Nephrotic syndrome is a kidney condition characterized by generalized edema, massive proteinuria, hypoproteinemia, hypoalbuminemia, and hyperlipidemia. The term was proposed in 1923. The diagnosis is based on the revealed changes in blood and urine tests (proteinuria, hyperlipemia, hypoproteinemia) on clinical data. It develops gradually, moreover, extra-renal symptoms predominate, especially edematous. There are increasing swelling, first in the eyelids, the lumbar region (it can eventually reach the degree of anasarca – the widespread edema of the subcutaneous tissue,) genitals, ascites, hydrothorax, and, more rarely, hydropericardium. The clinical picture is composed of the following symptoms. In the first place, proteinuria. Second, oliguria with a high relative density of urine. Third, and last, cylinders.
A significant hepatomegaly due to liver dystrophy is common. The skin becomes pale in the absence of anemia and dries. There are signs of hypovitaminosis A, C, B1, B2, and several dystrophic changes. There may be fragility and tarnishing of the hair, on the skin – cracks, from which fluid flows. In children, the patients become listless, a lack of appetite is frequent, as well as shortness of breath, tachycardia, and systolic murmur at the apex.
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A severe complication in patients with an anasarca, that is, expressed hypoproteinemia, may be a hypovolemic shock preceded by anorexia, vomiting, and abdominal pain. Arterial blood pressure is usually normal. Only up to 10% of children may have short-term arterial hypertension. The serum albumin level in such children is less than 10 g / l.
The content of total protein in blood plasma is sometimes reduced to 40 g / l. In particular, the concentration of albumins and g-globulin is sharply reduced, whereas the level of a2-globulins is increased and sharp dysproteinemia is observed. The blood serum has a milky color, it has a high level of lipids, cholesterol, and fibrinogen. The level of nitrogenous slag is usually normal, and the content of potassium and sodium is reduced. Renal symptomatology is an oliguria with a high relative density of urine and pronounced proteinuria. In the study of glomerular filtration by endogenous creatinine, normal and even elevated parameters are obtained, but this may be also a false impression.
The main treatment consists of a proper diet, infusion therapy, and diuretics. A diet is an option in case of kidney failure, with a restriction of fluid intake, salt-free, and with a precise amount of proteins. The infusion therapy consists mainly of albumin and rheopolyglucin. The diuretics play a big role in the treatment of kidney disease, but an uncontrolled and prolonged use of them may lead to a sharp loss of sodium and a decrease in the volume of circulating blood, as well as hypokalemia and metabolic acidosis. Forced diuresis with large doses of diuretics, like ultrafiltration, in conditions of severe hypoalbuminemia or severe renal failure may be complicated by a hard-to-control hypovolemic shock or a further decrease in glomerular filtration. Therefore, treatment with diuretics is recommended to be carried out as briefly as possible and to resume only in cases of a marked decrease in diuresis and swelling.
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The treatment of nephrotic edema usually includes furosemide which has a fairly powerful and rapid albeit short-term action. Like furosemide, ethacrynic acid is also recommended (50-200 mg/day.) Hypothiazide has a weaker effect, though: The diuretic effect of which is observed 1 to 2 hours after taking 25-100 mg of the drug. An important role in the fight against edema is played by potassium-sparing diuretics – triamterene, amiloride, especially spironolactone. Verospiron used in a dose of 25 to 200-300 mg per day, it’s most effective in combination with thiazide diuretics, furosemide. Edema, with nephrotic syndrome caused by amyloidosis, is characterized by great resistance to diuretics.
The introduction of large doses of HA in the form of pulses inhibits the formation of DNA antibodies, stops the formation of immune complexes, reduces their mass, and promotes the release from the sub-endothelial layers of the glomerular basement membrane while increasing the glomerular filtration and renal blood flow. GK are assigned to children in all cases of newly emerged HC, with relapses of hormone-sensitive NS, with a progressive course of GV, in combination with other immunosuppressants. In practice, three regimens of GC are used.
The continuous oral supply of PP at a dose of 1-2 mg / kg in 2-4 doses is the general measure, taking into account the daily activity of the adrenal cortex (the maximum dose of the drug in the morning, followed by a decrease, the latest reception no later than 4 pm,) which is prescribed at the beginning of treatment to achieve remission.
Finally, an alternative mode of reception of PP is used in the transition to maintenance therapy. It consists of taking a daily dose of PZ in a day, which, while maintaining the clinical effect, significantly reduces side effects: Acute – insomnia, euphoria, psychosis, increased appetite, chronic edema, obesity, myopathy, skin atrophy, hirsutism, acne, osteoporosis, cataract, increased blood pressure, steroid diabetes, adrenal crisis, and acute adrenal insufficiency with abrupt withdrawal of the drug.
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