Faculty of Medicine, Damascus University, Damascus, Syria.
Department of Pediatric Nephrology, Hemodialysis, and Kidney Transplantation, Children's University Hospital, Damascus University, Damascus, Syria.
Pediatr Nephrol. 2024 Aug;39(8):2413-2422. doi: 10.1007/s00467-024-06333-5. Epub 2024 Apr 8.
BACKGROUND: Idiopathic nephrotic syndrome (INS) is the most common glomerular disease in children. We performed this study to report histopathological findings, the correlation between clinical and histopathological features, and the response to steroids and other immunosuppressive drugs and outcomes in Syrian children with INS. METHODS: A single-center retrospective observational cohort study was conducted at Children's University Hospital in Damascus, and included all patients aged 1-14 years, admitted from January 2013 to December 2022, with INS and who underwent kidney biopsy. RESULTS: The study included 109 patients, with a male/female ratio of 1.13:1, and a median age of 5 years with interquartile range (2.8-10). The main indication of kidney biopsy was steroid-resistant nephrotic syndrome (SRNS) (57.8%). The main histopathological patterns were minimal change disease (MCD) (45%) and focal segmental glomerulosclerosis (FSGS) (37.6%). FSGS was the most common histopathological pattern in SRNS (44.3%). In SRNS, we used calcineurin inhibitors to induce remission. Tacrolimus was used in 49 patients with response rate (complete remission of proteinuria) of 69.4% and cyclosporine in 20 patients with response rate of 50%. In steroid-dependent nephrotic syndrome (SDNS), we used mycophenolate mofetil (MMF) and cyclophosphamide to prevent relapses; MMF was used in 9 patients with response rate (maintaining sustained remission) of 89% and cyclophosphamide in 3 patients with response rate of 66.7%. Rituximab was used in four patients with FSGS, two SRNS patients and two SDNS patients, with sustained remission rate of 100%. Fifteen patients (13.7%) progressed to chronic kidney disease stage 5. Of them, 7 patients had FSGS and 8 patients had focal and global glomerulosclerosis;14 of them were steroid-resistant and one patient was steroid-dependent with persistent relapses. The most common outcome was sustained remission (47%) in MCD and frequent relapses (31.7%) in FSGS. CONCLUSIONS: FSGS was the most common histopathological pattern in idiopathic SRNS and had the worst prognosis. Calcineurin inhibitors could be an effective therapy to induce complete remission in SRNS. Rituximab may be an effective treatment to achieve sustained remission in SDNS and frequently relapsing NS and may have a potential role in SRNS with further studies required.
背景：特发性肾病综合征（INS）是儿童中最常见的肾小球疾病。我们进行这项研究旨在报告叙利亚儿童 INS 的组织病理学发现、临床与组织病理学特征的相关性，以及对类固醇和其他免疫抑制剂的反应和结局。
方法：这是一项在大马士革儿童大学医院进行的单中心回顾性观察队列研究，纳入了 2013 年 1 月至 2022 年 12 月期间因 INS 接受肾脏活检的所有 1-14 岁儿童患者。
结果：该研究纳入了 109 例患者，男女比例为 1.13:1，中位年龄为 5 岁（四分位距 2.8-10）。肾脏活检的主要适应证为类固醇抵抗性肾病综合征（SRNS）（57.8%）。主要的组织病理学模式为微小病变性肾病（MCD）（45%）和局灶节段性肾小球硬化症（FSGS）（37.6%）。FSGS 是 SRNS 中最常见的组织病理学模式（44.3%）。在 SRNS 中，我们使用钙调磷酸酶抑制剂来诱导缓解。他克莫司用于 49 例患者，完全缓解蛋白尿的应答率为 69.4%，环孢素用于 20 例患者，应答率为 50%。在类固醇依赖性肾病综合征（SDNS）中，我们使用霉酚酸酯（MMF）和环磷酰胺来预防复发；MMF 用于 9 例患者，持续缓解（维持缓解）的应答率为 89%，环磷酰胺用于 3 例患者，应答率为 66.7%。利妥昔单抗用于 4 例 FSGS、2 例 SRNS 和 2 例 SDNS 患者，持续缓解率为 100%。15 例患者（13.7%）进展为慢性肾脏病 5 期。其中，7 例为 FSGS，8 例为局灶和全球性肾小球硬化症；14 例为类固醇抵抗性，1 例为类固醇依赖性，持续复发。最常见的结局是 MCD 中的持续缓解（47%）和 FSGS 中的频繁复发（31.7%）。
结论：FSGS 是特发性 SRNS 中最常见的组织病理学模式，预后最差。钙调磷酸酶抑制剂可能是诱导 SRNS 完全缓解的有效治疗方法。利妥昔单抗可能是 SDNS 和频繁复发的 NS 中实现持续缓解的有效治疗方法，在进一步研究后可能在 SRNS 中发挥作用。
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