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What is the cause of congenital nephrotic syndrome in children?

2017-05-29 10:13

First, the cause of the disease

According to the cause of classification, usually pided into two categories:

1, primary

Including Finnish congenital nephrotic syndrome, diffuse mesangial sclerosis, minimal lesions, focal segmental sclerosis.

2, secondary

Can be secondary to infection (congenital syphilis, congenital poisonous protozoa disease, congenital giant cell inclusion body disease, rubella, hepatitis, malaria, AIDS, etc.), mercury poisoning, infant systemic lupus erythematosus, hemolytic uremic syndrome, Patellar syndrome, Drash syndrome, renal vein thrombosis and so on.

Second, the pathogenesis

1, the pathogenesis

It has been clarified that the congenital nephrotic syndrome in Finland is an autosomal recessive inherited disease whose genes are located on the long arm of chromosome 19 and the pathogenesis is gradually elucidated. In 1966, 57 Finnish families were genetically surveyed to identify the autosomal recessive hereditary disease. The defective gene was found to be located on the long arm of chromosome 19 13.13. In 1983, 5 cases of congenital nephropathy were detected by cationic probe PEI Syndrome, found that the number of anionic sites of GBM decreased, the authors believe that the reduction of heparin sulfate is caused by the intrinsic glomerular filtration membrane on the protein permeability of the reasons. 1998 reported that the disease has glomerular foot between the cracks in the hole nephrin on the septum, encoding nephrin gene NpHSI mutation caused.

2, pathological changes

Pathology seen by the disease sooner or later. In the early stages of the disease, glomeruli may be normal, may also show focal segmental sclerosis, mesangial cells and mesangial matrix hyperplasia; showing diffuse tubular cystic expansion. In the late stages of the disease, glomerular capillary loop collapse, showing diffuse hardening; renal tubular expansion, atrophy; interstitial inflammatory cell infiltration and fibrosis. It has been said that the most characteristic change in the eigenvalue is the proximal tubule cystic dilatation, known as "small cystic disease", but found that the renal tubular lesions may be acquired, due to sustained large amounts of proteinuria and (Or) tubules within the urine flow blocked, resulting in incomplete nephrons appear tubular cystic changes. Immunofluorescence examination of early disease without Ig and complement calm. Immunoelectron microscopy showed nephrin abnormality on the septum of the foot. Electron microscopy showed endothelial cell swelling, epithelial cell fossa fusion, basement membrane shrinkage and so on.

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