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What is congenital nephrotic syndrome?

2017-06-01 14:56
Congenital nephrotic syndrome refers to born shortly after about 3 ~ 6 months have disease of a kind of kidney disease, it has four major characteristics of nephrotic syndrome, namely (1) a large amount of proteinuria, qualitative check (+ + +), or quantitative daily more than o. 1 g/kg; Hypoalbuminemia, serum albumin < 3g/l; Hypercholesterolemia, serum cholesterol exceeds 5. Tendency for 72 / l; (4) edema. Congenital nephrotic syndrome can be divided into primary and secondary, primary type is mainly Finland congenital nephrotic syndrome, the hair can be secondary to infection (such as congenital syphilis, CMV inclusions), rubella, hepatitis virus, poisoning, hemolysis urine toxin syndrome, etc.
Finland is autosomal recessive congenital nephrotic syndrome, often for premature infants, children have larger placenta, small nose, the bridge of the nose is low, wide between the eyes, muscle tension. Have proteinuria at birth, soon appear edema and ascites, and there is often an umbilical hernia, feeding difficulties, children are children, growth retardation, some children can show high coagulation state, lead to thromboembolic complications. Early kidney function was normal but prone to infectious diseases. There is no specific treatment for the disease, which can only be used to treat disease and support, prevent infection, reduce edema, steroid and immunosuppressant. The prognosis of this disease is poor, more than 6 months to 1 year old to die from infection, if can survive to 2 ~ 3 years old often die from uremia. So the most radical treatment is a kidney transplant after the age of two.

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