2015年11月16日星期一

Congenital Nephrotic Syndrome in Children

Congenital Nephrotic Syndrome is a genetic disease with autosomal recessive inheritance. The girls and boys have the same chance of inheriting the disease. Congenital Nephrotic Syndrome presents at birth or within the first three months birth.

In most cases, the infants with Congenital Nephrotic Syndrome are born prematurely so they often have a lower weight than the healthy infants. In more than half of cases of Congenital Nephrotic Syndrome, the babies have edema at birth or during the first week after birth. In severe case, they may even have marked ascites. Most of the babies have selective proteinuria, but hematuria is not common. As they may loss a large amount of protein in urine, they are often accompanied by profound hypoproteinemia. As a result, the babies can not get sufficient nutrition supplement thus influencing their growth seriously. Moreover, they are very susceptible to bacterial infections like respiratory infection, urine tract infection and so on. Generally speaking, Congenital Nephrotic Syndrome progresses into End Stage Renal Failure between 3 and 8 years of age. Then the children have to prolong their survival by effective treatment.

In the conservative treatments, there are no effective therapies for Congenital Nephrotic Syndrome. The children are usually recommended to keep albumin infusion, increase protein intake, keep a low-salt diet, keep enough vitamin supplement, and prevent infections and other complications. However, those treatments can not stop the disease from progressing at all. With the disease aggravation, some children have to receive bilateral nephrectomy. When the children grow 8~9 kg, dialysis is available for them. At that time, the parents should make preparations for Kidney Transplant. However, after Kidney Transplant, the children have to endure serious rejection reaction and depend on immunosuppressive agents in the rest of their life.


Therefore, the parents of children with Congenital Nephrotic Syndrome should choose a better treatment when their diseases are still mild so as to avoid dialysis and Kidney Transplant in the later years. If you are still worrying about your children’ s health problem and want to know more information about Congenital Nephrotic Syndrome, please email to kidney-treatment@hotmail.com.

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