Li Xinrui is suffering from pediatric primary nephrotic syndrome.
1, nephrotic syndrome (NS) is a common pediatric renal disease, is due to a variety of etiological factors caused by increased permeability of the glomerular basement membrane, a large amount of protein from the urine loss of clinical syndromes.
2. To be characterized by massive proteinuria, hypoalbuminemia, severe edema and hypercholesterolemia, it is classified into three types, namely, simple nephropathy, nephritic nephropathy and congenital nephropathy, according to its clinical manifestations. In children under 5 years of age, the pathologic type of nephrotic syndrome is mostly the microscopic lesion type, whereas in older children, the pathologic type is predominantly the non-microscopic lesion type (including tethered proliferative nephritis, focal segmental sclerosis, etc.).
3, the diagnosis of nephrotic syndrome is mainly based on the clinical manifestations, where there is a large amount of proteinuria (24h urine protein quantitative >0.1g/kg, or >3.5g/kg), a high degree of edema, hypercholesterolemia (>5.7mmol/L,<220mg%), hypoalbuminemia (<30g/L,<3Gg%) can be diagnosed as nephrotic syndrome. ) can be diagnosed as nephrotic syndrome, typical cases of serum protein electrophoresis more suggestive of α2↑↑, and γ↓, some children may have low complement blood, microscopic or microscopic hematuria, azotemia or hypertension, preschool children >1610.6kPa (120/80mmHg), school age >17.3/12kPa (130/90mmHg), is nephritic nephropathy.
4, to hormone resistance (full hormone 8 weeks ineffective or partial effect), frequent relapse or recurrent (off year ≥ 2 times, 1 year ≥ 3 times the person) and hormone-dependent nephropathy, known as refractory nephropathy, refractory nephropathy is one of the indications of renal biopsy, can be clarified in its pathologic subtyping, renal pathology severity to guide the treatment of the child with hypercoagulable state of the shortened prothrombinogen time, plasma fibrinogen The children with hypercoagulable state have shortened prothrombin time, elevated plasma fibrinogen level, higher than normal platelet count, and during the course of hormone therapy, if there is actual lumbar pain with hematuria, elevated blood pressure, worsening of edema, or deterioration of renal function, it suggests that renal vein thrombosis.
5, the age of onset and gender, to the peak of the incidence of preschool age, simple onset of the age of small, tightly 腅 long, men than women, men: women about 1.5 ~ 3.7:1.
6, edema is the most common clinical manifestations, often the earliest for the parents to discover, starting from the eyelids, the face, and gradually and limbs all over the body, edema can be concave, can also be seen in plasma cavities, such as hydrothorax, ascites, boys often have a significant nether, the first time to find out the cause of the disease. Ascites, boys often have significant scrotal edema, weight gain of 30% to 50%, severe edema in the thighs and upper arms and abdominal wall skin can be seen in the skin white or purple lines, edema severity is usually not related to the prognosis of edema is often accompanied by a decrease in urine output.
7, in addition to edema, the child can be due to long-term protein loss protein malnutrition, manifested as pale, dry skin, dry and yellow hair, white transverse lines on the toenails, ear shells and nasal cartilage is weak, the child's spirit is depressed, lethargy and weakness, loss of appetite, and sometimes diarrhea, may be associated with intestinal mucosal edema and or associated with infections, the disease is prolonged or recurrent authors of backward development, nephritic. Children may have increased blood pressure and hematuria.
Pediatric primary nephrotic syndrome pathogenesis
(I), the cause
Nephrotic syndrome can be divided into primary, secondary and congenital according to the cause of the three kinds of primary nephrotic syndrome accounted for more than 90%, followed by a variety of secondary nephrotic syndrome, congenital nephrotic syndrome is extremely rare.
The cause of primary nephrotic syndrome is not clear, and it is often triggered by respiratory infections, allergic reactions, etc. The causes of secondary nephrotic syndrome are mainly infections, medications, poisonings, etc., or secondary to tumors, hereditary and metabolic disorders, and systemic systemic diseases.
(2) Pathogenesis
Pathogenesis The pathogenesis of this disease is not yet fully understood, it is generally believed that proteinuria is due to the glomerular capillary tubular wall charge and/or sieve barrier damage caused by the normal glomerular filtration membrane is negatively charged, the charge barrier is composed of fixed anionic sites on the basolateral membrane (mainly heparan sulfate polysaccharides) and the endothelium, the surface of the epithelium of the anion (mainly salivary acid proteins). The charge barrier is composed of fixed anionic sites on the basement membrane (mainly heparan sulfate polysaccharide) and polyanions on the surface of endothelial and epithelial cells (mainly salivary acid proteins), while the sieve barrier is composed of endothelial cell pores on the inner side of the filtration membrane, the basolateral membrane, and epithelial cell lenticels, with the basolateral membrane playing the main role.