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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 investigate the potential of the antineutrophil cytoplasmic antibody (ANCA) renal risk score (ARRS) in predicting the prognosis of children with ANCA-associated glomerulonephritis (AAGN).Methods:Laboratory testing, renal pathology results, treatment and prognosis of 61 children with AAGN diagnosed by renal biopsy from June 2007 to May 2022 in General Hospital of Eastern Theater Command were retrospectively analyzed.The Kaplan-Meier method was used to evaluate the overall and renal survival of children with AAGN, and risk factors of progression to end stage renal disease (ESRD) were analyzed by Cox regression analysis. Results:Among the 61 children with AAGN, there were 14 males and 47 females with the age of (15.65±3.74) years.According to ARRS, AAGN children were assigned into low-risk group (27 cases), medium-risk group (21 cases) and high-risk group (13 cases). During a median follow-up duration of 46.36 (14.58, 95.62) months, the number of ESRD cases in the high-risk group (9 cases) was significantly higher than that of low-risk group (2 cases) and medium-risk group (3 cases) ( χ2=13.079, P<0.001). Kaplan-Meier survival analysis showed that AAGN children in the high-risk group had the worst renal prognosis ( χ2=5.796, P=0.016), while no significant difference was detected in the overall survival among the 3 groups ( χ2=2.883, P=0.237). Multivariate Cox regression showed that estimate glomerular filtration rate(eGFR)≤15 mL/(min·1.73 m 2) ( HR=9.574, 95% CI: 4.205-25.187, P=0.015) and ARRS ( HR=2.115, 95% CI: 1.206-4.174, P=0.012) were independent risk factors for children with AAGN progress to ESRD.Receiver operating characteristic (ROC) curve analysis results showed that the area under the curve of ARRS for predicting the risk of progressing to ESRD in AAGN children was 0.880 (95% CI: 0.759-1.000), and the optimal cutoff value of ARRS was 5.50, with the sensitivity and specificity of 85.71% and 82.98%, respectively. Conclusions:ARRS was an independent risk factor for children with AAGN progress to ESRD, which had a predictive value for the progression of AAGN to ESRD.
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Objective:To analyze the long-term prognosis of IgA nephropathy (IgAN) with focal segmental glomerulosclerosis (FSGS) and the risk factors related to renal prognosis in children with IgAN-FSGS.Methods:A retrospective study was concluded in IgAN-FSGS children who were followed up for more than 5 years and diagnosed by renal biopsy for the first time in the Eastern Theater General Hospital from January, 2004 to December, 2018. The end-point events of the study were entering end-stage kidney disease (ESKD) or estimated glomerular filtration rate (eGFR) decreased by ≥50% from baseline, which were defined as poor renal prognosis. Baseline clinicopathologic data of IgAN-FSGS children were compared between the end-point event group and the non-end-point event group. The cumulative renal survival rate of IgAN-FSGS children was calculated by Kaplan-Meier survival analysis. The influencing factors of poor renal prognosis in IgAN-FSGS children were analyzed by Cox proportional hazards model, and the diagnostic value was evaluated by the receiver operating characteristic curve (ROC curve) and area under the curve (AUC). The diagnostic value was verified by time dependent-ROC and time dependent-AUC.Results:A total of 204 IgAN-FSGS children were enrolled in this study, of whom 132 cases were males (64.7%). The median age of renal biopsy was 16 (14, 17) years old. During a median follow-up time of 90.7 (71.7, 114.8) months, 57 cases (27.9%) reached the end-point events. Compared with the non-end-point event group ( n=147), the end-point event group ( n=57) had higher proportions of males and hypertension, higher levels of 24-hour urinary protein, serum creatinine, serum uric acid, urinary N-acetyl-β- D-glucosaminidase, urinary retinol binding protein, higher proportions of glomerular segmental sclerosis (S1) ≥25% and tubular atrophy/interstitial fibrosis (T1/T2), and lower levels of serum albumin, serum IgA, and serum IgG (all P<0.05). There was no statistical difference between the two groups in treatment (all P>0.05). Kaplan-Meier survival analysis showed that with entry of ESKD or eGFR decreased by ≥50% from baseline as the end-point events, the 5-year, 10-year, and 15-year cumulative renal survival rates in IgAN-FSGS children were 88.7%, 67.6%, and 50.7%, respectively. Multivariate Cox regression analysis showed that proteinuria >1 g/24 h ( HR=3.702, 95% CI 1.657-8.272, P=0.001), hyperuricemia ( HR=3.066, 95% CI 1.793-5.245, P<0.001), S1≥25% ( HR=2.017, 95% CI 1.050-3.874, P=0.035), T1/T2 ( HR=1.863, 95% CI 1.021-3.158, P=0.016) were the independent related factors for poor renal prognosis. ROC curve analysis showed that S1≥25% ( AUC=0.605, P=0.021, sensitivity 26.3%, specificity 94.6%), T1/T2 ( AUC=0.624, P=0.006, sensitivity 43.9%, specificity 81.0%), hyperuricemia ( AUC=0.658, P<0.001, sensitivity 52.6%, specificity 78.9%), proteinuria>1 g/24 h ( AUC=0.670, P<0.001, sensitivity 87.7%, specificity 46.3%) could accurately predict the renal outcome of IgAN-FSGS. Time dependent-ROC curve validation showed that the combined diagnosis of S1≥25%, T1/T2, hyperuricemia and proteinuria>1 g/24 h had a good predictive value for renal prognosis (3-year AUC=0.846 and 5-year AUC=0.777, respectively). Conclusions:During a median follow-up of 90.7 months, 27.9% of IgAN-FSGS children have poor renal prognosis, and the 5-year, 10-year, and 15-year cumulative renal survival rates are 88.7%, 67.6%, and 50.7%, respectively. Urinary protein >1 g/24 h, hyperuricemia, T1/T2, and S1 ≥25% are the risk factors for renal prognosis in IgAN-FSGS children.
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The paper reports two cases of lipoprotein glomerulopathy (LPG) in children. The Sanger sequencing results in 2 cases indicated apolipoprotein E gene mutation[c.127 (exon3) C>T, p.R43C (p.Arg43Cys); c.494 (exon4) G>C, p.R165P (p.Arg165Pro),respectively]. Renal pathological presentation of two children showed that a large number of lipoprotein emboli were formed in the glomerular capillary loop, and the diagnosis of LPG was confirmed. The onset of LPG has no specific clinical manifestation, which is easy to be undiagnosed or misdiagnosed. Renal biopsy is a diagnostic means, glucocorticoid treatment is ineffective, and long-term lipid-lowering treatment may be required for LPG.
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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 analyze the clinical and pathological characteristics in children diagnosed with primary focal segmental glomerulosclerosis (FSGS) after repeated renal biopsy.Methods:The clinicopathological data of children who ever experienced renal biopsy in Jinling Hospital from January 1, 2000 to December 31, 2020 were retrospectively reviewed. Clinical manifestations, pathological characteristics and treatment responses were analyzed.Results:Of the 34 enrolled patients, there were 22 males and 12 females. The median age of the first renal biopsy was 14 years old (1-18 years old), and the median interval between repeat renal biopsy and first renal biopsy was 6 months (1-151 months). Thirty-one showed nephrotic syndrome, of which 22 had microscopic hematuria, and 4 had elevated serum creatinine. Among the other 3 patients, 2 had hematuria and proteinuria, and 1 had proteinuria. In the first renal biopsy, 16 cases were diagnosed as minimal change disease, 14 cases were diagnosed as mesangial proliferative glomerulonephritis, 2 cases were diagnosed as IgA nephropathy, and 2 cases were diagnosed as IgM nephropathy. All 34 children showed poor responses to hormone and immunosuppressive therapies. The pathological features of the first renal biopsy in some patients were adhesion (2/34), decreased loop podocyte attachment (2/34), peripheral loop extension to the urinary pole (2/34), renal tubular reflux (4/34), capillary thrombosis (2/34) and IgM deposition (12/34).Conclusions:The initial diagnosis of FSGS is difficult, and the lesions are atypical and easily misdiagnosed. The patients have poor responses to hormone and immunosuppressive therapies. For patients with the pathological changes of adhesion, decreased loop podocyte attachment, peripheral loop extension to the urinary pole, renal tubular reflux, capillary thrombosis and IgM deposition, follow-up is required, and if necessary, repeat renal biopsy needs be performed to determine whether it is FSGS.
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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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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.
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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.
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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 evaluate the enrollment rate, mutation rate and causes of variability the clinical pathway of bronchopneumonia.Methods:The enrollment rate, completion rate, variation and reasons of the clinical pathway in Beijing Children′s Hospital, Capital Medical University from January 2012 to December 2016 were retrospectively collected.Data of patients after the clinical pathway of bronchopneumonia in other tertiary class A hospitals were gathered by questionnaires, and the enrollment rate, completion rate, variation rate and reasons were analyzed.Results:(1)At the end of 2016, 11 of the 13 hospitals included in this study had implemented the clinical pathway for 5 years, 1 hospital for 3 years, and 1 hospital for 2 years.(2) Eleven hospitals provided their enrollment rates.The enrollement rate of 2 hospitals was<50%, and that of 9 hospitals was>80%.The annual completion rate of Beijing Children′s Hospital was ≥75%, and the completion rates offered by 8 hospitals were basically >70%.(3) Since the implementation of the clinical pathway for 5 years in Beijing Children′s Hospital, a total of 427 cases were enrolled of which 93 cases were mutated (variability 21.78%). The variability of 5 hospitals was maintained at <15%.The variability of 3 hospitals decreased with the implementation years, and became qualified.The variability of 1 hospital first rebounded and then controlled; 1 hospital increased by 27.65%; 1 hospital was first controlled and rebounded; 1 hospital was always >15%.The main cause of the mutation was coexisting diseases, complications, progression of the disease, or correction of the first diagnosis, etc.Conclusions:The completion rate of tertiary class A hospitals meets the requirements of national policy.However, the enrollment rate needs to be improved, and the variation rate among different hospitals differs a lot.Further implementation of the clinical pathway should be strengthened and monitored.
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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.
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Novel Coronavirus Pneumonia (NCP) is a class B infectious disease, which is prevented and controlled according to class A infectious diseases.Recently, children′s NCP cases have gradually increased, and children′s fever outpatient department has become the first strategic pass to stop the epidemic.Strengthening the management of the fever diagnosis process is very important for early detection of suspected children, early isolation, early treatment and prevention of cross-infection.This article proposes prevention and control strategies for fever diagnosis, optimizes processes, prevents cross-infection, health protection and disinfection of medical staff, based on the relevant diagnosis, treatment, prevention and control programs of the National Health and Health Commission and on the diagnosis and treatment experience of experts in various provinces and cities.The present guidance summarizes current strategies on pre-diagnosis; triage, diagnosis, treatment, and prevention of 2019-nCoV infection in common fever, suspected and confirmed children, which provide practical suggestions on strengthening the management processes of children′s fever in outpatient department during the novel coronavirus pneumonia epidemic period.
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Novel Coronavirus Pneumonia (NCP) is a class B infectious disease, which is prevented and controlled according to class A infectious diseases. Recently, children′s NCP cases have gradually increased, and children′s fever outpatient department has become the first strategic pass to stop the epidemic. Strengthening the management of the fever diagnosis process is very important for early detection of suspected children, early isolation, early treatment and prevention of cross-infection. This article proposes prevention and control strategies for fever diagnosis, optimizes processes, prevents cross-infection, health protection and disinfection of medical staff, based on the relevant diagnosis, treatment, prevention and control programs of the National Health and Health Commission and on the diagnosis and treatment experience of experts in various provinces and cities. The present guidance summarizes current strategies on pre-diagnosis; triage, diagnosis, treatment, and prevention of 2019-nCoV infection in common fever, suspected and confirmed children, which provide practical suggestions on strengthening the management processes of children′s fever in outpatient department during the novel coronavirus pneumonia epidemic period.
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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 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.
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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.
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Chronic kidney disease (CKD) is a global public health problem, which endangers human health, and it is also one of the important causes of hyperuricemia (HUA). In recent years, the incidence of HUA in children is increasing globally, and which badly affect the physical and mental health of children.HUA is not only a sign of kidney damage, but also a independent risk factors for CKD, cardio-cerebrovascular diseases and metabolic diseases.Therefore, there is a very important clinical significance for early diagnosis and early intervention in patients with CKD combined with HUA.This paper describes the standard diagnosis and treatment of CKD combined with HUA in the light of the latest diagnosis and treatment progress.