- primary - cells change in the presence of monoclonal hypermoglobuliomy, myeloma, and Waldenstromic macroglobulomia,
- secondary causes of this type of amyloidosis are inflammatory processes of a chronic nature (for example, rheumatoid arthritis, malaria, leprosy, tuberculosis, osteomyelitis, bronchiectasis),
- idiopathic (familial) - enzymes have defects since childhood, from generation to generation,
- age (senile) - violations begin already in old age, due to various functional failures in the body,
- dialysis - this type develops due to blood purification in acute and chronic kidney disease (their insufficiency) - hemodialysis.
Signs of amyloidosis:
- Gastrointestinal tract: tongue increases in size, swallowing function is disturbed, an upset stomach or, conversely, constipation, there may be deposits in the form of a tumor in the intestine or stomach (this happens very rarely), belching, heaviness in the stomach, nausea after eating,
- cardiovascular system: heart failure, disturbed rhythm of the heartbeat, myocardium,
- CNS: frequent headaches, increased sweating, dizziness, impaired sensitivity of the extremities, tingling of the tips of the fingers and toes, impotence, enuresis, fecal incontinence,
- cartilaginous system: dense swelling of the joints, numbness of the tips of the hands and feet, throbbing pain in the fingers, polyarthritis, periarthritis,
- tissue amyloidosis: enlarged spleen,
- respiratory system: persistent bronchitis, hoarse voice, lung tumors,
- associated symptoms: skin lesions (various nodes, papules, "symptom of glasses" - bruises around the eyes), thyroid disorder, adrenal insufficiency, kidney dysfunction (present in all forms of amyloidosis), anemia, elevated ESR, cholesterol.
Useful products for amyloidosis
Patients with amyloidosis must follow a diet in which the body must be saturated with potassium, starch, and vitamin C.
Foods that will help fill the potassium deficiency:
- vegetables (cucumbers, potatoes, zucchini, legumes, parsnips, rutabaga, carrots, green leafy vegetables),
- honey and its products (especially perga – bee pollen in comb),
- Apple vinegar,
- fresh fruits, berries (watermelons, oranges, melons, bananas,
- dried fruits: dried apricots, raisins, figs, prunes,
- rye flour, wheat bran bread,
- nuts (pine, almond, peanuts),
- wheat and oatmeal,
- animal products (beef, fish, liver (raw), dairy products),
For starchy foods include:
- porridge: buckwheat, oatmeal, millet, wheat, rice (brown), semolina, barley,
- macaroni and bakery products, galetny and oatmeal cookies,
- grain crops (rye, wheat, oats, corn,
- peas and beans,
- root vegetables of horseradish and ginger.
- rose hips, sea buckthorn, black currant, wild garlic, viburnum, mountain ash, strawberry, honeysuckle,
- all kinds of cabbage
- hot and sweet pepper,
- horseradish roots
- garlic greens
Means of traditional medicine for amyloidosis
The most effective folk remedy for the treatment of amyloidosis is considered a long course of taking a raw liver (100 grams per day). The course of treatment should be long and lasts a year and a half. Thanks to the vitamins of groups A, B, C, E, glycogen, carotene, niacin, biotin contained in the raw liver, it helps restore the functioning of the kidneys, heart, nervous and digestive systems.
Also, one should not neglect treatment with the help of various herbs and fees from:
- 1 camomile pharmaceutical, immortelle, birch buds, St. John's wort,
- 2 to clean the blood will help decoction of nettle (you can cook both from leaves and flowers),
- 3 have a cleansing effect on the blood and juniper berries (they should be eaten with 5 pieces, add one berry each day, bring to 15 berries),
- 4 a good heart remedy is green oats (grass), you can drink in the form of juice, decoction, tinctures,
- 5 healing properties for amyloidosis have tea made from dry leaves and berries of strawberries or raspberries, currants, rowan, mint and St. John's wort (2 grams of each available ingredient are needed, which need to be poured with hot water and allowed to brew for 10 minutes, the portion is calculated for one a cup / glass).
Dangerous and harmful products for amyloidosis
Potassium washed out pastries and various sweets, caffeinated products and alcoholic beverages. Also, potassium leaves the body due to excessive physical exertion and stress.
- egg whites,
- soy milk
- low-fat veal and beef,
- rabbit meat, chicken,
In small quantities, starch is found in pumpkin, carrots, tomatoes, garlic, asparagus, cabbage, radishes, parsley. Therefore, the emphasis on these products is not worth doing.
It is necessary to limit the consumption of table salt and salty food (especially for people with a sick heart, kidneys).
What is kidney amyloidosis?
Modern technology allows you to diagnose diseases, the presence of which in the body, people do not even realize. It turns out that humanity itself is looking for adventure. This is not true.
Take, for example, kidney amyloidosis. This disease is difficult to detect in its early stages; when diagnosed without special laboratory tests, it is often confused with other diseases, including urological ones. As a result, treatment of kidney amyloidosis is not performed, at best, the patient’s well-being does not deteriorate if he follows a diet. Discuss the situation with the appointment of antibiotics or other drugs is not necessary.
Meanwhile, it is kidney amyloidosis that leads to renal failure.
What is amyloid?
To touch on the topic of what is amyloid, will have a pretty deep. This is done to understand the seriousness of the situation and possible ways out of it. In addition, the disease itself raises many questions that can not be answered without basic knowledge.
Many mistakenly call amyloid starch, but it would be more correct to say that it is very similar to starch, especially under the action of various dyes. Amyloid is defined as a modified protein substance that is deposited in the body by polysaccharide chains.
Amyloid can accumulate in various organs:
and also on the skin.
Its formations are able to multiply through the body, displacing substances that must be contained in the space occupied by it. This leads to organ dysfunction and often to its complete loss.
In inflammatory processes, injuries, tuberculosis, the content of this substance in the blood increases dramatically. And although the substance itself has been studied in some detail, the reasons for its formation in the human body have not yet been studied enough.
Symptoms of amyloidosis
The disease is characterized mainly by 4 stages of flow:
The latent stage of the disease is dangerous because it proceeds like various infectious diseases. Amyloid in the blood is elevated, but rarely detected on time. Symptoms of rheumatic diseases may occur. The duration of this stage is 3-5 years.
The second stage (proteinuric) amyloidosis of the kidney is manifested in tests in the form of protein in the urine, elevated levels of ESR. Already lymphoid thickening of the skin can be observed, accompanied by various inflammatory processes, swelling of the hands, more often of the legs, and these are the first obvious signs of amyloid disease. An ultrasound should show an increase in the kidneys, their color is gray-pink.
Nephrotic stage occurs with obvious and frequent edema. Blood pressure is normal, but more often low. In the urine, the constant presence of protein, and in the blood plasma, on the contrary, low, elevated cholesterol levels, show resistance to diuretin treatment.
The symptoms of the fourth, azotemic stage on ultrasound are manifested in the reduction of the kidneys to a shriveled appearance; they can be covered with scars. Doctors call this condition amyloid kidney. Edema is observed at this stage of the disease. Often manifested painful anuria. Often the patient is lost due to the onset of azotemic uremia. In fact, there is a poisoning of the body with products of its vital activity.
Diagnosis and testing
As already noted, in order to diagnose amyloidosis in the kidneys, conduct clinical studies:
Regardless of whether amyloidosis, secondary or primary, is observed, an increase in the amount of protein is clearly expressed in the urine. But in the initial stages, the disease is not even diagnosed as simple pyelonephritis.
Performing an ELISA blood test, observe an increase in alkaline phosphatase activity. In parallel, they note leukocytosis, hypocalcemia, and hypercholesterolemia.
- Advanced feces analysis shows increased stool fat content, the presence of muscle fibers and starch.
- An ECG is also assigned, which often results in arrhythmia and diastolic dysfunction.
- The patient must undergo an abdominal ultrasound scan. Thyroid function may also be impaired.
- But the most important analysis for amyloidosis, which uniquely removes questions about the final diagnosis, is a kidney biopsy.
- If the observed kidney amyloidosis is secondary, then it is necessary to concentrate on identifying the underlying disease and its treatment.
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Traditional treatment and traditional medicine
Since the process of accumulation of abnormal proteins in the human body is still unexplored, the possible treatment may vary significantly depending on the doctoral school, ethical understanding of the problem. The doctor prescribes the treatment in accordance with the results of the tests, and they may differ significantly, even if the same diagnosis is made.
First, all forces are sent to stop the process, while therapeutic measures are appointed. When kidney amyloidosis requires a very specific diet, for example, a raw liver, which is not tolerated by all patients. Light heat treatment is allowed, but if this does not help, injections are prescribed that can replace this moment of the diet. The use of modern drugs can slow down the accumulation of abnormal protein in the kidneys. At this stage, there may be preparation for surgical treatment.
Although kidney amyloidosis often attempts to prescribe diuretics, it often turns out that the patient is opposed to their intervention, repulsing the influence of these drugs, just like antibiotics. However, this method of influencing the disease takes place in medical practice.
Depending on how the kidneys look in the diagnosis (ultrasound), the doctor prescribes a macropreparation or a microscopic preparation, since the appearance of the amyloid kidney can vary significantly at different stages of the disease.
After the spread of the disease can be stopped, they proceed to the restoration of the affected organ. In addition to the kidneys, adjacent organs may be affected. Here surgical treatment, physiotherapy, special massage can be prescribed, it is fixed by the whole diet. Recommended therapeutic exercises.
So that the treatment does not turn out to be meaningless, patients are recommended to undergo prophylactic procedures in boarding houses and visit a doctor in the terms established by a specialist.
But you can not neglect the methods of treatment of traditional medicine. Protein products should be excluded from food. Oddly enough, saturate the food with starch and potassium.
It is recommended to undergo a course of purification of the blood, liver, since this organ is always affected by the course of this disease. Used broths nettle, chamomile. You can approach the problem in a complex, using the principle of collection 5, in which in a particular case should be included in equal shares:
A comprehensive approach to the treatment of kidney amyloidosis, which does not preclude the use of medicinal herbs, is welcomed.
Preventive measures against amyloidosis
Talking about previous measures of kidney disease, referring to amyloidosis, is extremely difficult.
If there is amyloidosis of the kidney of a secondary nature, then prevention will be provided by the treatment of the primary disease. Already during the course of amyloidosis of the kidney, recovery in a particular case depends on how quickly the primary disease can be overcome, to stop its spread.
If the patient faces a familial version of this disease, then a specific diet throughout the life course can serve as prevention.
Primary amyloidosis, perhaps, has no specific preventive measures. In addition to the timely visit to the doctor when there is pain in the kidneys, attentive attitude to the infectious diseases.
The fulfillment of the doctor's prescriptions, the observance of the daily regimen, gymnastics in the morning, the right balanced diet - this is what is required to comply with for prevention.
Summing up, it is necessary to note once again how important it is to come to the doctor and undergo the necessary research on time. After all, the first stage of the disease is treatable, not only in a medical way, but also by following a certain diet.
Symptoms and treatment of kidney amyloidosis
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Among the diseases of the urinary system, renal amyloidosis occupies a special place. The phenomenon is characterized by the deposition in the parenchyma of the kidneys of an insoluble protein, amyloid, large aggregations of which disrupt the functioning of the organ. At the initial stages of development, the symptoms are not expressed and often the pathology is found after the formation of renal failure.
The accumulation of protein and its seal on the kidneys is fraught with impaired organ functions.
As a result of a metabolic disorder in the body, excessive formation and accumulation of amyloid organ tissues begins. The presence of this substance is in itself a pathology, since it is absent in a healthy organism. Once in the tissue, amyloid quickly grows and fills a lot of space, because of which the substances necessary tissues are forced out, dystrophy of the organ occurs. Therefore, this phenomenon is called amyloid dystrophy. The efficiency of the kidneys decreases and then stops, which leads to their loss. Pathology is considered rare and accounts for 2% of the total number of kidney diseases. There are two cases per 100 thousand people.
Amyloidosis provokes the occurrence of severe complications and is a dangerous disease for both human health and life in general. The level of amyloid in the blood sometimes increases with the presence of associated pathologies: tuberculosis, syphilis. Inflammation of any tissue also contributes to its formation. At the same time it is able to affect the liver and blood vessels. Scientists managed to study the structure of this substance, but the factors of occurrence have not yet been fully established.
Classification of forms
Amyloidosis has several forms that differ in the factors of the development of pathology:
Features of the structure of amyloid divide the pathology into several types:
Causes of kidney amyloidosis
There is a high probability of developing amyloidosis in men over 50 years old, in people suffering from tuberculosis, inflammatory processes in the joints, as well as in the case of systematic use of hemodialysis (extrarenal cleaning of the blood) or during a bone marrow tumor.
The reasons for the formation of this rare pathology are still being studied, but a number of factors affecting the occurrence of amyloidosis are known. It has been established that the chronic inflammatory process caused by infection is a catalyst for this disease. These include tuberculosis, osteomyelitis and other joint inflammations, syphilis, malaria, myeloma (a bone marrow tumor), and malignant neoplasms in the lymphoid tissue.
The occurrence of amyloidosis is due to the pathology of protein metabolism at the cellular level, which is accompanied by a violation of the quantity and quality of protein in the plasma (liquid part) of the blood. During clinical experiments, it was found that amyloidosis has an autoimmune nature, i.e., the immune system “makes a mistake” amid prolonged inflammation and protein synthesis is distorted. As a result, the protein acquires the signs of an antigen (a substance with signs of foreign information) and antibodies begin to be produced to combat it. The association of antigens and antibodies forms an amyloid substance in tissues.
To explain the mechanism of the onset and development of the disease, there are a number of scientific theories:
Some pituitary hormones contribute to the formation of amyloid.
Protein "clogging" of the kidneys is accompanied by back pain, swelling, painful urination.
Deposition in the parenchyma of the kidneys of amyloid is complicated by damage to the organ, functional failure. The symptoms of amyloidosis of the kidneys, regardless of the form, are combined with the standard symptoms of kidney disease (pain, impaired urination, intoxication, etc.). Often there are no manifestations of pathology until the onset of the acute phase of renal failure.
Primary renal amyloidosis occurs in people under 40 years of age, progresses rapidly, and often ends in death. The secondary type is diagnosed in people aged about 60 years and is accompanied by symptoms of the diseases that caused amyloidosis. There are stages of amyloidosis of the kidneys, describing the symptoms of the disease in accordance with the level of its development.
Preclinical (latent) stage
The accumulation of amyloid is not critical, functional disorders of the kidneys are not observed. Symptoms of pathology coincide with the symptoms of pathologies that led to the accumulation of insoluble protein. These include functional disorders of the lungs in tuberculosis, bone damage in osteomyelitis or arthritis, etc. It is not possible to diagnose abnormalities using a hardware examination (MRI, X-ray). The duration of the stage is not more than 5 years.
Signs of protinuric (albuminuric) stage
The accumulation of amyloid in the renal parenchyma reaches the level at which its permeability increases and protein from the blood enters the urine. This is detected with the help of a laboratory analysis of urine on planned medical examinations. Protein in the urine gradually increases, because the process of kidney damage can not be reversed, the condition gradually worsens. The developmental stage of the stage is about 10–15 years.
The clinical picture of the nephrotic (edematous) stage
The nephrotic stage of amyloidosis of the kidneys provokes a loss of weight, appetite, strength in the patient, the pressure is disturbed, nausea and emetic desires appear.
There is a pronounced symptoms. In the blood, the protein level is lowered due to its active elimination with urine. There is a sharp weight loss, the patient complains of fatigue, nausea, thirst. Appetite is getting worse. Lowering blood pressure with a sharp change in body position from horizontal to vertical provokes syncope. The amyloid accumulated in the intestinal tissues affects the nerve endings, which causes diarrhea. Appear and progress edema. Immediately, the legs and face swell, and then shortness of breath appears, since fluid is collected in the lungs. There are also accumulations of fluid in the pleura, in the pericardium (pericardial bag). The urination is broken up to the complete absence. As a result of accumulation of amyloid, the liver, spleen, lymph nodes are enlarged.
Symptoms of uremic (azothermic, terminal) stage of amyloidosis
The final stage. Renal amyloid does not allow the organs to function, they are modified. Renal failure is detected. Observed severe swelling, intoxication, fever, lack of urination. HELL lowered, the pulse is weak. Hemodialysis is recommended (hardware blood purification). Unfortunately, it does not completely release the body from amyloid. The probability of death is high, because a number of vital organs are also affected.
Diagnosis of kidney amyloidosis involves a series of tests:
What can be seen on the ultrasound?
Ultrasound examines the state of all the internal organs, fixes their size and structure, and disrupts their functioning. This information is necessary to determine the degree of pathology. Ultrasound reveals:
Sometimes heart disease is diagnosed earlier. Amyloid sediment in the myocardium (heart muscle) causes heart failure. Cardiac muscle increases significantly, the valves are disturbed. The functioning of the body is disturbed, there is stagnation of blood. There is a number of heart diseases that can lead to death, blood pressure is consistently low.
Normalize the protein balance in the kidneys by following a special diet and taking aminoquinolines.
Treatment of kidney amyloidosis involves nutritional correction, a diet is prescribed. For 1.5–2 years, you need to eat 80–120 mg of raw liver every day. Products containing protein and salt, from the diet to eliminate or minimize their use, especially if diagnosed deficiency. Most of the diet should be carbohydrates. At the same time, it is important for the body to obtain vitamins, especially vitamin C and potassium salts.
To restore the metabolism, the correction of the internal organs, improve immunity in the treatment of amyloidosis prescribe the use of drugs such as "Delagil", "Rezokhin", "Hingamin." Suprastin, diphenhydramine, pipolfen are recommended to alleviate the symptoms of allergy. Symptoms of the disease are eliminated with the help of diuretic drugs, drug-induced pressure reduction, plasma transfusions, etc. Treatment of the disease is possible only under the supervision of a doctor, and in the later stages in the hospital.
Prognosis and prevention
Amyloidosis is a steadily progressive pathology. Life expectancy with such a diagnosis depends on the state of all internal organs that could simultaneously suffer (heart, liver). With AL amyloidosis, patients do not live longer than 13 months. Despite the fact that this type of pathology is characterized by the presence of kidney failure, heart failure and blood infection leads to death.
AA-amyloidosis has more favorable projections. The determining factor is the underlying disease that provoked this pathology. With successful therapy, amyloidosis regresses first (development slows down). Life expectancy in this case ranges from 3–5 years. To prevent the development of pathology should be promptly treated inflammatory processes in the body, periodically undergo ultrasound to monitor the state of internal organs. Early diagnosis of amyloidosis contributes to effective therapy.
How to treat kidney amyloidosis?
Amyloidosis treatment should be aimed at reducing the production, delivery and accumulation of proteins - precursors of amyloid in the serum. If amyloid deposits are not common, do not pose a threat to life and cause little clinical manifestations (senile amyloidosis, skin and some types of localized tumor form), therapeutic interventions are not necessary and are carried out depending on the specific situation.
Therapy is obligatory in cases when the amyloid deposits become generalized, and the progression of the disease leads to severe impairment of the functions of the kidneys, the heart, the digestive tract, the peripheral nervous system. With sufficient nitrogen-excreting kidney function, patients with amyloidosis should adhere to a gentle regimen (physical exercise restriction, semi-bed rest), and diet.
Parenteral administration of blood or erythrocyte mass in case of anemia, correction of impaired electrolyte balance, careful administration of diuretic and antihypertensive drugs, and the use of appropriate drugs are recommended during the period of renal failure.
However, neither a rational diet, nor the use of modern active diuretic and antihypertensive drugs of different mechanisms of action can significantly suspend the progression of amyloidosis with the development of renal failure.
The use of so-called hepatic therapy, which with a certain conditionality can be called pathogenetic, is promising. A daily intake of 80-120 g of raw liver or liver preparations should be considered as optimal (a cream of 4-5 ml intramuscularly for a long time). The main ways of pathogenetic treatment:
Important is the active treatment of the underlying disease, in which amyloidosis develops. This applies mainly to secondary amyloidosis in chronic infections and purulent processes. The question of the effect of corticosteroids on the course of amyloidosis is not fully clarified. There is evidence to accelerate the development of amyloidosis under the action of conventional doses of ACTT, cortisone and prednisolone. This gives grounds to consider amyloidosis and the nephrotic syndrome caused by it, rather as contraindications to steroid therapy.
It is advisable to use melphalan, which suppresses the function of some clones of cells, in particular those that synthesize the light chains of immunoglobulins involved in the formation of amyloid fiber. Under the influence of melphalan (0.25 mg per 1 kg of body weight per day) proteinuria disappears, although this preparation has no significant effect on the further course of primary amyloidosis.
The specific treatment of amyloidosis include the use of colchicine. The mechanism of action of this drug has not been fully elucidated yet, it is suggested that it inhibits protein synthesis by hepatocytes. With periodic illness, colchicine is able to prevent its attacks. With kidney amyloidosis, it reduces proteinuria and, when treatment is started in a timely manner, it can completely cure nephrotic syndrome. The drug can cause nausea, vomiting, diarrhea, hair loss, leukopenia, thrombocytopenia, skin rashes.
In case of amyloid nephropathy, unitiol (sodium dimercaptopropanesulfonate) is used. The drug binds sulfhydryl groups of proteins - precursors of amyloid, preventing the formation of amyloid fibrils. Unithiol in complex treatment can slow down and stabilize the development of secondary amyloidosis of the kidneys. The side effects of its use include nausea, dizziness and tachycardia, so the dose should be increased gradually.
For the treatment of secondary amyloidosis, occurring against the background of rheumatoid arthritis, the anti-inflammatory drug dimexide is also used, which has been used as an external agent for a long time.
In the treatment of amylodosis, as well as myeloma, various polychemotherapy regimens are used to reduce the production of amyloid precursors - light chains of immunoglobulins.The progression of amyloidosis delayed immunostimulatory drugs (thymosin, levamisole), drugs increase the T-suppressor function of lymphocytes. Medications with a pronounced tropism for tissue cell elements include preparations of the aminoquinoline series (hingamin, resoquine, chloroquine, delagil, plaquenil), which inhibit the synthesis of nucleic acids, the activity of some enzymes, the formation of acidic mucopolysaccharides, the stabilization of lysosomal membranes. These drugs can affect the processes that play an important role, on the one hand, in the synthesis of amyloid fiber, on the other - in creating the main substance in which these fibrils are located.
Symptomatic treatment does not depend on the type of amyloidosis, but on the nature of the target organ damage. Modern transplantation and extracorporal methods essentially introduce changes in the treatment of systemic amyloidosis, in particular, in case of amyloid nephropathy at the stage of renal failure.
What diseases can be associated with
Arterial hypertension develops in 20-25% of patients, mainly in the presence of prolonged amyloidosis. Among the associated tubular dysfunctions, canalic acidosis and renal diabetes are observed. On the background of amyloidosis of the kidneys, thrombosis of the renal veins may develop.
Renal failure may develop and be fatal in the proteinuric, nephrotic, and even latent stage. The most common cause of its rapid development is the exacerbation of the underlying disease, the addition of an infection, or a complication such as renal vein thrombosis.
A feature of renal amyloidosis is the preservation of nephrotic syndrome and the large size of the kidneys during the development of chronic renal failure. A slight urinary sediment is also characteristic. Hematuria is rare.
The development of renal failure, arterial hypertension, the addition of complications or the exacerbation of the disease, in which amyloidosis has developed, make the complaints of patients with amyloidosis very diverse.
Damage to the cardiovascular system. observed in all patients. Vessels of any caliber can be involved in the process - both arterial and venous. Prolonged arterial hypertension leads to lesion of the left heart with the development of heart failure. Heart failure is typical of primary generalized amyloidosis, and heart failure often becomes the immediate cause of death.
Lung damage is diagnosed in half of patients with amyloidosis. It manifests itself shortness of breath, hemoptysis, hemorrhagic heart attacks, recurrent pneumonia. pulmonary insufficiency, development of the picture of fibrous alveolitis and alveolar capillary block. The combination of pulmonary and cardiac insufficiency complicates the picture of the disease and the assessment of pulmonary pathology, however, progressive dyspnea, recurrent pneumonia, along with other clinical signs, suggest that amyloidosis of the lungs is suspected.
Most patients have changes in the digestive tract: abdominal pain, constipation, alternating diarrhea, flatulence. vomiting, nausea, intestinal atony and stomach, etc. The digestive tract in amyloidosis affects all over.
Macroglossia. what happens with a frequency of 22% is a very characteristic symptom, it is sometimes the main problem for patients. The syndrome of malabsorption occurs not so much due to the damage of the villi, but rather from a violation of innervation.
Quite often, the liver is involved in the process, which is manifested by hepatomegaly, increased serum alkaline phosphatase activity and other symptoms of cholestasis (centrolobular amyloid deposits), rarely, at the developed stage, portal hypertension syndrome, bleeding from esophageal varicose veins. Often find splenomegaly, which is accompanied by functional hyposplenism.
Often, organ damage of the reticuloendothelial system is observed. A marked increase in lymph nodes usually gives grounds to suspect lymphogranulomatosis, sarcoidosis, tuberculosis, however, the probability of amyloid genesis and an increase in lymph nodes should be taken into account.
The involvement of the peripheral and autonomic (autonomous) nervous system, rarely the central nervous system is noted in systemic amyloidosis of various types. Neurological symptoms characteristic of individual forms of hereditary and primary amyloidosis, with secondary amyloidosis, may appear in the terminal (uremic) stage of the disease. Mental disorders in the form of dementia are noted in local forms of amyloidosis (Alzheimer's disease).
It is believed that a possible connection in the development of amyloidosis, atherosclerosis and diabetes mellitus due to similar metabolic disorders.
What doctors to contact if you have kidney amyloidosis
Laboratory indicators in primary amyloidosis are not specific:
Proteinuria ranges from microalbuminemia to massive proteinuria that accompanies nephrotic syndrome. Hematuria is rare, leukocyturia is not massive and is not associated with co-infection (minor changes in urinary sediment).
With liver damage, hypercholesterolemia is evident, in some patients hyperbilirubinemia, an increase in the activity of alkaline phosphaiase.
Coprological examination reveals pronounced amilorrhea and creators.
From instrumental studies are used:
The achievement of recent years is the introduction into clinical practice of the method of scintigraphy with labeled serum p-component to assess the entire distribution of amyloid in the body. In assessing the severity of systemic amyloidosis, visualization methods play an important role, especially ultrasound. They allow you to identify hepato-and splenomegaly, to identify pronounced changes in the form of hyperechoic sites in the walls of the intestine or thyroid gland.
It should be borne in mind the probability of a combination of renal amyloidosis with other diseases (glomerulonephritis, pyelonephritis), as well as relatively frequent renal vascular thrombosis, due to the deposition of amyloid masses and hypercoagulation. For the differential diagnosis should use the complex of all modern methods of research, including morphological study of tissue obtained by a biopsy of the kidney.
Types of amyloidosis
- AL - amyloidosis - the primary type. The consequence of the accumulation in the blood of abnormal immunoglobulin chains
- AA - amyloidosis - secondary type. It arises against the background of protracted diseases,
- AF - amyloidosis - a type of hereditary disease (transmitted by an autosomal recessive mode of inheritance,
- AH - amyloidosis - a consequence of immunoglobulin nonfiltration. The accumulation of immunoglobulins in tissue.
- AE - amyloidosis - is formed in the thyroid gland.
- The Finnish form of amyloidosis is a gene mutation. The kidneys are mainly affected, in some cases it affects the spleen, intestines, stomach.
Causes of disease
The reasons are a violation of protein metabolism. The accumulation of amyloid leads to the displacement of enzymes, the functioning of organs, which leads to their death.
Amyloidosis occurs due to the transmission of infectious diseases - tuberculosis, arthritis, syphilis, osteomyelitis, inflammatory processes. Amyloids can mutate in the body, which causes the development of a hereditary form of the disease.
This is a rare disease, the risk group is males - from 40 years.
Symptoms of the disease
All symptoms are identical in manifestation:
- body weight decreases
- feeling of fast fatigue
- depressive states, states of aggression
- enlarged liver
In the case of the discovery of such a series of symptoms, you should immediately go to a specialist. Amyloidosis is diagnosed by biopsy, X-rays, echocardia, feces, blood and urine.
Organic lesions with amyloidosis lead to deterioration of certain body systems. If urinary tracts are affected - the appearance of kidney failure, skin lesions - leads to wrinkling and redness of the skin, and lymph nodes.
From the digestive system:
- difficulty swallowing
- language increase
- enlarged liver
Hitting the nervous system leads to dementia.
With sediment in the airways:
- shortness of breath
- difficulty breathing
- hoarseness in voice
Skin lesions. The appearance of papules, plaques, nodes, hemorrhages near the eyes.
Since the cardiovascular system - arrhythmia, swelling in the heart, an increase. In the case of cardiac arrest - fatal.
Hepatic amyloidosis is quite common. The liver increases and thickens, the occurrence of pain in the right side, nausea, belching.
Amyloid tendon and cartilage
It hurts in the fingers, swelling of the joints.
It is much more dangerous when the disease is without symptoms, most often it leads to sudden death.
The disease is diagnosed: anemia, leukocytosis, in the eve of the disease, a decrease in the protein level, hyperglobulinemia, the platelet level in the blood, calcium and sodium are reduced.
The functional ability of the thyroid decreases. In half of the cases of detection of the disease, kidney failure first occurs. In the urine, the detection of cylinders, an increased number of red blood cells and white blood cells.
To reduce amyloid in the blood can be surgically. The spleen is removed, after which the patient's general condition improves, as the level of the substance in the blood plasma falls.
Predict amyloidosis: on average the course is from 12 to 14 months, then death occurs. Longer life expectancy for women. Older people, worse suffer the disease. Today, to overcome the disease is beyond the power of medicine. Therefore, attention is paid to the treatment of groups of related diseases. It is highly controlled symptoms and complications, this leads to an improvement in the course of the disease.
Chemotherapy improves and slows down the symptoms and the course of the disease.
In cases of AL-amyloidosis and AF-amyloidosis, undifferentiated stem cells transplant in the body. The patient receives these cells from the organs of blood donation and blood transfusion.
Protein production in amyloidosis is inhibited by groups of corticosteroids and immunosuppressants.
Treatment of amyloidosis disease
In the early stages of treatment, AA-amyloidosis carries a positive prognosis. In the case of complete disappearance of the underlying disease, the manifestation of amyloidosis disappears. Consequently - its course is directly proportional to the nature of the underlying disease, the degree of complication, the quality of treatment. An unfavorable course of the disease most often ends in death, from exhaustion, of heart and kidney failure. Recommended adherence to dietary diet. It should be consumed in the liver raw. Periodically, for a long time, you should go to the doctor-oculist and monitor the blood leukocyte count.
Treatment in the amyloidosis complex is a therapeutically long period. Every day, eating raw liver in an amount of about 100 grams is a prophylactic and therapeutic method.
As you know, in this disease, the function of the most important systems of the body, such as the digestive, nervous, cardiovascular, suffers. The use of the liver improves and improves the function of diseased organs.
For efficiency, it is worth consuming the liver for about one and a half to two years.
In addition to the therapeutic effects of this disease, it is necessary to observe a strict diet regime, that is, diet. Patients who suffer from chronic types of renal and heart failure should reduce the use of protein foods and salt. Better in the diet add starch. It is found in foods such as bread, flour, potatoes, cereals. Lean on vitamin C intake. Potassium salts should be added to the diet.
Vitamin C contributes to the formation of collagen. It participates in the process of development and repair of cells of woven areas and vascular walls. It acts anti-inflammatory, improves blood circulation.
With the help of starch, you can fix the problems with digestion, cholesterol, gall-acid metabolism.
Pick up a diet should only the attending physician, in any case should not be engaged in self-medication.
Treatment with Medicinal Plants
Plants of medicinal origin support the immunity of the patient, with this disease it is necessary to take drugs with strengthening, astringent and anti-inflammatory properties.
Chamomile, animal harvesting, birch buds and immortelle have a healing effect. The method of preparation is as follows: 100 grams of each collection, pour 500 ml of freshly boiled water, insist about 4 hours. After strain, squeeze. Accepted 200 ml overnight.
Deaf nettle helps clean the blood. Boil nettle flowers and leaves for 15 minutes. It is used for inflammation of the kidneys, enlarged lymph nodes, spleen. The use of juniper berries can also help clean up the blood. You can start using 5 berries, add 1 berry every day, add up to the amount of 15.
Nervous, cardiovascular systems can increase their efficiency by consuming green oats. To do in two ways: juice or insist on alcohol. The recipe is simple: grind in a blender, fill a jar, add alcohol. The drug should be infused for 3 weeks, in a place inaccessible to light. Tincture need to filter and shake. After taking 3 times a day from 20 to 30 drops, spreading with a spoonful of water.
You can pamper yourself with delicious and vitamin tea and at the same time get a therapeutic effect. The recipe is: take dried strawberry leaves, about 10 grams, St. John's wort 2 grams, mint 2 grams. Pour a glass of boiling water into the mixture and infuse for about 10 minutes. This can be done with rowan, raspberry, currant, cooked in a similar way.
Any folk remedy should be discussed with your doctor, for the appointment of a combined course with drug therapy.
Herbal treatment process
Astringent leaves, mountain ash, blueberries and mint have astringent and anti-inflammatory effects.
St. John's wort, in a dry form, crushed, poured boiling water (about a glass). Insist 40 min. Strain and consume 1 tbsp. spoon before meals / 3 times a day.
Rowan and blueberries are also great for infusion. The proportions of the fruits of plants are 3 fruits of mountain ash and 2 blueberries, pour 200 ml of freshly boiling water, leave for about 10 minutes, strain, let cool. Drink 100 ml / 3 times a day.
This mixture is prepared as follows: nettle, mint, calamus root, valerian root, chop, pour hot water (about 200 milliliters), let it brew for 45 minutes. After straining, take as needed. Helps with diarrhea, stomach pain.
Prevention of amyloidosis
This terrible disease can be avoided by using special diets and various nutritional supplements. The first step is to:
- reduce eating high protein foods. This mainly concerns dishes of animal origin,
- increase the consumption of fish dishes, products containing whole grains, as well as vegetables, fruits and nuts,
- refuse dairy products, sugars and foods containing caffeine,
- consume more foods containing vitamin C. These are citrus fruits, cabbage, tomatoes, apples, gooseberries, baked potatoes and many other foods.
Treatment depends on the type of disease. If primary amyloidosis, an anti-protein diet will lead to a good result. Chemotherapy and the use of colchicine can also give a positive result. Secondary amyloidosis involves the appointment of antibiotics, antiarrhythmic and diuretic drugs that act on a particular organ.
If drugs do not help, surgery is prescribed. In this way, bone marrow, kidney or liver transplantation, heart transplantation and even pacemaker implantation can be performed.
Affection of organs with amyloidosis
- Kidney damage. Frequent and very dangerous disease. Symptoms of amyloidosis of the kidneys have 4 stages: latent, proteinuric, nephrotic, and azotemic.
- Liver damage. The size of the body increases, which can be determined by probing the abdomen. Amyloid liver damage is manifested by an increase in liver, jaundice, and portal hypertension.
- Amyloidosis of the heart. Its manifestation is accompanied by arrhythmia, shortness of breath, fainting, and cardiomyopathy. Amyloid deposition may be present both in the heart muscle and in its membranes.
- Amyloidosis of the skin. Contributes to the violation of the blood supply in the vessels, which is expressed in dryness and peeling of the skin. There may be a rash in the form of small acne red.
- Joint disease. When amyloidosis of the joints affects first the hands and feet, and then the knees and elbows. Characterized by pain, swelling of tissues, increased temperature of the skin in places of inflammation.
- Muscle damage Amyloidosis of these organs is manifested in their weakness, increase in size and compaction. Accompanied by pain and the appearance of tight nodules. If the disease is started, the muscles are squeezed, the blood supply is disturbed, as a result of which their death occurs.
- Disease of the nervous system. Amyloidosis of the central nervous system is characterized by the deposition of amyloid in brain tissue, a violation of the patient’s mental abilities becomes a direct consequence of this process.
- Amyloid GIT. Changes in the absorption of nutrients and vitamins. This is manifested in the form of diarrhea, sudden weight loss, fatigue, mental abnormalities, as well as hair loss and anemia.
- Enlarged spleen. Amyloidosis of this organ leads to an increase in its size and the development of hypersplenism, which is characterized by anemia and leukopenia.
- Amyloidosis of the lungs. It occurs with pneumonia, pleurisy, cyst. Most often located on the walls of the blood vessels of the lungs and bronchi.
Treatment of amyloidosis can be carried out only with an accurate examination of the patient in a hospital.
The signs of amyloidosis observed in people with this disease are directly dependent on the affected organ. In this regard, it is impossible to isolate any particular group of symptoms and, depending on the amyloidosis of one or another organ, distinguish the whole symptom complexes. It should also be noted that the primary generalized amyloidosis is characterized by a polymorphism of the clinical picture, since along with the intestine, the heart, skin, kidneys, endocrine and nervous systems can be affected.
Often the first symptoms can be prolonged elevated body temperature, significantly increased ESR.
Kidney damage with amyloidosis.
The most frequent localization is renal amyloidosis. Symptoms in this pathology are very diverse and very dangerous without prescribing adequate treatment. The clinical picture of renal amyloidosis is manifested by some symptoms of chronic renal failure, namely:
- Quantitative changes in urine: oliguria is observed (less than 500 ml of urine is excreted per day) and anuria (less than 50 ml during the same period).
- Disorders of general well-being: the appearance of irritability, anxiety, weakness and increased fatigue.
- The appearance of edemas - due to a decrease in the concentration of proteins, fluid is transferred to tissue structures, which leads to swelling. With prolonged disease progression, fluid accumulates in the abdominal and thoracic cavities, as well as the pericardial cavity, leading to ascites, hydrothorax and hydropericardium.
- Due to the defeat of the kidney parenchyma, an increase in blood pressure occurs, arrhythmias and cardiac muscle hypertrophy appear.
- Disruption of the mental-functional state - manifested as a result of the damaging effect of urea with non-functioning kidneys - leads to impaired psychological background, insomnia, impaired memory and mental abilities.
- Anemia: due to kidney amyloidosis damage, the required amount of erythropoietin ceases to be produced, which stimulates the production of red blood cells in the bone marrow.
Liver damage with amyloidosis.
In generalized forms of the disease, so-called liver amyloidosis develops. Symptoms are caused by the deposition of amyloid in the liver tissue and, as a result, the compression of hepatocytes (liver cells) and the structures of the hepatic lobe (vein, artery, bile duct). The liver during the accumulation of amyloid increases in size, portal hypertension occurs as a result of compression of blood vessels. Next, there is an increase in pressure in the portal vein, the veins of the spleen, esophagus and other internal organs are dilated. All this is manifested by the appearance of ascites, the appearance of bleeding from the dilated veins of the esophagus (often), bloody stools (melena), and vomiting with blood.
When the amyloid is pressed, the jaundice occurs (as a result of the accumulation of bilirubin), which is accompanied by severe pruritus and yellow sclera.
Intestinal lesion in amyloidosis.
One of the defeat variants is intestinal amyloidosis. Symptoms of this form are characterized by:
- Chronic diarrhea - persistent, frequent and prolonged diarrhea - leads to depletion of the body, and a person begins to lose weight.
- Syndromes of exudative enteropathy and impaired absorption - the most frequent symptom of intestinal amyloidosis - in this case, a huge amount of protein is lost, which migrates from the blood into the intestinal lumen, standing out. Syndrome impaired absorption differs rapidly progressive course. Due to the simultaneous exudation (release) of protein into the intestinal lumen, disturbances of protein metabolism in the form of persistent hypoproteinemia (decrease in the amount of protein in the bloodstream) and edema prevail. In this case, hypoproteinemia is very difficult to correct with replacement therapy.
- Intestinal obstruction - is formed as a result of local (local) amyloid deposition and the development of a tumor-like formation, which, by squeezing the intestinal loops, leads to a violation of the evacuation of feces. Such a manifestation can lead to severe complications, up to sepsis.
- Gastrointestinal bleeding is a fairly frequent manifestation of intestinal amyloidosis, which is characterized by the development of various forms of anemia.
The defeat of the nervous system in amyloidosis.
The most frequent initial symptoms of primary amyloidosis on the part of the nervous system include general weakness, fatigue, and fairly rapid weight loss. In about 40% of cases, polyneuropathy develops with minimal involvement of internal organs, and in 15% of cases it may be the only manifestation of the disease. But in 60% of cases, primary systemic amyloidosis leads to polyneuropathy in the background of pronounced manifestations of amyloidosis of other localization (with existing amyloidosis of the heart, kidneys, intestines, and others). Polyneuropathy is axonal in nature, with predominantly subtle myelinated (myelin-coated) and non-myelinated fibers, and the clinical process is reminiscent of diabetic polyneuropathy. The first to suffer are sensory fibers, which conduct pain and temperature sensitivity, as well as vegetative fibers.
The initial symptoms of polyneuropathy in amyloidosis of the nervous system are:
- Dysesthesia (sensitivity disorders). The sensitivity here suffers more superficial than deep
- Burning pain in hands and feet.
The defeat of vegetative fibers and ganglia by amyloidosis is manifested by orthostatic hypotension, fixed pulse, erectile dysfunction, impaired trophism of the skin, and decreased sweating.
At a later stage, muscle weakness and amyotrophy (impaired muscle function and nutrition) develop. In this case, motor disturbances are less pronounced in comparison with sensitive ones.
The defeat of muscle tissue in amyloidosis.
The severity of motor disorders increases if amyloidosis directly affects the muscle tissue with involvement of the proximal muscles of the limbs.
Amyloid myopathy is manifested by higher-quality disorders mainly in children, which is associated with weakness and fatigue of the already weakly expressed muscular layer, which arise as a result of their thickening and hypertrophy. If amyloid begins to be deposited in the muscles of the tongue, so-called macroglossia develops (an increase in the tongue to abnormally large sizes). Dysphagia (impossibility of swallowing) and coarsening of the voice may also appear. In some patients, respiratory failure occurs due to amyloidosis of the respiratory muscles.
Skin lesions in amyloidosis.
In primary amyloidosis, it is often possible to note these or other skin rashes: most often they are petechiae, purpura, nodules and plaques, while some general criterion characteristic of skin amyloidosis cannot be noted. When the nodular-patchy form of amyloidosis of the skin is observed a large number of plaques and nodes, which are located mainly on the legs and ankles. A characteristic feature of this form of amyloidosis is a symmetrical arrangement of isolated nodules on both legs. This form occurs mainly in women.
Modern methods of diagnosis of amyloidosis
Amyloidosis is a disease manifested as a pathology of protein metabolism and the performance of the immune system. Amyloid (a protein-saccharide complex) resulting from this disorder can be deposited in the cells of any body tissue. With the development of the disease, it gradually replaces healthy cells, and the organ ceases to function. In the case of a severe form of pathology, multiple organ failure occurs (lesion of 50% and more organs), leading to death.
The hereditary form of the disease is observed in the area of the Mediterranean Sea, as well as among people of Jewish and Armenian nationality. Among men, the disease is twice as common.
Of the most common forms of the disease, nephropathic can be called, in which deposits are observed in the kidneys, and systemic - amyloid deposits are found in many organs.
Amyloid, deposited in various organs, differs in structure. In total there are about 15 types, differing in structure and composition. They are based on two types:
- AA amyloid. Chronic inflammatory diseases cause high levels of SAA protein in the plasma and maintain it for a long period. In the case of incomplete protein cleavage, fibrillar AA-amyloid is formed.
- AL amyloid. Proteins of this type appear in the breakdown of amyloidoblasts (degenerate plasma cells). They are abnormal immunoglobulin compounds.
- The appearance of other types of amyloid is determined by the form of amyloidosis.
Types of disease
Amyloidosis occurs as an independent, and concomitant disease. Diagnosis of amyloidosis distinguishes several types:
Primary or idiopathic. When this type of deposits are found in all internal organs. It is impossible to determine the exact cause of the appearance. Multiple abnormalities are observed in cells of the immune system with the accumulation of AL-amyloid in the skin, in the tissues of the muscular, nervous, and cardiovascular systems. It can be caused by plasmacytoma (myeloma) - a malignant tumor pathology.
Symptoms of primary amyloidosis:
- Myasthenia gravis followed by muscle atrophy
- Dyspepsia and diarrhea,
- Pathology of the genitourinary and reproductive system,
- Damage of the organs of sight.
It is a complication of any inflammatory disease. The reason for its appearance can be:
- Chronic infectious diseases: pyelonephritis, tuberculosis, bronchiectasis, malaria, syphilis or leprosy (leprosy),
- Chronic purulent diseases: osteomyelitis, purulent ulcers and wounds,
- Ulcerative colitis - inflammation of the colon,
- Tumor lesions of the blood-forming organs: leukemia, lymphogranulomatosis, etc.
- Rheumatological pathology: various arthritis and so on.
Secondary amyloidosis is formed in the internal organs. There is a violation of the organs with the greatest amyloid deposition - in the area of the kidneys, spleen, liver or lymph nodes. In the future, the lesion spreads to other organs, followed by death.
This form is formed due to genetic abnormalities in the cells of the immune system, leading to the appearance of amyloidoblasts. Similar pathology is diagnosed in some national groups or in a specific geographic area. To hereditary form include:
- Periodic illness - Mediterranean family fever,
- Familial nephropathic or English amyloidosis,
- Hereditary neuropathic amyloidosis - Portuguese, American or Finnish,
- Hereditary cardiopathic or Danish amyloidosis.
A systematic approach allows us to identify this pathology in people after 80 years. It includes:
- Cerebral or cerebral amyloidosis. Diagnosed with Alzheimer's disease,
- Amyloidosis of the heart. It affects the heart muscle. Deposits are also formed in the lungs, liver and pancreas.
General urine analysis
Diagnosis of kidney amyloidosis shows the likelihood of the development of inflammatory processes occurring in the kidneys.
When the pathology of the kidney is detected:
- Proteinuria - urine protein content over 3 g / l,
- Hematuria - the detection of red blood cells in the urine
- Leukocyturia - the presence of leukocytes in the urine,
- Cylindruria - the content in the urine of cylinders formed during amyloidosis from proteins, epithelial cells of the kidneys, leukocytes and erythrocytes,
- Decreased urine density.
It gives an opportunity to assess the general condition of the body and determine the cause of amyloidosis. In this analysis are determined:
- Proteins of the common phase of inflammation produced by the liver or certain leukocytes during the inflammatory process. Special attention should be paid to the amount of fibrinogen.
- Hepatic tests indicate the status of this organ.
- An increase in cholesterol is a sign of nephrotic syndrome.
- A decrease in protein levels indicates nephrotic syndrome or liver failure.
- An increase in urea and creatinine is an indicator of renal dysfunction in amyloidosis.
This method makes it possible to determine the structure and structure of tissues of internal organs, the degree and distribution of pathological processes.
Diagnosis of amyloidosis using ultrasound shows:
- Compacting and resizing the kidneys,
- The presence of cysts in the kidneys,
- Compaction and enlargement of the spleen and liver, accompanied by pathology of blood flow,
- Cardiac muscle hypertrophy,
- The presence of amyloid deposits in the walls of the main blood vessels,
- Increased fluid volume in various body cavities — ascites, hydropericardium, or hydrothorax.
Removal of a small part of the tissue for research using special methods. Its use allows you to diagnose amyloidosis in 90% of cases. The study takes the muscle tissue, internal organs and mucous membranes.
Conducted with the likelihood of hereditary amyloidosis. The study takes the genetic material of the patient, which is checked for the presence of genetic abnormalities in some chromosomes. When a pathology is detected, it is recommended that all the relatives of the patient be examined to identify the disease.
Treatment of amyloidosis is symptomatic and supportive - it is impossible to completely cure the disease. Hospitalization is recommended for the patient, since with severe signs of the disease, there may be a dangerous sharp deterioration and complications. After stabilization of the patient, therapy is carried out at home.
If the disease is of a secondary form and is at an early stage, then treatment of the underlying disease is effective. In this case, the painful symptoms of amyloidosis subside.
Drug treatment of amyloidosis tends whenever possible to slow down or limit the formation of amyloids and alleviate the course of the disease. For this drugs are used:
- steroidal anti-inflammatory,
- antitumor and aminoquinoline, which inhibit protein synthesis,
- anti-gouty (with a hereditary form of the disease), which slow down the rate of leukocyte formation.
If amyloidosis is diagnosed, the diet is prescribed as soon as they learn about it: it helps protect the heart, kidneys and other affected organs from metabolic products and normalizes the water-salt balance, equalizes blood pressure. Food should be taken often, every three hours, in small portions. Emphasis should be placed on vegetable broths, fresh vegetables and fruits, lean meats, fish, dairy products. It is recommended to avoid strong broths based on fish and meat, the use of cheese, egg yolk, baking, coffee and alcohol is undesirable. It is considered mandatory to limit salt intake (up to 2 grams per day) to prevent edema.
Some patients believe that fasting treatment can be a panacea for amyloidosis, but doctors dispel these notions: the patient needs regular food intake, and her lack threatens with complications.
At the same time, they are not opposed to the patient using folk remedies in the form of herbal preparations, which have an anti-inflammatory and diuretic effect, but warn that they can only serve as ancillary measures, but not replace the main treatment.
The disease progresses, and often taking certain medications is not enough.
Types of dialysis, which is carried out with amyloidosis
If the patient is diagnosed with amyloidosis of the kidneys, treatment, in order to avoid the development of renal failure, is implemented through dialysis. It provides the removal of toxic products from the body and contributes to the normalization of water-salt, acid-base balance. When amyloidosis can be dialysis of two types:
The procedure is performed using an artificial semi-permeable membrane that separates the patient’s blood and dialysis solution. The patient’s blood contains high concentrations of harmful substances (urea, proteins, toxins) that are not in solution. As a result of diffusion, these substances penetrate through the membrane, saturating the liquid with their lower concentration. The necessary vital elements (ions of sodium, magnesium, calcium, potassium, chlorine) are replenished with dialysate in the necessary concentration so that they do not remain in the blood. Thus, the device replaces the broken kidney. If a person suffers from swelling of the joints, lungs (such swelling can also be under the skin or around the heart), then due to the transfer of excess fluid into the solution due to the difference in pressure, they pass.
Treatment of kidney amyloidosis is effective with peritoneal dialysis: doctors believe that the removal of B2-microglobulin, which affects the formation of amyloid, is more effective than with hemodialysis. The principle of this procedure is similar to the principle of hemodialysis, but the peritoneum acts as the membrane through which the products of metabolism are removed. So called thin shell that covers the inner surface of the abdominal cavity and its organs.
For this procedure, it is necessary to place the catheter in the pelvic cavity. This manipulation is carried out with the help of surgical intervention. The outer part of the tube is removed under the skin outside the front or side of the abdominal cavity.
Due to this, treatment of amyloidosis using dialysis becomes possible at home: the patient can cope with the procedure that needs to be done every day. This is considered a big plus compared with hemodialysis. The catheter is tightly fixed, the risk of developing an infection in this case is minimal.
Dialysate is introduced into the abdominal cavity using a catheter, and toxins from the vessels of the peritoneal wall begin to flow into it (this area has a good blood supply). The solution is contaminated after a few hours, so it is replaced with the next dose (usually it is 2 liters). While the liquid is inside, no other measures are required - the patient can go about his daily business.
Transplant tissue or organ
In the event that organ failure arises, transplantation of the diseased organ (if it is a kidney or heart) or tissue (when the liver, skin is affected) is necessary. If amyloidosis develops in the spleen, it must be removed.
After a donor transplant, the patient for the rest of his life takes drugs that suppress immunity - this is necessary so that the body itself does not reject the transplanted tissue or organ. However, the use of a donor organ is not a panacea, since in most cases a recurrence of the disease is possible, which worsens the prognosis.
Organ transplantation is carried out both in domestic clinics and abroad (in Germany, Israel), where this area of medicine is developed especially.
At first, when the disease has only manifested itself, aminoquinoline medications help. Only a doctor can prescribe these drugs. Also remember that they are not effective in acute or late stages of the disease.
If blood pressure is significantly increased from amyloidosis, medications are prescribed that normalize it. In the formation of edema, diuretics of a certain dosage are used, depending on the severity of the nephrotic syndrome. If the patient has decreased hemoglobin, you need to take drugs with iron content to normalize the composition of the blood.
When treating this disease, immunosuppressants are often prescribed. In primary amyloidosis, it is recommended to take Melfalan, Prednisolone. In the presence of AA-amyloidosis prescribe Colchicine, Unithiol. For the treatment of many patients used desensitizing drugs - Suprastin,
Pipolfen. In secondary amyloidosis, which appears as a complication of rheumatoid arthritis, specific anti-inflammatory drugs are prescribed.
Medication in the early stages of the disease
In the treatment of amyloidosis, preparations of the aminoquinoline series are used, which are aimed at preventing the formation of amyloid in the tissues of the body. They need to be taken from 2-3 months to several years to prevent side effects.
Aminoquinoline drugs can adversely affect vision. Therefore, in the process of treatment, it is necessary to periodically undergo examination by an oculist in order to identify violations in time. Other side effects are also possible - diarrhea, vomiting, psychosis, rashes on the skin. With a pronounced negative effect of drugs in this group they are canceled.
Colchicine with amyloidosis
Using Colchicine for a long time, you can forget about new bouts of the disease, it is an excellent prophylactic. If this drug is taken for life, you can achieve the following results:
- prolonged remission
- re-development of the disease is prevented during kidney transplantation,
- complete disappearance of nephrotic syndrome,
- elimination of proteinuria, if the kidney works well for the patient.
The recommended daily dose for amyloidosis is 1.8-2 mg. In the presence of renal insufficiency, the amount of Colchicine is slightly reduced. This drug is safe enough for humans. It can be taken for a long time. In rare cases, possible side effects in the form of digestive disorders, abdominal pain. If these symptoms are present, there is no need to discontinue treatment with colchicine. In a few days everything should be normal. In order to alleviate the condition of the patient, the additional intake of enzyme preparations for 2-3 days is allowed.
Colchicine is not recommended for the treatment of secondary amyloidosis. In this case, it will not give the desired effect.
Preparations for the treatment of amyloidosis caused by rheumatoid arthritis
Dimexide is used for secondary amyloidosis. This anti-inflammatory agent should be consumed orally in the amount of 1-5 ml. In the presence of kidney amyloidosis, the agent is prescribed with great care, starting with the minimum dosage. The whole process of treatment should take place under the supervision of a physician.
Treatment of primary amyloidosis
To help the patient with primary amyloidosis, Melphalan is used, which is combined with another drug, Prednisolone. The course of treatment is long: at least a year. The medication takes 4 to 7 days, after which you need to take a break for 28-45 days. The number of treatment courses is determined by the doctor.
The dosage of drugs depends on the weight of the person. 0.2 mg of Melphalan and 0.8 mg of Prednisolone are prescribed per kg of weight. This dosage is indicated for taking the drugs for 24 hours.
The positive effect of treatment with these drugs:
- significant decrease in urine protein,
- normalization of the creatinine content in the patient’s blood,
- improving blood circulation
- reducing the amount of immunoglobulin.
In primary amyloidosis, it is often not possible to conduct long-term treatment with these drugs, since the condition of a person can rapidly deteriorate. This scheme is acceptable for patients without severe renal failure.
Treatment of amyloidosis with folk remedies can have a positive effect on health, but should be carried out solely with the consent of the attending physician.
In many diseases, it is necessary to follow a diet without fail, and amyloidosis is no exception to this rule. It is necessary to exclude from your diet proteins and salt, because they affect renal and heart failure. It is desirable to increase the use of products containing starch, ascorbic acid, potassium salts. These products include bakery products, potatoes, cereals, bell peppers, green vegetables (spinach, broccoli, Brussels sprouts, dill), citrus fruits, garlic, carrots, liver, fish and dairy products.
Traditional medicine recommends in the treatment of amyloidosis to eat raw liver, which contains a large number of useful and necessary for our body substances (ascorbic acid, tocopherol, B vitamins, carotene, axeroftol). Also in the liver is a rich composition of mineral elements: P, Fe, Zn, Cu. As a result of daily ingestion of a raw liver (over one and a half to two years), improvement in the functioning of the kidneys, the gastrointestinal tract and the nervous system can be observed.
To strengthen the immune system used medicinal plants that have anti-inflammatory properties. The following remedies are popular in folk medicine:
- Healing infusion of chamomile, birch buds, immortelle, hypericum. These plants are mixed, and the mixture is placed in a thermos and filled with boiling water, after which it is allowed to infuse, filter and consume overnight.
- Decoction of nettle (aimed at cleansing the blood, also has anti-inflammatory effects).
- Brewed tea from the flowers and leaves of nettle, which boil before consumption.
- Tea made from strawberries, mint, St. John's wort. Used as a general tonic.
Alcohol tincture of oat grass. It is believed that this drug has a beneficial effect on the functional activity of the heart, blood vessels, nervous system. When cooking, the grass is crushed, poured into a bottle and filled with alcohol, and then put in a dark place (it is recommended to periodically filter). Before use, dilute the tincture with water.