Lipoid nephrosis

Description:

The lipoid nephrosis is a rare disease, one of forms of a nephrotic syndrome which is characterized by damage of kidneys of preferential dystrophic character. Earlier the lipoid nephrosis was considered as an independent type of pathology of kidneys. Further it became clear that this group of damages of kidneys is heterogeneous. Its most part is made by cases of the nephrotic syndrome representing one of displays of any general disease (tuberculosis, osteomyelitis, syphilis, malaria, diphtheria, dysentery, a chronic coloenteritis, polyarthritis, a lymphogranulomatosis, lead and mercury intoxications etc.). Cases when the origin of dystrophic changes in kidneys remains not clear, are designated as a lipoid nephrosis.

Symptoms of the Lipoid nephrosis:

Kidneys are increased (the mass of one kidney reaches 250 g), the capsule easily is removed, a surface smooth, pale gray color (a big white kidney). The bast layer is considerably expanded, yellowish-gray color.

At a histologic research find changes, mainly in cells of proximal department of renal tubules. In basal parts of epithelial cells find deposits of lipids. Balls dystrophic are changed, especially podocytes and endothelial cells with which permeability of a glomerular membrane is connected.

In late stages of a disease destruction of epithelial cells of tubules with development of connecting fabric and the subsequent atrophy, hyalinization of balls is observed. As a result of it kidneys decrease in volume and nephrotically contracted kidney is formed.

The lipoid nephrosis develops gradually. Treat early complaints of patients: weakness, decrease in working capacity, appetite loss, thirst, chill.

Usually patients see a doctor in connection with emergence of hypostases. The preedematous period more often long, is characterized by the expressed proteinuria. Then gradually puffiness and pallor of the person, decrease in a mimicry appear. Hypostases accrue and take all hypodermic cellulose (anasarca), deforming a face and a trunk, but especially they are expressed on a face.

Face of the patient bloated, eyelids the bulked-up, palpebral fissures narrow, morning of an eye open hardly. Liquid collects also in internals and serous cavities - belly, pleural, is more rare in a pericardium. Hypostases keep a long time (months and even years). They are very soft, mobile, when pressing quickly disappearing deep pole remains a finger. The amount of edematous liquid in some cases reaches 20 l and more.

Skin over places of hypostases becomes brilliant from a tension, can burst with the liquid expiration. Further infection is possible (an erysipelatous inflammation, etc.). Emergence of hypostases is connected with a hypoproteinemia and decrease in colloid osmotic pressure. Edematous liquid is poor in proteins and is rich with salts, in particular chlorides. Liquid has a milk shade, the quantity of lipoproteins in it is increased.

Arterial pressure normal or lowered. Muting of cordial tones, bradycardia is noted. Angiospastic symptoms and symptoms of heart failure are absent. The liver is sometimes slightly increased. At disease height at the expressed hypostases the oliguria is observed (the amount of urine decreases to 400-300 ml a day), significant increase in relative density of urine (to 1,040-1,060).

Urine of brown color also contains a large amount of protein (to 10-20% and above). Sometimes daily loss of protein reaches 10-15 g. The hyperpermeability of balls, reduction of a reabsorption of protein in tubules and the strengthened protein filtering connected with change of its properties is the cornerstone of a proteinuria. Existence of the resistant and expressed albuminuria is one of the most characteristic symptoms of a disease. Among proteins in urine finely divided molecules of albumine prevail.

In an urocheras a large number of hyaline, granular and wax-like cylinders and cells of a renal epithelium is defined. In an urocheras there are no erythrocytes or single meet. Quite often there is a lot of leukocytes, but they differ from the leukocytes of urine found at infectious and inflammatory diseases of kidneys, pyelonephritises as are well painted by safranin on Shtengaymera - to Malbin.

The amount of the chlorides emitted with urine (to 1 g) which normal makes 10-15 g a day is reduced. It is explained by their delay with liquid, and also the strengthened products of Aldosteronum increasing a reabsorption of ions of sodium in tubules. The expressed disproteinemia and a pshoproteinemiya are inherent to a nephrosis (to 3-5 g of %). Especially strongly the content of albumine in a blood plasma (at the expense of the expressed albuminuria) that coefficient albumine-globulinovogo to 1-0,5 leads to change and decreases below (1,5-2 is normal).

Respectively in blood the amount of globulins increases, it is preferential for the account alfa-2-and beta globulins. Level of gamma-globulins is reduced. The disproteinemia and a hypoproteinemia lead to decrease in osmotic pressure of proteins, at the same time liquid does not keep in blood and comes to fabrics.

Constant symptom of a disease is the sharp lipidemiya, a cholesterinemia. Cholesterol level in a blood plasma increases to 500 mg (1360 mmol/l) and above. The amount of lecithin and fatty acids increases. These changes, obviously, have a secondary origin and are connected with disturbance of protein metabolism and a hypoproteinemia. Besides, hypochromia anemia, a lymphocytosis, an eosinophilia, increase in SOE are observed. Krovoochistitelny function of kidneys is not broken, the azotemia is not observed. Only in late stages, at a contracted kidney, in an organism nitrogenous slags are late and uraemia develops. The main functional trials of kidneys a long time remain normal. Glomerular filtering and a canalicular reabsorption are usually strengthened. The increased hydrophily of fabrics that is confirmed by blisteral test of Mac-Klyura - Aldrich at which the rassasyvaniye vnutrikozhno of the entered isotonic solution of sodium of chloride (in number of 0,2 ml) occurs in an expedited manner - in 10-20 min. is noted (30-40 min. are normal). The disease is long, up to 15-20 years and more. Alternation of a long preedematous stage with a stage edematous at which patients lose working capacity is observed. Hypostases differ in extreme persistence and can keep for years. Patients in an edematous stage because of the expressed hypogammaglobulinemia and puffiness of fabrics are very sensitive to infectious agents. They quite often observe repeated pneumonia, erysipelatous inflammations of skin etc. of which earlier, before opening of antibiotics, patients died.

Reasons of the Lipoid nephrosis:

It is considered that the reason of a lipoid nephrosis and a nephrotic syndrome are sharp disorders of exchange, generally fatty and proteinaceous therefore the trophicity and permeability of a wall of capillaries of renal balls is broken. The proteinaceous particles and lipoida which in a large number are contained in primary urine of patients infiltrirut a wall of tubules, causing rough dystrophic changes in epithelial cells. Besides, recently the great value in development of this pathology of kidneys is allocated for the autoimmune mechanism.

Treatment of the Lipoid nephrosis:

Now these complications will respond to treatment antibiotics and sulfanamide drugs. Besides, frequent emergence at sick vascular thromboses is noted. At a combination of a lipoid nephrosis to an amyloidosis of kidneys the renal failure from the outcome can develop in uraemia.