ABSTRACT
Nephrotic syndrome is a clinical syndrome characterized by massive proteinuria, serum hypoalbuminemia, edema, hyperlipidemia.Most children can get relief with hormone therapy, but 15%~20% of children with hormone resistance eventually reach to end-stage nephropathy.Genes associated with steroid resistant nephrotic syndrome(SRNS)include NPHS1, NPHS2, CD2AP, TRPC6 encoding the slit diaphragm of podocytes, ACTN4 and INF2 encoding podocyte cytoskeleton proteins, WT1 encoding the nucleus of podocytes, COQ2 and COQ6 encoding mitochondrial proteins of podocytes, LAMB2 encoding glomerular basement membrane proteins.Research have found that one mutation in one of these molecules is enough to cause SRNS, so SRNS is also known as podocyte molecular disease.
ABSTRACT
C1q nephropathy is a special type of glomerulonephritis, which characterized by massive deposition of C1q on glomerular mesangial.The pathological manifestations of C1q nephropathy are minimal change disease, focal segmental glomerulosclerosis and proliferative glomerulonephritis.C1q nephropathy is prone to hormone dependence or resistance, and the prognosis is not good relatively.The pathogenesis of the disease is not clear.The clinical manifestations are quite different.Glucocorticoid is still the first choice for treatment.Immunosuppressive agents are often used in patients with hormone dependence or resistance.It has been found that the rituximab has good curative effect on C1q nephropathy in recent years.Although the understanding and research on C1q nephropathy is deepening, there is still much controversy about whether to classify C1q nephropathy into a class of independent diseases and the clinical significance of their classification.In this paper, the pathogenesis, pathological histology, clinical characteristics, treatment and prognosis of C1q nephropathy are reviewed in order to have a better understanding of C1q nephropathy.