2018 Volume 31 Issue 2 Pages 96-108
IgA nephropathy (IgAN) and Henoch-Schönlein purpura nephritis (HSPN) are most frequently found chronic glomerulonephritis (CGN) in childhood. Recently, it has been reported that the pathogenesis of IgAN and HSPN may be associated with the circulation of defective forms of IgA1 or the presence of in situ immune complexes, and the onset and progression of inflammation are thought to be associated with complement components, activated macrophages and mesangial cells. To prevent progression to chronic renal injury, it is important to control these abnormal immunoresponse. For children with severe CGN, the long-term prognosis was improved by multi-drug combination therapy including steroid and immunosuppressive drugs, plasmapheresis and LDL-apheresis. On the other hand, hemolytic uremic syndrome (HUS) is defined as a triad of microangiopathic hemolytic anemia, thrombocytopenia, and acute renal failure and the cause is Shiga toxin-producing Escherichia coli infection in most infant cases. We produced an adequate HUS model for the study of the recovery process from acute renal injury, and studied its mechanism, renal regeneration factor, and the treatment of HUS by using this model.
