Urinary excretion of sphingomyelinase phosphodiesterase acid‐like 3b in children with intractable nephrotic syndrome
Urinary excretion of sphingomyelinase phosphodiesterase acid‐like 3b in children with intractable nephrotic syndrome
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顽固性肾病综合征患儿尿中鞘磷脂酶磷酸二酯酶酸3b的排泄
DOI：
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发表时间：
2017
影响因子：
1.4
通讯作者：
Hiroshi Tanaka
中科院分区：
文献类型：
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作者：
Shojiro Watanabe;K. Tsugawa;K. Tsuruga;T. Imaizumi;Hiroshi Tanaka
Rituximab （RTX）是一种针对人CD20的特异性抗体，已成功用于治疗难治性肾病综合征（NS）。最近的研究表明，RTX通过靶向鞘磷脂酶磷酸二酯酶酸样3b （SMPDL‐3b）直接作用于足细胞。因此，我们在顽固性NS患儿的活检标本中检测了SMPDL‐3b的尿排泄及其免疫反应性。我们检测了6名患者（5名NS变化最小，1名局灶节段性肾小球硬化）和4名健康成人的尿液样本。与对照组相比，蛋白尿NS患者肾小球免疫反应性和尿中SMPDL3b的排泄量下降。有趣的是，同一患者在RTX治疗后缓解期的尿液样本显示，与蛋白尿期相比，尿SMPDL‐3b排泄增加。因此，尿中SMPDL‐3b水平可用于预测RTX治疗NS患者的临床疗效。
Rituximab (RTX), a specific antibody to human CD20, has been successfully used to treat intractable nephrotic syndrome (NS). Recent studies have suggested a direct effect of RTX on podocytes by targeting sphingomyelinase phosphodiesterase acid‐like 3b (SMPDL‐3b). Thus, we examined the urinary excretion of SMPDL‐3b as well as its immunoreactivity in biopsy specimens from children with intractable NS. Urine samples from six patients (five with minimal‐change NS and one with focal segmental glomerulosclerosis) and from four healthy adults were examined. Glomerular immunoreactivity and urinary excretion of SMPDL3b in proteinuric NS patients decreased compared with controls. Interestingly, urine samples obtained from the same patients at the remission stage after RTX treatment showed an increase in urinary SMPDL‐3b excretion compared with the proteinuric stage. Urinary excretion level of SMPDL‐3b could thus be used to predict the clinical efficacy of RTX treatment in NS patients.
影响因子：
13.6
作者：
Yoo, Tae-Hyun;Pedigo, Christopher E.;Merscher, Sandra
通讯作者：
Merscher, Sandra