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目的 了解肝豆状核变性(HLD)患儿的体液免疫和急性期反应蛋白变化。方法 用琼脂音向免疫扩散法定量测定11例HLD患儿血清补体成分、免疫球蛋白、急性期反应蛋白水平。结果 与正常儿童比较,HLD患儿的C1q、C3、IgA、Tf均未见明显差异,C-1INH、IgG、IgM、FN、Pg、a2M的含量均极显著升高,CP、HP、C4、C5、C9、Bf、PA、ALb、a1AG、a1AT的含量均析显著降低。结论 提示HLD不仅是CP显著改变和铜代谢异常,HLD有抗原刺激机体介导体液免疫应答,HLD患儿的血清蛋白成分含量变化不与机体在急性期应激反应时急性期蛋白的变化相符,可能C-1INH、FN、a2M有利于加强患儿机体的防御保护。
Objective To understand the changes of humoral immunity and acute phase reaction protein in children with hepatolenticular degeneration (HLD). Methods Serum complement components, immunoglobulin and acute phase reaction protein levels in 11 HLD children were quantitatively determined by agar diffusion to immunodiffusion. Results There was no significant difference in C1q, C3, IgA and Tf among children with HLD compared with normal children. The contents of C-1INH, IgG, IgM, FN, Pg and a2M were all significantly increased. The contents of C5, C9, Bf, PA, ALb, a1AG and a1AT were significantly decreased. Conclusions HLD is not only a significant change in CP and abnormal copper metabolism, HLD antigen-stimulated body humoral immune response, HLD children with serum protein content does not change with the body in the acute phase of stress response acute phase protein changes in line, May C-1INH, FN, a2M is conducive to strengthening the body’s defensive protection in children.