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1.
Article in Chinese | WPRIM | ID: wpr-908024

ABSTRACT

Objective:To analyse the clinical and prognosis of C1q deposition in children with primary membranous nephropathy (PMN).Methods:A retrospective analysis was conducted in 177 children with PMN who were diagnosed by renal biopsy in the Eastern Theater Cornmand General Hospital from July 2005 to September 2013.Patients were divided into C1q deposit group and C1q non-deposit group according to the immunofluorescence staining of C1q.Clinical and pathological characteristics, treatment response, and long-term renal prognosis were compared between the 2 groups.Results:A total of 177 pediatric patients with PMN were included, involving 98 boys and 79 girls with a median age of 192.0 months.During an follow-up of (52.4±35.6) months, 7 cases(4.0%) progressed end-stage renal disease (ESRD), and 14 cases(7.9%) developed ESRD or renal dysfunction.The blood IgG level of C1q deposit group was higher than that of C1q non-deposit group [(5.10±2.51) g/L vs.(4.34±2.10) g/L, t=2.110, P=0.036]. The frequency of glomerular C4 deposits in C1q deposit group was significantly higher than that of C1q non-deposit group (34.7% vs.2.9%, χ2=32.567, P<0.001). The Kaplan-Meier survival analysis showed that there were no differences in cumulative renal survival rate of ESRD ( P=0.561) and cumulative incidence rate of remission ( P=0.291) between groups.The Logistic regression analysis demonstrated that C1q deposition was not correlated with treatment responses ( P=0.587). Univariate COX regression analysis demonstrated that the male gender ( HR=8.578, 95% CI: 1.120-65.689, P=0.039) and no remission ( HR=0.053, 95% CI: 0.017-0.171, P<0.001) were risk factors for renal dysfunction in children with PMN.Multivariate COX regression analysis reveled that no remission ( HR=21.858, 95% CI: 5.595-85.387, P<0.001) and C1q deposition ( HR=0.116, 95% CI: 0.023-0.584, P=0.009) were independent risk factors for renal dysfunction in children with PMN. Conclusions:C1q deposition was an independent risk factor for renal dysfunction in children with PMN.The classical pathway does occur in some PMN patients, which plays an essential role in mediating kidney injury.

2.
Article in Chinese | WPRIM | ID: wpr-907256

ABSTRACT

The formation of crescent is a typical pathological change characterized by a rapid deterioration of renal function.T lymphocytes are involved in the formation of crescent and the pathological progression of crescent nephritis.CD4 + and CD8 + cells in T lymphocyte subsets, and T cell costimulatory factors mediate immune responses in different ways.They participate in the occurrence, development and fibrosis of crescent, or delay their deterioration.In order to provide a new target for clinical treatment of crescent nephritis, we review the mechanism of T lymphocytes in the formation and development of crescentic body.

3.
Chinese Journal of Nephrology ; (12): 176-182, 2021.
Article in Chinese | WPRIM | ID: wpr-885492

ABSTRACT

Objective:To discuss the etiology, clinical manifestations and renal pathological features of acute interstitial nephritis (AIN) in children.Methods:The etiology, clinical manifestations, pathological characteristics, clinical effects and outcome of the children with AIN diagnosed by renal biopsy from January 2010 to December 2019 in Nanjing Jinling Hospital were analyzed retrospectively. The Kaplan-Meier method was used to evaluate the kidney survival rate. Cox regression model was built to analyze the risk factors for developing end-stage renal disease (ESRD) at baseline in AIN children.Results:A total of 51 children with AIN were diagnosed by renal biopsy, including 36 males and 15 females. The age was (12.94±2.55) years old (2-17 years old). The clinical manifestations of AIN in children were various and lack of specificity. Only 2 cases (3.92%) had triad. All of the 51 children with AIN showed acute renal injury (AKI), accompanied by increased serum creatinine and decreased estimated glomerular filtration rate. The stage of AKI was mainly stageⅢ(33 cases, 64.71%). Infection was the main cause (38 cases, 74.51%) and drug factor was the second cause (27 cases, 52.94%) in children with AIN. Nonsteroidal antiinflammatory drugs (NSAIDs) were the main inducers of drug-induced AIN (18 cases, 35.29%). The interstitial inflammatory cell infiltration or interstitial edema was found in 51 children. The infiltration of inflammatory cells was mainly mononuclear cells (46 cases, 90.20%). After 4 weeks of treatment, 32 cases (62.75%), 11 cases (21.57%) and 8 cases (15.69%) showed complete, partial and no recovery of renal function, respectively. After 12 weeks of treatment, 49 cases (96.08%), 0 cases (0) and 2 cases (3.92%) showed complete, partial and no recovery of renal function, respectively. After an average follow-up of 4.0(2.0-15.0) months, 2 case (3.92%) patients developed ESRD. The cumulative survival rates of ESRD at 1 year and 2 years after renal biopsy both were 100%, and renal survival rates at 5 years and 10 years were 96.55% and 72.41%, respectively. Multivariate Cox regression analysis results showed that N-acetyl-β-D-glucosidase (NAG) enzyme level>17.6 U/g·cr ( HR=15.729, 95% CI 1.045-15.977, P=0.042) and IgM deposition in renal tissue ( HR=7.523, 95% CI 1.142-9.541, P=0.033) were independent risk factors for developing ESRD in AIN children. Conclusions:AKI is the main clinical manifestation of AIN in children. The characteritic of renal pathology in AIN is tubulointerstitial lesions. After active treatment, most of the patients have a good prognosis. Prevention of infection and rational use of drugs are the key to reduce the incidence rate of AIN in children. The N-acetyl-β-D-glucosidase enzyme level>17.6 U/g·cr and IgM deposition in renal tissue are independent risk factors for developing ESRD in AIN children.

4.
Article in Chinese | WPRIM | ID: wpr-885056

ABSTRACT

Objective:To evaluate the role of nuclear factor erythroid 2-related factor/ heme oxygenase-1 (Nrf2/HO-1) signaling pathway in dexmedetomidine-induced reduction of oxygen-glucose deprivation and restoration (OGD/R) injury to microglia.Methods:BV-2 microglia were cultured in high-glucose DMEM culture medium supplemented with 10% fetal bovine serum in an normal culture incubator at 37 ℃ (5%CO 2-21%O 2-74 %N 2). The cells were seeded in 96-well plates at a density of 1.5×10 4 cells/ml (200 μl/well) or 6-well plates at a density of 2×10 5 cells/ml (2 ml/well) and divided into 5 groups ( n=30 each) using a random number table method: control group (group C), dexmedetomidine group (group D), group OGD/R, OGD/R+ dexmedetomidine group (group OGD/R+ D) and OGD/R+ dexmedetomidine+ ML385 group (group OGD/R+ D+ ML). The cells in group C were continuously cultured in a normal culture incubator for 26 h. In group D, dexmedetomidine at the final concentration of 10 μmol/L was added, cells were incubated for 2 h, and then were continuously incubated in a normal culture incubator for 26 h. In OGD/R, OGD/R+ D and OGD/R+ D+ ML groups, the culture medium was replaced with glucose-free DMEM culture medium, cells were cultured for 2 h in an incubator at 37 ℃ (5%CO 2-1%O 2-94 %N 2), the culture medium was replaced with high-glucose DMEM culture medium containing 10% fetal bovine serum and then the cells were cultured for 24 h in a normal incubator.Dexmedetomidine at the final concentration of 10 μmol/L was added at 2 h before OGD in OGD/R+ D and OGD/R+ D+ ML groups.Nrf-2 inhibitor ML385 at the final concentration of 4 μmol/L was added at 30 min before dexmedetomidine was added in group OGD/R+ D+ ML.Cells in 6 wells in each group were selected randomly for assessment of cell viability (by methyl thiazolyl tetrazolium assay) and apoptosis (using flow cytometry), and for determination of the concentrations of tumor necrosis factor (TNF)-α, interleukin (IL)-6, and IL-10 in the supernatant (using enzyme-linked immunosorbent assay), the expression of Nrf2 in nucleus, Nrf2 and HO-1(by Western blot ) and the expression of HO-1 mRNA (by real-time polymerase chain reaction). Results:Compared with group C, the cell viability was significantly decreased, cell apoptosis rate and concentrations of TNF-α, IL-6 and IL-10 in the supernatant were increased, and the expression of Nrf2 in nucleus, Nrf2, HO-1 and its mRNA was up-regulated in OGD/R and OGD/R+ D groups ( P<0.05), and no significant change was found in each parameter mentioned above in group D ( P>0.05). Compared with group OGD/R, the cell viability and IL-10 in the supernatant concentration were significantly increased, cell apoptosis rate and concentrations of TNF-α and IL-6 in the supernatant were decreased and the expression of Nrf2 in nucleus, Nrf2, HO-1 and its mRNA was up-regulated in group OGD/R+ D ( P<0.05), and no significant changes were found in the parameters mentioned above in group OGD/R+ D+ ML ( P>0.05). Compared with group OGD/R+ D, the cell viability and concentration of IL-10 in the supernatant were significantly decreased, cell apoptosis rate and concentrations of TNF-α and IL-6 in the supernatant were increased and the expression of Nrf2 in nucleus, Nrf2, HO-1 and its mRNA was down-regulated in group OGD/R+ D+ ML ( P<0.05). Conclusion:The mechanism by which dexmedetomidine alleviates OGD/R injury to microglia may be related to promoting the activation of Nrf2/HO-1 signaling pathway and inhibition of inflammatory responses.

5.
Article in Chinese | WPRIM | ID: wpr-882829

ABSTRACT

Objective:To investigate the efficacy and safety of Rituximab (RTX) in treating children with refractory steroid-resistant nephrotic syndrome (SRNS).Methods:The clinical data of 10 children with refractory SRNS receiving RTX in the Department of Pediatrics, Jinling Hospital from September 2013 to March 2018 were analyzed retrospectively.Results:The age of onset of 10 children (including 5 males and 5 females) was (4.47±2.75) years old.The renal biopsy showed focal segmental glomerular sclerosis in 5 cases (50%), minimal change nephropathy in 3 cases (30%), IgM nephropathy in 1 case (10%), and mesangial proliferative glomerulonephritis in 1 case (10%). Ten children received RTX treatment (1 or 4 doses; 375 mg/m 2 once; maximum: 500 mg) at the age of (6.74±2.62) years old.There were 8 patients (80%) receiving a single dose of RTX, 1 patient (10%) receiving 3 doses, and 1 patient (10%) receiving 8 doses.The follow-up time was 11.93 (5.17, 25.66) months.The remission rates at the 3-month follow-up, 6-month follow-up and last follow-up were 30% (3 patients), 40% (4 patients), and 40% (4 patients), respectively.The 24-hour urinary proteinuria and serum albumin levels were improved in 10 children after RTX treatment, but there were no significant statistical difference(all P>0.05). No significant difference was found in humoral immunity and renal function before and after RTX treatment (all P>0.05). During the treatment and follow-up, 3 patients (30%) developed infusion reaction, 2 patients (20%) showed severe pulmonary infection, and 1 patient (10%) died of severe pulmonary infection. Conclusions:RTX is effective in treating some children with refractory SRNS, and a long-term follow-up should be conducted to prevent infection.

6.
Chinese Journal of Nephrology ; (12): 834-843, 2020.
Article in Chinese | WPRIM | ID: wpr-871012

ABSTRACT

Objective:To analyze the clinical and pathological features, treatment and prognosis of primary membranous nephropathy (PMN) in children.Methods:A retrospective study was conducted in patients with PMN diagnosed by renal biopsy in the Eastern Theater General Hospital from July 1, 2008 to September 30, 2017. The data of patients' general information, laboratory examination, renal pathology and therapeutic regimen were collected. The effects of different drugs in treatment and prognosis of PMN children were analyzed.Results:Among 218 patients with PMN, the ratio of male to female was about 1.32∶1. The age group from 13 to 18 years old (adolescent) accounted for 87.6%, and there was no significant difference in age between the sexes ( P=0.839). The main clinical manifestation was nephrotic syndrome (157 cases, 72.0%). The most common renal pathology stage was stage Ⅱ (101 cases, 46.3%). The positive rates of IgG1 and IgG4 in immunofluorescence staining were 100.0% and 98.5%, respectively, and IgG4 (45 cases, 33.8%) was the most common deposit. The positive rates of serum anti-PLA2R-Ab and kidney tissue PLA2R immunostaining were 53.97% and 82.54%, respectively. The total remission rate of PMN in children treated with tacrolimus combined with steroid was 83.6% and the recurrence rate was 33.3%. After follow-up time of 45.0(23.5-74.0) months, 11 cases (5.0%) developed end-stage renal disease (ESRD). The cumulative survival rates of ESRD at 5 and 10 years after renal biopsy were 95.4% and 63.7%, respectively. The cumulative renal survival rates of ESRD or a 30% decline in eGFR at 5 and 10 years after renal biopsy were 92.7% or 55.9%. Univariate Cox regression analysis demonstrated that hypertension and heavy proteinuria (24-hour urinary protein≥50 mg/kg) predicted a high risk of ESRD, and renal pathologic parameters were not associated with disease progression. Multivariate Cox regression analysis showed that hypertension ( HR=9.517, 95% CI 1.181-76.715, P=0.034) and heavy proteinuria ( HR=3.946, 95% CI 1.126-13.832, P=0.032) were independent risk factors for developing ESRD in PMN patients. However, the effectiveness of Cox regression analysis was analyzed by PASS software, and it was concluded that hypertension was not related with disease progression. Conclusions:PMN should be considered in adolescent patients with nephrotic syndrome. Tacrolimus combined with steroid is more effective than steroid combined with other immunosuppressive agents in treating PMN. After follow-up time of 45.0(23.5-74.0) months, the prognosis of PMN children is acceptable. Heavy proteinuria is an independent risk factor for developing ESRD in children with PMN.

7.
Chinese Journal of Nephrology ; (12): 766-772, 2020.
Article in Chinese | WPRIM | ID: wpr-871008

ABSTRACT

Objective:To investigate the effect of tonsillectomy combined with glucocorticoids therapy on long-term clinical remission and renal prognosis in IgA nephropathy (IgAN) children with recurrent acute onset history of tonsillitis.Methods:The clinical data of children who were diagnosed with primary IgAN from January 2000 to December 2017 in Jinling Hospital were retrospectively analyzed. All participants were treated with long course therapy of glucocorticoids. The children with recurrent acute onset history of tonsillitis were divided into tonsillectomy group and non-tonsillectomy group according to whether to perform tonsillectomy, followed up until the patients' serum creatinine doubled, the estimated glomerular filtration rate decreased by more than 50%, progression to end-stage renal disease, renal replacement therapy or death. The renal survival rate was calculated and compared by Kaplan-Meier method. Univariate and multivariate Cox regression models were used to analyze the effect of tonsillectomy on the renal prognosis of IgAN children.Results:A total of 120 children with IgAN were enrolled in this study, including 40 cases in tonsillectomy group and 80 cases in non-tonsillectomy group. The median follow-up time was 97.5(57.3, 132.0) months. The clinical remission rate in the tonsillectomy group was higher than that in the non-tonsillectomy group (72.5% vs 45.0%, χ2=8.123, P=0.004). The Kaplan-Meier survival curve showed that there was no significant difference in renal survival rate between the two groups (Log-rank test χ2=0.070, P=0.791). Multivariate Cox regression analysis showed that tonsillectomy was not an independent risk factor affecting renal end-point events in IgAN children ( HR=0.986, 95% CI 0.499-1.948, P=0.967). Conclusions:The clinical remission rate of IgAN children undergoing tonsillectomy is higher than that of children without tonsillectomy. Tonsillectomy is not an independent factor affecting renal end-point events in IgAN children. Tonsillectomy does not delay the time of entry into end-stage renal disease for children with IgAN.

8.
Chinese Journal of Nephrology ; (12): 264-270, 2020.
Article in Chinese | WPRIM | ID: wpr-870962

ABSTRACT

Objective:To observe the clinical efficacy of tacrolimus (TAC) and mycophenolate mofetil (MMF) in children with refractory IgA nephropathy (IgAN).Methods:The diagnosis of refractory IgAN was defined as urinary protein level ≥ 50 mg·kg -1·d -1 after treatment with renin-angiotensin system (RAS) blocker and prednisone. Following the case-control matching method, 76 children with renal biopsy diagnosed as refractory IgAN in the Jinling Hospital from January 1, 2012 to December 31, 2016 were retrospectively selected, and the children were divided into TAC group (38 cases) and MMF group (38 cases). The 24 h urinary protein quantity (24hUP), serum albumin (Alb), serum creatinine (Scr), serum uric acid (UA), serum glucose (Glu), adverse reactions and treatment effects were compared between the two groups. Results:There were no significant differences in the age, sex ratio, blood pressure, estimated glomerular filtration rate (eGFR), 24hUP, urine red blood cell count (U-RBC), Scr, Alb, BUN, aspartate transarninase (AST), alanine transarninase (ALT), Glu, pathological Oxford classification, and the proportions of big-dose methylprednisolone treatment before using immunosuppressants between the two groups (all P>0.05), and they were comparable. From 3 months after treatment, the 24hUP levels of the two groups were significantly lower than those of the baseline (all P<0.05), and the 24hUP levels of TAC group were lower than those of MMF group at 3, 6 and 12 months (all P<0.05). The Alb level of TAC group was significantly higher than the baseline value from 1 month of treatment ( P<0.05), while the Alb level in the MMF group was significantly higher from 3 months of treatment ( P<0.05). The Alb levels in the TAC group were higher than those in MMF group after 1, 3, and 6 month of treatment (all P<0.05), and there was no significant difference in Alb level at 12 months between the two groups. The total effective rate, complete remission rate and ineffectiveness rate of the TAC group all showed significant differences with the MMF group from 3 month of treatment (all P<0.05), but there was no difference between the two groups during the follow-up period of partial remission rate, point recurrence rate and cumulative recurrence rate (all P>0.05). The TAC group achieved the maximum effective rate at 6 months (94.7%), while the MMF group achieved the maximum effective rate at 12 months (68.4%), and the difference was statistically significant ( χ2=8.756, P=0.003). The incidence of adverse reactions in two groups had not significant difference (15.8% vs 21.1%, χ2=0.350, P=0.554). However, the blood glucose of TAC group was higher than that of MMF group in the third month of treatment, and the difference was statistically significant [5.02(4.72, 5.22) mmol/L vs 4.42 (4.19, 5.07) mmol/L, Z=-2.745, P=0.006]. Conclusion:Both TAC and MMF in the treatment of refractory IgAN result in a good treatment effect in children, but the TAC reaches the response level faster and the response rate is higher.

9.
Article in Chinese | WPRIM | ID: wpr-864220

ABSTRACT

Focal segmental glomerulosclerosis (FSGS) is characterized by the fusion of foot processes of podocytes, and can lead to end-stage kidney disease in children.The pathogenesis of FSGS has not been fully clarified, but more than 30 pathogenic genes have been identified in FSGS patients in recent years with the development of molecular genetics.These findings prove that the destruction of the structure and function of podocytes plays a role in the pathogenesis of FSGS.In this paper, the research progress of common pathogenic genes of FSGS was reviewed.

10.
Article in Chinese | WPRIM | ID: wpr-864017

ABSTRACT

Objective:To investigate the clinical manifestations, auxiliary examination results, prognosis and treatment of children with typical hemolytic uremic syndrome (D + HUS). Methods:The clinical data of 36 patients diagnosed as D + HUS in the Department of Pediatrics of Nanjing Jinling Hospital from January 2001 to January 2019 were collected, and the laboratory results including blood routine, liver and kidney function, coagulation function, humoral immunity and urine were compared before and after treatment. Results:The white blood cell count[ (9.28±6.77)×10 9/L vs.(11.20±5.93) ×10 9/ L ], C-reactive protein [7.15(3.34, 29.33) mg/L vs.31.83(25.03, 39.75) mg/L], reticulocyte count [(112.49±76.25)×10 9/L vs. (206.49±147.99)×10 9/L], erythrocyte sedimentation[15.02(11.79, 22.83) mm/1 h vs.28.06(24.13, 40.52) mm/1 h] , aspartate aminotransferase[50.04(41.92, 60.11) U/L vs.62.61(54.58, 83.52) U/L], alanine aminotransferase [16.72(11.80, 24.74) U/L vs.24.54(20.30, 34.36) U/L], uric acid [(532.84±309.06) μmol/L vs.(606.64±327.23) μmol/L], serum creatinine[160.07(124.87, 221.18) μmol/L vs.200.56(160.62, 283.01)μmol/L ], blood urea nitrogen [20.74(15.77, 28.40) mmol/L vs.33.67(25.91, 45.84) mmol/L], lactate dehydrogenase [488.21(337.59, 692.82) U/L vs.1 520.68(734.24, 2 272.10) U/L ], prothrombin time [(12.14±5.89) s vs. (17.91±6.12) s ], activated partial thrombin time [(25.05±6.26) s vs.(32.38±5.49) s], fibrinogen [ (3.79±2.17) g/L vs.(5.17±3.88) g/L], D-dimer [0.92(0.30, 1.13) mg/L vs. 1.27(1.01, 1.90) mg/L ], 24-hour urinary proteinuria [ (84.05±44.19) mg/(kg·24 h) vs.(112.18±78.26) mg/(kg·24 h) ], urinary sediment [175.73(79.72, 258.66)×10 7/L vs. 160.38(118.68, 361.83)×10 7/L], N-acetyl-β-D-glucosaminidase [25.10(18.84, 33.02) U/(g·cr) vs. 41.57(29.49, 58.61) U/(g·cr)], urinary retinol binding protein [0.35(0.18, 1.33) mg/L vs 1.05(0.66, 1.68) mg/L.] in patients after treatment were significantly lower than those before treatment, and the differences were all statistically significant(all P<0.05); patients had higher levels of red blood cell count [ (4.51±1.73)×10 9/L vs.(2.43±1.40) ×10 9/L], platelet[(126.82±78.35)×10 9/L vs. (85.21±69.38)×10 9/L], hemoglobin[(118.46±18.27) g/L vs. (62.36±16.11) g/L], and complement C 3levels [(0.74±0.39) g/L vs.(0.58±0.27) g/L ] after treatment, and the differences were all all statistically significant(all P<0.05). Children with D + HUS showed multiple system injuries.Among 36 cases, 17 cases (47.22%) had fever, 31 cases (86.11%) had abdominal pain and diarrhea, 29 cases (80.56%) had nausea and vomiting, 8 cases (22.22%) had headache and dizziness, 36 cases (100.00%) had proteinuria and hematuria, 34 cases (94.44%) had renal insufficiency, and 21 cases (58.33%) had yellow staining of skin and sclera.The auxiliary examination for abnormal results mainly included renal pathology (100.00%) (mesangial proliferation endothelial cell proliferation and swelling, and shedding of renal tubular brush borders), bone marrow pathology (100.00%) (active bone marrow hyperplasia), and renal B-ultrasound (86.67 %) (kidney injury-like sound image). Conclusions:D + HUS in children shows multiple system damage.Digestive system abnormalities are the main causative factor of D + HUS in children, and the disease is dangerous.Therefore, early diagnosis and active treatment can improve the prognosis.

11.
Chinese Journal of Nephrology ; (12): 655-661, 2019.
Article in Chinese | WPRIM | ID: wpr-756093

ABSTRACT

Objective To investigate the clinical and pathological features and prognosis of children with IgA nephropathy with C1q deposition. Methods The children with IgA nephropathy diagnosed by renal biopsy from January 1, 2000 to December 30, 2017 were retrospectively analyzed and divided into C1q deposit group and C1q negative group according to glomerular immunofluorescence examination. Follow-up until the patient's serum creatinine doubled, glomerular filtration rate decreased by more than 50%, entering end-stage kidney disease, renal replacement therapy or death. Kaplan-Meier survival analysis was used to evaluate the renal survival rate in two groups. Univariate and multivariate Cox proportional hazard regression models were used to analyze the effect of C1q deposition on the prognosis of patients with IgA nephropathy. Results There were 60 cases in C1q deposition group and 60 cases in C1q negative group. (1) the initial eGFR and plasma albumin in C1q deposition group were lower than those in C1q negative group, while the levels of serum creatinine, serum cholesterol and 24 hour urinary protein in C1q group were higher than those in C1q negative group (all P<0.05). (2) pathological indexes:Mesangial cell proliferation, tubular atrophy/interstitial fibrosis, and cell/fibrocytic crescein score in C1q negative group were significantly higher than those in C1q negative group (all P<0.0.5). (3) Kaplan-Meier analysis showed that there was significant difference in renal cumulative survival rate between the two groups (Log-rank test:χ2=6.801, P=0.009). Cox proportional hazard regression model showed that the risk of renal end-point events in IgAN children with C1q deposition group was 5.772 times higher than that in C1q negative group (HR=5.772, 95%CI: 1.353-24.6211, P=0.018). Conclusion C1q deposition is an independent risk factor for the progress of renal function in IgA nephropathy children.

12.
Article in Chinese | WPRIM | ID: wpr-752240

ABSTRACT

Objective To observe the long_term efficacy and adverse reactions of Rituximab( RTX)in the treatment of children with frequently relapsing nephrotic syndrome(PRNS),and to explore the feasible treatment plan of RTX in children with PRNS. Methods PRNS children with RTX[375 mg∕(m2·time),2_3 times]from Depart_ment of Dediatrics,Jinling Hospital,Nanjing Clinical School of Southern Medical University between Pebruary 2011 and December 2017 were retrospectively reviewed,and followed up for 12 _36 months. Age,gender,number of relapses, dose of steroids and immunosuppressants,adverse reactions and laboratory indicators(peripheral blood CD20 ﹢B lympho_cyte count,24_hour urine protein quantification,etc)were observed. Results Thirty_four patients(23 males and 11 females)with PRNS were included in the present study,and the median age for the first RTX treatment was 6 years (2_12 years). After the first treatment,there was complete remission in 34 patients(100%,34∕34 cases),and 12 pa_tients(35%,12∕34 cases)relapsed during follow_up. The number of relapse after treatment[(0. 27 ± 0. 45)times] significantly decreased compared with that before treatment[(2. 94 ± 1. 08)times;t﹦11. 9,P〈0. 05]. After the second treatment,3 children relapsed due to "infection" and no discomfort was found in the first 6 months;5 of 23 cases (21. 7%,5∕23 cases)relapsed once and 11 were unclear in the following 6 months. There was a difference between the 2 treatment intervals 〈12 months(12. 5%,2∕16 cases)and ≥12 months(55. 5%,10∕18 cases). After the third treatment,with an interval of 6 to 15 months,1 of 15 patients(6. 67%)relapsed and the rest were stable. In addition, there was a significant difference in the mean accumulated steroid dose of 20 patients between 6 months before treatment [(2. 50 ± 0. 87)g ]and 6 months after treatment[(1. 30 ± 0. 97)g;t﹦6. 05,P﹦0. 001]. Of the 15 patients after RTX treatment for 6_12 months Tacrolimus was reduced from[(1. 62 ± 0. 77)mg∕24 h ]to[(0. 62 ± 0. 96)mg∕24 h;t﹦6. 80,P﹦0. 000]. Two patients after RTX first infusion had chest tightness,palpitations,nausea,vomiting,dizzi_ness,and headache,3 cases had mild upper respiratory tract infection and 1 case had severe pulmonary infection. Conclusion Long_term follow_up of PRNS children treated with RTX turns out to be safe and effective.

13.
Chinese Journal of Nephrology ; (12): 177-183, 2019.
Article in Chinese | WPRIM | ID: wpr-745962

ABSTRACT

Objective To analyze the spectrum of children's kidney pathology by renal biopsy.Methods The clinical and pathological data of the cases in Jinling Hospital involving the patients younger than 18 years old who received renal biopsy from April 1st,2004 to December 31th,2017 were retrospectively collected,and compared with the renal pathological data of 1611 children aged 0-18 years from June 1982 to March 2004.Results This study included 9925 cases of kidney diseases proven by renal biopsy.The ratio of male to female was 1.79∶ 1.Primary glomerulonephritis (PGN) accounted for 66.14%,and secondary glomerulonephritis (SGN) accounted for 28.00%.Top five of the PGN were IgA nephropathy (IgAN,19.11%),mesangial proliferative glomerulonephritis (MsPGN,16.07%),minimal change disease (MCD,14.20%),focal segmental glomerulosclerosis (FSGS,6.19%)and membranous nephropathy (MN,4.70%) in whole children,IgAN (13.12%),MsPGN (11.20%),MCD (10.63%),FSGS (4.55%) and MN (2.54%) in males,and IgAN (5.99%),MsPGN (4.87%),MCD (3.57%),MN (2.16%) and FSGS (1.63%) in females.Top three of the SGN were Henoch-Schonlein purpura nephritis (HSPN,17.74%),lupus nephritis (LN,8.23%) and vasculitis nephropathy (1.82%).The male was in a dominant position in all kinds of pathologic types than female except LN.HSPN was the most frequent type in adolescents between 6-13 years old.LN was the commonest one in 14-18-year-old girls,while IgAN was the the most common in 14-18-year-old boys.Post infective nephritis was the most popular in 12-14-year-old teenagers.It was also found that MN ascended in female.When compared with the data before 2004,HSPN and LN accounted for a greater proportion in SGN,post infective nephritis displayed a smaller proportion.Conclusions PGN is the mainly kind of glomerular disease as before,and immune disorder related to glomerular diseases increase and post infective nephritis decreases in proportion.This study provides the reference and epidemic data for diagnosis,treatment and prevention of children's renal diseases.

14.
Article in Chinese | WPRIM | ID: wpr-802862

ABSTRACT

Magnesium is the fourth metal ion in the body, and magnesium deficiency has been neglected for a long time.In addition to the large amount of enzyme metabolism needs magnesium, it is also an important factor for bone health.There are limited means of assessing magnesium deficiency, and active prevention and treatment are needed in clinical practice.Now, the biological activities of magnesium deficiency and osteoporosis as well as osteoblasts, osteoclasts and chondrocytes, and its treatment and prevention are reviewed.

15.
Chinese Journal of Nephrology ; (12): 655-661, 2019.
Article in Chinese | WPRIM | ID: wpr-797935

ABSTRACT

Objective@#To investigate the clinical and pathological features and prognosis of children with IgA nephropathy with C1q deposition.@*Methods@#The children with IgA nephropathy diagnosed by renal biopsy from January 1, 2000 to December 30, 2017 were retrospectively analyzed and divided into C1q deposit group and C1q negative group according to glomerular immunofluorescence examination. Follow-up until the patient's serum creatinine doubled, glomerular filtration rate decreased by more than 50%, entering end-stage kidney disease, renal replacement therapy or death. Kaplan-Meier survival analysis was used to evaluate the renal survival rate in two groups. Univariate and multivariate Cox proportional hazard regression models were used to analyze the effect of C1q deposition on the prognosis of patients with IgA nephropathy.@*Results@#There were 60 cases in C1q deposition group and 60 cases in C1q negative group. (1) the initial eGFR and plasma albumin in C1q deposition group were lower than those in C1q negative group, while the levels of serum creatinine, serum cholesterol and 24 hour urinary protein in C1q group were higher than those in C1q negative group (all P<0.05). (2) pathological indexes: Mesangial cell proliferation, tubular atrophy/interstitial fibrosis, and cell/fibrocytic crescein score in C1q negative group were significantly higher than those in C1q negative group (all P<0.0.5). (3) Kaplan-Meier analysis showed that there was significant difference in renal cumulative survival rate between the two groups (Log-rank test: χ2=6.801, P=0.009). Cox proportional hazard regression model showed that the risk of renal end-point events in IgAN children with C1q deposition group was 5.772 times higher than that in C1q negative group (HR=5.772, 95%CI: 1.353-24.6211, P=0.018).@*Conclusion@#C1q deposition is an independent risk factor for the progress of renal function in IgA nephropathy children.

16.
Article in Chinese | WPRIM | ID: wpr-696392

ABSTRACT

Objective To investigate the renoprotective effect of aspirin-triggered lipoxins(ATL)on kidney of mice with acute kidney injury(AKI).Methods Eighty-eight male specific pathogen-free(SPF)C57BL/6J mice were randomly divided into lipopolysaccharide(LPS)groups(including 2 h group,4 h group,8 h group,12 h group, 24 h group),ATL+LPS(including 2 h group,4 h group,8 h group,12 h group,24 h group)and normal control group according to random numble table,and each group had 8 mice.The mice in LPS groups were given LPS intraperitoneal injection to establish AKI animal models,while the mice in ATL+LPS groups were given ATL intraperitoneal injection 30 minutes before LPS intraperitoneal injection.The enzyme linked immunosorbent assay was used to test the serum creatinine(Scr),serum urea nitrogen(BUN),tumor necrosis factor-α(TNF-α),interleukin-1β(IL-1β)and urine neutrophil gelatinase-associated lipocalin(NGAL),kidney injury molecule-1(KIM-1),cysteine-rich protein-61 (Cyr61)and netrin-1 levels of mice.Results The kidney tissue injury scores of mice of ATL+LPS group[4 h:(22.32 ± 1.04)scores,8 h:(31.11 ± 1.86)scores,12 h:(18.22 ± 0.92)scores,24 h:(20.87 ± 3.18)scores] were lower than those of LPS group at the corresponding time points[4 h:(35.47 ± 2.27)scores,8 h:(52.28 ± 2.82) scores,12 h:(54.99 ± 4.56)scores,24 h:(53.41 ± 4.76)scores],and the differences were statistically significant(all P<0.01).The values of Scr,BUN,TNF-α and IL-1β in ATL+LPS group[Scr 8 h:(143.07 ± 5.02)μmol/L, BUN 12 h:(33.07 ± 3.52)mmol/L,TNF-α 4 h:(196.33 ± 14.181)ng/L and 8 h:(221.77 ± 10.11)ng/L,IL-1β 4 h:(50.25 ± 2.67 ng/L)]were lower than those in LPS group at the corresponding time points[Scr 8 h:(227.43 ± 11.17)μmol/L,BUN 12 h:(59.68 ± 3.84)mmol/L,TNF-α 4 h:(267.87 ± 26.48)ng/L and 8 h:(334.78 ± 21.08)ng/L,IL-1β 4 h:(89.45 ± 5.87)ng/L],and the differences were statistically significant(all P<0.01). The urine NGAL[4 h:(56.76 ± 4.01)μg/L,8 h:(65.44 ± 7.81)μg/L],KIM-1[8 h:(78.19 ± 9.48)μg/L] and netrin-1[8 h:(40.12 ± 2.01)ng/L,12 h:(36.87 ± 2.87)ng/L]of mice in ATL+LPS group were lower than those in LPS group at the corresponding time points[NGAL 4 h:(168.77 ± 10.77)μg/L,8 h:(155.33 ± 8.26) μg/L;KIM-1 8 h:(124.73 ± 13.47)μg/L;netrin-1 8 h:(89.17 ± 2.74)ng/L,12 h:(81.11 ± 3.88)ng/L],and the differences were statistically significant(all P<0.01).Conclusions ATL can treat LPS-induced AKI and play a renoprotective role in the kidney.

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Journal of Clinical Pediatrics ; (12): 411-415, 2018.
Article in Chinese | WPRIM | ID: wpr-694694

ABSTRACT

Objective To explore the diagnosis and treatment of Dent disease I. Method The clinical data of 4 children with Dent diseaseⅠand the direct sequencing results of Dent disease-related genes CLCN5, OCRL1 exons and nearby regulatory regions were retrospectively analyzed. Results All the four children were male, the age at onset was 1.5~4 years and the age at diagnosis was 3~10 years. All of them had the clinical manifestations of proteinuria, among which 2 cases were accompanied by rickets symptoms. Gene detection showed that all of them had CLCN5 mutations, L263F, R104X, S244L and exon 9-13 deletion respectively. S244L is the most common mutation in patients with Dent disease I, and the rest are newly discovered mutation sites. Conclusion Dent disease I is mainly manifested as low molecular weight proteinuria and hypercalciuria. Gene detection contributes to the early and clear diagnosis.

18.
Article in Chinese | WPRIM | ID: wpr-514832

ABSTRACT

Steroid-resistant nephrotic syndrome (SRNS) is a relatively difficult clinical type of treatment.The major therapy measures in present include steroid and immunosuppressant.Commonly used immunosuppressant include tacrolimus,cyclosporin,cyclophosphamide,mycophenolate mofetil,ect.Tacrolimus-induced clinical remission rate is superior to other immunosuppressive agents,has been the first-line agent of SRNS.Because of the individual difference in metabolism,the drug concentration of tacrolimus should be determined periodically.In order to obtain optimal efficacy of tacrolimus and reduce renal toxicity,the treatment protocols of small doses with long courses for children with SRNS were recommended.

19.
Article in Chinese | WPRIM | ID: wpr-514831

ABSTRACT

microRNAs play an important regulative role in body's growth and development,and the development of the disease process.Much microRNAs can maintain normal kidney function and regulate kidney pathological process,the miR-30a has extensive effect on kidney development and progression of renal diseases.In this review,a brief overview on the role of miR-30a in renal pathology is presented.

20.
Journal of Medical Postgraduates ; (12): 266-270, 2017.
Article in Chinese | WPRIM | ID: wpr-511536

ABSTRACT

Objective Few researches have been reported on the gene methylation in children with steroid-sensitive nephrot-ic syndrome (SSNS) or steroid-dependent nephrotic syndrome (SDNS).This study aimed to investigate the possible pathogenesis and therapeutic target of SSNS and SDNS by screening differentially methylated genes ( DMGs) and bioinformatic analysis using DNA meth-ylation microarray. Methods This study included 3 hospitalized children with SSNS and another 4 with SDNS, all treated with full dose of prednisone ( 2 mg per kilogram of the body weight per day or 60 mg per m2 per day).Negative urine protein was achieved within 4 weeks in the former group , while the latter , though sensitive to hor-monal therapy , relapsed within 2 weeks after drug withdrawal or dose reduction .DNA was extracted from the peripheral blood of the patients in both groups for screening DMGs and bioinformatic analysis using DNA methylation microarray . Results Compared with the patients with SSNS, 318 DMGs were found in the SDNS group , among which 193 were hypermethylated and the other 125 hypomethylated .These abnormal genes were mainly located in the open reading frame of DNA and the CpG island region .DMGs were mainly involved in Rho guanyl-nucleotide exchange factor activity , nucleoside-triphosphatase regulator activity , GTPase activator activity , and other molecular functions .The biological processes were chiefly associ-ated with the regulation of the generation of precursor metabolites and energy , antigen processing and presentation , regulation of Rho and Ras protein signal transduction , lamellipodium assembly , regeneration , and other biological processes .The cell composition was mainly related to MHC protein complexes , perichromatin fibrils , and the MHC class I protein complex .Analysis of the KEGG signaling pathway showed that DMGs participated in 9 signaling pathways , involving type I diabetes , starch and sucrose metabolism , allograft re-jection, autoimmune thyroid disease , and others. Conclusion The heterogeneity of methylation is widespread in children with SDNS and may be one of the causes of steroid dependence , which has provided a basis for searching for potential therapeutic targets .

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