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.

Objective:To explore the efficacy and safety of high-titer plasma in the treatment of pediatric patients with severe adenovirus pneumonia.Methods:The clinical data of 92 pediatric patients with severe adenovirus pneumonia admitted to pediatric intensive care unit (PICU) in Guangzhou Women and Children′s Medical Center from January 2016 to October 2019 were retrospectively collected. According to the treatment with or without high-titer plasma, the patients were divided into plasma treatment group ( n=41) and non-plasma treatment group ( n=51). The 51 patients with chest radiograph showing more than half the lungs involved were divided into plasma treatment group ( n=29) and non-plasma treatment group ( n=22). According to fever duration before plasma treatment, patients were divided into early group (≤5 days, n=5), middle group (>5-10 days, n=14), and late group (>10 days, n=22). Baseline data, therapeutic effects, and prognosis of patients in each group were analyzed with t test, non-parametric rank sum test, one-way ANOVA and chi-square test. Results:Ninety-two patients were included. There were no significant differences in age, gender, body weight, fever duration, sequential organ failure assessment, and Murray lung injury score between plasma treatment group and non-plasma treatment group before admission (all P>0.05). The proportion of patients whose temperature drop to normal within 5 days was higher in plasma treatment group than that in non-plasma treatment group (88% (36/41) vs. 69% (35/51), χ 2=4.745, P=0.029). However, there were no significant differences between the two groups in the proportions of invasive ventilator weaning within 14 days (63% (26/41) vs. 76% (39/51), χ 2=1.868, P=0.172), transfer out from PICU within 14 days (49% (20/41) vs. 69% (35/51), χ 2=3.724, P=0.054), discharge within 28 days (51% (21/41) vs. 61%(31/51), χ 2=0.846, P=0.358) and survived patients (85% (35/41) vs. 76%(39/51), χ 2=1.143, P=0.285). Among patients with severe chest radiograph, the proportions of patients whose temperature drop to normal within 5 days and survived patients were higher in plasma treatment group than those in non-plasma treatment group (86% (25/29) vs. 59% (13/22), χ 2=4.843, P=0.028; 83% (24/29) vs. 55%(12/22), χ 2=4.796, P=0.029, respectively). However, there were no significant differences between the two groups in the proportions of invasive ventilator weaning within 14 days (52% (15/29) vs. 59% (13/22), χ 2=0.274, P=0.601), transfer out from PICU within 14 days (34% (10/29) vs. 45% (10/22), χ 2=0.632, P=0.427), and discharge within 28 days (45% (13/29) vs. 45% (10/22), χ 2=0.002, P=0.964). Among early, middle and late group, the proportions of invasive ventilator weaning within 14 days were 2/5, 13/14 and 50% (11/22), respectively, with statistically significant difference (χ 2=8.119, P=0.017). There were no significant differences in the proportions of patients whose temperature drop to normal within 5 days (4/5, 14/14, 82% (18/22), χ 2=2.965, P=0.227), transfer out from PICU within 14 days (2/5, 10/14, 36%(8/22), χ 2=4.386, P=0.112), discharge within 28 days (2/5, 8/14, 50% (11/22), χ 2=0.462, P=0.794) and survived patients (4/5, 13/14, 82% (18/22), χ 2=0.966, P=0.617) in the three groups. Only one case with high-titer plasma therapy had rash in the course of infusing plasma and no other adverse reactions were observed. Conclusions:High-titer plasma can shorten the fever time and improve the proportion of survival patients in pediatric severe adenovirus pneumonia. The clinical effect of high-titer plasma is better in 5-10 days of fever course. High-titer plasma is an effective and safe treatment.

Based upon the experiences of planning and implementation of "Dynamic changes of Torque Teno virus load in Chinese renal transplant recipients with immunosuppressive therapy: A multi-center prospective observational double-blind cohort study", this article explored the current status and major difficulties of investigator-initiated multi-center prospective clinical trials of kidney transplantation in China. Various issues of organization design and implementation process management were discussed for enhancing the quality of implementing the relevant trials.