Koivisto P V, Miettinen T A
Second Department of Medicine, University of Helsinki, Finland.
Clin Chim Acta. 1988 May 31;174(2):197-205. doi: 10.1016/0009-8981(88)90386-5.
Plasma cholestanol is increased in cerebrotendinous xanthomatosis and in sitosterolemia with xanthomatosis. We measured plasma and biliary cholestanol in heterozygous familial hypercholesterolemia patients with (n = 10) and without (n = 12) ileal exclusion. In the unoperated patients plasma cholestanol concentration (12.9 mumol/l) and content (1.2 mmol/mol cholesterol) were slightly higher than in the nonhypercholesterolemic control subjects studied by us. Ileal exclusion had lowered plasma cholestanol concentration but only in proportion to the lowering of plasma cholesterol concentration, and plasma cholestanol content (mmol/mol cholesterol) was similar in the operated and unoperated subjects. Plasma and biliary cholestanol contents were positively associated. In the unoperated patients the fractional cholesterol absorption and plasma plant sterols, also reflecting sterol absorption, were positively correlated with plasma cholestanol content. Our study suggests, that plasma cholestanol is slightly elevated in familial hypercholesterolemia and that, in addition to plasma lipoprotein level, sterol absorption is important in the regulation of plasma cholestanol level. Ileal exclusion decreases plasma cholestanol in proportion to the decrement in the plasma cholesterol concentration.
在脑腱性黄瘤病以及伴有黄瘤病的谷甾醇血症中，血浆胆甾烷醇会升高。我们检测了有回肠排除术（n = 10）和无回肠排除术（n = 12）的杂合子家族性高胆固醇血症患者的血浆和胆汁胆甾烷醇。在未接受手术的患者中，血浆胆甾烷醇浓度（12.9 μmol/L）和含量（1.2 mmol/mol胆固醇）略高于我们研究的非高胆固醇血症对照受试者。回肠排除术降低了血浆胆甾烷醇浓度，但仅与血浆胆固醇浓度的降低成比例，并且手术组和未手术组受试者的血浆胆甾烷醇含量（mmol/mol胆固醇）相似。血浆和胆汁胆甾烷醇含量呈正相关。在未接受手术的患者中，胆固醇吸收分数和血浆植物甾醇（也反映甾醇吸收）与血浆胆甾烷醇含量呈正相关。我们的研究表明，家族性高胆固醇血症患者的血浆胆甾烷醇略有升高，并且除了血浆脂蛋白水平外，甾醇吸收在血浆胆甾烷醇水平的调节中也很重要。回肠排除术会使血浆胆甾烷醇与血浆胆固醇浓度的降低成比例地减少。
