A brief introduction of congenital nephrotic syndrome

A brief introduction of congenital nephrotic syndrome

A brief introduction of congenital nephrotic syndrome 

Many children born soon suffered from congenital nephrotic syndrome, regret, so what is a congenital nephrotic syndrome? 

Congenital nephrotic syndrome (congenital nephrotic syndrome, CNS) refers to the birth of the disease within three months after nephrotic syndrome, with the same clinical manifestations of childhood nephrotic syndrome, which appears to be a massive proteinuria or within 3 months after birth, highly edema, hyperlipidemia, and hypoalbuminemia, etc., according to genetic nephrotic syndrome and the causes are usually pided into two types: hereditary nephrotic syndrome. 

No special treatment, steroid hormones and cytotoxic drugs no curative effect, and is often characterized by hormone resistance. So can't regular treatment by nephrotic syndrome. Most children died within 1 year after onset, but rarely because of kidney failure, but more died of serious infections, malnutrition, diarrhea, electrolyte disorder, etc. So to actively give support therapy, thorough treatment should be performed in 2 years after renal transplantation. 

1. Reduce the edema Main should limit salt and diuretic use. For serious low serum albumin leels or associated with low blood volume performers, salt-free human blood albumin infusion. There are 4 weeks after the main Zhang Zisheng Finland vein type blood albumin, maintain its plasma protein in more than 15 g/L, is now generally is no edema and the growth of close to normal. 

2. Keep to the high quantity of heat and enough protein diet. 

3. The prevention and control of infection, infection as the main cause of death, should pay attention to protect, in the event of infection should be timely and positive treatment, usually do not prophylactic antibiotics putting-in-service proactively. When necessary, can be continuously used blood ivig preparations. 

4. The prevention and treatment of complications in the treatment of secondary thyroid function is low, have high condensation to dipyridamole (dipyridamole) and a small amount of aspirin or treatment with warfarin, but complications, such as the prevention and treatment of embolism. General case blood pressure is normal, later to give regular antihypertensive treatment increased blood pressure. 

5. Other In recent years have reported application of angiotensin converting enzyme inhibition (ACEI) medicine, also has a report accompanied with indomethacin (indomethacin), can reduce the proteinuria. 

6. The treatment of renal transplant renal transplantation is the only completely, usually in after 2 years of age or weight 7 kg. Nephrectomy can go ahead for severe proteinuria (termination of proteinuria), rely on dialysis to sustain life waiting for transplantation. As Mattoo and report line in 1992 a side nephrectomy, reduce the urine protein excretion, and another kidney to maintain renal function, which can reduce the human blood albumin infusion of a month. 

Drash syndrome do not respond to treatment, and recurrence after renal homograft. The recurrence after transplantation in patients with nephrotic syndrome is due to cytomegalovirus infection or transplant rejection. Due to a high incidence of bilateral Wilms tumor, therefore, some people recommend preventive renal resection. 

In order to children's health, it is suggested that completes the related inspection before pregnancy. 

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