Aviles Diego H, Vehaskari Matti V, Culotta Kirk S, Manning Jennifer, Ochoa Augusto C, Zea Arnold H
Department of Pediatrics, LSU Health Sciences Center, New Orleans, LA 70112, USA.
Pediatr Nephrol. 2009 Apr;24(4):769-73. doi: 10.1007/s00467-008-1061-1. Epub 2008 Dec 10.
Children with idiopathic nephrotic syndrome (INS) have an increased risk of developing life-threatening infections. Several studies have demonstrated functional abnormalities in the T lymphocytes of patients with nephrotic syndrome. Although T cells are activated in INS during relapse, as indicated by an increased expression of interleukin (IL)-2 receptor, these cells have a decreased ability to proliferate. The T-cell receptor (TCR) plays an important role in signal transduction and T cell activation, with the TCR-zeta (TCRzeta) chain being a key element in early signaling. We measured the expression of the TCRzeta chain in patients with INS (steroid resistant and steroid sensitive) during relapse and remission by flow cytometry and by PCR ELISA. The results showed a significant decrease in the expression of the TCRzeta chain at both the protein and mRNA level in INS patients during relapse as compared with normal controls (p < 0.05). In contrast, when patients with INS achieved remission, the expression of TCRzeta normalized and was similar to that expressed in normal controls. Therefore, a decreased expression of the TCRzeta chain may explain the abnormal function of T cells in patients with INS, and it may also contribute to the increased risk for infections seen in these patients.
患有特发性肾病综合征（INS）的儿童发生危及生命感染的风险增加。多项研究已证实肾病综合征患者的T淋巴细胞存在功能异常。虽然在复发期间INS患者的T细胞被激活，如白细胞介素（IL）-2受体表达增加所示，但这些细胞的增殖能力下降。T细胞受体（TCR）在信号转导和T细胞激活中起重要作用，TCR-ζ（TCRzeta）链是早期信号传导的关键元件。我们通过流式细胞术和PCR ELISA测量了复发期和缓解期INS患者（激素抵抗型和激素敏感型）中TCRzeta链的表达。结果显示，与正常对照相比，复发期INS患者的TCRzeta链在蛋白质和mRNA水平的表达均显著降低（p < 0.05）。相反，当INS患者达到缓解时，TCRzeta的表达恢复正常，与正常对照中的表达相似。因此，TCRzeta链表达降低可能解释了INS患者T细胞的异常功能，也可能导致这些患者感染风险增加。
