GLOMERULOSCLEROSIS DIABETIC (Latin glomerulus ball + sclerosis; grech, diabetes, from diabainein to pass; synonym: interkapillyarny glomerulosclerosis, Kimmelstil's syndrome — Wilson) — a specific and most frequent form of damage of kidneys at a diabetes mellitus; it is for the first time described in
1936 is one of the heaviest complications of a diabetes mellitus (see a diabetes mellitus). G.'s frequency of on materials of autopsy fluctuates from 19,5% [L. Henderson] to 50,9% [White] at the patients suffering from a diabetes mellitus over 20 years.
In G.'s clinic of, according to R. A. Heifetz and L. I. Kaminskaya, comes to light in 6%, according to E. G. Moscovitch — in 17,8% and even in 48% of cases (V. V. Sur) of number of the patients suffering from a diabetes mellitus.
At the women suffering from a diabetes mellitus, G. of occurs in 30%, at men — in 19,5% of cases.
Histologic changes of kidneys at G. of very polimorfna; three main forms of defeat — nodular, diffusion and exudative are allocated. Some authors instead of exudative allocate the mixed form.
The nodular form is characterized by existence in balls of eosinophilic educations (small knots) of a rounded or oval shape (fig. 1) containing vacuoles. They can occupy a part or all ball, on the periphery to-rogo in the latter case the remained squeezed capillary loops are located. Expansion and aneurisms of capillaries of balls, a thickening of their basal membranes is at the same time observed. At a histochemical research of small knots they are painted as fibrin in the beginning, and further as collagen. Small knots contain a large amount of high-molecular mucopolysaccharides, a small amount of acid mucopolysaccharides, fatty substances, generally unsaturated fatty acids and holesterinester. At electronic microscopic examination it is visible that formation of small knots happens in a mezangiya in the form of accumulation in it glybok and the trabeculas similar to substance of a basal membrane.
The diffusion form is expressed in homogeneous expansion and consolidation a mezangiya with involvement in process of basal membranes of capillaries which are sharply thickened. The membranopodobny structures which are formed in a mezangiya do not merge in continuous masses, and nodulation does not occur. Basal membranes of capillary loops of balls are thickened, their structure disappears (fig. 2).
The mixed form is characterized by a combination of typical small knots to diffusion consolidation a mezangiya and a thickening of basal membranes of capillaries of balls (fig. 3). According to electronic microscopic examinations the thickening of basal membranes of capillaries of balls arises early, is frequent before emergence of any clinical manifestations of damage of kidneys, and takes place, probably, at all forms G. of. The thickening of membranes is observed not only at patients, but also at young people from diabetic the burdened families.
The exudative form meets less than previous, and it is characterized first of all by so-called «fibrinoid hats» which represent PAS-nolozhitelny's deposits of material between an endothelium and a basal membrane of capillaries (fig. 4). At an immunohistochemical research in these educations a significant amount of complement-fixing immunoglobulins is found that gives the grounds to consider them a complex antigen — an antibody, but not serumal exudate. «Fibrinoid hats» are not specific to G., but at the same time they are found at the heavy and quickly progressing its forms. The so-called «capsular drops» which are located on the interior of the boumenovy capsule are often combined with them.
Changes of tubules are characterized by glikogenovy infiltration with localization of process in a kortikomedullyarny zone, in a terminal segment of proximal gyrose tubules with distribution on thin Department of a Henle's loop in the beginning. The brush border of an epithelium is loosened, in cytoplasm also proteinaceous granules are found. In heavy, far come G.'s cases of the expressed atrophy of tubules, expansion of their gleams with existence of cylinders in them is observed. Basal membranes of tubules of a razvoloknena are also thickened. In small blood vessels of kidneys and other bodies plasmatic treatment, proliferation of an endothelium, a thickening of basal membranes with gradual increase of both changes up to hyalinization of arterioles is noted.
Pathogeny. Of — damage of kidneys, specific to a diabetes mellitus, is also connected with endocrine and exchange disturbances.
The disproteinemia arising at patients with increase in alfa2-globulins and emergence of pathological paraproteins, increase in level of mucopolysaccharides, the general lipids, triglycerides, p-lipoproteids in combination with increase in vascular permeability can lead to their penetration into a mezangialny matrix, a delay there, polymerizations and, in particular, to nodulation. Similarity of damage of kidneys at G. with the changes of kidneys observed at introduction by an animal of high doses of corticosteroids gives the grounds to connect these defeats with hyperfunction of adrenal glands, in particular with hypersecretion of Aldosteronum.
A clinical picture
existence of a proteinuria, arterial hypertension, hypostases Is characteristic. Proteinuria (see) is the first and most continuous manifestation of G. of. Losses of protein reach 40 g a day. Diabetic is almost constantly observed retinopathy (see), characterized by microaneurysms with dot hemorrhages, exudates, sites of a degeneration in the form of white maculas lutea, and at the most severe forms — proliferating retinitis (see). The urocheras is usually scanty. Arterial hypertension occurs at 60% of patients and the same as the proteinuria, can be its first clinical manifestation. Frequency of arterial hypertension increases with increase in term of a disease. At young people the nephrotic syndrome, at elderly — arterial hypertension is more often observed, edges it can be combined with heart failure. In these cases hypostases have the mixed cordial and renal character.
Moderate hypostases of a gipoproteinemichesky origin are observed often — at 47% of patients according to L. Henderson et al.; nephrotic syndrome (see) — much more rare — from 6 to 26%. A nephrotic syndrome at patients with a diabetes mellitus more terrible predictive sign, than at patients with hron, nephrite.
Level of protein and a ratio of protein fractions of blood serum in the absence of the expressed proteinuria remains within norm, at a nephrotic syndrome along with a hypoproteinemia and a hypoalbuminemia increase in level alfa2-less often than gamma-globulins is observed. Level of lipids increases at a nephrotic syndrome. Also the level of mucopolysaccharides and mukoproteid is increased.
Pathogenetic therapy of G. of does not exist. There are messages on favorable effect of anabolic hormones (retabolil, Nerobolum, etc.), heparin. Replacement of digestible carbohydrates with fructose at sufficient total quantity of carbohydrates and proteins is reasonable (in the absence of a renal failure) and restriction of fat. Symptomatic therapy is defined a wedge, manifestations: hypertension, hypostases, functional capacity of kidneys and cardiovascular system. Surgical methods of treatment — a hypophysis - and the adrenalectomy, transplantation of kidneys along with a pancreas did not gain distribution.
G.'s Current of variously depending on age of patients, weight of diabetes and its correction. At young people the disease progresses quicker. Development of a renal failure is promoted by accession of pyelonephritis. Average life expectancy of patients after emergence of the first symptoms of G. of — 5 — 6 years, but can fluctuate from 2 to 12 years.
In G.'s prevention by the main place belongs to careful correction of diabetes.
Bibliography: Vikhert A. M. and Sokolov and R. I, Diabetic glomerulosclerosis, Arkh. patol., t. 34, No. 2, page 3, 1972, bibliogr.; Moscovitch E. G. Questions of clinic and treatment of a diabetic nephropathy, Probl, endocrinins, and gormonoter., t. 15, No. 5, page 120,1969; Moscovitch E. G., Anosova L. N. and Kogan T. N. A diabetic nephropathy, in book: Aktualn, probl. Diabetolum., under the editorship of E. A. Vasyuko-va, page 101, M., 1972; Fundamentals of nephrology, under the editorship of E. M. Tareeva, t. 2, page 692, M., 1972; With e r about in V. V., etc. A diabetic mikroangiopatiya according to clinic and a biopsy (kidneys and skin). Arkh. patol., t. 34, KV 2, page 15, 1972, bibliogr.; Heyets R. A. and Kamensk L. I. to a question of a diabetic nephropathy (kliniko-morphological characteristic), Works Center, in-that moustaches, doctors, t. 104, page 164, M., 1967, bibliogr.; R e u b i P. Nierenkrankheiten, Bern, 1960; Sarre H. u. a. Nephrotisehes Syndrom des Erwachsenenalters, Dtsch, med. Wschr., S. 225, 1971.