Salen G, Kwiterovich P O, Shefer S, Tint G S, Horak I, Shore V, Dayal B, Horak E
J Lipid Res. 1985 Feb;26(2):203-9.
We have measured plasma sterol composition in 14 subjects with sitosterolemia and xanthomatosis. In addition to elevated plasma phytosterol (campesterol 16 +/- 7 mg/dl and sitosterol 35 +/- 16 mg/dl) and normal to moderately high cholesterol levels (258 +/- 96 mg/dl), concentrations of 5 alpha-saturated stanols, cholestanol, 5 alpha-campestanol, and 5 alpha-sitostanol were at least 10 times greater than controls. Diets contained plentiful quantities of cholesterol and plant sterols, but only trace amounts of cholestanol (less than 2 mg/day) and no detectable 5 alpha-campestanol and 5 alpha-sitostanol, which indicated that the 5 alpha-saturated stanols were formed endogenously. Treatment with cholestyramine reduced plasma cholesterol and phytosterol levels by 45% and 5 alpha-saturated stanols by 55%. These results indicate that abnormally high plasma concentrations of cholestanol, 5 alpha-campestanol, and 5 alpha-sitostanol are found in subjects with sitosterolemia and xanthomatosis, and that treatment with cholestyramine effectively reduced elevated plasma sterol levels.
我们测量了14例患有谷甾醇血症和黄瘤病患者的血浆甾醇成分。除了血浆植物甾醇升高（菜油甾醇16±7mg/dl，谷甾醇35±16mg/dl）以及胆固醇水平正常至中度升高（258±96mg/dl）外，5α-饱和甾烷醇、胆甾烷醇、5α-菜油甾烷醇和5α-谷甾烷醇的浓度至少比对照组高10倍。饮食中含有大量胆固醇和植物甾醇，但仅含微量胆甾烷醇（每天少于2mg），且未检测到5α-菜油甾烷醇和5α-谷甾烷醇，这表明5α-饱和甾烷醇是内源性形成的。用消胆胺治疗可使血浆胆固醇和植物甾醇水平降低45%，5α-饱和甾烷醇降低55%。这些结果表明，在患有谷甾醇血症和黄瘤病的患者中发现血浆胆甾烷醇、5α-菜油甾烷醇和5α-谷甾烷醇浓度异常升高，并且用消胆胺治疗可有效降低升高的血浆甾醇水平。
