Clinical Course of Children and Adolescents with Primary Focal Segmental Glomerulosclerosis and the Predictors of Their Outcome


ABSTRACT:BACKGROUND:The aim of this retrospective study is to report the clinical course of children and adolescents with primary focal segmental glomerulosclerosis (FSGS), and to study their outcome and to identify the predictors of progression to end stage renal disease (ESRD).PATIENTS AND METHODS:This is a retrospective study of 50 patients with biopsy-proven primary FSGS who were admitted from April 1995 - January 2007, during the study period from May 2005 - June 2007. Clinical, Laboratory and histopathological data were recorded. The median follow-up time of 4.5 year.RESULTS:The commonest age and sex group is male between 1 - 5 year. At presentation all patients had nephrotic-range proteinuria, hypertension was noted in 22 (44%) of patients, microscopic haematuria was detected in 20 (40%) of patients, five patients had evidence of abnormal renal function. The distribution of patients according to steroid responsiveness show that the steroid sensitive patients were 21 (42%), 8 (38.09%) of them were frequent relapsers and 11 (52.38%) of them were steroid dependant and 2 (9.52%) of them developed secondary steroid resistance. But those who had steroid resistance from the start of treatment were 29 (58%) patients. During follow-up 30 (62%) patients had complete remission, 15 patients (30%) developed chronic kidney disease (9 of them stage 5).At the end of follow-up, 24 (80%) of 30 patients with normal renal function had short stature. The univariate analysis identified the presence of hypertension (P=0.0027), heamaturia (P=0.0107) and presence of abnormal renal function (P=0.0001) at presentation, also presence of initial steroid resistance (P=0.0383), resistance to cytotoxic therapy (P=0.0032), capsular adhesions in renal biopsy (P=0.0066), tubular atrophy (P=0.0027), interstitial fibrosis (P=0.0010), all expect to be significant predictors of progression to ESRD.CONCLUSION:Considering the clinical and histological characteristics of studied patients, apparently our results are comparable to other published series. The progression to chronic kidney disease (CKD) occurs in 30% of patients after 5 years follow-up, must of them with ESRD, this is relatively good out come compared to other studies.