Nephrotic syndrome

Description:

Nephrotic syndrome - the state which is characterized by generalized hypostases, a massive proteinuria (higher than 50 мг*кг / or it is higher than 3,5 g/days of days), a hypoproteinemia  and a hypoalbuminemia (less than 20 g/l), a lipidemia (cholesterol is higher than 6,5 mmol/l). The term is offered by E. M. Tareev in 1923.

The Nephrotic Syndrome (NS) — the clinical laboratory symptom complex which is clinically characterized by peripheral or generalized hypostases up to ascites and an anasarca, and laboratory — a proteinuria more than 3,5 g/days or more than 50 mg/kg/days, a hypoproteinemia, a hypoalbuminemia (it is lower than 20 g/l), a disproteinemia, a lipidemia and a lipiduriya.

Symptoms of the Nephrotic syndrome:

The nephrotic syndrome has a set of manifestations. It is first of all  a high proteinuria (usually more than 3,5 g/days, sometimes are slightly less), a hypoalbuminemia,  hypostases,  a giperlipoproteidemiya,  a lipiduriya and  increase in coagulability of blood. It is important to note that primary disturbance is a  proteinuria which arises because of a hyperpermeability of the glomerular filter at damage of a glomerular basal membrane and filtrational cracks between legs of podocytes. All other manifestations of a nephrotic syndrome are the investigations  of a proteinuria though they can be it is available at a moderate proteinuria and be absent when it is very high.

Hypoalbuminemia - a direct consequence of a proteinuria; level of albumine of subjects is lower, than more its excretion with urine. Other reasons of a hypoalbuminemia - disintegration of reabsorbirovanny albumine in proximal tubules and insufficient increase in synthesis of albumine in a liver.

The reasons of formation of hypostases at a nephrotic syndrome are not absolutely clear. The most widespread "hypovolemic" hypothesis describes this process so. At a hypoalbuminemia the oncotic pressure of plasma decreases that leads to a water exit from vessels in an interstitium. In response to reduction of OTsK the renin-angiotenzinovaya system is activated, the sympathetic tone raises, secretion of ADG grows, and secretion of atrial natriuretic hormone decreases. All this leads to a delay of sodium and a delay of water which continues to leave in an interstitium. Not clearly, however, why hypostases at those patients at whom OTsK is raised in that case develop, and a renin-angiotenzinovaya the system is suppressed. Possibly, in such cases formation of hypostases is caused by a delay of salt and water.

Giperlipoproteidemiya, as believe, develops because the liver strengthens development of lipoproteids in response to decrease in oncotic pressure of plasma, and also because of loss with urine of the proteins regulating exchange of lipoproteids. The LPNP levels and cholesterol are increased at most of patients, and LPONP and triglycerides - in the most hard cases. Possibly, though it is not proved that the giperlipoproteidemiya promotes development of atherosclerosis and progressing of HPN.

Increase in coagulability of blood has several reasons: loss with urine of antithrombin III, a protein With and S protein, the strengthened synthesis of fibrinogen in a liver, weakening of a fibrinolysis and the increased aggregation of thrombocytes. Clinically these disturbances are shown by TELA, fibrinferments of peripheral vessels and thrombosis of renal veins.

Symptoms of acute thrombosis of renal veins - a sudden back pain or a stomach, a gross hematuria, left-side to a varikotsela (the left ovarian vein falls into a renal vein), sharp increase of a proteinuria and falling of SKF. Chronic thrombosis of renal veins usually proceeds asymptomatically. Thrombosis of renal veins especially often (to 40% of cases) develops at patients with a nephrotic syndrome at a hymenoid nephropathy, a mezangiokapillyarny glomerulonephritis and an amyloidosis.

Also proteinaceous insufficiency and microcytic hypochromia anemia which develops because of loss of transferrin meet and will not respond to treatment iron preparations. Losses of the serum proteins transferring vitamin D lead to avitaminosis of D with a hypocalcemia and a secondary hyperparathyreosis, transthyretin losses - to decrease in the T4 level, immunoglobulins - to decrease in resilience to infections.

Reasons of the Nephrotic syndrome:

Only the glomerular proteinuria can be the cause of a nephrotic syndrome. It can arise at any defeat of the glomerular filter when the basal membrane or filtrational cracks between legs of podocytes are damaged or lose a negative charge.

Six main reasons to which share more than 90% of all cases of a nephrotic syndrome fall are a disease of the minimum changes, a focal and segmented glomerulosclerosis, a hymenoid nephropathy, a mezangiokapillyarny glomerulonephritis, a diabetic glomerulosclerosis and an amyloidosis.

Treatment of the Nephrotic syndrome:

    * A diet — at a renal failure liquid reception restriction, electrolyte-deficient, amount of protein, optimum on age
    * Infusional therapy (albumine, реополиглюкин and др)
    * Diuretics

diuretics play a large role in treatment of a disease of kidneys, however at uncontrolled and their prolonged use there can occur sharp loss of sodium and decrease in volume of the circulating blood, a hypopotassemia and a metabolic acidosis. The artificial diuresis by means of high doses of diuretics, as well as ultrafiltration, in the conditions of a sharp hypoalbuminemia or the expressed renal failure can be complicated by unhandy hypovolemic shock or further decrease in glomerular filtering. Therefore treatment by diuretics is recommended to be carried out most quickly and to renew only in cases of noticeable decrease in a diuresis and increase of hypostases.

Usually apply furosemide to treatment of nephrotic hypostases – 20 – 400 mg inside, 20 – 1200 mg intravenously) which renders rather powerful and bystry, though short-term, action. Like furosemide also Acidum etacrynicum (50 – 200 mg/days) works. More weakly hypothiazid which diuretic effect is observed in 1 – 2 h after reception of 25 - 100 mg of drug works. An important role in fight against hypostases is played by kaliysberegayushchy diuretics – Triamterenum, amiloride, especially Spironolactonums (Aldactonum, верошпирон). Veroshpiron apply in a dose from 25 to 200 - 300 mg a day, He most эффектииен in combination with tiazidovy diuretics, furosemide. Hypostases – at the nephrotic syndrome caused by an amyloidosis differ in big resistance to diuretics.

    * Heparin
    * Antibacterial therapy
    * Corticosteroids