Clinicopathological Features And Prognosis Of 67 Children With Primary Focal Segmental Glomerulosclerosis

Objective This study investigate the clinicopathological features and prognosis evaluation and their connections primary focal segmental glomerulosclerosis (FSGS)in southwest of Chinese children.Methods This retrospective study evaluated clinical features and outcomes of pathologic FSGS variants in 67 children patients between January 2001 and December 2013. We analyzed histological, clinical and follow-up data and compared among variants.Result1)There were 38 males (56.7%) and 29 females (43.3%), The average onset age of those children was 7.49±3.53.2)49 children (73.1%) onset of the nephrotic syndrome,12 children (17.9%) onset of glomerulonephritis,5 children (7.5%) onset of isolated Hematuria,1 case onset onset of isolated proteinuria. There are 3 cases (4.5%) of 67 children onset of chronic kidney disease.3)There were 28 (41.8%),14 (20.9%),11 (16.4%),9(13.4%), and 5 (7.5%) patients with not-otherwise specified (NOS), glomerular tip lesion (TIP), collapsing (COLL),cellular (CELL),and perihilar variants, respectively.The most common type is NOS,the most rare common type is perihilar.4)The average age of perihlar was older than tip variant.The amount of proteinuria was higher in the glomerular collapsing variant than cellular variant (P=0.049< 0.05).There was no significant difference in clinical manifestation and other laboratory examination among five pathological types.5)40 patients were followed up for 2-140 months,the average time is 43.7±35.1 months,8 patients (20%) were complete remission,5 patients (12.5%) were significant remission,5 patients (12.5%) were partial remission and 22 patient (55%) was no remission. There was no significant difference of the five variant’s outcome.18 patients were followed up of endogenous creatinine clearance,There were 7 cases (38.9%) progression to end-stage renal disease(ESRD),the average time is 31.1±26.8 months,the median time is 24.0±47.3 months.50 percent of those children progression to ESRD or death over 2 years.The average time of the four pathological type with renal failure or death is 16.1±9.1 months with COLL,21.3±20.0 months with CELL,37.4±40.9 months with NOS,55.0±11.8 months with TIP,respectively.CELL progression to ESRD or death is earlier than TIP.6)The significant risk factors for bad term of prognosis were the serum creatinine levels,urea nitrogen levels,and severe tubulointerstitial changes.Conclusion1) FSGS were mainly male,with more common in school-age children.2)The main clinical manifestation of primary FSGS was nephritic syndrome in children. Nephritis type of nephrotic syndrome was the most common presentation. A few of children onset of ESRD.3)The NOS variant was the commonest pathological variant seen in primary FSGS.Perihilar is more common in older children.There was no significant difference in clinical manifestation and other laboratory examination among five pathological types. The amount of proteinuria was higher in COLL variant than CELL variant.4)For these individuals, the prospect of long-term survival is poor. Treatment without efficiency is as high as 55%.50% of those children develop to ESRD or death.CELL progression to ESRD or death is earlier than other types.5)urea nitrogen levels,Creatinine levels and tubulointerstitial lesion were risk factors of poor prognosis.