Objective To investigate the clinicopathological features and treatment of children with C3 glomerulopathy (C3 G).Methods Seven children diagnosed as C3 G by clinical and pathological characteristics were enrolled in this study.The clinicopathological data and the prognosis were analyzed.Results Of the 7 cases,4 cases were female and 3 cases were male,with the mean age of (7.7-± 3.1) years old (1.5-10.4 years old) at onset,the duration from onset to renal biopsy was (3.4 ± 2.4) months (1-6 months) and 1 of them had a second renal biopsy 4.2 years later,and mean age was (8.4 ± 3.6) years old (1.8-13.3 years old) on diagnosis.Clinical features:among the 7 patients,6 cases had hematuria,among them 1 case had gross hematuria and 5 cases had microscopic hematuria;6 cases had low level of serum complement C3,5 cases had heavy proteinuria and low serum albumin,and anemia was observed in 2 cases respectively.Five cases had complement factor H and H factor antibody by examination,and 1 of them had low serum factor H,but none of them had serum antibody to factor H.Four cases had genetic evaluation,and only 1 case revealed risk variants in the C3 gene(R304R,T612T,V807V,A915A,P1632P)and CFH gene(p.H402Y,p.E936D).Clinically,4 cases were diagnosed as nephrotic syndrome of nephritis type,2 cases were diagnosed as nephritic syndrome,and 1 case was diagnosed as nephrotic syndrome of simple type.Immunofluorescence study showed that all the cases had intense deposition of C3,and 6 cases were accompanied by the deposition of immunoglobulin.Under light microscopy,3 cases showed the feature of membrane proliferative glomemlonephritis,2 cases with endocapillary prolifera-tive glomerulonephritis,1 case with mesangial proliferative glomerulonephritis,and 1 case with endoeapillary proliferative IgA nephropathy.Under electron microscopy,3 cases who had typical ribbon-like dense deposits in glomerular basement membrane were of dense deposit disease,and the rest were C3 glomerulonephritis.All patients had steroid and immune inhibitor treatment,and during the follow-up stage of (2.6 ± 1.8) years(1.1-5.6 years),4 cases showed normal urinalysis,2 cases had microproteinurine and microscopic hematuria,and 1 case had urinary protein ± to + + and microscopic hematuria.Conclusions C3G has variety of pathological-clinical manifestation.Interpretation of individual cases depends on integration of information from the biopsy together with clinical,serological,and genetic features.Patients with steroid and immune inhibitor treatment had some clinical improvement of their urinalysis.

Objective:To investigate the protective effect of ulinastatin on sepsis-acute kidney injury (SA-AKI) by NF-κB signaling pathway.Methods:Total of 60 mice were randomly(random number) divided into sham group, cecal ligation puncture group (CLP group) and ulinastatin treatment group (CLP+UTI group). Ulinastatin treatment group was intraperitoneally injected with ulinastatin 50 000 U/kg once a day. 24 hours after operation, five mice were sacrificed, the kidney tissues were collected to observe renal histopathology by HE staining. The macrophage infiltration was observed by immunohistochemistry. The remaining mice in each group were used to calculate the survival rate of 7-day after operation. HK-2 cells were stimulated by LPS to obtain the SA-AKI model, and the cells were divided into control group, LPS group and LPS + UTI group. CCK-8 assay was used to detect cell viability, EdU assay was used to detect cell proliferation, and JC-1 assay was used to detect mitochondrial damage. The phosphorylation degree of NF-κB was detected by western blot. Inflammatory factors concentrations of cellular supernatant were detected by ELISA assay.Results:Compared with the sham group, the kidney tissue of mice in CLP group showed that kidney pathological obvious changed, the infiltration of macrophages increased, and the survival rate of mice decreased. CLP+ UTI group reduced the pathological changes and the infiltration of macrophages, improved the survival rate of mice. Compared with control group, LPS group obviously inhibited the cells activity and proliferation of HK-2 cells, damaged the mitochondrial membrane potential of HK-2 cells. Compared with LPS group, LPS+ UTI group attenuated the phosphorylation of NF-κB, decreased the secretion of inflammatory factors, rescued the activity and proliferation of HK-2 cells, and reduced the damage of HK-2 mitochondrial membrane potential.Conclusions:Ulinastatin can reduce mitochondrial damage, inhibit the secretion of inflammatory factors and improve the function of renal tubular epithelial cells through regulating NF-κB signaling pathway.