Retrospective evaluation of steroi̇d resi̇stant nephrotic syndrome cases

In this study, during 2000-2015, 50 patients who were followed at the Pediatric Nephrology clinic and outpatient clinic diagnosed as steroid-resistant nephrotic syndrome were evaluated retrospectively. In this study 44% (n=22) of the patients were female and 56% (n=28) of the patients were male and ages ranged from 1 to 14 (6.38±3.95) years. Diagnostic renal biopsy is done to all of our patients, the most frequent pathology was FSGS (44%). FSGS is followed by MPGN (30%), MPGN (22%) and MCD (4%). In 8 of 22 patients diagnosed with FSGS participated in the study positive podocin gene mutation was present, 10 of this FSGS patients were negative and in 4 patients was not any data about podocin gene mutation. In MGN patient history of consanguineous marriage and family history of NS was seen more frequently (81.8%). Consanguineous marriage rates was lower in FSGS patients than other patients (45.5%). Consanguineous marriage rate was higher in patients who have positive podocin gene mutation (87.5%). At admission blood pressure elevation (36.3%) and decrease in GFR (40.9%) was common in FSGS patients and decrease in complement was more prominent in MPGN patients. 50 SRNS patients followed between 2000 and 2015, with an average follow-up period of our 5.8±3.8 years (1-14 years), 18 (36.0%) were in remission, 21 patients (42.0%) had nephrotic proteinuria and 11 patients (22.0%) had developed the chronic renal failure. Cyclosporine therapy in patients who mere in remission had emerged as the most effective treatment. 3 of our patients who did not respond to cyclosporine treatment were in remission with tacrolimus therapy. There were no patients in remission following treatment with Rituximab, MMF, cyclophosphamide and plasmapheresis. As a result our study showed FSGS is responsible for a significant part of childhood nephrotic syndrome and majority of steroid-resistant nephrotic syndrome. Podocin gene mutation positive FSGS is responsible from the important part of FSGS. Our study showed that cyclosporine is an effective treatment option in steroid resistant nephrotic syndrome of cyclosporine, in cases where this treatment was found to be insufficient, tacrolimus can be an alternative option. However, despite recent advances in the diagnosis and treatment process, some of the SRNS patients was unable to get rid of chronic renal failure process. For the diagnosis, treatment and prognosis multicenter prospective studies are needed.