目的　探讨家族性混合型高脂血症与家族性高胆固醇血症血浆载脂蛋白A1(apoA1)、载脂蛋白B10 0 (apoB10 0 )水平的异同。方法　病例 -对照 /家系设计 ,家族性混合型高脂血症家系 15个 (93人 ) ;家族性高胆固醇血症家系 11个 (94人 ) ;对照家系 12个 (6 7人 )。比较家系间及家系内受累组与未受累组血浆apoA1与apoB10 0 水平。结果　两种高脂血症家系间及两种家系内受累组间血浆apoA1与apoB10 0 水平未见统计学显著性差异 ;与对照家系相比 ,两种高脂血症家系血浆apoB10 0 水平均显著升高 (P <0 0 1) ,同时 ,家系内受累组血浆apoB10 0 水平显著高于未受累组 (P <0 0 1)。结论　两种高脂血症家系受累组血浆apoB10 0 水平均显著升高 ,故血浆apoB10 0 水平升高不宜作为家族性混合型高脂血症与家族性高胆固醇血症鉴别诊断的指标 Objective To investigate the similarities and differences between plasma levels of apolipoprotein A1 (apoA1) and apolipoprotein B10 0 (apoB10 0) in familial mixed hyperlipidemia and familial hypercholesterolemia. Methods A total of 15 familial mixed hyperlipidemic pedigrees (93), 11 familial hypercholesterolemic pedigrees (94), and 12 (67) familial mixed hyperlipidemic families were enrolled in this study. The plasma levels of apoA1 and apoB10 0 were compared between affected and non-affected families. Results There were no statistically significant differences in plasma levels of apoA1 and apoB10 0 between the two families of hyperlipidemia and those of the two families. The levels of apoB10 0 in both hyperlipidemic families were significantly higher than those in the control families (P <0.01). Meanwhile, the level of plasma apoB10 0 in the affected pedigree was significantly higher than that in the non-affected group (P <0.01). Conclusions Plasma levels of apoB10 0 are significantly elevated in both affected families of pediatric hyperlipidemic subjects. Therefore, it is not appropriate to use elevated plasma apoB10 0 as a marker for the differential diagnosis between familial mixed hyperlipidemia and familial hypercholesterolemia