Options of the treatment in steroid resistant nephrotic syndrome in children in central child teaching hospital-Baghdad

The therapy of steroid resistant nephritic syndrome [SRNS] is still a matter of controversy. To assess the options of treatment in SRNS. A retrospective study to 50 patient randomly selected in the Central Child Teaching Hospital during study period from Jan. 2006 till July 2008. The patient age was between 6 months - 18 years. All patients who had failed to achieve an improvement in proteinuria after minimum of 4 weeks [up to 8 weeks] of prednisolone [PDN] in a dosage 2 mg/kg/day were taken. Only the patients with idiopathic nephritic syndrome [45 patients] were involved in the study but the patients with secondary nephritic syndrome and congenital neprosis were excluded from the study. Each patient were individualized to the type of pathology and to the type of medication used. Forty five patients were included in the study, the age range between 6 months -18 years. Twenty eight patients were male and 17 were female, M: F ration 1.64: 1 regarding the type of pathology, 20 patients with focal segmental glomerulosclerosis [FSGS], 11 patients with minimal change nephritic syndrome [MCNS], 8 patients with diffuse mesagnial proliferation [DMP] and 6 patients were unknown biopsy [not down biopsy]. The drugs that used were methyl prednisolone [MP] in 17 patients. Every other day steroid [EODS] in 10 patients, cyclosporine A [CsA] plus EODS in 16 patients [10 patients as first option and 6 patients as second option, cyclophosphamide [CYS] used in 8 patients [6 patients as first option and 2 patients as second option] and chlorambucil wee used in 2 patients only. The response was higher in patients who received EODS [50%], followed by the patients who received CsA plus EODS [25%] then the patients who received MP [23.5%] and the patients who received CYS [12.5%] and chlorambucil [zero%]. The response to treatment was higher in females than males, 11 out of 28 males [39.28%] responded to treatment while 7 of 17 female [41.17%] responded to treatment. The patients with early presentation responded to treatment higher than those with late presentation, so 12 of 19 patients [63.15%] presented early while 10 of 26 patients [38.46%] presented ate. According to histopathology, the patients with unknown etiology had higher rate of response, 3 out of 6 patients [50%] responded to treatment followed by 7 of 20 [35%] patients with FSGS, then 2 of 8 [25%] patients with DMP, then 2 of 11 [18.18%] patients with MCNS. The drugs used are the common drugs and EODS is preferable type of medication used in SRNS

Clinical course of children and adolescents with primary focal segmental glomerulosclerosis and the predictors of their outcome

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]. 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. 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 followup 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. 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