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What about congenital nephrotic syndrome?

What about congenital nephrotic syndrome?

Have you ever heard of congenital nephrotic syndrome? This is a rare kidney disease in infants, but it can be serious if the disease is present. Congenital nephrotic syndrome refers to a disease shortly after birth, about 6 months of onset of 3 to kidney,...


Have you ever heard of congenital nephrotic syndrome? This is a rare kidney disease in infants, but it can be serious if the disease is present.

Congenital nephrotic syndrome refers to a disease shortly after birth, about 6 months of onset of 3 to kidney, it has four characteristics: the nephrotic syndrome, proteinuria, qualitative check (=), quantitative daily more than o. 1g/kg; hypoproteinemia, serum albumin, <3g/L, hypercholesterolemia, serum cholesterol exceeding 5. 72mmol/L. Edema. Congenital nephrotic syndrome is pided into primary and secondary, primary is the main type of congenital nephrotic syndrome in Finland, following the onset may be secondary to infection (such as congenital syphilis, rubella and CMV, Gan Yan), poisoning, hemolytic uremic syndrome.

Finland congenital nephrotic syndrome belongs to autosomal recessive inheritance. The children are usually premature, have large placenta, small nose, low nasal bridge, wide eye distance and poor muscle tone. At birth have proteinuria, edema and ascites occurred quickly, and often with umbilical hernia, children with feeding difficulties, prone to vomiting and diarrhea, growth retardation, in hypercoagulable state, causing thromboembolic complications. Early renal function is normal, but it is susceptible to infectious diseases. There is no specific treatment for this disease. Only symptomatic and supportive treatment can be used to prevent infection, reduce edema, and steroids and immunosuppressive agents are ineffective. The prognosis of this disease is poor, more than June to 1 years old, died of infection, such as can survive to 2~3 years old, often die of uremia. Therefore, the most thorough treatment is renal transplantation after the age of 2.

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