Résumé
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.
Résumé
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.
Résumé
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.