TY  - JOUR
TI  - Rituximab treatment for difficult-to-treat nephrotic syndrome in children: a multicenter, retrospective study
AB  - Background/aim: This study aimed to evaluate the efficacy of rituximab in children with difficult-to-treat nephrotic syndrome, considering the type of disease (steroid-sensitive or –resistant) and the dosing regimen. Materials and methods: This multicenter retrospective study enrolled children with difficult-to-treat nephrotic syndrome on rituximab treatment from 13 centers. The patients were classified based on low (single dose of 375 mg/m2) or high (2-4 doses of 375 mg/m2) initial dose of rituximab and the steroid response. Clinical outcomes were compared. Results: Data from 42 children [20 steroid-sensitive (frequent relapsing / steroid-dependent) and 22 steroid-resistant nephrotic syndrome, aged 1.9–17.3 years] were analyzed. Eleven patients with steroid-sensitive nephrotic syndrome (55%) had a relapse following initial rituximab therapy, with the mean time to first relapse of 8.4 ± 5.2 months. Complete remission was achieved in 41% and 36% of steroid-resistant patients, with the median remission time of 3.65 months. At Year 2, eight patients in steroid-sensitive group (40%) and four in steroid-resistant group (18%) were drug-free. Total cumulative doses of rituximab were higher in steroid-resistant group (p = 001). Relapse rates and time to first relapse in steroid-sensitive group or remission rates in steroid-resistant group did not differ between the low and high initial dose groups. Conclusion: The current study reveals that rituximab therapy may provide a lower relapse rate and prolonged relapse-free survival in the steroid-sensitive group, increased remission rates in the steroid-resistant group, and a significant number of drug-free patients in both groups. The optimal regimen for initial treatment and maintenance needs to be determined.Key words: Frequently relapsing nephrotic syndrome, immunosuppressive agents, steroid-dependent nephrotic syndrome, steroidresistant nephrotic syndrome, remission
AU  - BALAT, AYSE
AU  - Goknar, Nilufer
AU  - AKGUN, CIHANGIR
AU  - çelakıl, mehtap
AU  - Sever, Fatma lale
AU  - Taşdemir, Mehmet
AU  - Türkkan, Özde nisa
AU  - BENZER, MERYEM
AU  - Saygili, Seha Kamil
AU  - ALPAY, HARİKA
AU  - AKINCI, NURVER
AU  - Candan, Cengiz
AU  - Yavuz, Sevgi
AU  - Bilge, Ilmay
AU  - Özçelik, Gül
AU  - Ozkayin, Emine Nese
AU  - Yıldız, Nurdan
AU  - Canpolat, Nur
DO  - 10.3906/sag-2012-297
PY  - 2021
JO  - Turkish Journal of Medical Sciences
VL  - 51
IS  - 4
SN  - 1300-0144
SP  - 1781
EP  - 1790
DB  - TRDizin
UR  - http://search/yayin/detay/480445
ER  -