Renal amyloidosis - Causes, Symptoms and Treatment .MF .
renal amyloidosis - a disease in which the notes deposited in kidney tissue specific insoluble proteins - amyloid.
Depending on the development mechanism of the disease, and structural characteristics of amyloid identify several types of amyloidosis.
AA amyloidosis refers to the most common type of renal amyloidosis.This group includes secondary amyloidosis, a cause of which usually is rheumatoid arthritis;chronic suppurative destructive diseases such as osteomyelitis, tuberculosis;inflammatory bowel diseases such as ulcerative colitis, Crohn's disease;as well as tumors, particularly malignant.AA formed from the amyloid protein, which is released by liver cells and some blood cells during inflammation.In the presence of a genetic structure that breakage instead of the normal protein produced soluble protein insoluble amyloid.The conditions expressed or prolonged inflammation current amyloid concentration in blood is increased, it begins to sag and accumulate in the kidney tissu
AL-amyloidosis is another type of kidney disease.It is based on primary amyloidosis and / or multiple myeloma.In this case, due to genetic abnormalities protein structure under normal conditions (no predisposing factors, such as inflammation) produced defective insoluble protein, which also accumulates in various organs.
ATTR- amyloidosis .Rarely encountered a group of diseases, which include diseases such as familial amyloid polyneuropathy and systemic senile amyloidosis.Familial amyloid polyneuropathy - a hereditary disease that occurs when the accumulation of amyloid in neural tissue and is characterized by severe damage to the nervous system.System senile amyloidosis occurs in people older than 70 years as a result of age-related changes of normal proteins that become insoluble.
Symptoms of renal amyloidosis amyloidosis
When insoluble protein, usually formed in large quantities, accumulates in the kidney tissue, causing a violation of its basic functions.
main clinical feature of both primary and secondary amyloidosis is kidney damage.Very often, kidney disease is asymptomatic and is detected at stage III-IV renal failure.
In primary amyloidosis disease develops early, around the age of 40 years, is characterized by progressive course, and high mortality.Secondary amyloidosis is detected at the age of 60 years, in the course of the disease is dominated by symptoms of the disease, which was the cause of amyloidosis.
There are several successive stages of the disease.This division is more characteristic of secondary amyloidosis.
1. Preclinical .In this stage the amount of amyloid in kidneys insufficient for significant violations of their function.So dominant are the signs of the disease - causes amyloidosis.It can be a pain in the bones and osteomyelitis with fever, changes in respiratory function in pulmonary tuberculosis, joint damage in rheumatoid arthritis.Urinalysis and instrumental methods of research, such as the US does not reveal kidney abnormalities.This stage, depending on the underlying disease, lasts on average 3-5 years.
2. proteinuric .Amyloid accumulates in kidney cells in sufficient quantities, thereby increasing their permeability for high molecular weight substances.The result is a blood protein in the urine, which is defined in its study.The amount of protein in the urine is constantly increasing over time, as the damage is irreversible kidney cells.At this stage there are no external manifestations of kidney disease.Protein in the urine is detected by chance during routine medical examinations or in the diagnosis of other diseases.Proteinuric stage lasts an average of 10-15 years.
3. nephrotic stage is characterized by severe external manifestations, which in most cases are caused by low levels of protein in the blood due to its massive urinary excretion.Characterized by a significant complaints of general weakness, nausea, loss of appetite, thirst, dry mouth.Patients lose weight rapidly.With a sharp transition from a prone position to a vertical position falls sharply blood pressure, up to fainting.Characterized by persistent diarrhea due to the accumulation of amyloid in the gut wall and destruction of the nerve endings.Arise and rapidly developing edema.In the beginning, they are identified in the area of the lower extremities, on the face, shortness of breath may be detected during a routine physical load (due to the accumulation of fluid in the lung tissue).Then take swelling common, until the accumulation of large volumes of fluid in the pleural cavity (hydrothorax), in the abdomen (ascites) in the cavity of the outer lining of the heart (hydropericardium).
Exterior patients with nephrotic syndrome.
sharp reduction in the amount of urine until her absence.Along with the swelling revealed enlarged lymph nodes, liver, spleen, indicating that amyloid deposition in them.At the same time these bodies are dense, painless, often, especially the liver, reach enormous proportions.
4. uremic stage - finals of the disease.Kidney wrinkled, loses its normal structure and the ability to perform all of its functions.At this stage of renal failure is diagnosed or IV degree V chronic kidney disease stage.Against the background of pronounced edema uremic symptoms occur (due to the exchange of the products that are normally excreted in the urine) intoxication.Nausea, vomiting, not bringing relief, weakness, abdominal pain, possibly raising the temperature to 37-37,5 degrees.Urine is usually absent.Often reduced pressure and rare pulse weak filling.At this stage, with the aim of removing toxic metabolic products is a renal replacement therapy (peritoneal dialysis, hemodialysis and its variants).
But, unfortunately, at the present stage, there are no methods that allow full display of amyloid from the body.Therefore, even in the face of the sessions of dialysis mortality in these patients remains high because of the defeat of other organs (heart, blood vessels, liver, pancreas, etc.).
In describing amyloidosis not to mention the damage to the heart, which begins at the same time, and sometimes even before the kidney disease.Amyloid is accumulated in heart tissue, cause the development of heart failure.At the same time the heart increases in size, its wall becomes stiff, affected valves.As a result, the heart can not cope with normal load, the blood stagnates in the lungs or on the periphery, which exacerbates the edema and respiratory failure.In addition, an enlarged heart due to a violation of the nervous regulation often develop arrhythmias, many of which are potentially fatal.Also, due to heart disease and vascular pressure in the beginning of the disease increased, and with the lack of effect of the drugs in the final stage of firmness decreased.
diagnosis of amyloidosis
following changes are detected in the laboratory analyzes:
• Complete blood count .Persistent and significant increase in ESR (erythrocyte sedimentation rate) in the early stages of the disease.In renal insufficiency, the development of a decrease in hemoglobin and red blood cell count.The increase in the number of platelets suggests involvement in the pathological process of the spleen.
• Biochemical analysis of blood .At the stage of the disease nephrotic noteworthy reduction in the number of proteins, including albumin, elevated cholesterol and low density lipoprotein.electrophoresis method can be released from the defective blood-insoluble protein.
• urine analysis.Since the proteinuric stage is characterized by large amounts of protein.The method may immuno protein identification Bens-Jones, which is typical of AL-amyloidosis with multiple myeloma.
To confirm the diagnosis requires a biopsy (tissue sampling modified area with the purpose of microscopic examination).If you suspect an AL-amyloidosis is conducted bone marrow biopsy of the sternum (sternal puncture) or iliac bone (biopsy).In all cases, shows a biopsy of the rectal mucosa, the web portion of the intestine or kidneys, liver.In the study of possible typing amyloid that determines the further treatment tactics.
treatment of amyloidosis
purpose of amyloidosis therapy - reduction in the formation of pathological protein and protecting organs from exposure.
In AA amyloidosis main role belongs to the anti-inflammatory actions, including with the use of surgical methods.So, when osteomyelitis is necessary to remove portions of purulent fusion bone, abscess (cavity filled with pus) light - drainage of an abscess.It is especially important early treatment of rheumatoid arthritis, as one of the main causes of the disease.
the presence of renal amyloidosis shows treatment with cytostatics (methotrexate, cyclophosphamide), which allows you to delay the onset and slow the progression of kidney failure.The greatest effect in all forms of amyloidosis was observed in some plant cytostatics (colchicine).
When AL-amyloidosis used combination therapy with cytostatic and hormonal therapy (prednisone).
In secondary amyloidosis is conducted in the first treatment of the underlying disease, after which often disappear and symptoms of amyloidosis.Held also symptomatic therapy: a multivitamin (parenteral), diuretics (when expressed edema), transfusion of plasma.When diarrhea is prescribed astringents (bismuth nitrate, adsorbents).
At the end of the disease is vital dialysis sessions.But still, the main method of treatment of nephrotic and uremic stage of the disease is a kidney transplant.
common mode is the same as in chronic glomerulonephritis When renal amyloidosis.
Prevention of amyloidosis
Prevention should be directed to the treatment of diseases associated with chronic inflammation, as well as timely diagnosis and competent treatment of amyloidosis in view of its type.
physician therapist, nephrologist Sirotkin EV