中华皮肤科杂志 ›› 1990, Vol. 23 ›› Issue (1): 36-38.

• 论著 • 上一篇    下一篇

Fabry病患者血清HDL2-C, HDL3-C, LPO含量分析及皮疹

刘德文1, 姜树山2, 陈丽瑛3, 张悦红1, 杨建学2, 叶培明3, 鲍丽玲4, 郭玲玲4, 杨美荣5   

  1. 1. 山西医学院生物化学教研室;
    2. 山西医学院病理解剖教研室;
    3. 山西医学院附属一院皮肤科;
    4. 山西医学院附属一院眼科;
    5. 太原市卫生防疫站
  • 收稿日期:1988-05-04 修回日期:1988-10-10 出版日期:1990-02-15 发布日期:1990-02-15

DETERMINATION OF CONCENTRAION OF HDL2-G, HDL3-C AND LPO IN PATIENTS WITH FABRY’S DISEASE LIU

LIU De-Wen1, JIANG Shu-Shan2   

  1. Shanxi Medical College, Taiyuan
  • Received:1988-05-04 Revised:1988-10-10 Online:1990-02-15 Published:1990-02-15

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摘要： 本文对两个Fahy病家族的4名患者进行血清高密度脂蛋自亚组分(HDL2-C,HDL3-C)及脂质过氧化物(LPO)含量分析,并将病变组织作了超微结构观察.发现Fabrp病患者除α-半乳糖苷脊酶缺陷引起糖脂代谢素乱之外,还可能涉及外周血中胆固醇回输肝脏的过程障碍及脂质过氧化反应速率增快致使细胞膜损伤.血清HDL2-C含量降低,血清LPO升高.并证实Fahry病特异的电子致密包含体的存在.

Abstract: The concentration of nigh-density lipoprotein subfractions and the level of lipid peroxides were determined in four cases of Fabry's disease in two families.The results showed that the concentration of H.DL2-C decreased,the HDL3-G/HDL2-C ratio and the content of lipid peroxides in the sera were significantly-elevated in lour patients.Electron xnicrascopy showed that there were different kinds of electron dense inclusion in endothelial cells,pericytes,smooth muscle cells and febroblast.In the cytoplasra of these cells,there were round lipid granules in different sizes.This study suggests that the pathogenesis of Fabry's.disease may be caused by the accumulation of sphingolipids and metabolic disorder of HDL-C subfractions (HDL3-C and HDL2-C) and lipid peroxides.