40例肝豆状核变性患者血浆CP、α_2-M、MDA及红细胞SOD的测定  

THE DETERMINATION OF PLASMA CERULOPLASMIN,α2-MACROGLOBULIN, MALONALDEHYDE ANDERYTHROCYTE SUPEROXIDE DISMUTASELEVELS IN 40 PATIENTS WITHHEPATOLENTICULAR DEGENERATION

作　　者：李乃忠[1] 刘道宽[1] 姚景莉[1] 陈识杰[2] 乐敏[2] 胡昱兴[2] 

机构地区：[1]上海医科大学神经病学研究所 [2]上海医科大学放射医学研究所

出　　处：《上海医科大学学报》1991年第3期205-208,共4页Journal of Fudan University(Medical Science)

摘　　要：本文检测了40例肝豆状核变性(HLD)患者的血浆铜蓝蛋白(CP)、α_2-巨球蛋白(α_2-M)、丙二醛(MDA)含量及红细胞超氧化物歧化酶(SOD)的活力。发现HLD患者血浆α_2-M含量增高,CP含量及红细胞SOD活力降低,重症患者血浆MDA含量增高,而轻症者则无明显改变。经青霉胺治疗后CP含量仍无改变,α_2-M含量降低,SOD活力上升。认为CP含量为HLD的异常基团表达,而α_2-M、MDA及SOD的变化继发于自由基清除障碍。对上述指标监测的临床意义进行了讨论。Plasma ceruloplasmin(CP),α2-maoroglobulin(α2-M),malonaldehyde(MDA) levels and erythrocyte superoxide dismutase(SOD) activity were measured in 40 pabients with hepatolenticular degeneration(HLD).Results revealed plasma α2-M level to be increased above normal,CP level and erythrocyte SOD activity decreased.Plasma MDA levels were increased in those patients with severe clinical status and unchanged in mild patients.After treatment with D-penicillamine,plasma CP concentrations were unchanged in both mild and severe patients,but their plasma levels of α2-M were lowered,erythrocyte SOD activities increased.It is considered that reduction of plasma CP level is the abnormal gene expression of HLD,but the changes of plasma α2-M,MDA levels and erythrocyte SOD activity result from chronic clearance disturbance of free radicals.The clinical significance of continuous monitoring of all above items was discussed.

关 键 词：肝豆状核变性 超氧物歧化酶 自由基 

分 类 号：R575.240.2[医药卫生—消化系统]