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Objective:To explore the diagnostic potential of magnetic resonance imaging (MRI) in children with nutcracker syndrome (NCS).Methods:A retrospective analysis was performed in patients with suspected NCS(155 cases) diagnosed in the Department of Pediatrics, General Hospital of Eastern Theater Command from January 2017 to July 2020.Suspected NCS was diagnosed primarily based on clinical signs or symptoms, laboratory testing, and imaging reports, and other conditions that may cause hematuria and/or proteinuria were excluded.MRI examination was performed in all patients.According to the diagnostic criteria of NCS, patients diagnosed as NCS with the compression of the left renal vein (LRV) were included in the NCS group(58 cases), and those without the compression of the LRV or with the compression of the LRV but was not consistent with the diagnosis of NCS were included in the control group(97 cases). t test, Mann- Whitney U test and χ2 test were used to compare the baseline characteristics, clinical characteristics and imaging characteristics of the children in the nutcracker group and the control group.Receiver operating characteristic curves were plotted to explore the diagnostic potential of MRI in children with NCS. Results:(1)The area under curve of the angle between the superior mesenteric artery (SMA) and the aorta, compression ratio (CR) and beak sign in diagnosing NCS in children were 0.870, 0.895 and 0.878, respectively.(2)The optimal cut-off values of the angle between the SMA and the aorta and CR were 36.8° and 3.99, respectively.(3)The specificity of the angle between the SMA and the aorta<36.8°, beak sign, CR>3.99, the angle between the SMA and the aorta combined with beak sign, the angle between the SMA and the aorta<36.8° combined with CR>3.99, and beak sign combined with CR>3.99 in diagnosing NCS in children were 82.5%, 93.8%, 93.5%, 97.9%, 95.9% and 97.9%, respectively.Conclusions:Children with the angle between the SMA and the aorta<36.8°, beak sign and CR>3.99 suggested on MRI scans should be highly suspected of NCS.The beak sign has the highest specificity in the diagnosis of NCS in children, and the combination of any two parameters has a higher specificity than a single parameter.
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Objective:To analyze the clinicopathologic features and prognosis of children with Henoch-Sch?nlein purpura nephritis (HSPN).Methods:The clinicopathological data of children with HSPN who were followed up for more than 5 years and underwent renal biopsy in Jinling Hospital affiliated to Medical School of Nanjing University from January 2001 to June 2015 were retrospectively analyzed. The follow-up endpoint event was defined as estimated glomerular filtration rate (eGFR)<90 ml·min -1·(1.73 m 2) -1. Participants were divided into two groups according to whether the children had reached the primary endpoint event or not. Cox proportional hazards model was used to analyze the influencing factors of renal poor prognosis in children with HSPN. Kaplan-Meier survival curve method was used for survival analysis, and log-rank test was used to compare the difference of renal cumulative survival rate between segmental sclerosis/adhesion (S1) group and non-segmental sclerosis/adhesion (S0) group. Receiver operating characteristic curve (ROC curve) and area under the curve ( AUC) were used to evaluate the diagnostic value. Results:A total of 130 children with HSPN were enrolled in the study. The median onset age was 11.7(8.6, 13.3) years old, of whom 71 cases were males (54.6%). At a median follow-up time of 100.0(75.8, 119.0) months, 12 cases (9.23%) with HSPN reached the primary endpoint event. Compared with the non-endpoint event group, the endpoint event group had higher proportion of hypertension, higher levels of 24-hour urinary protein, serum cholesterol, serum uric acid, and serum creatinine, and lower levels of serum albumin (all P<0.05). There was no statistical difference in treatment between the two groups (all P>0.05). In terms of pathological features, compared with the non-endpoint event group, the endpoint event group had higher proportion of mesangial hyperplasia (M1), S1, tubular atrophy/interstitial fibrosis (T1/T2) and Glomerulus-Bowman's capsule adhesion (all P<0.05). Multivariate Cox regression model showed that S1 was significantly correlated with renal poor prognosis ( HR=7.739, 95% CI 1.422-42.114, P=0.018). As was revealed in a Kaplan-Meier plot, renal cumulative survival rate in the S1 group was significantly lower than that in the S0 group (log-rank χ2=17.069, P<0.001). The ROC curve showed S1 accurately predicted the outcome ( AUC=0.710, 95% CI 0.549-0.872) with specificity of 0.667(95% CI 0.349-0.901) and specificity of 0.754(95% CI 0.667-0.829). Conclusions:S1 is an independent risk factor affecting renal poor prognosis and has a diagnostic value.
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Objective:To analyze the clinical and prognosis of primary membranous nephropathy (PMN) in children with positive glomerular M-type phospholipase A2 receptor (PLA2R).Methods:A total of 69 children diagnosed with PMN by renal biopsy admitted to the Department of Pediatrics of Eastern Theater Command General Hospital from January 2006 to December 2018 were retrospectively analyzed, including 40 males and 29 females, with an average age of 14.86 years.According to the immunofluorescence of renal pathology, they were divided into PLA2R positive group and PLA2R negative group.Pathological features between 2 groups were compared by the t test, Mann- Whitney U test and Chi- square test.Kaplan-Meier method was used to compare the long-term renal survival rate and cumulative remission rate between 2 groups. Results:A total of 69 pediatric PMN patients were included.The po-sitive rates of serum anti-PLA2R antibody and positive expression of PLA2R in renal tissues were 53.6% (37 cases) and 82.6% (57 cases), respectively.The proportion of children with clinical manifestations of large proteinuria [55 cases(96.5% ) vs.9 cases(75.0%), P=0.034] was significantly higher in the PLA2R positive group than that of the PLA2R negative group.Blood urea nitrogen level was significantly higher in the PLA2R positive group than that of PLA2R negative group[1.14(0.93, 1.54) mg/L vs.0.80 (0.44, 1.18) mg/L, P=0.049], while estimate glomerular filtration rate(eGFR) [162.26 (139.81, 185.53) mL/(min·1.73 m 2) vs.199.52 (157.58, 212.01) mL/(min·1.73 m 2), P=0.034] and serum IgG [3.58 (2.50, 5.43) g/L vs.5.14 (4.35, 6.03) g/L, P=0.016] were significantly lower.The cumulative remission rate was significantly higher in the PLA2R negative group than that of PLA2R positive group ( P<0.001). The 24 h urinary protein ≥50 mg/kg ( HR=0.119, 95% CI: 0.021-0.595, P=0.010)was an independent risk factor for renal prognosis, and PLA2R( HR=0.263, 95% CI: 0.125-0.551, P<0.001) and 24 h urinary protein ≥50 mg/kg ( HR=0.568, 95% CI: 0.125-0.551, P=0.041)were independent predictors of urinary protein remission.PLA2R ( HR=1.020, 95% CI: 0.698-1.682, P=0.656)was not associated with renal prognosis. Conclusions:The severity of PMN in children with positive PLA2R was higher than that in those with negative PLA2R.The long-term cumulative remission rate of PLA2R negative children with PMN was higher than that of PLA2R positive children.
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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.
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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.
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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.
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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.
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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.