Nephrotic syndrome is a common disease in children during the urinary system. Some children after the onset of the disease repeatedly, prolonged course, seriously affecting the health of children. The disease has four characteristics: ① proteinuria, +++ qualitative and quantitative> 0.1 g / kg body weight / day; ② hypoalbuminemia, serum albumin <30 g / liter; ③ edema; 5 hypercholesterolemia disease (hyperlipidemia), serum cholesterol> 2.2 g / liter.
Nephrotic syndrome, according to the etiology and pathogenesis of age can be divided into three categories: ① congenital nephrotic syndrome; ② primary nephrotic syndrome; ③ secondary nephrotic syndrome, such as secondary to allergic purpura, systemic lupus erythematosus, glomerulonephritis, drugs and metal poisoning.
Children with primary nephrotic syndrome has according to clinical manifestations, course and laboratory examination process is divided into two categories, namely simple and nephritis, nephrotic kidney. In addition to the former by the latter with the above-mentioned four clinical features, but also has the following four in one or more abnormal findings. ① red blood cells in the urine over 10 / HPF; ② recurrent hypertension (schoolchildren> 17.3 / 12.0Kpa (130 / 90mmHg), preschoolers> 16.0 / 10.7Kpa (120 / 80mmHg); ③ azotemia, plasma non-protein nitrogen than 35.7mmol / l (500 mg / liter), or urea nitrogen than 10.71mmol / l (300 mg / liter); 4 total blood complement C3 repeatedly reduced or clinically to simple common, accounting. 68.4%; nephritis 31.6%.
Children with nephrotic syndrome by renal biopsy, lesions and progression can be in a variety of pathological changes, but with minimal change in the majority, accounting for 76.4%. In addition, the multi-mesangial proliferative lesions. Pathological classification of nephrotic syndrome treatment options and prognosis may provide some help. Such as small lesions usually simple, corticosteroids and immunosuppressants sensitive; rather small lesions can be expressed as nephritis, often not sensitive to the hormone. Clinically regular enough by corticosteroids after 8 weeks of therapy, the continuous examination of urine protein results in a 3 times a week to determine. Urine protein were overcast for hormone-sensitive, urinary protein (+) - (+) were partially sensitive urinary protein still ≥ (+++) were insensitive.
It is generally believed, nephrotic syndrome is an autoimmune disease, immune-mediated and inflammatory damage plays a major role in the pathogenesis. Under the pathogenic factors causing immune dysfunction in children, cytokine production and immune regulation imbalance caused by glomerular basement membrane damage lesions, resulting in increased permeability, massive proteinuria. Under normal circumstances, daily urine protein little, about 150 mg. However, patients with nephrotic syndrome daily urine protein up to 2 to 20 grams, so nephrotic syndrome, proteinuria is the most important clinical manifestations, and become the most important factor affecting the prognosis of nephrotic syndrome. For this reason, in recent years, focusing on how to control proteinuria, reduce disease recurrence, minimizing the use of hormones and immunosuppressants become a key issue in today's treatment of nephrotic syndrome.
Because a lot of blood in the urine albumin, causing hypoalbuminemia and reduce swelling pressure vessels, fluid water into the interstitial space, the occurrence of edema. Eyelid edema seen in the early morning after, then be spread to the legs, back, abdomen, or even the whole body. Edema is one of the major clinical manifestations of nephrotic syndrome have a characteristic, and often parents to bring children for treatment reasons.
Hypoalbuminemia and edema caused by hypovolemia, resulting in the renin - angiotensin - aldosterone axis stimulated, cause salt and water retention. That some patients have high blood pressure, oliguria, tachycardia and other symptoms, especially common in patients with minimal change nephrotic syndrome. Therefore, children with nephrotic syndrome edema must be given full attention. Edema is a difficult work but should treat the symptoms. The treatment measures include limiting salt diet and taking diuretics. Pleural effusion, ascites affect breathing and oliguria available furosemide, hydrochlorothiazide, or spironolactone. Proteinuria can make all kinds of plasma protein loss, causing metabolic disorders and abnormal functions of several systems, and even the occurrence of various complications. For example hypoalbuminemia itself or swelling associated with low pressure can produce changes in lipid metabolism, with lecithin - cholesterol acyl-converting enzyme from the urine, resulting in hypercholesterolemia, and then make the proportion of different types of lipoproteins and activity change, prone to cause injury and glomerular endothelial dysfunction, cardiovascular disease risks become.
Because urinary protein excretion and changes in protein synthesis, so that the body of coagulation factors and anticoagulant factors out of balance, clotting factor Ⅱ, increased Ⅲ, Ⅳ, Ⅴ and Ⅹ level, clotting mechanism of the body, platelet activity, thrombolytic process disorders. Therefore, the blood of children with nephrotic syndrome in a hypercoagulable state, belonging to high-risk pathological thrombosis.
With urinary protein loss but also have a variety of other proteins, will cause the corresponding results, such as vitamin D-binding globulin is lost, resulting in abnormal metabolism of vitamin D and calcium, vitamin D deficiency, affecting bone development in children. Lack of thyroid-binding globulin and albumin cause thyroid dysfunction, thereby affecting the growth and development of children.
Minimal change nephrotic syndrome in children prone to bacterial infections. The reason is that a large number of immune globulin in the urine resulting in reduced immune defenses; plus edema prone to skin damage, so easy to invasive pathogens. Bacterial infections, pneumonia cocci, gram-negative bacilli have (such as E. coli) infection. Common respiratory tract infections, urinary tract infections, skin type erysipelas and primary peritonitis. susceptible to viral infection in children, especially corticosteroids and immunosuppressants accept the treatment process, such as concurrent chicken pox, measles, herpes zoster time, indicating serious condition.
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