Overview
In accordance with the classification of amyloidosis are categorized disproteinozov system. Amyloidosis occurs with synthetic and accumulation in different organs of the polysaccharide protein, known as amyloid. This leads to disruption of the functioning of the affected organ.
If we talk about the structure of amyloid, is a complex chemical compound that consists of a protein fraction and a polysaccharide. It It can accumulate in virtually every tissue and organ of the human. If it is a small amount, it does not lead to the development of pathological disorders. In this case, the pathology can be detected only on the histological examination of tissue sections.
However, if the amyloid accumulates in large quantities in a particular organ, such a phenomenon is a violation of its structure. As a result, develop atrophic changes in tissues and multiple sclerosisThat clinic manifested dysfunction of the affected organ.
Suffer from this disorder mostly elderly people living in Mediterranean countries, Japan and China.
Causes
The pathogenesis of this disease process is not completely understood. There are several theories:
- According to the theory of cell genesis in macrophage cells synthesized chemicals - amyloid precursor from which subsequently produces the protein polysaccharide.
- Theorists disproteinoza considered that amyloid is formed in the body due to disturbance of metabolism, in particular protein.
- Immune theory explains the accumulation of the substance due to the immune response of getting into the organism foreign protein or compound having a large number of its own degradation products tissues.
- The theory of mutations suggests that amyloid production occurs under the influence of mutational factors that disrupt the natural protein in the body to synthesize the patient.
However, there are so-called predisposing factors that lead to the formation of secondary amyloidosis. These include:
- Infectious diseases which are chronic (tuberculosis, malariaAnd syphilis);
- inflammatory processes accompanied by the development of suppuration (osteomyelitis, Or empyema endocarditis bacterial);
- tumors of various origins (e.g., cancer process in the kidneys or lungs);
- genetic predisposition to amyloidosis.
Classification
One common classification no. Some authors take as a basis the reasons for the development of the pathological process, the following groups:
- Idiopathic amyloidosis, which occurs in patients with no obvious primary prerequisites for this.
- Reactive process is considered to be secondary, that is, it becomes an extension or complication of any disease.
- Hereditary disease linked to the presence in the patient's genetic predisposition thereto.
- If amyloidosis develops in old age, a patient diagnosed with senile amyloidosis.
Also pathology classified by a primary lesion of a particular organ. For example, if the kidneys are affected, we speak of nefropaticheskom amyloidosis, and the pathological condition diagnosed kardiopatichesky amyloidosis in the heart. If struck by more than one body, and a few, talk about mixed form.
Many foreign authors amyloidosis are divided into two types:
- localized form, which mostly occurs in older patients;
- systemic amyloidosis.
In the latter case, the diagnosis is complemented by a special marking that characterizes the biochemical composition of amyloid:
- Marking «AL» appears in the presence of an immunoglobulin light;
- «AA» - if in structure of amyloid compounds appears α-globulin;
- marker "Aβ2M" distinguished history of the disease in patients with β2-microglobulin, which is part of fibrils;
- «ATTR» is placed when the fibrils in the analysis reveal the transport protein compounds.
symptoms
The clinical manifestation of renal amyloidosis depends on the order in which the organ or tissue and the substance on the degree of injury begins to accumulate. Amyloid heart failure is characterized by such features:
- conductivity suffers cardiac tissue;
- there is an arrhythmia;
- focal changes observed in muscle tissue of the heart;
- gradually increasing heart failure.
With the defeat of intestine following symptoms:
- often suffers from the esophagus;
- disturbed swallowing and absorption, resulting in reduced weight and avitaminosis varying severity;
- language often increased;
- by increasing the size of the liver and spleen of their functions, as a rule, do not suffer;
- in gastric tissues may appear tumor-like growths consisting of amyloid deposits.
amyloid liver damage is quite rare. Symptomatically, it appears:
- increase in body;
- pain symptoms in the area of its location;
- nausea and vomiting;
- belching;
- jaundice varying intensity;
- in severe hemorrhagic syndrome may develop;
- palpation revealed an increase in the liver, it tight to the touch.
Renal disease is usually a secondary process and proceeds sufficiently hard. Experts identify three stages of the disease:
- The first stage of amyloidosis characterized by the presence of proteinuria.
- When work stage renal nephrotic violated, as manifested edema, change indicators urinalysis (protein appears oliguria and nocturia) and blood (abnormal protein ratio fractions). Suffering heart (reduced blood pressure index), gastrointestinal tract, and breaks down the protein-carbohydrate liver function.
- At step develops azotemicheskoy chronic renal failure with the defeat mocheobrazovatelnoy and excretory functions.
Amyloidosis genitals symptomatology of lesions appears quite late, about the second year disease. As a rule, production of gametes is not broken, but may reveal uterine bleeding, diseases of the prostate or urinary system.
Skin amyloidosis is characterized by:
- pale skin;
- presence of characteristic plaques colored in yellow;
- the emergence of small areas of hemorrhage;
- thinning of the skin, which is starting to come into the fold.
The accumulation of amyloid in neural tissue is characterized by:
- headaches;
- dizziness;
- increased sweating;
- tremor limbs;
- loss of neural sensitivity;
- incontinence of urine and feces;
- increasing symptoms of impotence.
Diagnostics
- The survey of the patient in handling refinement of complaints, the time the first symptoms, the presence of chronic diseases, including inflammatory disorders. Also specifies the presence of close relatives symptoms of amyloidosis.
- Palpatory percussion and medical examination.
- If necessary - consultation with other specialists.
- Appointed urinalysis, and biochemical analysis of blood.
- Performed a biopsy of the affected organ or tissue.
Treatment
Developed tactics treatment of patients with renal amyloidosis hitherto not. This is due to the fact that the exact the cause of this pathology is not clear. In the early stages of the disease drugs recommended aminohinolinovogo series immunosuppressants which inhibit the intensity of the immunological reaction. Also, being symptomatic therapy, which depends on the affected organ disease. In addition, they can be assigned to cytotoxic anticancer drugs and hemodialysis.
In more severe course of the pathological process operative treatment. It provides for the complete replacement of the affected organ disease - transplantation.
prevention
The basis for the prevention of secondary forms of amyloidosis is timely chronic infectious inflammation therapy. As a precaution prolongation of an existing disease is recommended timely medication and diet therapy. It is necessary to reduce the amount of protein consumed and replace it with carbohydrates, to give preference to starchy. It should also be possible to use less salt food.
Forecast
Amyloidosis is characterized by a gradual progression of symptoms. The probability of full treatment of amyloidosis quite low. The overall prognosis depends entirely on the degree of organ damage. With timely treatment and implementation of directions of the doctor patient can long enough to have an initial stage of the disease and to maintain the ability to work. In more severe cases the patient may be partially or completely disability.
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