Hulton S A, Shah V, Byrne M R, Morgan G, Barratt T M, Dillon M J
Division of Medicine (Medical Unit), Institute of Child Health, London, UK.
Pediatr Nephrol. 1994 Apr;8(2):135-9. doi: 10.1007/BF00865458.
Abnormal T lymphocyte function and reduced interleukin-2 (IL-2) production have been implicated in the pathogenesis of the nephrotic syndrome (NS). We investigated: (1) lymphocyte subpopulations and expression of IL-2 receptor (IL-2R) on T cells using two-colour flow cytometry, (2) serum IL-2 and (3) the soluble component of IL-2R (sIL-2R) in serum, using enzyme-linked immunosorbent assay, in 38 children with NS. All children, except those in remission, had marked proteinuria. They were divided into groups according to renal pathology: (1) steroid-sensitive NS (SSNS) not receiving prednisolone therapy, (2) SSNS on prednisolone, (3) focal segmental glomerulosclerosis (FSGS), (4) SSNS in remission and not receiving prednisolone therapy, (5) congenital NS (CNS). Results were compared with 26 age-matched controls. Total T lymphocytes (CD3) were reduced in groups 1 and 2; CD4 count was reduced in groups 1-4; CD8 count increased in groups 2 and 3; CD8 and CD19 (B lymphocytes) were significantly reduced in group 5. Increased IL-2R expression (CD25) on CD4 lymphocytes was noted in groups 1, 2 and 3 implying activation of these cells. In patients with SSNS, increased serum sIL-2R was recorded during relapse (1,273 +/- 497 U/l vs. 913 +/- 401 U/l in remission, P < 0.005) but free serum IL-2 was not detectable at any stage. The specific alterations in lymphocyte subpopulations in SSNS and FSGS would imply an involvement of the immune system distinct from that in CNS.
异常的T淋巴细胞功能和白细胞介素-2（IL-2）产生减少与肾病综合征（NS）的发病机制有关。我们对38例NS患儿进行了研究：（1）采用双色流式细胞术检测淋巴细胞亚群及T细胞上IL-2受体（IL-2R）的表达；（2）检测血清IL-2；（3）采用酶联免疫吸附测定法检测血清中IL-2R的可溶性成分（sIL-2R）。所有患儿，除缓解期患儿外，均有明显蛋白尿。根据肾脏病理将他们分为几组：（1）未接受泼尼松龙治疗的激素敏感型NS（SSNS）；（2）接受泼尼松龙治疗的SSNS；（3）局灶节段性肾小球硬化（FSGS）；（4）缓解期且未接受泼尼松龙治疗的SSNS；（5）先天性NS（CNS）。将结果与26例年龄匹配的对照组进行比较。第1组和第2组总T淋巴细胞（CD3）减少；第1 - 4组CD4计数减少；第2组和第3组CD8计数增加；第5组CD8和CD19（B淋巴细胞）显著减少。第1、2和3组CD4淋巴细胞上IL-2R表达（CD25）增加，提示这些细胞被激活。在SSNS患者中，复发期间血清sIL-2R升高（1273±497 U/l，缓解期为913±401 U/l，P<0.005），但在任何阶段均未检测到游离血清IL-2。SSNS和FSGS中淋巴细胞亚群的特异性改变表明其免疫系统受累情况与CNS不同。
