Nephrotic syndrome

It is characterised by presence of a high proteinuria (more than 3,5 gramme a day), hypoalbuminemias (more low 30 gramme/litre), a hypoproteinemia and edemas.

Abjection of a nephrotic syndrome extremely important as it is accompanied by appreciable changes of a homeostasis, often is very hardly tolerated by the patient because of widespread edemas, can become complicated an infection contamination, vascular clottages, and also worsens the forecast, Usually even at the expressed signs of a nephrotic syndrome at the patient long time remains a high glomerular filtration and there is no arterial hypertensia, however spontaneous remissions at adults are rare also illness progresses with development Chronic renal insufficiency, that always forces to solve a question on active treatment.

Specification of nosological essence so, and aetiologies of the nephrotic syndrome which signs have nonspecific character, is a paramount problem. In overwhelming majority of cases the nephrotic syndrome arises at an acute and chronic glomerulonephritis, but can be observed at a lesion of nephroses against system - Systemic lupus erythematosus, the hemorrhagic vasculitis, a pseudorheumatism, is rarer a nodous periarteritis, a system scleroderma, granulomatoses), infectious (pyeses, a tuberculosis, an actinomycosis, a subacute infectious endocarditis), parasitosises, at illnesses of a liver (especially virus aetiology), blood, a diabetes, a vascular clottage, allergic responses. It is necessary to remember possibility of development of a nephrotic syndrome of a medicinal genesis (antibiotics, gold preparations, D - Penicillaminum, etc.), within the limits of paraneoplastic (a bronchogenic cancer, a nephros cancer, etc.) And paratubercular reactions (a paraspecific nephritis). There are rare genetical forms of a nephrotic syndrome.

Morphological basis of a nephrotic syndrome are in overwhelming majority of cases two variants of a lesion of nephroses - changes of a type of a glomerulonephritis (various morphological types) and an amyloidosis.

At the heart of modern understanding of a pathogenesis of a nephrotic syndrome representation about immunely inflammatory nature of illnesses of nephroses lays. Sedimentation of cell-bound immune complexes on a basal membrane of glomuluses causes a number of cellular reactions of an immune inflammation that conducts to disturbance of the glomerular filter and a massive proteinuria. Protein of urine can be presented only an albumin (at the minimum changes of a glomulus) or largly molecular globulins (at more serious lesions of a glomulus). After a proteinuria educe a hypoproteinemia (45 - 55 gramme/litre and more low) and a hypoalbuminemia (to 15 gramme/litre, sometimes to 10 gramme/litre). The maintenance a - globulins is usually raised, the maintenance of β₂ globulins is is more often lowered, but can be normal or even raised (at general diseases, an amyloidosis). Very often find a lipidemia (the raised maintenance of cholesterol, triglycerides, lipoproteins in blood). The maintenance of the general lipids in plasma can reach 20 gramme/litre. To a lipidemia it is closely bound lipiduria. The lipidemia usually correlates with size of a hypoalbuminemia and is, as assume, a consequence of mobilisation of Adepses from depot for the power purposes in the conditions of a hypoalbuminemia. It can promote development in the patient with a nephrotic syndrome of an atherosclerosis, and also to disease advance. The lipidemia is not an obligatory sign of a nephrotic syndrome, can be absent at an acute glomerulonephritis with a nephrotic syndrome, in some cases at a lupoid glomerulonephritis. In parallel with these disturbances hemostasis changes often educe, the hypercoagulation causes possibility of vascular clottages, increase of changes in glomuluses.

Two mechanisms take part in development of nephrotic edemas:

1. a hypoalbuminemia accompanied by depression of oncotic pressure of plasma, a hypovolemia and a hyperaldosteronism leading to a delay of sodium and through rising of synthesis Adh to a delay of water (a hypovolemic variant)
2. intrarenal disturbance of abjection of sodium - depression of size of a glomerular filtration of sodium and augmentation of its canalicular reabsorbtion (a hypervolemic variant)

Condition of patients various complications - infection contaminations (worsen a pneumonia, a pneumococcal peritonitis, a sepsis), including at immunodepressive therapy (a pyelonephritis, a tuberculosis), vascular clottages, nephrotic crises in the form of episodes of erythemas of various localisation and the peritoneal phenomena, a hypovolemic collapse. Consequences of a nephrotic proteinuria which are bound to loss with hepatocuprein urine (deficiency of copper), a transferrin (a microcytic anaemia), immunoglobulins (bacteriemic infection contaminations), an antithrombin III and other proteins are less known.

The most frequent cause of a nephrotic syndrome there is a glomerulonephritis, therefore the basic place in treatment belongs to glucocorticoids and cytostatics. At the nephrotic syndrome caused by an amyloidosis, a diabetic glomerulosclerosis, a clottage of renal veins, paraneoplastic and paratubercular processes, immunodepressants are contraindicative, therefore the specified conditions should be excluded before the beginning of such treatment.

Treatment of a hydropic syndrome depends first of all on success of treatment of a basic disease. So, at «steroid-sensitive» forms of a nephrotic syndrome for 5-7 day of treatment by adequate doses of glucocorticoids the diuresis sharply increases ("a steroid diuresis"). However other methods - a diet, reduction of a hypovolemia and application of diuretics have great value also. It is necessary to notice, that diuretic therapy is shown first of all at appreciable edemas; at the moderate edemas which are not causing to the patient of inconveniences, in some cases it is possible to recommend a confinement to bed causing augmentation of a diuresis, use of elastic stockings, infusions and decoctions of the medicinal plants possessing diuretic properties (parsley, a bearberry, a foxberry, a juniper). It is necessary to confine necessarily considerably salt consumption - to 3 grammes/days. At the expressed edemas the quantity of sodium deduced by nephroses is sharply circumscribed, sometimes to 25 mekv/days At such patients sodium introduction should not exceed its deducing (1 gramme of sodium corresponds 23 milliequivalent), it is necessary to exclude as much as possible the foodstuff containing salt, sometimes to change usual drinking water distilled. At development of renal insufficiency salt reception should be enlarged.

The high-protein diet (100 - 120 gramme of protein a day) at conservation of function of nephroses can promote some rising of level of blood proteins, but can and enhance a proteinuria. Besides, reduction of quantity of consumed protein slows down glomerulonephritis advance. Therefore patients with a nephrotic syndrome should recommend a physiological quota of animal protein in nutrition or even its some restriction.

Diuretics play the big role in treatment of disease of nephroses, however at uncontrolled and their long application there can come sharp loss of sodium and depression of volume of circulating blood, a hypopotassemia and a metabolic acidosis. The artificial diuresis by means of the big doses of diuretics, as well as an ultrafiltration, in the conditions of a sharp hypoalbuminemia or the expressed renal insufficiency can become complicated an unhandy hypovolemic shock or the further depression of a glomerular filtration. Therefore treatment by diuretics is recommended to be spent as much as possible quickly and to renew only in cases of appreciable depression of a diuresis and increase of edemas.

To treatment of nephrotic edemas usually apply Furosemidum - 20 - 400 mg inside, 20 - 1200 mg intravenously) which renders powerful enough and fast though also short-term, action. Like Furosemidum Acidum etacrynicum (50 - 200 mg/sut) reacts also. Hypothiazidum which diuretic effect is observed through 1 - 2 hours after reception 25 - 100 mg of a preparation more weakly reacts. The important role in struggle against edemas saving up diuretics - Triamterenum, amiloride, especially Spironolactonums (Aldactonum, a verospiron) play a potassium. A verospiron apply in a dose from 25 to 200 - 300 mg a day, It is most effective in a combination with thiazide diuretics, Furosemidum. Edemas - at the nephrotic syndrome caused by an amyloidosis, differ the big. Resistance to diuretics.