Changes In Clinicopathological Features Of Children With Primary Nephrotic Syndrome In Single Center

BackgroundPrimary nephrotic syndrome is one of the most common kidney diseases in children.The long treatment cycle and repeated recurrences have brought huge economic and psychological burdens to families and society.The characteristics of the disease may change over time,but studies on changes in the characteristics of primary nephrotic syndrome in children have rarely been reported at home and abroad.ObjectiveWe collected the clinicopathological data of children with primary nephrotic syndrome who were admitted to the Kidney Rheumatology Immunology Division of Shandong Provincial Hospital affiliated to Shandong University in the last 10 years.The clinical manifestations,characteristics of renal pathology were analyzed and summarized.And we compared the changes in clinical pathology before and after the study of children with primary nephrotic syndrome in children,we followed up these patients in long-time to analyze the long-term prognosis and risk factors for serious prognosis,in order to provide valuable clinical data for pediatricians in the region.MethodsA retrospective analysis was performed on 1072 children with primary nephrotic syndrome who were admitted to Pediatrics of Shandong Provincial Hospital between January 01,2006 and December 31,2015.The clinicopathological data of the children were collected through the electronic medical records system,and the data were collated and entered into the EXCEL database.According to the admission date of children,the children were divided into two groups,specifically,January 01,2006 to December 31,2010 was Group A and January 01,2011 to December 31,2015 was Group B.We used SPSS 22.0 to analysis and compare the clinical manifestations,pathological features,and treatment regimens of A and B groups.We followed up the long-term prognosis of children with primary nephrotic syndrome,and analyzed the risk factors that affected prognosis.Results1.A total of 1072 children with primary nephrotic syndrome were included,including 806 males and 266 females.The male to female ratio was 3.03:1;the onset age was 1-14 years and the average was(4.92�2.87)years.There were 597 cases(55.69%)with initial onset,475 cases with recurrence,accounting for 44.31%.There was no significant difference in baseline data between groups A and B(P>0.05).2.Clinical manifestations:There were 886 cases of simple nephrotic syndrome,accounting for 82.65%;186 cases of nephritis nephrotic syndrome,accounting for 17.35%.Specifically there were 308 cases of simple nephrotic syndrome,64 cases of nephritis nephrotic syndrome in group A;and there were 578 cases of simple nephrotic syndrome and 122 cases of nephritis nephrotic syndrome in group B.There was no statistical difference between A and B groups(P>0.05).There were 783 patients with steroid-sensitive nephrotic syndrome(SSNS),241 patients with steroid-resistant nephrotic syndrome(SRNS),and 48 with unknown steroid effects.Specifically,there were 258 cases SSNS,91 SRNS,and 23 unknown steroid effects in Group A and there were 525 cases of SSNS and 150 cases of SRNS and 25 unknown steroid effects in Group B.There was no significant difference between groups A and B(P=0.27,P>0.05).3.Pathological features:A total of 136 children underwent renal biopsy,accounting for 12.69%(136/1072)of children with PNS.The most common is minimal change(MCD)in 60 cases(44.12%),followed by mesangial proliferative glomerulonephritis(MsPGN)in 31 cases(22.79%),focal segmental glomerulosclerosis(FSGS)19 Cases(13.97%),membranous nephropathy(MN)in 15 cases(11.03%),membrane proliferative glomerulonephritis(MPGN)in 6 cases(4.41%),IgM nephropathy(IgMN)in 4 cases(2.94%),One case of focal roliferative glomerulonephritis(FGN)(0.73%).There was a statistically significant difference in MsPGN and MN between group A and B(P<0.05),suggesting that the proportion of MsPGN in children with PNS gradually decreased while the MN gradually increased(P<0.05).There was no significant change in other types of pathological types.Repeated renal biopsy was performed in 2 patients;1 case of primary renal biopsy was stage ?-? membranous nephropathy.The children were not treated regularly and stopped on their own.After 3 years,the second renal biopsy was progressed to stage? membranous nephropathy with focal segmental kidneys.The other one case was diagnosed as MCD by initial renal biopsy.However,she has not get the completely relieved after regular treatment.The secondary renal biopsy progressed to FSGS after 3 years.4.Complications:A total of 512 children with primary nephrotic syndrome had complications,accounting for 47.76%(512/1072)of the total number of cases.Among them,139 cases had complications in group A accounted for 37.36%(139/372);373 cases had complications in group B accounted for 53.29%(373/700),which was significantly higher than group A(P<0.01).The various types of complications were 449 cases of infection,36 cases of acute renal insufficiency,21 cases of adrenal crisis,24 cases of hypertension,7 cases of thromboembolism,and 7 cases of other complications.The incidence of infection in group B was higher than that group A(P<0.05).There was no significant difference between the two groups in the other types of complications(P>0.05).5.Treatment:Four out of 1072 children were not treated with steroid(parents refused to use it),and other patients were treated with steroid sequentially.Among them,immunosuppressive agents were used in 292 cases due to steroid resistance,steroid dependence,or frequent relapse,accounting for 27.24%(292/1072).69.18%(202/292)of them used an immunosuppressive agent,30.82%(90/292)uses two or more immunosuppressants one after another.The proportion of cyclosporine A and Tripterygium glycosides was decreased(P<0.05),the proportion of tacrolimus used was increased(P<0.05),and other immunosuppressive agents(such as Mycophenolate mofetil and cyclophosphamide,etc.)were no statistically different between the two groups.6.Follow-up:End of follow-up time In October 2017,156 cases were lost to follow-up,the loss rate was 14.55%,and 4 cases without steroid treatment were excluded.Finally,the prognosis of 912 children with primary nephrotic syndrome was analyzed.Among the 912 children with primary nephrotic syndrome,71.60%(653/921)were stopped using hormones,27.74%(235/912)were treated with sequential hormones,and 1.74%(16/912)were deaths.Chronic kidneys The failure accounted for 0.88%(8/912).7.Clinical Outcomes:480 cases(52.63%)were clinically cured(over 3 years);868(95.17%)completely relieved,17(1.86%)partially relieved,and 16(1.75%)died,slow renal failure 8 Example(0.88%).Among the 912 children,156(17.10%)had no recurrence,429(47.04%)had recurrent frequency,and 327(35.85%)recurred frequently.The overall recurrence rate was 82.89%,the frequency recurrence rate was 35.85%,and there was no relapse.Only 17.10%.8.Risk factor analysis:Binary logistic regression analysis found that the risk factors affecting the prognosis of children with primary nephrotic syndrome are hormone effects,serum creatinine,blood pressure,and serious complications.Conclusion1.The demographic data,clinical types and hormone effects of children with primary nephrotic syndrome have not changed significantly in recent years.2.The results of renal biopsy in children with primary nephrotic syndrome showed that the proportion of MsPGN decreased,while the proportion of MN gradually increased.Repeated renal biopsy can detect the pathological changes of the kidney in time and adjust the treatment plan in time.3.Children with primary nephrotic syndrome had a marked increase in complications in recent years,mainly due to increased infection complications,acute renal insufficiency,adrenal crisis,hypertension,thromboembolism and other no significant changes.4.In terms of treatment,glucocorticoids are still the first-line treatment,and immunosuppressants are needed for refractory primary nephrotic syndrome.The selection of immunosuppressive agents requires comprehensive consideration of many factors.In recent years,the proportion of tacrolimus used has increased,while the use of triptolide and cyclosporine A has decreased.5.The overall prognosis of children with primary nephrotic syndrome is good,but the recurrence rate is high.6.Hypertension,hormonal effects,serum creatinine,and severe complications are independent risk factors for poor prognosis in children with primary nephrotic syndrome.