AMYLOIDOSIS

From Big Medical Encyclopedia

Amyloidosis (amyloidosis; Greek amylon — starch, eidos — a look + - Usis), amyloid dystrophy — the disturbance of protein metabolism which is expressed in adjournment and accumulation in fabrics of proteic matters with characteristic physical. - chemical properties. Etiologically and pathogenetic combines the different processes leading to education in fabrics of a difficult glucoproteid — amyloid.

To studying And. descriptions Rokitansky (S. promoted Rokitansky, 1844) «a grease disease» and Virkhovy (R. Virchow, 1853) amyloid, and also creation of a pilot model of an amyloidosis by N. P. Kravkov and Kuchinsky (M. of N. Kuczynski). The casein model A is most widespread., received by introduction to mice or rabbits subcutaneously of 5 — 10% of a suspension or solution of caseinate of sodium. However experimental And. it is possible to cause also by means of culture of golden staphylococcus, pyocyanic and diphtheritic sticks, a gonokokk, a cholera vibrio, turpentine, proteolytic enzymes, colloidal solutions of sulfur, selenium, etc.

Distribution And. in various countries it is unequal. Its frequency in Spain — 1,9% of openings, in Portugal — 1,4%, Israel — 0,55%, in Japan — only 0,1% that some authors are inclined to explain with feeding habits of the population.

The most frequent localization And. — kidneys where it is found, by data A. A. Dyomina (1970), in 1,4% of cases, according to G. P. Shultsev (1970), covering section observations 60 — the 70th years — in 1,9% of cases.

Amyloid and its properties

Amyloid has complex structure. Its main component are proteins among which both fibrous (fabric) proteins like collagen, and plasma proteins — α-and γ-globulins are found, fibrinogen. Polysaccharides of amyloid are presented by chondroitinsulphuric and hyaluronic acids, heparin, neuraminic to - that, and hondroitinsulfata prevail. Amyloid has antigenic properties; it is steady against effect of many enzymes, acids, alkalis thanks to durability of bonds between proteinaceous and polisakharidny components.

The chemical composition of amyloid is not identical at various forms and types A., than its various relation to dyes (congo red, marked - or a methylrosanilinum chloride, iodine and iodine-gryun) and different intensity of characteristic metachromatic reaction speaks; in some cases this reaction is absent (achromatic amyloid, or akhroamiloid, paraamyloid). The most specific to amyloid is its luminescence with thioflavin S or T.

Fig. 1. Amyloidosis of a spleen. Positive anisotropy of amyloid deposits

Amyloid has a fibrillar paracrystalline structure. In this regard it possesses a dichroism and anisotropy (see); the last most clearly is expressed during the coloring to congo red (fig. 1). The range of positive double refraction of amyloid lies within 540 — 560 nanometers. These polarizing and optical properties of amyloid allow to distinguish it from collagen, reticuline, elastin.

Fig. 2. Fibrilla of amyloid (1) on the periphery of reticular macrophages (2) in connection with invaginations of a cytoplasmic membrane (culture of fabrics; × 53000)

The fibrilla of amyloid revealed at an elektronnomikroskopichesky research has diameter of 7,5 nanometers and length to 800 nanometers; they are deprived of cross striation. Each fibrilla consists of two subfibrilla (filaments) on 2,5 nanometers in the diameter which are located in parallel at distance of 2,5 nanometers from each other. The intracellular fibrillogenesis of amyloid, and also participation in a fibrillogenesis of mesenchymal cells — a reticular macrophage, a fibroblast (fig. 2) is established that allowed to consider amyloid fibrous abnormal protein.

Unlike collagen, structural proteins of fibrilla of amyloid are rich with tryptophane and do not contain hydroxyproline, are connected with a small amount of neutral Sugars and sialine to - you.

Structural chemical properties of fibrilla define specific coloring of amyloid congo red, molecules to-rogo strongly are kept between filaments of fibrilla by hydrogen bindings of sulfonate groups of dye with basic groups of protein.

In addition to fibrilla, in amyloid specific rhabdoid structures («periodic sticks», or the R-component) with a diameter of 10 nanometers and up to 400 nanometers long are allocated. They consist of the separate pentagonal educations with a diameter of 9 — 10 nanometers located from each other at distance of 4 nanometers. Each such education is presented by five triangular components to 2,5 nanometers in the diameter. Rhabdoid structures treat glucoproteids of a serumal origin, in them in comparison with fibrilla of amyloid there are much higher than contents of neutral Sugars and sialine to - you; they define antigenic properties of amyloid.

Etiology and pathogeny

Aetiology and pathogeny And. are not quite clear. Three main theories of a pathogeny are discussed And.

I. The theory of local cellular genesis of Taylum (G. Teilum, 1954) explains amiloidogenez only at the level of a cell. At the same time it is about synthesis by a cell of a reticuloendothelium not of amyloid — the complex heterogeneous substance consisting of fibrillar and not fibrillar structures, and only his fibrillar predecessors. Amyloid is formed out of a cell in close connection with fibrous structures of connecting fabric. According to this theory in an amiloidogeneza it is possible to allocate two phases: before amyloid and actually amyloid. Before emergence of amyloid in reticuloendothelial system proliferation and plazmotsitarny transformation with formation of pironinofilny cells, rich RNA is observed. In actually amyloid phase there is a suppression of proliferation of cells of a reticuloendothelium, exhaustion of pironinofilny cells, emergence of the cells rich with polysaccharides (PAS cell) which «build» amyloid substance. The theory of «local cellular genesis» explains a number of the facts known from kliniko-experimental observations. From positions of this theory it is possible to explain, in particular, selectivity of defeat of elements of reticuloendothelial system at secondary And., first of all areas, the most active in the functional relation: marginal zones of follicles of a spleen, sinusoid of a liver. An essential argument in favor of the theory of local cellular genesis are works in which formation of amyloid in culture of fabric is convincingly shown. However proofs of an intracellular fibrillogenesis of amyloid a cell of a reticuloendothelium do not resolve (PAS cell) an issue in favor of recognition of the theory of «local cellular genesis» universal.

II. According to the immunological theory of Leshke — Letterera (N. of Loeschke, E. Letterer, 1927) formation of amyloid is considered as result of reaction antigen — an antibody where antigen — decomposition product of fabrics or a foreign protein, and amyloid — the proteinaceous precipitated calcium superphosphate which is laid first of all in places of antibody formation. Amyloid arises on condition of bad development of antibodies and excess of antigen. In favor of the immunological theory testify a hypergammaglobulinemia in before an amyloid stage, falling of immunoglobulins during formation of amyloid, the nature of morphological changes in bodies etc. However this theory does not explain cases of development And. at persons with hypo - and an agammaglobulinemia. Participation of immunologic mechanisms in development And. demands further studying.

III. The theory of a disproteinoz, or Kagli's organoproteidoz (V. Cagli, 1961), considers amyloid as a product of the perverted protein metabolism. From positions of this theory a fundamental unit in a pathogeny And. — a disproteinemia with accumulation in plasma of coarse-dispersion protein fractions and abnormal proteins. The fibrinosis can be also a condition of formation of amyloid.

Pathological anatomy

1. Amyloidosis of a spleen. Adjournment of amyloid on the periphery of a follicle (coloring by red Congo)
2. Adjournment of amyloid in a mozgovomveshchestvo of a kidney (coloring by red Congo)
3. Adjournment of amyloid in kapillyarnykhpetlyakh a ball of a kidney (coloring by red Congo)

Amyloid deposits have typical localization in walls circulatory and limf, capillaries and vessels, in an intima or in an adventitia; in a stroma of bodies for the course of reticular or collagenic fibers; in own cover of ferruterous structures.

Depending on the relation of amyloid to various cells (a reticular macrophage, a fibroblast) or various fibers of connecting fabric (reticular, collagenic) among which amyloid drops out, distinguish periretikulyarny and perikollagenovy And.

For periretikulyarny And., at Krom amyloid drops out on the course of the membranes of vessels and glands, and also a reticular stroma of parenchymatous bodies containing reticuline, preferential damage of a spleen, liver, kidneys (see color fig. 1 — 3), adrenal glands, intestines, an intima of vessels of small and average caliber is characteristic (so-called parenchymatous, typical And.). For perikollagenovy And., at Krom amyloid drops out on the course of collagenic fibers, preferential defeat of an adventitia of vessels of average and large caliber, a myocardium, cross-striped and smooth muscles, nerves, skin is characteristic (so-called mesenchymal, system And.).

The insignificant deposits of amyloid which are coming to light only at microscopic examination usually do not lead to functional disturbances (clinically latent stage And.) also do not change outward of bodies.

Fig. 3. Amyloid contracted kidney. Amyloid (light sites) in capillary loops of a ball (a luminescence with thioflavin T)

Progressing And., as a rule, leads to functional insufficiency of body that is connected with an atrophy of its parenchymatous elements and a sclerosis. The body increases in volume, becomes to dense t fragile, has a peculiar wax-like or grease appearance on a section («a grease spleen», «a wax-like liver»). In the final amyloid wrinkling of body, e.g. amyloid wrinkling of kidneys (fig. 3) develops. Thus, variety of origins And. and mechanisms of formation of amyloid does low-justified searches of the uniform theory of an amiloidogenez. No, obviously, a uniform amyloidosis as a kliniko-morphological concept, there are amyloidoses. The competing theories And. explain only separate links of its pathogeny (humoral, fabric, cellular).

However there is a number of signs, the general for all forms A. Treat them: the disproteinemia which is expression of the broken protein metabolism and process of updating of proteins of an organism; transformation of cells of reticuloendothelial system with emergence of a fibrillar structure of amyloid; the typical submicroscopic changes preceding emergence of amyloid; uniform submicroscopic structure of amyloid.

Standard classification And. does not exist. Lyubarsh (O. of Lubarsch, 1929) and Reymann (N. of A. Reimann, 1935) allocated primary (idiopathic) and secondary amyloidosis. Briggs (G. W. Briggs, 1961) distinguishes the following types And.: 1. Primary: a) generalized; b) family; c) respiratory — tumorous (knotty) and diffusion. 2. Secondary. 3. Senile cardial. 4. And. at a multiple myeloma. 5. Tumorous local And. (excepting respiratory).

According to Heller (N. of Heller, 1966), three groups A exist., in each of which a number of forms is allocated:

I. Genetic (hereditary) And.: 1) family Mediterranean fever (periodic disease); 2) family Ampere-second fever, urticaria and deafness (Makkl's form — Wells); 3) neyropaticheskiya with preferential damage of the lower or upper extremities; 4) kardiopaticheskiya.

II. Acquired And.: 1) as complication hroya. infections (bronchoectatic disease, tuberculosis, osteomyelitis), collagenic diseases (pseudorheumatism, etc.), malignant tumors; 2) as manifestation of a multiple myeloma.

III. Idiopathic And.: 1) classical primary; 2) nefropaticheskiya; 3) neyropaticheskiya; 4) kardiopaticheskiya; 5) localized.

At various forms in each group the type of adjournment of amyloid (periretikulyarny or perikollagenovy) can be a miscellaneous.

Heller's classification in comparison with Briggs's classification is more progressive. It outlines group idiopathic more strictly And., including in it, in addition to a generalized form primary And., its various clinical options with preferential damage of kidneys, a nervous system, heart. Allocation in independent group genetic is justified And., the origin to-rogo is connected with the hereditary fermentopatiya defining defect of synthesis of fibrous proteins of an organism. Among forms hereditary And. the most widespread is And. at a periodic disease (family Mediterranean fever), especially often described at Armenians, Jews, Arabs and inherited on autosomal recessively type. At the same time And. can be the only manifestation of this genetically caused suffering (the II phenotype) or to develop after the period of fever and attacks of pains (the I phenotype), leading in either case to a renal failure. Allocate also so a groove. the Portuguese amyloidosis proceeding with preferential peripheral neuropathy and which is transferred on dominant type and the Ampere-second preferential damage of heart (kardiopaticheskiya) which is coming to an end with death from heart failure.

Gaffney (J. Gafni, 1964) reduces all variety hereditary And. to three kliniko-morphological types (forms): 1) nefropaticheskiya, shown a proteinuria, a nephrotic syndrome and uraemia in the final of a disease; 2) neyropaticheskiya, characterized by the progressing polyneuritis with a muscular atrophy, impotence, intestinal frustration and a cachexia; 3) kardiopaticheskiya, the accruing heart failure is inherent to Krom.

The frequent reason acquired (secondary) And. are hron. specific infections (tuberculosis, syphilis), especially hron. suppurations (osteomyelitis, a bronchoectatic disease), a pseudorheumatism, are more rare nonspecific ulcer colitis, a lymphogranulomatosis, tumors («cancer-amyloidosis»).

In group secondary And. refer as well the perikollagenovy deposits of so-called paraamyloid at a multiple myeloma and Valdenstrem's macroglobulinemia connected with the perverted synthesis of globulins tumor cells.

It is shown that in development secondary And., complicating hron. infections, collagenic diseases, hron. a glomerulonephritis, the role of immunological (autoimmune) reactions is big. The mechanism of formation of paraamyloid comes down to adjournment in fabric of a paraprotein which reacts protein — protein or protein — polysaccharide that conducts to precipitation of insoluble paraamyloid.

Localized (local) And., which is carried to primary, has some features. Dropping out in a mucous membrane of a nose, a throat, phonatory bands, a trachea and bronchial tubes, in a wall of a bladder and ureters, in cellulose a century and language, and also skin, amyloid forms tumorous structures (tumorous And.).

Heller's classification should be added senile And. (group acquired And.). Most often occurs at old people And. a brain, heart and islets of Langerhans that, on Shvarttsa (P. Schwartz, 1970), makes the characteristic pathoanatomical triad causing senile mental and physical degradation. Amyloid deposits in other bodies and tissues of old people meet much less often. Shvartts considers a senile amyloidosis as a disease of age and considers that old people have an undoubted communication And., atherosclerosis and diabetes which combine uniform metabolic disturbances.

Clinical picture

Clinical picture A. it is diverse and depends on localization and intensity of amyloid deposits in bodies, duration of a disease, existence of complications. Especially demonstrative it becomes at damage of kidneys, hearts, a nervous system, intestines.

An amyloidosis of kidneys

Kidneys are surprised often as at secondary And., and at primary. Gradual distribution of amyloid deposits with involvement of a vascular wall leads to the accruing proteinuria with emergence further of a nephrotic syndrome, decrease in a renal blood-groove, glomerular filtering, development of a nephrogenic hypertension and an azotemia. At the same time can be observed, and sometimes and to prevail manifestations of the basic disease which brought to And. — a pseudorheumatism, a periodic disease, a lymphogranulomatosis, etc. that complicates recognition And.

Patients And. kidneys complain of the general weakness, a loss of appetite, hypostases, often for the first time arising standing; further they extend on all body, complicate breath, digestion, a mocheotdeleniye. There are pains in a waist, especially sharp and acute, at fibrinferment of renal veins. The arterial hypertension, a renal failure develop.

At And. the oliguria of the period of big hypostases can be replaced in a stage hron. a renal failure a polyuria, but quite often the oliguria together with hypostases remains also in the terminal period of a disease. Sometimes there is diarrhea. At considerable defeat of distal tubules excess allocation with urine of sodium and water can be observed, just as at And. adrenal glands (nephrogenic insipidarny syndrome), and canalicular metabolic acidosis.

For And. kidneys it is characteristic proteinuria (see), developing at all forms of a disease, but the most expressed at secondary And. At the same time from 2 to 20 also even 40 g of protein, generally albumine and in smaller quantities — globulins, glycoproteins, especially α1-гликопротеидов, etc. are allocated per day. The considerable proteinuria remains at development of chronic and even terminal renal failure.

Long loss of protein kidneys, it is frequent together with its smaller reception with food lowered by absorption, and sometimes and its strengthened removal through went. - kish. a path as well as the raised proteinaceous catabolism, leads to a hypoproteinemia and hypostases.

As a rule, hypostases develop early enough and gain widespread and persistent character. Decrease in content in blood of albumine and increase α2-and γ-globulins — — the disproteinemia strengthened and a basic disease (active tuberculosis, an aggravation of a pseudorheumatism) is especially continuous.

Increase in maintenance of glycoproteins and lipoproteids respectively in α1-and β-fractions with simultaneous decrease in glycoproteins in albumine takes place, the content of serumal immunoglobulins changes and the caption of a complement decreases. Often sharply ROE accelerates and sedimentary tests change. The lipidemia with the increased content of cholesterol and considerable dominance of β-lipoproteids of blood is characteristic. The hypercholesterolemia remains usually and at the exhausted patients (e.g., at cavernous tuberculosis), just as is frequent also in an uraemic stage together with sharply expressed proteinuria and hypostases.

The combination of a massive proteinuria, a hypoproteinemia, a disproteinemia, a hypercholesterolemia and hypostases makes very characteristic for And. kidneys the classical nephrotic syndrome observed on average at 60% of patients after quite often very long latent stage of a moderate proteinuria. Emergence of the developed nephrotic syndrome can be provoked by an intercurrent infection, cooling, an injury, vaccination, medicinal influences, an aggravation of a basic disease.

At And. also the fibrinosis, hypercoagulation meets development of thrombosis of renal veins, etc. It is possible to find also a leukocytosis in blood, anemia, a marrowy plasmocytosis, a syndrome of nephrogenic diabetes.

Rather often the resistant microhematuria, and sometimes and a gross hematuria as well as a leukocyturia, without the accompanying pyelonephritis comes to light. Find also a lipoiduriya with existence of birefringent crystals in an urocheras.

According to a number of clinical physicians, the most typical for And. kidneys transition of the early, bezotechny period with an insignificant proteinuria in an edematous stage, and then during the cachectic or uraemic period is. And. kidneys repeats in general evolution of a classical disease of Brayt.

Stages hron. renal failure (see) all main lines of disturbance of concentration and azotovydelitelny function of kidneys — falling of specific weight of urine, decrease in glomerular filtering, a delay of removal of creatinine and other nitrogenous slags are inherent. The contracted kidney can be revealed also by means of the survey roentgenogram, scanning, a technique of pneumoren.

Arterial hypertension at And. develops more often in parallel with a resistant azotemia, anemia, i.e. in a final stage of a disease as manifestation of diffusion damage of kidneys, but maybe at the beginning of it that is connected with defeat of vessels of kidneys.

Long existence of arterial hypertension leads to a hypertrophy of a left heart with development of heart failure. Vessels of an eyeground are involved. However a malignant hypertension with a blindness and sharply changed ECG at And. it is rare. At And. also the collapse as a result of accession of infectious and trombotichesky complications or the progressing arterial hypotension is possible owing to And. adrenal glands.

An amyloidosis of heart

At secondary And. damage of heart meets seldom, and at primary, including at family, idiopathic and far come senile And. — at 80 — 100% of patients. Clinically And. hearts it is shown in the form of the restrictive myocardiopathy which is characterized by the moderate increase in weight (which is not finding other pathogenetic explanation) and the progressing heart failure quickly enough becoming refractory to treatment cardiac glycosides. On an ECG note a reduced voltage of QRS complexes, various disturbances of endocardiac conductivity and other signs of damage of a myocardium. Find a proteinuria in many patients, considerably big, than at a congestive kidney. Quite often the hypoproteinemia, a hypoalbuminemia and a hyperglobulinemia at the normal content of cholesterol in blood serum comes to light. Except the progressing heart failure, at And. hearts are possible (as rare syndromes) chronic cardial compression, defeat of an endocardium. Sometimes amyloid is laid in walls of intramural coronary arteries, narrowing their gleam; it can be shown by corresponding changes of a myocardium on an ECG, including and focal character.

A number of authors revealed Amyloidosis of a thyroid gland. Externally condensed and increased thyroid gland at the same time reminds Hashimoto's thyroiditis (see. Hashimoto disease ).

The amyloidosis of digestive tract

Data on the frequency of involvement went. - kish. a path in And. are contradictory. Frequency of defeat went. - kish. a path at And. it is difficult to define, t. to, some symptoms are connected not with actually And., and with those diseases on the basis of which it developed.

Disturbances from outside went. - kish. a path (abdominal distention, a loss of appetite, dysfunction of intestines) there can be already in an initial stage A. Pokazatel defeats of capillaries of intestines the increased removal from blood of albumine, marked is I 131 , steatorrhea.

A sprue at And. it is described often and it is connected with adjournment of amyloid in an internal cover of intestines (see. Malabsorption syndrome ). Ponosa, resulting from intestinal dysbacteriosis, and also nephrotic hypostasis of a mucous membrane of intestines and its uraemic changes, considerably worsen a condition of patients since lead to further decrease in food, changes in water and electrolytic balance. Quite often noted locks are connected with development And. in a wall of intestines that leads to its thickening and loss of motor function.

These rektoromanoskopiya do not give a specific picture; histologic studying of a biopsirovanny mucous membrane and finding of amyloid — more valuable diagnostic indicators. In literature a number of cases with sharply expressed symptomatology of defeat is described went. - kish. path. The macroglossia which is followed concerns to them dysarthtia (see), dysphagy (see), difficulty of the act of chewing. Cases of Ampere-second by signs of involvement of a gullet, stomach, with the expressed disturbances of their motility, with pains in «epigastric» area, nausea, anorexia, vomiting are described. Massive deposits of amyloid can lead to obturation of a gleam of vessels, local heart attacks, ulcerations with bleeding. It is known that on the frequency of defeat the liver is the third body (after a spleen and kidneys) at And. An essential difference in character of a patotopografiya of amyloid in a liver and in clinical manifestations at various types A. no. The hepatomegalia is considered one of pathognomonic symptoms. As a rule, the liver happens plotnovaty to the pointed edge, painless at a palpation.

Gepato-and a splenomegaly can be connected as well with features of a course of those diseases at which And. developed (e.g., a pseudorheumatism, a septic endocarditis, heart failure). And. the liver usually is not followed by functional disturbances: casuistic jaundice and portal hypertensia are rare; activity of enzymes (including transaminases), test with bromsulfaleiny does not change and skanogramma are not changed. At involution And. the liver can be reduced to the normal sizes. The puncture biopsy of a liver has diagnostic advantages before other methods.

Amyloidosis of a pancreas glands it is a little shown. Sometimes it is possible to find a passing small glucosuria and changes of activity of some enzymes in such patients — trypsin, etc.

Defeats of a nervous system at an amyloidosis

the Expressed neurologic symptomatology is inherent to separate forms genetically caused And., when the clinical picture reminds heavy symptomatology of a multiple sclerosis and includes early loss of a thermoesthesia, disturbance of a mocheotdeleniye, impotence, the progressing paralyzes of the lower extremities, the extending trophic frustration. At secondary And. defeat of a nervous system usually arises in a terminal (uraemic) stage of a disease.

Hereditary And. it is allocated only in recent years, and to a crust, there is no time integral idea of a clinical picture of a disease. There are attempts to combine hereditary forms in groups (see above).

An amyloidosis primary urinogenital bodies

In addition to systemic lesion And. kidneys — secondary (owing to any hron, patol, process), meets primary And. urinogenital bodies — idiopathic, localized, on Heller's classification (N. of Heller, 1966).

Its first descriptions were made at the end of 19 century by pathologists. Hvalla (R. Chwalla, 1932) for the first time distinguished clinically primary

And. bladder and prostatic department of an urethra.

By 1976 in literature it is described apprx. 180 observations primary And. bodies of urinogenital system. It was found in a renal pelvis, an ureter, a bladder, an urethra, seed bubbles, a prostate, a small egg, a penis. Most often the bladder and seed bubbles are surprised. The age of most of patients is more senior than 60 years; more often men get sick.

An etiology localized primary And. it is in many respects unknown. Some researchers emphasize value of a senile factor as the frequency of this disease considerably increases with age; other authors note a frequent combination of a tumor and And. (V. V. Serov, A. Yu. Gritsman, 1975).

Diagnosis is based on tests with dyes (congo red, methylene blue, etc.) though results of these tests are changeable, and also on a biopsy.

Conservative treatment primary And. urinogenital bodies do not exist. The nature of surgery depends on localization of process.

The forecast after operation is usually favorable.

Primary amyloidosis of a renal pelvis meets seldom. Clinically it is shown by a pyuria, a hamaturia. Function of a kidney is broken, at bilateral defeat the renal failure can develop. On roentgenograms according to an arrangement of kidneys find shadows, suspicious on stones (in connection with calcification of amyloid deposits) and defects of filling. The diagnosis to a thicket is established later nephrectomies (see) or on autopsy. Find consolidation and a thickening of the wall covered by kroshkovidny deposits in a pelvis. Except a nephrectomy, also organ-preserving operation — removal of the calcific amyloid center can be executed from a pelvis and nephrostomy (see) what G. D. Chisholm et al. reported (1967) about.

Primary amyloidosis of an ureter also meets seldom. The disease can develop at any age, a thicket at women, affects exclusively pelvic department of an ureter.

Diagnostic characters: pain in lumbar area and a hamaturia; radiological — defect of filling or is defined a stenosis of an ureter above which a megaloureter and a pelvis. At a tsistoskopiya — a picture of hemorrhagic cystitis and puffiness of a mucous membrane of a bladder in a circle of the mouth of the struck ureter. Differential diagnosis should be carried out with a tumor, endometriosis, tuberculosis of an ureter. During operation find sharp consolidation and a thickening of the struck segment of an ureter. Intervention consists in a resection of an ureter and imposing of an ureterotsistoanastomoz — direct or by Boari's method (see. Boari operation , Ureterotsistokeostomiya ).

Primary amyloidosis of a bladder was described for the first time by Solomin (P. Solomin, 1897) who found at autopsy in a bladder «tumor», edges at gistol, a research it was amyloid.

In domestic literature this disease was described by Ch. A. Sinkevichus and L. Yu. Lyutkus (1966). In the world literature St. 80 observations are published. Amyloid is laid preferential in a submucosal layer of a bladder, in walls of vessels and among muscle fibers. Macroscopically — the hilly education with an ulceration of a mucous membrane which is very reminding a malignant new growth.

The most frequent symptom — the hamaturia caused by adjournment of amyloid in walls of the blood vessels losing ability to reduction. The dysuria, dull aches in lumbar area are less often observed. At a tsistoskopiya — the hilly tumorous formations of yellow color which are gradually getting dark red coloring and exposed to necrotic changes in the center with a hemorrhagic zone for a circle. On the roentgenogram — the defect of filling characteristic of a new growth.

At most of patients mistakenly make the diagnosis of cancer of bladder, is more rare than cystitis, endometriosis, a malakoplakia, tuberculosis, a hemangioma.

The main role in recognition of a disease is played by a biopsy (including and endovesical), and during operation — gistol, a research of a wall of a bubble.

Treatment surgical: segmented cystectomy. Occasionally — at total damage of a bladder And. — make cystectomy (see).

Primary amyloidosis of an urethra. Amyloid is laid under an epithelium of front department in the form of the small tumorous nodes narrowing a gleam of an urethra. Symptoms of a disease, as well as change on an uretrogramma, are similar to symptoms of a tumor of an urethra. In some cases the disease is taken for cancer and only the biopsy clears up the diagnosis. Most efficiently treatments the curettage is [Tripati, Dezotels (V. N. Tripathi, R. E. Desautels), 1969].

The amyloidosis of a prostate was considered an accidental pathoanatomical find earlier, however it meets also in a wedge, practice. So-called colloid corpuscles of a prostate (corpora amylacea prostatae) of any relation to And. have no and should not be taken into account at diagnosis. Amyloid is laid in an epithelium of channels of a prostate or in blood vessels; defeat of vessels at patients of advanced age can be the cause of severe bleeding at prostate gland operations.

The main diagnostic method of a disease — a biopsy, and the most reasonable way of treatment — a transurethral resection of gland (see. Adenoma of a prostate ).

Primary local amyloidosis of seed bubbles. Adjournment of amyloid usually considerable also gains tumorous character. The disease proceeds asymptomatically more often, but emergence of a hemospermia at advanced and senile age (in the absence of other reasons causing it) can be explained And. seed bubbles.

It is necessary to differentiate Ampere-second a tumor, tuberculosis, nonspecific inflammatory process in seed bubbles. Symptomatic treatment. Spontaneous regression patol is seldom observed. changes.

Primary amyloidosis of a small egg meets seldom. It is necessary to differentiate it with a tumor that is reached by means of a biopsy of a small egg.

Treatment — an orkhiektomiya or resection of a small egg (see).

Primary amyloidosis of a penis — extremely rare disease (only one observation with localization of process on a head of the member is described).

A cerebral senile amyloidosis

the Concept is entered in patol, anatomy of a brain of P. Divry in 1942.

The most important stage of studying of a senile atrophy of a brain was opening in 1892. P. Blocq and G. Marinesku of the vessels of a cerebral cortex of small small knots or roundish little bodies disseminated around to dia. apprx. 60 microns. They received the name of senile plaques. Amyloid nature of senile plaques and other manifestations And. a brain P. Divry opened and studied. It showed that senile plaques possess all physical. - chemical properties of amyloid, are connected with a wall of small vessels. Are allocated meningovaskulyarny and cerebrovascular senile And., and also senile And. ependymas of ventricles and vascular textures.

At a submicroscopy of the biopsy material taken from a brain by means of a needle the fibrilla of amyloid similar to that are found in vessels and senile plaques at the earliest stages of their development at secondary, primary and experimental And.

The mass of fibrilla of amyloid are found in the center of plaques vnekletochno and in adjacent cells of a microglia that allows to assume their participation in products of protein of amyloid fibrilla. The ultrastructure of altsgeymerovsky balls of neurofibrilla is presented by the so-called twisted tubules. The origin of these educations remains not clear (change of microtubules, formation of new protein), but communication with And. it is rejected.

P. Schwartz does not carry cerebral senile And. to secondary or primary, including it an independent form with preferential damage of a brain, heart, vessels and a pancreas (a so-called triad of P. Schwartz).

For cerebral senile And. the atrophy of a cerebral cortex and his edema is characteristic. The weight of a brain considerably decreases. An edema results in translucency of a funnel of a brain. Sharply expressed cachexia is noted. Survey colourings (hematoxylin-eosine and across Nissl) reveal rare pink and blue specks of plaques. Amyloid comes to light by means of red Congo in alkaline solution with the subsequent studying of drugs in the polarized light; by a selective method for identification of amyloid consider a flyuorokhromirovaniye of cuts thioflavin S or thioflavin T and tiazinovy red on Haytingera and to the Geyser. Communication of senile plaques with capillaries well comes to light by means of impregnation of cuts across Snesarev.

The forecast

the Forecast depends on the nature of a disease, at Krom developed And., the complications connected both with a basic disease and with And. or with the carried-out treatment (use of corticosteroids). Existence of a widespread lymphogranulomatosis, inoperable tumor, myeloma defines a zlokachestvennost of a current and a lethal outcome And. already in the nearest future after emergence of a proteinuria.

Diagnosis

Diagnosis primary system, hereditary And. it is based on the careful genetic analysis. Existence of a disease at other family members or in several generations resolves an issue in favor of the hereditary nature of a disease. Senile And. has polymorphic clinical manifestations depending on localization of deposits of amyloid and dysfunction of bodies.

Emergence and progressing of a proteinuria, emergence of a nephrotic syndrome or a renal failure when the essence of a nephropathy remains not clear or when there are persistent heavy heart failure, a neuropathy, not explainable other reasons, force to think first of all of A.

Veroyatnost of the diagnosis And. increases at detection gepato-and splenomegalies.

The most reliable diagnostic method And. the biopsy of bodies (kidneys, a mucous membrane of a rectum, a gingiva, limf, nodes, a liver) promoting recognition and a basic disease admits, at Krom developed And. (tuberculosis, malignant tumor). The biopsy of kidneys possible to find amyloid in a medullary layer for several years prior to the beginning of a proteinuria that allowed to allocate the preclinical period of a disease and to put forward a question of timely prevention of an amyloidosis.

Treatment

Treatment And. — it most often treatment of the disease of kidneys revealed in a stage of a nephrotic syndrome. At sufficient azotovydelitelny function of kidneys the full-fledged feeding schedule with restriction of sodium chloride is appointed, the broken balance of electrolytes is regulated, diuretic and hypotensive drugs are carefully used. In the period of a renal failure all arsenal of the means applied at uraemia is used. In a stage of a terminal renal failure treatment is shown by a hemodialysis. Further perspectives of treatment And. open using transplantation of kidneys.

The available data on a pathogeny And. allow to plan the next main ways of treatment: 1) elimination of the factors promoting formation of amyloid; 2) braking of products of amyloid; 3) the influences leading to a rassasyvaniye of amyloid.

It is necessary to emphasize importance of active treatment of a basic disease, at Krom A. Eto developed belongs to secondary And. at hron. infections and suppurative processes.

Removal of the center hron. suppurations, use of high doses of antibiotics and streptocides sometimes so improve a condition of the patient and indicators of functions of kidneys that it is possible to speak about involution And. E.g., removal of a malignant tumor of kidneys led within 20 months to reduction of the sizes of an amyloid liver (the diagnosis was confirmed histologically). Active treatment of a long septic endocarditis caused the rassasyvaniye of amyloid in tissue of a liver stated at repeated biopsies.

It is possible that by a pathogenetic method of influence on developing And. prolonged use of a crude liver and drugs of a 4-am1shokhinolinovy row serves. It is about additional administration of some amino acids, enzymes, and synthetic antimalarial means, such as Chingaminum (delagil), influencing function of a reticuloendothelium, promote braking of education, and it is possible, and resorptions of already dropped out amyloid.

Introduction of corticosteroids, tsitostatik and antplimfotsitarny serum at And. it is not shown.

Amyloidosis of skin

And. skin — adjournment of amyloid masses in various layers of skin. Distinguish And. skin localized and system, primary and secondary.

Primary localized amyloidosis of skin (only skin is affected) has the following versions:

5. Amyloid lichenins

1. Amyloid lichenins — lichen amyloidosus (Freudenthal) or amy-loidosis cutis localis nodularis et dissemuiata (Gutmann) — the numerous, small, closely located, but not merging, dense, partially brilliant, semi-translucent, conic or flat small knots of color of normal skin or cyanotic-violet. Are localized on an extensor surface of extremities, it is preferential in shins (tsvetn. fig. 5). The disease is followed by a painful itch. As a result of constants having combed there is sharply expressed likhenization and quite often cicatricial atrophy of the affected skin («a biopsnruyushchy itch»).

2. Spotty And. skin — amyloidosis maculosa (Palitz, Peck) — the single or multiple, roundish or linear, slightly towering pinkish-brown color spots of the small sizes which are localized more often on skin of extremities. The itch is not constant.

3. Tumorous And. skin — tumorous formations of various sizes, single or multiple, are located in a derma and a hypoderma, a thicket in a face, a breast, genitalias. Skin over them of an atrofichn, easily gathers in folds, reminds tissue paper; on it bubbles with a sluggish tire and transparent contents can be formed.

Are ill primary localized And. skin are more often elderly men, cases in several generations are known.

The secondary localized amyloidosis of skin — adjournment of amyloid in the skin affected before with other dermatosis (seborrheal warts, keratoza, ulcers trophic and piodermichesky, Bowen a disease, etc.).

Primary System amyloidosis of face skin
Primary System amyloidosis of skin

Primary system amyloids of skin — amyloidosis cutis metabolica (Königstein) — adjournment in skin of amyloid at the general Ampere-second damage of internals (see above). It is characterized by emergence on pale, farforopodobny skin of various rashes: a) smooth wax-like small papules, skin color or yellowish, on a face, especially in eye-sockets, a neck and genitals (tsvetn. fig.), it is frequent with a hemorrhagic component; b) smooth dense yellowish plaques of various sizes, is more often on skin of extremities; c) nodes and tumorous educations with a preferential arrangement on a trunk; d) purpuras (petechias, ecchymomas), as a rule, on a face, a neck, mucous membranes. Besides, can be observed an alopecia, usually universal; dystrophy of nails (nail plates become dim, fragile, the anonychia is possible); a white acanthosis (Soreau) — warty growths and a thickening of skin pleated, similar acanthosis nigricans (see), but hypochromia; erythrosis of a yellowish-red shade [«the orange person» (Gougerot, Grupper)], makrokheyliya, macroglossia, xerostomia. The itch usually is absent. Secondary system And. skin meets extremely seldom.

Histopathology. Amyloid masses can be postponed in a papillary layer of a derma, directly under epidermis (amyloid lichenins, a spotty amyloidosis of skin) or to be located diffuzno in a derma and a hypoderma, around sebaceous glands and hair bags, striking deep vessels of a derma on perikollagenovy type (see above Primary system amyloidosis of skin).

For confirmation of the diagnosis And. skin use test with congo red (Markionini — Jonah) for what enter 1,5% into the affected skin solution of congo red (1 ml subcutaneously or 0,1 ml vnutrikozhno). Sites with adjournment of amyloid in 24 — 48 hours clearly are also with firmness painted in red color, and surrounding skin is painted very poorly.

Current And. skin chronic. The forecast at the localized forms favorable, at system And. skin — depends on extent of damage of internals. To differentiate And. skin follows with neurodermatitis, by red flat it is deprived, a scleroderma.

Treatment is quite often ineffectual. The concentrate of vitamin A, antimetabolites, diathermocoagulation, a roentgenotherapy are recommended. Outwardly appoint zhelatino-zinc bandages.

An amyloidosis at children

the General And. children are ill seldom, it almost does not occur at newborns. At children of preschool and school age And. develops as a complication (secondary And.) less often out of communication with any disease (primary).

In urine urobilin, insignificant proteinuria. Anemia, leukopenia, thrombocytopenia. Increase in zymohexase, transaminases, the changed sedimentary tests. Gipoalbumiyaemiya, hypergammaglobulinemia. The slowed-down removal bromine-sulfaleina. At a puncture biopsy of a liver lack of adjournment of amyloid

is more often And. occurs at children at a pseudorheumatism, is much more rare at osteomyelitis, tuberculosis, a system lupus erythematosus, malignant tumors, a lymphogranulomatosis, a periodic disease.

Frequency And. at the children sick with a pseudorheumatism, from 2,7 to 15,6% hesitate, averaging 5%.

M. P. Matveev and soavt. revealed And. at the children sick with nephrite with the expressed nephrotic syndrome (3,7% of cases).

According to B. M. Kavaliva, among children with bone and joint tuberculosis the amyloid nephrosis was noted by 10 times less than at adults (approximately in 0,2%).

And. at children proceeds hard, progresses quicker, than at adults. At patients with the smallest duration of a basic disease the isolated damage of kidneys, a spleen or a liver is observed.

At children kidneys generally are surprised. There is a proteinuria without pathological elements in draft. In the beginning it is changeable and insignificant, at a glomerulonephritis — resistant. Later the proteinuria tends to increase, and an urocheras scanty, with a microhematuria, a microleukocyturia and single hyaline and granular cylinders. Gradually pallor of integuments increases, there is a hepatomegalia, a splenomegaly, the accelerated ROE, a thrombocytosis keeps. The resistant disproteinemia due to increase α2-глобулинов is observed. In process of progressing And. hypostases, a proteinuria accrue, the polyuria, a nocturia, a hyposthenuria joins. The α-globulinemiya, to a lesser extent — a β-globulinemiya increases. The hypoproteinemia, hypoalbuminemia amplifies. At most of children the lipidemia is defined, is more rare — a giperkholesterolemiya. In blood the accelerated ROE, a neutrophylic leukocytosis, anemia. Further the azotemia accrues, there is a hemorrhagic syndrome, uraemia develops.

At a pseudorheumatism in process of progressing And., increase of humoral indicators of activity of process the exudative phenomena in joints abate. At a periodic disease hypertensia often meets and the hepatolienal syndrome is more rare. At other collagenic diseases And. usually is not followed by a nephrotic syndrome or it is poorly expressed.

For early diagnosis And. the electrophoretic research of serum proteins and a biopsy of bodies is of great importance. Test with congo red is a little sensitive, however it should be applied to differential diagnosis.

Differential diagnosis And. at children — see the table.


At an amyloidosis treatment of the basic pathological process which is the cornerstone of it development is carried out. Early the begun treatment and elimination of a basic disease can lead to involution And.

At development of a nephrotic syndrome apply treatment according to clinical symptoms.

Recently for treatment And. use drugs of a 4-aminoquinolinic row (chloroquine, Resochinum, delagil, etc.). Disputable is a question of use of steroid hormones. According to most of authors, corticosteroid drugs at treatment And. are a little effective.

Bibliography

Andreyeva N. E. and Alekseev G. A. Amiloidoz at a multiple myeloma (paraamiloidosis), Probl. gematol. and modulation, blood, t. 13, No. 3, page 16, 1968; Vinogradova O. M., etc. Primary family amyloidosis, Rubbed. arkh., t. 41, No. 2, page 105, 1969, bibliogr.; Davydovsky I. V. Bullet wound of the person, t. 2, page 351, M., 1954; Rukosuyev V. S. Immunomorphological identification of fibrin in amyloid masses, Arkh. patol., t. 27, No. 9, page 32, 1965; Serov V. V. Some controversial issues of classification of an amyloidosis, in the same place, t. 32, No. 6, page 8, 1970, bibliogr.; Tareev E. M, etc. To a problem of a nephrotic syndrome, Rubbed. arkh., t. 35, No. 11, page 9, 1963, bibliogr.; Andrade C. and. lake of Hereditary amyloidosis, Arthr. and Rheum., v. 13, p. 902, 1970, bibliogr; Briggs G. W. Amyloidosis, Ann. intern. Med., v.55, p. 943, 1961, bibliogr.; Cathcart E. S., Skinner M. Cohen A. S. Immunogenicity of amyloid, Immunology, v. 20, p. 945, 1971, bibliogr.; Cohen A. S. and. Calkins L. E. Electron microscopic observations on a fibrous component in amyloid of diverse origins, Nature (Lond.), v. 183, p. 1202, 1959; Cohen A. S., Gross E. Shirahama T. The light and electron microscopic autoradiographic demonstration of local amyloid formation in spleen explants, Amer. J. Path., v. 47, p. 1079, 1965, bibliogr.; Druet R. L. a. Janigan D. T. Rates of induction, lymphocyte depletion and thymic atrophy, ibid., v. 49, p. 911, 1966, bibliogr.; Gafni J., Sonar E. Heller H. The inherited amyloidoses, Lancet, v. 1, p. 71, 1964; Glenner G. G. a. o. Amyloid, J. Histochem. Cytochem., v. 16, p. 633, 1968, bibliogr.; Heller H., Sohar E. Gafni J. Classification of amyloidosis with special regard to the genetic types. Path, et Microbiol. (Basel), v. 27, p. 833, 1964, bibliogr.; Heller H. o. Amyloidosis, J. Path. Bact., v. 88, p. 15, 1964, bibliogr.; Kilpatrick T. River, Horack H. M. a. Moore C. B. «Stiff heart» syndrome, Med. clin. N. Amer., v. 51, p. 959, 1967, bibliogr.; Schmitz-Moormann P. Zur Biochemie des Amyloid, Virchows Arch. path. Anat., Bd 339, S. 45, 1965; Schwartz Ph. Amyloidosis, Springfield, 1970, bibliogr.; Senn H. I. u. a. Zur klinischen Diagnose der Amyloidose, Schweiz. med. Wschr., S. 1363, 1966, Bibliogr.; Shirahama T. Cohen A. S. Highresolution electron microscopic analysis of the amyloid fibril, J. cell. Biol., v. 33, p. 679, 1967, bibliogr.; Stiller D. u. Katenkanp D. Zur Pathogenese dcr senilen Amyloidose, Virchows Arch. path. Anat., Bd 352, S. 209, 1971; Vazquez J. J. a. Dixоn F. J. Immunohistochemical analysis of amyloid by the fluorescence technique, J. exp. Med., v. 104, p. 727, 1956.

And. skin

Lever U. F. Gistopatologiya of skin, the lane with English, page 291, M., 1958; Banerjee B. N. a. Duttа A. To. Lichen amyloidosis, Int. J. Derm., v. 9, p. 290, 1970, bibliogr.; Brownstein M. H. a. Helwig E. Century of Cutaneous amyloidoses, Arch. Derm., v. 102, p. 8, 1970, bibliogr.; Potter B. S. a. Johnson W. C. Primary localized amyloidosis cutis, ibid., v. 103, p. 448, 1971, bibliogr.

A. at children

Dumnova A. G. To clinic of an amyloidosis of kidneys at infectious nonspecific (rhematoid) arthritis at children, Pediatrics, No. 8, page 47, 1968, bibliogr.; Matveev M. P., etc. About a nephrotic syndrome at an amyloidosis at children, Urol. and nefrol., No. 5, page 23, 1971; Shcherba M. L. Obshchy of amyloids, L., 1957, bibliogr.; Yakovleva A. A. and Bryazgunov I. P. Amyloids at children, Pediatrics, No. 6, page 81, 1971, bibliogr.; Ansell B. M. and. Bywaters E. G. L. Rheumatoid arthritis (Still's disease), Pediat. Clin. N. Amer., v. 10, p. 921, 1963, bibliogr.; Strauss R. G., Schubert W. K. and. Ms of Adams A. I. Amyloidosis in childhood, J. Pediat., v. 74, p. 272, 1969, bibliogr. Pytel A. Ya. and A. L. O Sabbath primary amyloidosis of uric and men's generative organs, Urol-i nefrol., No. 1, page 64, 1976, bibliogr.; Serovv. Century of an igritsman. Yu. Amiloidoz, fabric, disproteinoz or tumor? Sov. medical, No. 7, page 13, 1975, bibliogr.; Sinkevichus Ch. A. and Lyutkus L. Yu. Primary amyloidosis of a bladder, Urol. and nefrol., No. 5, page 52, 1966, bibliogr.; Chisholm G. D., With about about t e of of N. Century of a. Dawson J. M of Primary amyloidosis of the renal pelvis, Brit. med. J., v. 1, p. 736, 1967; M a-1 e k R. S «, G r e e n e L. F. a. Farrow G. M. Amyloidosis of the urinary bladder, Brit. J. Urol., v. 43, p. 189, 1971, bibliogr.; Tripathi V. N. a. Desaute 1 s R. E. Primary amyloidosis of the urogenital system, J. Urol. (Baltimore), y. 102, p. 96, 1969, bibliogr.

Cerebral senile amyloidosis

Serov V. V. and Shamov I. A. Amyloidosis, M., 1977, bibliogr.; DivryP. De la nature de formations argentophiles des plexus choroides, Acta neurol. belg., t. 55, p. 282, 1955; Schwartz Ph. Amyloidosis, cause and manifestation of senile deterioration, Spring-fjeld, 1970, bibliogr.

B. V. Serov (properties of amyloid, etiol., stalemate., stalemate. An.); E. M. Tareev, N. A. Mukhin (C., prognrz to lay down.); S. S. Kryazheva (dermas.), E. I. Shcherbatova (ped.). A. Ya. Pytel, A. L. Shabad, A. I. Oyfa.

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