Comparison of immune suppressive treatments in steroid resistant nephrotic syndrome
2024
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Advisor: Prof. Dr. Mithat Büyükçelik
Abstract (EN)
Aim: This study aimed to compare the effects of immunosuppressive treatments used for the treatment of steroid-resistant nephrotic syndrome, an important nephrological disease of childhood. MaterialsandMethods: In this study, 55 cases with steroid-resistant nephrotic syndrome who were hospitalized in the Pediatric Health and Diseases Clinic of Gaziantep University Faculty of Medicine Hospital and followed up in the Pediatric Nephrology Polyclinic between 2011 and 2023 were retrospectively examined. Age, gender, family history, consanguinity, presence of hematuria, albumin level at the time of diagnosis, arterial blood pressure, history of nephrotic syndrome in siblings, biopsy diagnosis, immunosuppressive treatments given, duration of receiving immunosuppressive treatments, response to treatment, number of relapses under the given treatment of the children included in the study. and follow-up times were recorded. Results: The average age of the patients participating in the study was 48 (12-171) months, 54.5% were girls and 45.5% were boys, 80.0% had hematuria, 10.9% had C3, It was found that 9.1% had low C4 levels, 16.4% had high blood pressure values, and 7.3% had ANA positivity. Distribution of biopsy results of patients; It was FSGS in 40.0% , MPGN in 18.2% , MLH in 20% , IgA nephropathy in 3.6% , IgM nephropathy in 1.8% , and RPGN in 1.8% . It was determined that the use of cyclosporine was higher than other agents, 76.3% responded to the treatment, and the number of relapses was significantly lower (p = 0.003) (p = 0.019 and p = 0.021, respectively). It was determined that 63.6% treatment response occurred in patients using tacrolimus. At the same time, it was shown that patients who received both cyclosporine and tacrolimus treatment had a tacrolimus response (86.7% ), while patients who did not respond to cyclosporine treatment did not have a tacrolimus response (85.7% ). It was observed that the treatment responses of cyclosporine and tacrolimus were close. It was determined that 44.4% treatment response occurred in patients using MMF. It was determined that only the duration of mycophenolic acid use was statistically significantly higher in those with MMF treatment response (p=0.016), and although the number of relapses was lower in those with treatment response, this did not reflect statistical significance (p=0.786). It was determined that the patients who received both Cyclosporine and MMF treatment (57.1% ) also had an MMF response, while all of the 2 patients (100.0% ) who did not respond to cyclosporine treatment did not have an MMF response. Cyclosprine treatment was found to be more effective than MMF. It was determined that 77.7% treatment response occurred in patients using rituximab. In comparisons made according to treatment response for rituximab; It was determined that only the number of rituximab doses was statistically significantly higher in those with treatment response (p = 0.035). It was determined that patients using cyclophosphamide (60% ) responded to the treatment. Azathioprine and eculizumab were used in the treatment of one patient each, and no statistical studies could be conducted. Conclusion:We found that FSGS is responsible for the majority of steroid-resistant nephrotic syndrome, one of the important diseases of childhood, and that the hematuria finding, which we see more in nephritic syndrome, is a common finding in our patients with steroid-resistant nephrotic syndrome. Many immunosuppressive agents are used for treatment. Although the more potent cyclosporine was seen, we found that tacrolimus had a similar success rate, but we found that the number of relapses of cyclosporine was lower. Although other agents, MMF, rituximab, cyclophosphamide, azathioprine, and eculizumab, were used, we found that they were not as effective as tacrolimus and cyclosporine. However, multicenter prospective studies are required to evaluate these agents more clearly and demonstrate their effectiveness. Keywords:nephrotic syndrome, steroid-resistant nephrotic syndrome, ımmunesuppressıve treatments
Author
Mihriban Dağlar Söğütçü
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Mihriban Dağlar Söğütçü (Medical Specialty Thesis). Comparison of immune suppressive treatments in steroid resistant nephrotic syndrome, 2024, Gaziantep University.
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