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Sphingomyelin Phosphodiesterase Acid-Like 3b(SMPDL-3b)水平在肾病综合征患儿肾活检标本中的重要性。

The Importance of Sphingomyelin Phosphodiesterase Acid-Like 3b (SMPDL-3b) Levels in Kidney Biopsy Specimens of Children With Nephrotic Syndrome.

机构信息

Department of Pediatric Nephrology, Faculty of Medicine, Pamukkale University, Denizli, Turkey.

Department of Pathology, Faculty of Medicine, Pamukkale University, Denizli, Turkey.

出版信息

Fetal Pediatr Pathol. 2023 Dec;42(6):936-949. doi: 10.1080/15513815.2023.2267683. Epub 2023 Nov 22.

DOI:10.1080/15513815.2023.2267683
PMID:37818552
Abstract

OBJECTIVE

It remains unclear whether the low amount of SMPDL-3b required for rituximab binding is the cause of treatment resistance in patients with treatment-resistant nephrotic syndrome with advanced podocyte injury. Given the limited number of studies on the relationship between rituximab and SMPDL-3b, this study was conducted to assess whether SMPDL-3b levels in pretreatment renal biopsy specimens can be used to predict the clinical effectiveness of immunosuppressive drugs, especially rituximab, in children with nephrotic syndrome.

METHODS

Kidney biopsy specimens from 44 patients diagnosed with idiopatic nephrotic syndrome were analyzed using immunohistochemical staining with an anti-SMPDL-3b antibody and real-time polymerase chain reaction (PCR) for SMPDL-3b mRNA expression.

RESULTS

We showed that SMPDL-3b mRNA expression and anti-SMPDL-3b antibody staining did not differ significantly between the patient groups with different responses to immunosuppressive therapies.

CONCLUSION

Our results suggest that SMPDL-3b may actually be an indicator of disease progression rather than a marker for predicting response to a particular immunosuppressive agent.

摘要

目的

尚不清楚利妥昔单抗结合所需的 SMPDL-3b 量低是否是伴有晚期足细胞损伤的治疗抵抗性肾病综合征患者治疗抵抗的原因。鉴于关于利妥昔单抗和 SMPDL-3b 之间关系的研究数量有限,本研究旨在评估肾活检标本中 SMPDL-3b 水平是否可用于预测免疫抑制剂,特别是利妥昔单抗在肾病综合征患儿中的临床疗效。

方法

使用针对 SMPDL-3b 的抗 SMPDL-3b 抗体进行免疫组织化学染色和 SMPDL-3b mRNA 表达的实时聚合酶链反应(PCR)对 44 例诊断为特发性肾病综合征的患者的肾活检标本进行了分析。

结果

我们表明,对免疫抑制疗法有不同反应的患者组之间 SMPDL-3b mRNA 表达和抗 SMPDL-3b 抗体染色没有显着差异。

结论

我们的结果表明,SMPDL-3b 实际上可能是疾病进展的指标,而不是预测对特定免疫抑制剂反应的标志物。

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