Amyloidosis of kidneys

Description:

Amyloidosis of kidneys – the manifestation of a system amyloidosis which is characterized by disturbance of proteinaceous carbohydrate metabolism with extracellular adjournment in renal fabric of amyloid – difficult proteinaceous полисахаридного the connection leading to dysfunction of body. The amyloidosis of kidneys proceeds with development of a nephrotic syndrome (a proteinuria, hypostases, hypo - and a disproteinemia, a hypercholesterolemia) and an outcome in a chronic renal failure. Diagnosis of an amyloidosis of kidneys includes researches of urine, blood and a koprogramma; performing ultrasonography of kidneys and biopsy. At an amyloidosis of kidneys the diet is appointed, medicinal therapy and correction of primary disturbances is carried out; in hard cases the hemodialysis and transplantation of a kidney can be required.
In urology the amyloidosis of kidneys (amyloid dystrophy, an amyloid nephrosis) makes 1–2,8% of all diseases of kidneys. The amyloidosis of kidneys serves as the most frequent manifestation of a system amyloidosis at which in intercellular space of various bodies special glikoproteidny substance – amyloid collects, breaking their functions. The amyloidosis of kidneys, along with a glomerulonephritis, is the main reason for development of a nephrotic syndrome.

Reasons of an amyloidosis of kidneys:

The etiology of an idiopathic amyloidosis of kidneys in most cases remains to the unknown; sometimes the disease develops at a multiple myeloma. Except kidneys at primary amyloidosis language, skin, a thyroid gland, a liver, lungs, intestines, a spleen, heart can be surprised.
The secondary amyloidosis is usually associated with persistent infections (syphilis, tuberculosis, malaria), is purulent - destructive processes (a brokhoektatichesky disease, a pleura empyema, osteomyelitis, a long septic endocarditis), general diseases (a pseudorheumatism, psoriasis arthritis, Bekhterev's disease), intestines diseases (ulcer colitis, a disease Krone), tumors (a lymphogranulomatosis, a meningioma, new growths of kidneys), etc. A secondary amyloidosis kidneys, vessels, lymph nodes, a liver, etc. bodies are surprised.
As the reason of a dialysis amyloidosis of kidneys long carrying out to the patient of a hemodialysis acts. The family amyloidosis meets at a periodic disease, is frequent in the countries of the Mediterranean Basin (in particular, the Portuguese option). The senile amyloidosis is considered as the aging sign which is found after 80 years at 80% of people. Local forms of an amyloidosis can be caused by tumors of endocrine system, Alzheimer's disease, a diabetes mellitus of the 2nd type, etc. the reasons.
Among theories of a pathogeny of an amyloidosis of kidneys the immunological, mutational hypothesis and a hypothesis of local cellular synthesis are considered. The amyloidosis of kidneys is characterized by extracellular adjournment in renal fabric, is preferential in balls, amyloid – a special glycoprotein with the high content of insoluble fibrous protein.

Classification:

According to etiopatogenetichesky mechanisms allocate five forms of an amyloidosis of kidneys: the idiopathic, family, acquired, senile, local tumorous.
At primary (idiopathic) amyloidosis of kidneys of the reason and mechanisms remain unknown. Family (hereditary) forms are caused by genetic defect of education in an organism of fibrous proteins. The secondary (acquired) amyloidosis of kidneys develops at immunological disturbances (persistent infections, rheumatic diseases, malignant tumors etc.). Involute change of exchange of proteins is the cornerstone of a senile amyloidosis. The nature of a local tumorous amyloidosis of kidneys is not clear.
Depending on type of the fibrous protein which is contained in amyloid various forms of an amyloidosis ("And") can be designated the following abbreviations: AA-type (secondary, amyloid contains serumal a-globulin); AL-type (idiopathic, in amyloid - easy chains of Ig); ATTR-type (family, senile; in amyloid – protein transthyretin); A2M type (dialysis; in amyloid - β2-микроглобулин), etc.
Idiopathic, family, senile and local tumorous forms of an amyloidosis are allocated in independent nosological units. The secondary amyloidosis of kidneys is considered as a complication of a basic disease.
Taking into account the prevailing defeat of any body and development of its insufficiency allocate nefropaticheskiya (an amyloidosis of kidneys), the epinefropatichesky, neyropatichesky, gepatopatichesky, enteropatichesky, kardiopatichesky, pancreatic, mixed and generalized options of an amyloidosis. Irrespective of a form and type at an amyloidosis there is a replacement of specific structural elements of fabrics by amyloid substance, decrease, and as a result and loss of functions of appropriate authorities.
AL-, AA-, ATTR-, A2M amyloidoses belong to system (generalized) amyloidoses; to local – an amyloidosis of kidneys, auricles, pancreas islands, cerebral, etc.

Symptoms of an amyloidosis of kidneys:

At an amyloidosis symptoms can be quite various, I will present only some of them:
Baldness;
Asthma;
General breakdown;
Arrhythmias;
Hypostases standing;
Renal failure;
Stethalgia;
Heart failure;
Loss of weight;
Weakness and weakness;
Abnormal changes of nail plates;
Damages of skin: consolidations and small knots, ulcerations, not pruritic rash;
Anemia (insufficient level of hemoglobin);
Dizziness and numbness of extremities because of the increased ABP level.
Frequent bruises;
Vision disorder, owing to accumulation of abnormal protein behind lens (at an inborn amyloidosis);
The increased fragility of bones;
Sharp differences of arterial pressure;
Inflammation of lymph nodes;
The language size can sometimes increase;
Vegetative neuropathy, including diarrhea and abdominal distention (neuropathy of hereditary character);
The amyloidosis belongs to the category enough rare diseases. About two thirds of patients are ill a secondary amyloidosis. And only about 5% are the share of senile, inborn and primary amyloidosis.

Practically all patients treat an age group 45 years (68% are more senior than the man from them).

Diagnosis:

During the early preclinical period to diagnose an amyloidosis of kidneys extremely difficult. In this stage the priority is given to laboratory methods – to a research of urine and blood. In the general analysis of urine the proteinuria which tends to steady increase, a leukocyturia (in the absence of symptoms of pyelonephritis), a microhematuria, a cylindruria is early noted.
Biochemical indicators of blood are characterized by a hypoalbuminemia, a hyperglobulinemia, a hypercholesterolemia, increase in activity alkaline phosphotazy, a hyperbilirubinemia, an electrolytic imbalance (a hyponatremia and a hypocalcemia), increase in content of fibrinogen and b-lipoproteids. In the general blood test - a leukocytosis, anemia, increase in SOE. At a koprogramma research the expressed steatorrhea degree ("a fat chair"), creatorrheas (considerable contents in excrements of muscle fibers), amylorrheas (presence in Calais of a large amount of starch) quite often comes to light.
Due to the expressed metabolic disturbances at an amyloidosis of kidneys on an ECG arrhythmia and disturbances of conductivity can be registered; at EhoKG — a cardiomyopathy and diastolic dysfunction. Ultrasonography of an abdominal cavity reveals increase in a spleen and liver. At a X-ray analysis of a GIT gullet hypotonia, weakening of a vermicular movement of a stomach, acceleration or delay of a passage of barium is determined by intestines. On ultrasonography of kidneys their increase (big fatty kidneys) is visualized.
As reliable diagnostic method of an amyloidosis serves the kidney biopsy. The morphological research of a bioptat after coloring of Congo red at the subsequent submicroscopy in the polarized light reveals the green luminescence characteristic of an amyloidosis of kidneys. Amyloid can come to light on the course of vessels, tubules, in balls. The biopsy of a mucous rectum, skin, a gingiva, liver is in certain cases made.

Treatment of an amyloidosis of kidneys:

In therapy of a secondary amyloidosis of kidneys a considerable role is played by success of treatment of a basic disease. In case of a full resistant izlechivaniye of primary pathology also symptoms of an amyloidosis of kidneys quite often regress.
Patients with an amyloidosis of kidneys need change of a diet: the use of a crude liver (on 80-120 mg/days), restriction of protein and salt (is necessary long, within 1,5-2 years, especially at renal and heart failure); the increased consumption of carbohydrates; food, vitamin-rich (especially vitamin C) and potassium salts.
Pathogenetic drugs for treatment of an amyloidosis of kidneys are derivative 4 aminoquinolines (delagil, Resochinums, Chingaminum) desensibilizing means (Suprastinum, Pipolphenum, Dimedrol), colchicine, Unithiolum, etc. Symptomatic therapy at an amyloidosis of kidneys includes purpose of diuretics, hypotensive drugs, plasma transfusion, albumine etc. Expediency of use of corticosteroids of tsitostatik is discussed.
In an end-stage of an amyloidosis of kidneys chronic renal dialysis or transplantation of a kidney can be required.

Forecast:

The forecast in many respects is defined by a current of a basic disease and speed of progressing of an amyloidosis of kidneys. Development of thromboses, hemorrhages, accession of intercurrent infections, advanced age worsens the forecast. At development of a heart or renal failure survival makes less than 1 year. As conditions of recovery serve the timely address to the nephrologist and early diagnosis of an amyloidosis of kidneys, active treatment and full elimination of a basic disease.