Pediatric Chronic Kidney Disease Research Center, The Children's Hospital Medical Center, Tehran University of Medical Sciences, Tehran, Iran.
Department of Pediatrics, Division of Nephrology, Children's Hospital Medical Center, Tehran University of Medical Sciences, Tehran, Iran.
BMC Pediatr. 2022 Jan 12;22(1):36. doi: 10.1186/s12887-022-03109-4.
There are controversy results in the optimal management of children with steroid-dependent and steroid-resistant nephrotic syndrome (SDNS, SRNS). This study aimed to determine the efficacy and safety of rituximab (RTX) in these pediatric patients.
Medical records of 1-18-year-old Iranian children with SDNS (n = 26) and SRNS (n = 22) with a follow-up for at least 24 months were included from 2009 to 2019. The short- and long-term responses to RTX were respectively evaluated to determine the random protein-to-creatinine ratio after 6 and 24 months and classified as complete (CR) and partial (PR) remission or no response.
Male patients (n = 26) were slightly predominate. The median age of patients at the time of RTX therapy was 8.6 ± 4.01 years. At the end of the 6-month follow-up, CR and PR occurred in 23 (47.9%) and 12 (25%) patients, respectively. Of 23 patients with CR, 18 (69.2%) and 5(22.7%) had SDNS and SRNS, respectively (p < 0.005). However, only 18 (37.5%) of patients after 24 months had been in CR. No significant difference in the CR rate was found between the two groups. RTX was more effective when administered during the proteinuria-free period (p = 0.001).
In the short term, RTX significantly was efficient in inducing complete or PR in SDNS and SRNS patients. However, the favorable response rate in a long-term follow-up was insignificantly lower between the two groups.
在儿童激素依赖性和激素抵抗性肾病综合征（SDNS、SRNS）的最佳治疗方面，结果存在争议。本研究旨在确定利妥昔单抗（RTX）在这些儿科患者中的疗效和安全性。
纳入了 2009 年至 2019 年期间至少随访 24 个月的 26 例 SDNS 和 22 例 SRNS 的 1-18 岁伊朗儿童的病历。分别评估 RTX 治疗后的短期和长期反应，以确定治疗后 6 和 24 个月时随机蛋白尿与肌酐比值，并将其分类为完全缓解（CR）和部分缓解（PR）或无反应。
男性患者（n=26）略占优势。患者接受 RTX 治疗时的中位年龄为 8.6±4.01 岁。在 6 个月的随访结束时，分别有 23（47.9%）和 12（25%）例患者出现 CR 和 PR。在 23 例 CR 患者中，18（69.2%）例和 5（22.7%）例分别患有 SDNS 和 SRNS（p<0.005）。然而，只有 18（37.5%）例患者在 24 个月后仍处于 CR。两组之间的 CR 率无显著差异。在蛋白尿无期间使用 RTX 时效果更明显（p=0.001）。
在短期内，RTX 可显著诱导 SDNS 和 SRNS 患者完全缓解或 PR。然而，在长期随访中，两组之间的良好反应率无显著差异。
