Minimal change nephrotic syndrome (MCNS) is the most common nephrotic syndrome among children.Although the details of pathogenesis remain unknown, it is widely considered that upregulated expression of T lymphocyte cell cytokines contributes to the initiation of MCNS.Moreover, studies had revealed that altered number and function disorder of B lymphocyte cells could change the functions of antigen presentation, participating in the onset of MCNS by affecting the function of T lymphocyte cells.Recently, CD80 has emerged as a popular research topic which exerts its effects via the change of podocytes morphology, thereby affecting the glomerular permeability.However, neither immune disorder nor podocyte dysfunction is poorly demonstrated to be associated with the pathogenesis of MCNS, the hypothesis such as "a 'two hit disorder" and "γδT cells exacerbate podocyte injury via the CD28/B7-1-phosphor-SRC kinase pathway" are raised.In the current review, we summarized the related investigations to help us to understand the mechanisms and pathogenesis of MCNS.

Osteoporosis is often considered as a disease of the aging.In recent years, osteoporosis in children is increasingly recognized.Glucocorticoid (GC) is the most common factor for this disease, GC exposure during the pedia-tric age, in particular during the adolescent growth spurt, can significantly does harm to normal bone development and increases the risk of fracture, pain and disability, impairing the quality of life.Now, the recent advances on glucocorticoid-induced osteoporosis (GIOP) are discussed, and the importance of attention to GIOP in children is raised.

Objective To investigate the clinical efficacy and safety of continuous blood purification (CBP) in the treatment of severe sepsis in infants. Methods A retrospective analysis of 40 infants with severe sepsis treated with CBP was performed at PICU of Hunan Children's Hospital from January 2014 to July 2017,and 50 infants with severe sepsis who were not treated with CBP at the same period were enrolled as control group. The indicators included blood gas analysis,lactic acid (Lac),blood glucose,electrolytes, blood routine,C-reaction protein ( CRP) and procalcitonin ( PCT),alanine aminotransferase ( ALT) and aspartate aminotransferase (AST),total bilirubin(TB),urea nitrogen (BUN),serum creatinine (Scr) and creatine kinase isoenzyme MB (CK-MB),pediatric critical illness score (PCIS). Results (1) After 3 days of treatment,the recovery of body temperature,heart rate,respiratory rate and blood pressure in CBP group were better than those in the control group,and the differences between two groups were statistically signifi-cant(P < 0. 05). (2) After treatment,the levels of base excess(BE),Lac,white blood cell (WBC),PCT, CRP,ALT,AST,TB and BUN of CBP group recovered better than those of the control group,the differences were statistically significant (P < 0. 05). (3) The ΔPCIS (D3-D1) of CBP group was higher than that of the control group,and the difference between two groups was statistically significant (P < 0. 05). (4) The fatali-ty rate of CBP group was lower than that of the control group,and there was no significant difference between two groups (P > 0. 05). (5) Two cases of thrombocytopenia,2 cases of femoral vein thrombosis,2 cases of hypovolemic shock,and 1 case of blood coagulation in filter happened in CBP gruop,all cases had no punc-ture site infection. Conclusion CBP can improve the vital signs,internal environment,inflammatory reaction and organ function of infants with severe sepsis,and the effect is better than that of traditional methods. The complications of CBP in infants with severe sepsis are relatively large,so we should strictly master the indica-tions of CBP in the treatment of severe sepsis in children.

Objective To explore the risk factors of primary nephritic syndrome complicated with thrombosis in children. Methods Clinical data of 238 children with primary nephritic syndrome were retrospectively analyzed. The children were divided into thrombosis group and non-thrombosis group according to whether complicated with thromboembolism. Univariate and logistic regression analysis were performed.Results Among 238 children, there were 32 cases of primary nephritic syndrome complicated with thrombosis and the rate was 13.44%. Univariate analysis showed that infections, the use of diuretic, degree of edema, white blood cell count, IgG, C3, total protein, albumin, urea nitrogen, plasma ifbrinogen, D-dimer, antithrombinⅢ, and 24-hour proteinuria were signiifcantly different between two groups (allP< 0.05). Multivariate analysis showed that D-dimer and 24-hour proteinuria were the independent risk factors for children with primary nephrotic syndrome complicated with thrombosis.Conclusion The elvated level of D-dimer and 24-hour proteinuria were the risk factors of children with primary nephrotic syndrome complicated with thrombosis.

Objective To investigate the changes in the concentration of myocardin in children with lupus nephritis (LN) under different degree of vessel damage.Methods Forty-nine children diagnosed with LN by routine tissue immunolfuorescence, light microscope, and electron microscope were included, and 30 healthy children were included as control group. The pathological classiifcations were performed according to the ISN/RPS 2003 LN pathological classiifcation criterion. According to the Katafuchi evaluation method, the semi quantitative assessment of glomerular and kidney tubule damage was carried out, and the degree of vascular damage was evaluated at the same time. Double antibody sandwich method was used to detect the concentration of serum myocardin.Results The glomerular and kidney tubules damage in children with LN were signiifcantly aggravated with higher pathological classification (P<0.05). Glomerular damage was positively correlated with renal interstitial damage (r=0.96, P<0.01). The degree of vascular damage was related to the degree of glomerular injury and renal interstitial injury, while it was no related with the results of clinical tests. There were different concentrations of myocardin among mild-, moderate-, severe-vessel damage and control groups (F=378.61,P<0.001), and the concentration of myocardin in moderate- and severe-vessel damage groups were obviously lower than those in control group and mild-vessel damage group (P<0.01) while there was no difference between control group and mild-vessel damage group (P>0.05). According to pathological type, there were signiifcant differences in the concentration of myocardial between control group and different pathological types (F=626.793,P<0.01). FromⅡ,Ⅲ,Ⅲ+Ⅴ,Ⅳ toⅣ+Ⅴ, the concentrations of myocardial were decreased systematically, and there were statistic differences between groups (P all<0.05).Conclusion The concentration of myocardin in children with LN can relfect the renal vascular damage to a certain extent. Elevation of myocardin concentration may be helpful for the repair of vascular damage.

Objective To discuss the role of EB virus (EBV)in the pathogenesis of systemic lupus erythematosus(SLE) in children through investigating the copies of EBV DNA and expression of EBV genes in peripheral blood mononuclear cells(PBMCs).Methods (1)PBMCs were isolated from 30 patients with SLE and 12 healthy normal controls respectively and DNA was extracted from PBMCs.(2) PBMCs were co-cultured with EBV for 12 days and RNA was extracted from PBMCs.(3)Real-time fluorescence quantitative PCR(Real-time PCR) was applied to detect the copies of EBV DNA in PBMCs.(4)Reverse transcription PCR was applied to detect expression of EBV genes.Results (1) Compared with the healthy control group [(40.1 ± 11.6) copies/μg],a significant increase of EBV DNA copies was observed in SLE group[(658.6 ± 183.6) copies/μg] (P ＜0.05).The EBV DNA copies in the active SLE group [(785.2 ± 179.2) copies/μg] were significantly higher than those in the non-active SLE group [(586.0 ± 193.1) copies/μg] (P ＜ 0.05).(2)There was no correlation between EBV DNA copies and systemic lupus erythematosus disease activity index (r =0.03,P ＞ 0.05).(3) After PBMCs got co-cultured with EBV,expression of latent EBV genes and lytic genes were both increased in the patients and healthy controls.The latent EBV genes including latent membrane protein 1 (LMP1),LMP2,EBV nuclear antigen 1 and the lytic genes including BCRF1,BLLF1 were all increased significantly in the patients compared with the healthy controls (all P ＜ 0.05).Conclusions There is a significant increase of EBV DNA copies and aberrant expression of EBV genes in SLE patients,which suggests that EBV may contribute to the pathogenesis of SLE.

Objective To investigate the expression and significance of Epstein-Barr virus (EBV) genes in children with systemic lupus erythematosus (SLE).Methods Peripheral blood mononuclear cells (PBMCs) were isolated from 20 children with SLE and 12 healthy human controls.Enzyme-linked immunosorbent assay (ELISA) was conducted to detect anti-EBV viral capsid antigen (VCA) IgG/IgM antibodies.The culture supernatants of cells from patients with anti-EBV VCA IgG/IgM antibodies were collected,and PBMCs from the patients and controls were co-cultured with the supernatants respectively for 12 days.RNA was extracted from PBMCs before and after the coculture,and reverse transcription-PCR was performed to detect the expression of EBV genes,including LMP1,LMP2,EBNA1,BCRF1,BLLF1 and BILF1 genes.Results LMP1 gene was detected in fresh PBMCs from 10 out of 20 patients and 1 out of 12 controls (P ＜ 0.05).No significant differences were observed between the patients and controls in the detection rate of LMP2 gene (4/20 vs.1/12),EBNA1 gene (13/20 vs.3/12),BCRF1 gene (3/20 vs.1/12) or BLLF1 gene (5/20 vs.2/12) in fresh PBMCs.After co-culture with the supernatants of cells from patients with anti-EBV VCA IgG/IgM antibodies,the expressions of EBV genes in these PBMCs were increased to different degrees,and there was a significant difference in the expressions of EBV latent genes LMP1,LMP2 and EBNA-1 as well as EBV replicative genes BCRF1 and BLLF1 between the patient-derived and control-derived PBMCs (all P ＜ 0.05).Conclusions There is an aberrant expression of EBV genes in children with SLE,and EBV genes may contribute to the development of SLE.

The incidence of acute kidney injury(AKI) in neonate is not low,it occurs in many cases such as ischemic-hypoxic injury,infection,administration of nephrotoxicity drugs and urinary tract obstruction,of which perinatal asphyxia ranked first in China.Due to the severe food security crisis,the occurrence of urinary tract obstruction is rising in recent years.The child health care should pay attention to prevent and screen this kind of disease for the high risk group.The diagnosis of AKI is difficult for newborn because serum creatinine and urine are hard to make a definite boundary.So study of early markers of AKI seems to be of great importance,of which neutrophil gelatinase associated lipocalin and cystatin C are research focuses,treatments should aim at solving primary disease such as ischemic,and so on.Renal replacement therapy is recommended when it comes to severe cases,but mortality still remains high,corresponding to severe primary disease and complications.

Objective To research Henoch-Schonlein purpura purpura (HSP) and renal pathology in children.Methods 31 hospitalized HSP children that with normal urine routine and accepted renal biopsy in our hospital.Results There were different levels of kidney pathological damage in this group of 31 cases,the results of light microscope were from grade Ⅱ to grade Ⅵ The proportion was grade Ⅱ(35.48％,11 of 31),grade Ⅲ (54.83％,17 of 31),and grade Ⅳ,Ⅴ and Ⅵ (each 1 case of 31,3.23％ ).lmmunofluorescence pathology results were showed as following:merely IgA depositional (48.38％,15 of 31 ),IgA + IgG depositional ( 19.36％,6 of 31 ),IgA + IgM depositional ( 19.36％,6of 31 ),IgA + igG + IgM depositional ( 12.90％,4 of 31 ).Microalbuminuria had been founded in 14 cases,and the microalbuminuria level of 10 cases were higher than normal value( 10 of 14,71.43％ ).Conclusions HSP children had renal pathologic dysfunction,even the urine routine were normal,and the detection of urine microalbumin was a significant marker in the early stage.

ObjectiveStudy the relationship among CaSR expression, tubulointerstitial damage,metabolic disturbance of calcium and phosphorus and microvascular density around the tubulointerstitium in children with steroid-resistant nephrotic syndrome.Methods36 cases of children with primary nephrotic syndrome were divided into hormone-sensitive group and steroid-resistant group.Semi-quantitative scores for tubulointerstitial pathological evaluation of the extent of damage, automatic biochemical analyzer for the determination of serum calcium (Ca), phosphorus (P) concentration of renal tubular epithelial CaSR expression and microvessel microvascular density around the tubulointerstitium were determined by immunohistochemical assay.ResultsMore severe the tubulointerstitial damage, lower level of serum Ca and higher level of serum P were observed [(2.26 ± 0.15) mmol/L]in children of the steroid-resistant group and the steroid-sensitive group [(1.90 + 0.12) mmol/L, P ＜ 0.05].CaSR expression (4.63 + 0.78) of renal tubular epithelial cells in the steroid- sensitive group was significantly lower than that in the steroid-resistant group (6.56 + 1.22, P ＜ 0.05), but microvascular density was significantly higher in the steroid- sensitive group(2.98 +0.35 vs 2.02 +0.24, P ＜0.05).When the tubulointerstitial damage was mild, CaSR expression (4.15 +0.58) in renal tubular epithelial cells in the steroid- sensitive group (4.26 ±0.61) was lower than the steroid-resistant group(3.12 ± 0.33; 3.01 ± 0.21), and microvascular density was higher,but the difference was not significant(P ＞0.05).In the moderate tubulointerstitial damage, CaSR expression in renal tubular epithelial cells in the steroid- sensitive group (5.35 ± 0.64) was significantly lower than the resistant group (7.37 +0.81, P ＜0.01), and microvascular density was significantly higher than the resistant group (2.81 ±0.16, 2.02 ±0.14, P ＜0.05).Compared by mild and moderate tubulointerstitial damage in children with the steroid-resistant, CaSR expression (11.46 ± 1.38) in children with severe tubulointerstitial damage was significantly increased, and microvascular density (1.15 ± 0.11) was significantly decreased (all P ＜ 0.01).ConclusionsCaSR expression was increased and microvascular density around the tubulointerstitium was decreased in children with steroid-resistant nephrotic syndrome.Dut to steroid resistance, the cytotoxic of steroid damaged the renal tubular epithelial cells, the metabolic disturbance of calcium and phosphorus and the damage of blood vessel endothelium finally resulted in severe tubulointerstitial damage.