys. For AL amyloidosis, the appearance in the blood plasma of chains of amyloid immunoglobulins deposited in various tissues. A similar mechanism is observed in myeloma.
- Secondary amyloidosis( AA-type).More common kind. Primarily occurs due to inflammatory lesions of organs, chronic destructive diseases, tuberculosis. Secondary amyloidosis of the kidneys can be a consequence of chronic bowel diseases( ulcerative colitis, Crohn's disease), and as a result of tumor growth. Amyloid type AA is formed from the protein alpha-globulin, synthesized by the liver in the case of a prolonged inflammatory process. Genetic breakdown in the structure of the alpha-globulin protein just leads to the fact that instead of the usual soluble protein, an insoluble amyloid is produced.
- Family hereditary amyloidosis( AF-type).This disease is transmitted through several generations, often found among Jews, Armenians and Arabs. The disease is manifested by fever, pain in the abdomen, various rashes on the skin.
- Amyloidosis in patients on hemodialysis( AH-type).If the artificial kidney is kept for a long time in the blood, the concentration of a certain protein increases, which normally should be excreted by the kidneys. It binds to the plasma nucleoproteins, and then falls into the tissues as an insoluble precipitate, which aggravates the existing situation.
- Tumor amyloidosis( AE-type).It is a complication of some types of tumors.
- Aging amyloidosis. It develops in patients of senile age as a result of age-related changes.
Stages of
In the development of amyloidosis of the kidneys, clinicians distinguish several stages of development:
- latent( hidden);
- proteinuric;
- nephrotic, or edematous;
- is azotemic( uremic or terminal).
Latent stage of the development of the disease is not in vain called hidden. At this stage, signs of amyloidosis in no way manifest, the patient can only worry about the symptoms of the underlying disease( inflammatory processes in the intestine, rheumatism, etc.).The amount of amyloid is so small that it can not lead to any impairment of renal function. The disease does not appear in a standard examination, which includes a blood test and an ultrasound examination( ultrasound) of the kidneys. The latent phase can last from three to five years.
Proteinuric stage, the duration of which can be more than 10 years. This stage is characterized by the deposition of amyloid in different parts and cells of the kidney - in the glomeruli, vessels and intercellular space, as a result of which the main kidney cells - nephrons - begin to change in size and die off. The kidney filter becomes permeable, as a result of which the protein falls out in the urine. In urinalysis, in addition to protein, microhematuria and leukocyturia are observed. Over time, the kidneys increase, their color changes to gray-pink, the density increases.
The next stage of development of amyloid kidney disease is nephrotic. It is also called edematous due to the pronounced edema of the tissues. Edema appears on the face, in the genital area, near the waist. There is a large accumulation of fluid in the abdominal cavity, in the cavity of the heart bag and in the pleural cavity. All this is a consequence of the massive loss of protein, which in large quantities goes along with the urine. Reducing the number of proteins in the blood and is the reason for the accumulation of fluid in the tissues of the body.
For the azotemic stage, the kidneys decrease in size, they become wrinkled, the number of functional cells in them is no more than 25%.This is not enough for the organ to cope with its excretory function, so the concentration of uric acid begins to increase in the blood. The patient suffers from debilitating vomiting, abdominal pain, skin itching, high blood pressure. There is a picture of kidney failure, complications of the disease - pneumonia, thrombosis of renal veins, cardiac hypertrophy can occur.
Pathogenesis of the disease
Despite the fact that the level of development of medicine is quite high, physicians are still puzzling over the mechanism of the disease.
There are several theories explaining the causes of the disease:
- Theory of disproteinosis( organoproteinosis).According to this concept, amyloidosis occurs due to some disturbances in the correct correlation between blood protein fractions. Such a violation is called disproteinemia. Because of this disturbance in the blood plasma begin to accumulate coarsely dispersed protein fractions and paraproteins, which penetrate into the tissues and form there amyloid deposits.
- The theory of local cell genesis explains the formation of amyloidosis by gross violations of the protein-synthetic function of the reticuloendothelial system.
- The immune theory of the formation of amyloidosis suggests that as a result of the development of the underlying disease, the body accumulates toxins and products of tissue decay. They can play the role of autoantigens, and then be transformed into autoantibodies. The interaction of antibodies and antigens leads to the formation of amyloid and deposition of it in those places where there is a large accumulation of antibodies.
- The mutational theory tries to explain the excessive accumulation of insoluble protein by a gene mutation, which results in the formation of a clone of cells - amyloidoblast. They are responsible for the production of amyloid.
On the video of what amyloidosis of kidneys is:
Symptoms of
Amyloidosis is a rather insidious disease. The fact is that the latent stage of the disease does not presage any dangerous signs indicating an anomaly. Poor health and swelling, appearing at later stages, already indicate irreversible changes in the kidneys.
Proteinuria is regarded by specialists as an important sign of amyloidosis of the kidneys. The protein precipitate in the urine, as well as the admixture of blood, are considered dangerous signals indicating a possible failure of the organ.
The disease gradually progresses, resulting in new symptoms in patients:
- increased feeling of fatigue;
- sweating;
- bags under the eyes;
- swelling of the facial tissues;
- high pressure;
- anuria.
Prolonged and massive proteinuria along with hematuria and leukocyturia can be a serious reason to suspect the patient of the development of amyloidosis. After a long period of proteinuria, the nephrotic syndrome begins to develop. Its main manifestation is a strong puffiness, which does not decrease even after taking strong diuretics, appears regardless of the time of day and the position of the patient's body.
In the third stage, in addition to severe nephrotic syndrome, ascites is observed in patients, disorders in the work of the heart, intestine, spleen.
Diagnosis
It is extremely difficult to diagnose "amyloidosis of the kidney" at the latent stage. To suspect such a defeat of the kidneys, the clinician will be helped by prolonged growing proteinuria. Along with the appearance of protein in the urine, an important criterion in the diagnosis can be leukocyturia, especially if there is no obvious inflammatory kidney disease.
In the general analysis of blood there is a persistent increase in ESR already at an early stage of the disease. In a later period there is a drop in hemoglobin and the number of red blood cells.
Biochemical blood test indicates amyloidosis with a significant loss of albumin, elevated cholesterol and lipoprotein levels.
The most effective and reliable method of diagnosis is a biopsy of organs. Tissues taken with a biopsy stain the Congo red and examined under a microscope. Detection of amyloid in tissues will allow the diagnostician not only to make a correct diagnosis, but also to determine the clinical stage of the disease, which will allow choosing the correct treatment tactics.
For the detection of amyloid, it is possible to take a biopsy not only from the affected kidney, but also the mucous membrane of the liver, intestine or gum. Possible further typing of amyloid for the purpose of choosing a treatment regimen.
Treatment of
It should be understood that it is impossible to completely stop the formation and deposition of amyloid in tissues. Therefore, therapy with amyloidosis of the kidneys is aimed at reducing the formation of amyloid and protecting the organs from its harmful effects.
If we are talking about secondary amyloidosis, it will be correct to eliminate the symptoms of the underlying disease. Purulent formations should be removed in a timely manner, inflammatory processes are stopped with the help of anti-inflammatory drugs.
Therapy with cytostatics will help delay the progression of renal failure. With amyloidosis of the kidneys, good results are shown by plant cytostatics, one of which is Kolchicin. This drug helps to reduce protein loss and prevents seizures.
To stop the rapid progress of the disease will help the use of drugs from the group of amincholines( chloroquine or delagila).The use of drugs for several years in the detection of disease in the early stages will help significantly slow the progression of the disease.
It is equally important for patients suffering from amyloidosis of the kidneys to observe a special diet. At the initial stages of amyloidosis, patients showed a decrease in protein food in the diet and an increase in carbohydrate foods, especially fruits and berries rich in vitamins. In particular, the diet should be accompanied by the use of vitamins C, contained in black currant, wild rose broth, strawberries, Bulgarian pepper, and foods rich in potassium - bananas, potatoes in uniform, apricots, raisins, apricots, etc. It is very useful to consume raw liver daily in an amount of at least 80-100 grams, which helps to reduce proteinuria.
Kidney amyloidosis therapy includes the use of desensitizing drugs( dimedrol, suprastin, pilpofen), unitiol, steroidal anti-inflammatory drugs - dexamethasone and prednisolone.
In case of kidney failure, a real rescue for patients can be a transplant of donor organs. However, this treatment will not prevent further development of amyloidosis, but will only increase life expectancy.
Forecasts
For amyloidosis of the kidneys, the steady progression of the disease is a characteristic phenomenon. Survival of patients with this pathology directly depends on the type of disease, the defeat of vital organs - the liver and heart, the development of the underlying disease.
With the expanded clinical picture of amyloidosis, in the event that there is multiple organ dysfunction, most patients die within a year after diagnosis.
The lowest survival rate was observed in patients with AL type of amyloidosis. The average life expectancy of such patients is no more than 13 months. The most frequent causes of death in such patients are heart failure, uremia and sepsis. In this case, chronic renal failure develops in more than 60% of cases.
More favorable is the prognosis for secondary amyloidosis. Life expectancy depends on the nature of the underlying disease and the possibility of its treatment. The cure of the primary disease slows the progression of amyloidosis, but does not exclude the development of the disease in a later period. The average life expectancy of patients with secondary amyloidosis of the kidney is between three and five years.