| ObjectiveThe aim of study is to investigate the clinical features of children with steroid-resistant nephrotic syndrome(SRNS)retrospectively and explore the risk factors for poor prognosis.The clinicopathological feature,genetic characteristics and treatment experience of the steroid-resistant nephrotic syndrome were analyzed and summarized.,which can provide a theoretical basis for improving the prognosis of children with SRNS in the region.MethodsThe study analyzed the clinical data which included clinicopathological feature,genetic characteristics,treatment measure and efficacy and long-term prognosis of 268 patients who were diagnosed with SRNS between Jan 1st.2009 and Dec 31st.2018 in the Pediatric Kidney rheumatism immunology Department of Shandong Provincial Hospital retrospectively.According to drug resistance type,pathological type,gene mutation type,all children were divided into different groups.At the same time,we explored the risk factors affecting the prognosis of SRNS children.Results1.A total of 268 children with SRNS were included in this study,including 177 males and 91 females.The ratio of male to female was 1.95:1.The onset of age was 0.42-14 years and an average age of 5.77±3.54 years.There were 224 children diagnosed with primary steroid-resistant nephrotic syndrome(PSRNS)and 44 children diagnosed with late steroid-resistant nephrotic syndrome(LSRNS).There was no significant difference in age,sex and age distribution between the two groups(P>0.05).2.Clinical manifestations:There were 140 cases(52.2%)of simple nephrotic syndrome and 128 cases(47.8%)of nephritis nephrotic syndrome.The most common clinical manifestation of SRNS was edema(100%),followed by microscopic hematuria(46.6%)and hypertension(9.7%).The incidence of microscopic hematuria in children with PSRNS(52.7%)was significantly higher than that of children with LSRNS(15.9%),and the difference was statistically significant(P<0.05).3.Pathological characteristics:A total of 103 children with SRNS were biopsied,accounting for 38.4%of SRNS.The pathological types of 103 children with SRNS were minimal change disease(MCD)(42.7%),followed by focal segmental glomerulosclerosis(FSGS)(26.2%),membranous nephropathy(MN)(17.5%),membranoproliferative glomerulonephritis(MPGN)(6.8%),mesangial proliferative glomerulonephritis(MsPGN)(4.9%)and IgM nephropathy(IgMN)(1.9%),There was no significant difference in the distribution of different age groups and genders among all pathological types(P>0.05).The incidence of microscopic hematuria in the MCD group was lower than that in the non-MCD group,and the difference was significant(P<0.05).The total cholesterol level of the MCD group was higher than that of the non-MCD group.In this study,it was not found that the 24 hours urine protein level and triglyceride level were related to the pathological type.There was statistically significant difference between renal immune complex deposition type and SRNS pathological type(P<0.05).There was no significant difference between PSRNS and LSRNS children in the deposition types of immunocomplex deposition in renal tissues,and IgM was the main type of immunocomplex deposition in both groups.There were 3 cases(2.9%)with repeated renal biopsy,1 cases of MN Ⅱ-Ⅲ stage changed to stage MN Ⅲ with focal segmental glomerulosclerosis.2 cases of MCD changed to FSGS.4.Genetic testing:There were 21 cases of genetic testing.Within the 11 cases of renal biopsy,11 patients underwent renal biopsy,there were 6 cases of MCD,5 cases of FSGS.There were 9 cases of gene mutation,accounting for 42.86%,including 5 cases of COL4A5,1 case of NPHS1,1 case of ADCK4,1 case of FAT 1 and 1 case of WT1.All children of gene mutation were primary steroid-resistant.There were no statistically significant differences in gender,age,clinical type,steroid resistance type and pathological type between children with gene mutation group and children without gene mutation group(P>0.05).Most of the children with gene mutation had poor prognosis,1 cases(NPHS1 gene mutation)was lost to follow-up,1 cases(FAT1 gene mutation)of complete remission and the rest of no remission.5.Complications:A total of 126 children with SRNS had one or more complications in the last ten years,accounting for 47.0%.The number of complications in SRNS children was 98 cases of infection.30 cases of hypertension,20 cases of acute kidney injury,10 cases of adrenal crisis.5 cases of thromboembolism.4 cases of reversible posterior leukodystrophy,and 2 cases of hypovolemic shock.The incidence of ocular complications(cataract or glaucoma)in LSRNS group was higher than that in PSRNS group,and the difference was significant(P<0.05).6.Treatment:There were 194 cases(72.4%)of SRNS were treated with one or a combination of immunosuppressive agents on the basis of steroid therapy.We analyzed the use of immunosuppressive agents of 160 cases within 1 year after the diagnosis of SRNS.There was no significant difference in the therapeutic effects of cyclophosphamide(CTX),cyclosporine(CsA),tacrolimus(TAC),and metmecolphenol(MMF)between PSRNS groups and LSRNS groups(P>0.05).We analyzed the use of immunosuppressive agents of 50 cases undergoing renal biopsy within 1 year after the diagnosis of SRNS.The effective rate of CTX treatment in FSGS children was lower than MCD children with,the difference was significant(P<0.05).There was no significant difference in the curative effect of CTX,CsA,and TAC in Children with MCD and FSGS(P>0.05).7.Follow-up and Prognosis:End of follow-up time in August 2019,11 cases were lost to follow-up,accounting for 4.48%of 268 cases with SRNS.In the end,the prognosis of 257 cases were analyzed.92 cases(35.8%)stopped steroid therapy in the end.Clinical outcome:216(84.0%)children were maintaining complete remission,10(3.9%)maintaining partial remission,14(5.4%)maintaining no remission,5(2.0%)maintaining chronic renal failure and 12(4.7%)died.The prognosis of children with LSRNS was better than that of PSRNS children.The prognosis of different pathological types was significant(P<0.05).FSGS had the worst prognosis.Hypertension and renal pathology with FSGS were independent risk factors for the prognosis of SRNS children.Conclusion1.The most common renal biopsy types in SRNS children include MCD,FSGS and MN.The pathological types of PSRNS and LSRNS children are mainly MCD,but the pathological types can be converted,and renal biopsy should be repeated if necessary.When SRNS children with microscopic hematuria,the pathological type of is non-MCD.2.A small number of SRNS children have gene mutation,suggesting bad response to immunosuppressive therapy and poor prognosis.For SRNS children,especially children in small age groups,early genetic testing is very necessary.3.Compared with children with PSRNS,LSRNS had a better prognosis.The prognosis of SRNS is related to the pathological type,and the prognosis of FSGS is the worst4.Hypertension and FSGS are independent risk factors affecting the prognosis of children with steroid-resistant nephrotic syndrome. |
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