| Objective: To investigate the Clinical and pathological of primary focal segmental glomerulosclerosis(FSGS)in children.Methods: The data of 31 children who were diagnosed as primary FSGS were studied retrospectively between 2007-2017 in the First Hospital of Jilin University.Results: The incidence of primary FSGS is 5.9% on renal biopsy cases.The average age of onset was 7.74 ± 4.73 years and the average age of diagnostic was 9.58 ± 4.36 years.Of 31 children with primary FSGS,19 were boys and 12 were girls,the ratio of boy to girl was 1.58.Among them,the clinical manifestation include nephrotic syndrome in 21(68%)cases(nephritis nephropathy in 12,simple nephropathy in 9),glomerulonephritis in 3 cases(10%),isolated proteinuria in 3 cases(10 %),and isolated hematuria in 4 cases(12%).The distribution of histologics variant was 52%(n=16)FSGS not otherwise specified(NOS),32%(n=10)glomerular tip lesion(GTL),10%(n=3)of collapse(COLL),6%(n=2)cellular(CELL),and no perihilar type.28 cases were treated in our hospital after diagnosis of primary FSGS,20 cases with nephrotic syndrome were given steroids therapy include 6 steroidssensitive cases,10 steroids resistance cases and 4 steroids-dependent cases.13 patients combined with immunosuppressive agents,which tacrolimus were used in 5 patients,cyclophosphamide were used in 6 patients,1 patient was used cyclophosphamide and tacrolimus,1 patient was used tacrolimus and MMF,1 patient was used 3 immunosuppressive agents which were cyclophosphamide,tacrolimus and MMF.In this group of children,the follow-up time was 2 months-120 months,80% were complete remission,10% were partial remission and 10% were no remission.Conclusion:: 1.The primary clinical manifestations of primary FSGS in children is nephrotic syndrome.2.The most common pathological variants of primary FSGS in children is not otherwise specified(NOS).3.Prednisone combined with immunosuppressive agents could increase the primary FSGS remission rate in children. |
