How is hereditary nephritis treated?

summary

Hereditary glomerulonephritis (Alport syndrome, as) is a hereditary glomerular basement membrane disease characterized by hematuria, progressive renal dysfunction, sensorineural hearing loss and ocular abnormalities. It is caused by the mutation of collagen IV gene, the main collagen component of glomerular basement membrane. The incidence of gene mutation is about 1 / 10000 ~ 1 / 5000. So let's talk about how to treat hereditary nephritis?.

How is hereditary nephritis treated?

First: drug intervention in recent years, it has been reported that cyclosporine and angiotensin converting enzyme inhibitors have positive effects on reducing urinary protein and delaying the progression of renal disease to end-stage renal disease in patients with Alport syndrome. Aldosterone receptor blockers and angiotensin receptor antagonists can reduce urinary protein in patients with Alport syndrome. However, due to the lack of sufficient evidence-based medicine, the curative effect of these studies is still inconclusive.

Second, renal replacement therapy is feasible for patients with Alport syndrome progressing to end-stage renal disease. Kidney transplantation is an effective treatment for this disease. However, it is reported that about 3-5% of Alport syndrome patients who received kidney transplantation produce antibodies to the normal glomerular basement membrane of the transplanted kidney, and then develop anti glomerular basement membrane nephritis, which leads to the failure of transplantation. In addition, the authors reported that anti glomerular basement membrane glomerulonephritis could occur again after transplantation because of the occurrence of anti glomerular basement membrane glomerulonephritis after transplantation, and in the case of transplantation failure, anti glomerular basement membrane glomerulonephritis could still occur again after transplantation. However, if anti basement membrane nephritis has occurred after transplantation, living kidney is not used at the end of transplantation. Some studies suggest that female carriers of heterozygous COL4A5 gene, who have no clinical manifestations of proteinuria, hypertension, renal dysfunction and deafness, can be used as donors, but the probability of renal insufficiency after transplantation is higher than that of healthy donors.

Third: gene therapy although the mutation genes of various genotypes of Alport syndrome have been identified in recent years, and the gene therapy of animal models of Alport syndrome has achieved certain results, there are still a series of problems in gene therapy, such as the low efficiency of gene transfection, the way of target gene introduction, the selection of introduction time / opportunity, the survival time in vivo The safety of vectors and the regulation of target genes are not well resolved. Therefore, it is still a long time for gene therapy of Alport syndrome to be used in clinic.

matters needing attention

Early diagnosis and eugenics. Rest properly and avoid strenuous exercise. But when the condition is stable, appropriate exercise is necessary.