Amyloidosis

Description:

Amyloidosis (amyloid dystrophy) — the disturbance of protein metabolism which is followed by education and adjournment in fabrics specific proteinaceous полисахаридного a complex — amyloid. Amyloid persistirut long time in an organism and even after death is not exposed to rotting for a long time.
AL amyloidosis (immunoglobulin light chains derived) — primary amyloidosis caused by emergence in a blood plasma and adjournment in the most different body tissues of abnormal light chains of the immunoglobulins synthesized by malignizirovanny plasmocytes. The same process goes also at a multiple myeloma (Rustitsky-Kalera's disease, a plasmacytoma), but into the forefront pathological proliferation of a malignizirovanny clone of plasmocytes, infiltriruyushchy fabrics acts here (most often flat bones and vertebrae, with development of pathological changes).

AA amyloidosis (acquired) — the secondary amyloidosis caused by hypersecretion a liver of protein of an acute phase of alpha globulin in response to any chronic inflammation. This process develops at a pseudorheumatism, an ankylosing spondylarthritis, a bronchoectatic disease, chronic osteomyelitis, tuberculosis, a leprosy and some other diseases.

The ASC amyloidosis (systemic cardiovascular) developing at persons over 90 years is considered subspecies of this amyloidosis. The pathogeny in details is unknown.

AF amyloidosis (the Mediterranean alternating fever) — a hereditary form of an amyloidosis, with the autosomal and recessive mechanism of transfer. The people belonging to the certain ethnic groups living on the coast of the Mediterranean Sea (Sephardic Jews, Greeks, Arabs, Armenians) suffer from this type of an amyloidosis. There are kinds of an amyloidosis characteristic of a certain geographical area: "the Portuguese amyloidosis" (with preferential damage of nerves of the lower extremities), "the American amyloidosis" (with preferential damage of nerves of upper extremities), a family nefropatichesky amyloidosis, or the "English amyloidosis" proceeding with symptoms of a small tortoiseshell, deafness and fever.

The AH amyloidosis (hemodialisis-related) — is observed only at the patients who are on hemodialysis treatment. The pathogeny is connected with the fact that, normal utilized kidneys beta 2 - class MHC I microglobulin, is not filtered in the haemo dialyzer and collects in an organism.

AE amyloidosis — the form of a local amyloidosis developing in some tumors, for example, in medullary cancer of C-cells of a thyroid gland. In this case a predecessor of amyloid are pathological fragments of a calcitonin.

Amyloidosis of the Finnish type — the rare type of an amyloidosis caused by a mutation of a gene of GSN coding protein джелсолин.

Amyloidosis symptoms:

Intensity of clinical manifestations depends on localization and prevalence of amyloid deposits. It causes duration of a disease and existence of complications. Damage of kidneys is the most typical, sometimes there are damages of a gullet, a spleen, intestines, and also a stomach. At an amyloidosis of kidneys the long eclipse period of a disease at which there are no considerable symptoms is observed — only insignificant weakness and decrease of the activity are observed. After two weeks of a current of the eclipse period hypostases of kidneys, their dysfunction (emergence in urine of proteins — development of a proteinuria, and also uniform elements of blood), heart failure develop. At an amyloidosis of a stomach are observed weight in epigastric area, weakening of a vermicular movement of a stomach after the use of food. At an amyloidosis of intestines weight and dull aches in a stomach, diarrhea are observed.

Amyloidosis reasons:

Development of an amyloidosis is connected with disturbance of proteinaceous and synthetic function of reticuloendothelial system, accumulation in a blood plasma of the abnormal proteins serving as autoantigens and causing formation of autoantibodies. Interaction of antigen with an antibody is resulted by sedimentation of the coarse-dispersion proteins participating in formation of amyloid. Being postponed in fabrics (for example, in walls of vessels, ferruterous, etc.), amyloid forces out functionally specialized elements of body that leads to death of this body.

Treatment of the Amyloidosis:

General principles of treatment of an amyloidosis:

    * The mode is house, except for serious conditions (the expressed heart failure, a chronic renal failure)
    * The sick amyloidosis showed long (1,5 — 2 years) reception of a crude liver (on 100 — 120 g/days)
    * Restriction of consumption of protein, salt to patients with a chronic renal failure
    * Salt restriction to patients with heart failure
    * At a secondary amyloidosis — treatment of a basic disease (tuberculosis, osteomyelitis, a pleura empyema, etc.) after which treatment also manifestations of an amyloidosis quite often disappear
    * Transfer of the patient with a dialysis amyloidosis on peritoneal dialysis
    * At the intestines amyloidosis proceeding with persistent diarrhea — astringents (bismuth subnitrate, adsorbents)
    * At primary amyloidosis in initial stages of process — chloroquine of 0,25 g 1 р / days it is long, a combination of Melphalanum and Prednisolonum, Melphalanum, Prednisolonum and colchicine or only colchicine
    * At a secondary amyloidosis - specific treatment of a basic disease
    * At a family amyloidosis — colchicine (on 0,6 mg 2 — 3 р / days)
    * Symptomatic therapy: the vitamins, diuretic means, drugs reducing pressure, plasma transfusion, etc.

Surgical treatment of an amyloidosis:

    * Removal of a spleen can improve a state owing to reduction of amount of the amyloid which is formed in an organism