【摘要】 目的　探讨转化生长因子 β(TGFβ)在小儿原发性肾病综合征 (INS)中的变化及其意义。方法　应用酶联免疫吸附试验 (ELISA)及原位逆转录多聚酶链反应 (InSitu RTPCR)方法 ,分别检测了30例健康儿童和 35例原发性肾病综合征患儿活动期 (35例 )与缓解期 (2 5例 )外周血单个核细胞(PBMC)在内毒素 (LPS)刺激下TGFβ蛋白水平和TGFβmRNA表达的变化 ;同时观察激素治疗中PBMC的TGFβ的变化。结果　 (1)INS (单纯型和肾炎型 )患儿活动期TGFβ蛋白水平 (2 4 7± 2 6 )ng L和TGFβmRNA表达 (0 5 7± 0 18)明显高于缓解期和对照组 (P均 <0 0 1) ;而缓解期TGFβ蛋白水平(12 5± 16 )ng L和TGFβmRNA表达 (0 30± 0 12 )与对照组比较差异也有显著性 (P <0 0 5 )。 (2 )INS活动期肾炎型与单纯型比较 ,TGFβ蛋白水平 [(2 75± 2 6 )ng L、(2 2 0± 18)ng L]差异有显著性 (t =6 .4 5 ,P <0 0 1) ,mRNA表达 (0 5 8± 0 15、0 5 5± 0 16 )差异无显著性 ;肾炎型和单纯型与对照组比较差异均有显著性 (P均 <0 0 1)。 (3)激素敏感型治疗后TGFβ蛋白水平和mRNA表达明显低于治疗前 (P<0 0 1) ;而激素耐药型则变化不明显 (P >0 0 5 ) ;除治疗前mRNA两型差异无显著性外 ,激素耐药型无论是治疗前还是治疗后都明显高于?更多还原

【Abstract】 Objective Idiopathic nephrotic syndrome (INS) is a common glomerular disease. The pathogenesis of the disease remains unclear. Recent studies indicate that transforming growth factor β (TGF β) is the main cytokine involved in glomerular disease. It plays an important role in the development of INS and in occurrence of glomerulosclerosis. The present study aimed to study changes and significance of TGF β in children with idiopathic nephrotic syndrome (INS). Methods Totally 35 cases with INS (13 males, 22 females) were studied. The age of onset was between 2 years and 1 months and 14 years with an average of 8 years and 3 months. The active stage group had 35 cases and the remission stage groups had 25 cases. The cases in active stage group had first onset of the disease with obvious clinical symptoms and abnormal laboratory findings without use of corticosteroids. The cases in remission stage group were asymptomatic without abnormal laboratory findings. Protein in urine was negative over 4 weeks after oral administeration of prednisone for 8 weeks. Twenty five cases were steroid responsive and 10 cases were steroid non-responsive among the 35 cases. Thirty healthy young children were enrolled as control. TGF β was detected by ELISA in peripheral blood monoeuclear cell (PBMC) culture medium. The TGF β mRNA gene expression was measured by insitu PCR in PBMC. Results (1) Concentration of TGF β(247±26) ng/L and TGF β mRNA expression (0.57±0.18) in active stage of simple type or nephritis type INS were higher than those of remission stage and control (P<0.01). Concentration of TGF β[(125±16) ng/L] and TGF β mRNA expression(0.30±0.12)in remission stage were higher than that of control (P<0.05). (2)The level of TGF β protein in nephritis type [(275±26) ng/L] was significantly higher than that in simple type [(220±18) ng/L] in active stage INS (t=6.45,P<0.01). No significant difference in TGF β mRNA expression was found between the nephritis type(0.58±0.15)and simple type (0.55±0.16) in active stage INS, either (P>0.05). But these two types were different from the control (P< 0.01). (3) Concentration of TGF β and TGF β mRNA expression after therapy was clearly lower than that before therapy in steroid responsive group (P<0.01). Whereas no significant change was seen in steroid non-responsive group. Both indicators were higher in steroid non-responsive group than in steroid responsive group whether before or after therapy. Conclusion TGF β may play an important role in the mechanism of INS and its level in PBMC can be used as an immunological indicator for the illness state, therefore, determination of TGF β level and mRNA may be of some clinical significance.更多还原