Amiloidoz of kidneys
Amiloidoz of kidneys – the manifestation of a system amiloidoz which is characterized by violation of proteinaceous and carbohydrate exchange with extracellular adjournment in renal fabric of an amiloid – difficult proteinaceous the connection leading to malfunction of body. Amiloidoz of kidneys proceeds with development of a nephrotic syndrome (a proteinuria, hypostases, hypo - and a disproteinemiya, a hypercholesterolemia) and an outcome in a chronic renal failure. Diagnostics of an amiloidoz of kidneys includes researches of urine, blood and a koprogramma; performing ultrasonography of kidneys and biopsy. At an amiloidoza of kidneys the diet is appointed, medicinal therapy and correction of primary violations is carried out; in hard cases the hemodialysis and transplantation of a kidney can be required.
Amiloidoz of kidneys
In urology kidneys (amyloid dystrophy, amyloid ) makes 1–2,8% of all diseases of kidneys. Amiloidoz of kidneys serves as the most frequent manifestation of a system amiloidoz at which in intercellular space of various bodies special glikoproteidny substance – collects, breaking their functions. Amiloidoz of kidneys, along with glomerulonefrity, is the main reason for development of a nephrotic syndrome.
Classification of forms of an amiloidoz of kidneys
According to etiopatogenetichesky mechanisms allocate five forms of an amiloidoz of kidneys: the idiopathic, family, acquired, senile, local opukholevidny. At primary (idiopathic) amiloidoz of kidneys of the reason and mechanisms remain unknown. Family (hereditary) forms are caused by genetic defect of education in an organism of fibrillyarny proteins. Secondary (acquired) kidneys develops at immunological violations (chronic infections, rheumatic diseases, malignant tumors etc.). Involute change of exchange of proteins is the cornerstone of a senile amiloidoz. The nature of a local opukholevidny amiloidoz of kidneys is not clear.
Depending on type of the fibrillyarny protein which is contained in an amiloida various forms of an amiloidoz ("And") can be designated the following abbreviations: AA-type (secondary, the amiloida contains serumal a-globulin); AL-type (idiopathic, in an amiloida - easy chains of Ig); ATTR-type (family, senile; in an amiloida – protein ); A2M type (dialysis; in an amiloida - β2-), etc. Idiopathic, family, senile and local forms of an opukholevidny amiloidoz are allocated in independent nosological units. Secondary kidneys is considered as a complication of the main disease.
Taking into account the prevailing defeat of any body and development of its insufficiency allocate nefropaticheskiya ( kidneys), the epinefropatichesky, neyropatichesky, gepatopatichesky, enteropatichesky, kardiopatichesky, pancreatic, mixed and generalized options of an amiloidoz. Irrespective of a form and type at an amiloidoza 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. Amiloidoza AL-, AA-, ATTR-, A2M belong to system (generalized) amiloidoza; to local – kidneys, auricles, pancreas islands, cerebral, etc.
Reasons of development of an amiloidoz of kidneys
The etiology of an idiopathic amiloidoz of kidneys in most cases remains to the unknown; sometimes the disease develops at a multiple myeloma. Except kidneys at primary amiloidoz language, skin, a thyroid gland, a liver, lungs, intestines, a spleen, heart can be surprised.
Secondary it is usually associated with chronic infections (syphilis, tuberculosis, malaria), it is purulent - destructive processes (a brokhoektatichesky disease, an empiyemy pleura, osteomyelitis, a long septic endocarditis), system diseases (rheumatoid arthritis, psoriatichesky arthritis, Bekhterev's disease), intestines diseases (ulcer colitis, a disease Krone), tumors (limfogranulematozy, meningiomy, new growths of kidneys), etc. A secondary amiloidoz kidneys, vessels, lymph nodes, a liver, etc. bodies are surprised.
As the reason of a dialysis amiloidoz of kidneys long carrying out to the patient of a hemodialysis acts. Family hereditary meets at a periodic disease, it is frequent in the countries of the Mediterranean Basin (in particular, the Portuguese option). Senile is considered as the aging sign which is found after 80 years at 80% of people. Local forms of an amiloidoz can be caused by tumors of endocrine system, Alzheimer's disease, diabetes of the 2nd type, etc. the reasons.
Among theories of pathogenesis of an amiloidoz of kidneys the immunological, mutational hypothesis and a hypothesis of local cellular synthesis are considered. Amiloidoz of kidneys is characterized by extracellular adjournment in renal fabric, mainly in balls, an amiloida – a special glikoproteid with the high content of insoluble fibrillyarny protein.
Symptoms of an amiloidoz of kidneys
At an amiloidoza of kidneys the kidney and extra kidney manifestations causing polymorphism of a picture of a disease are combined. During an amiloidoz of kidneys allocate 4 stages (latent, proteinurichesky, nephrotic, azotemichesky) having characteristic clinical symptomatology.
In a latent stage, without watching presence of an amiloid kidneys, clinical manifestations of an amiloidoz are not observed. During this period the symptomatology of primary disease prevails (infections, purulent processes, rheumatic diseases, etc.). The latent stage can last up to 3-5 and more years.
In a proteinurichesky (albuminurichesky) stage there is an accruing loss of protein with urine, a mikrogematuriya, a leykotsittsriya, increase in SOE. Owing to a sclerosis and an atrophy of nefron, a limfostaz and hyperaemia of a kidney increase, become dense, gain opaque gray-pink color.
The nephrotic (edematous) stage is characterized by a sclerosis and amiloidozy a brain layer of a kidney and, as a result, development of a nephrotic syndrome with a typical tetrad of signs – a massive proteinuria, a hypercholesterolemia, a gipoproteinemiya, hypostases, resistant to diuretics. Arterial hypertension can be noted, but HELL is more often happens normal or lowered. Also the splenomegaliya is quite often noted gepato-.
In azotemichesky (terminal, uraemic) the stages of a kidney cicatricial wrinkled, dense reduced in sizes (an amyloid kidney). The Azotemichesky stage corresponds to development of a chronic renal failure. Unlike a glomerulonefrit, at an amiloidoza of kidneys persistent hypostases remain. Amiloidoz of kidneys can be complicated by thrombosis of renal veins with an anury and a pain syndrome. An outcome of this stage often is death of the patient from azotemichesky uraemia.
Dizziness, weakness, short wind, arrhythmia, anemia, etc. can be system manifestations of an amiloidoz of kidneys. In case of accession of an amiloidoz of intestines persistent diarrhea develops.
Diagnostics of an amiloidoz of kidneys
During the early preclinical period to diagnose 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 leykotsituriya (in the absence of symptoms of pyelonephritis), a mikrogematuriya, a tsilindruriya is early noted.
Biochemical indicators of blood are characterized by a gipoalbuminemiya, a giperglobulinemiya, a hypercholesterolemia, increase in activity alkaline phosphotazy, a giperbilirubinemiya, an electrolytic imbalance (a giponatriyemiya and a gipokaltsiyemiya), increase in content of fibrinogen and b-lipoproteidov. In the general blood test - , anemia, increase in SOE. At a koprogramma research the expressed steatorea degree ("a fat chair"), kreatore (considerable contents in excrements of muscle fibers), amilore (presence in Calais of a large amount of starch) quite often comes to light.
Due to the expressed metabolic violations at an amiloidoza of kidneys on the ECG arrhythmia and violations 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 method of diagnostics of an amiloidoz serves the kidney biopsy. The morphological research of a bioptat after coloring of Congo red at the subsequent electronic microscopy in the polarized light reveals the green luminescence characteristic of an amiloidoz of kidneys. Amiloid can come to light on the course of vessels, tubules, in balls. The biopsy of a mucous rectum, skin, gum, liver is in certain cases made.
Treatment of an amiloidoz of kidneys
In therapy of a secondary amiloidoz of kidneys a considerable role is played by success of treatment of the main disease. In case of a full resistant izlechivaniye of primary pathology also symptoms of an amiloidoz of kidneys quite often regress. Patients with amiloidozy 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 medicines for treatment of an amiloidoz of kidneys are the derivative 4 aminoquinolines () desensibilizing means (, , ), colchicine, , etc. Symptomatic therapy at an amiloidoza of kidneys includes purpose of diuretics, hypotensive medicines, transfusion of plasma, albumine etc. Expediency of use of corticosteroids of tsitostatik is discussed. In a terminal stage of an amiloidoz of kidneys chronic kidney dialysis or transplantation of a kidney can be required.
Forecast and prevention of an almiloidoz of kidneys
The forecast in many respects is defined by the course of the main disease and speed of progressing of an amiloidoz of kidneys. Development of thromboses, hemorrhages, accession of interkurrentny 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 diagnostics of an amiloidoz of kidneys, active treatment and full elimination of the main disease.
Prevention of an amiloidoz of kidneys demands timely treatment of any chronic pathology which can cause development of an amyloid nefroz.