LIPOPROTEIN ABNORMALITIES IN PRIMARY BILIARY-CIRRHOSIS - ASSOCIATION WITH HEPATIC LIPASE INHIBITION AS WELL AS ALTERED CHOLESTEROL ESTERIFICATION
LIPOPROTEIN ABNORMALITIES IN PRIMARY BILIARY-CIRRHOSIS - ASSOCIATION WITH HEPATIC LIPASE INHIBITION AS WELL AS ALTERED CHOLESTEROL ESTERIFICATION
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DOI：
10.1016/0016-5085(85)90642-0
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发表时间：
1985-01-01
期刊：
影响因子：
29.4
通讯作者：
BREWER, HB
中科院分区：
文献类型：
--
作者：
JAHN, CE;SCHAEFER, EJ;BREWER, HB
对不同阶段原发性胆汁性肝硬化（PBC）患者血浆脂蛋白异常的性质和病因进行了评估。观察到两种不同的脂蛋白模式。在PBC的早期和中期组织学阶段的患者中，经常注意到极低密度脂蛋白和低密度脂蛋白（LDL）的轻度升高以及高密度脂蛋白（HDL）的显著增加（组1）。相比之下，晚期疾病患者在存在脂蛋白-X的情况下LDL显著升高，HDL显著降低（第2组）。第1组中的脂蛋白模式的特征在于游离胆固醇与总胆固醇的正常比率，而在第2组中，该比率升高。在第1组患者的血浆中，仅在电子显微镜下观察到球形胶束，而在第2组患者的血浆中，LDL和HDL中可以观察到双层圆盘。在第1组受试者中观察到的HDL升高与HDL 2升高相关，而在第2组受试者中，两组中仅极少量的HDL 3升高;但载脂蛋白A-I和A-II值不同，第1组受试者的值升高，第2组受试者的值降低。由于PBC血浆中存在抑制剂，两组PBC患者的平均肝素后肝脂肪酶活性均降低，而仅在第2组中观察到胆固醇酯化改变。这些数据表明，肝脂酶抑制可能在早期和中期PBC中观察到的脂蛋白异常的发病机制中起重要作用，并且这些异常随后通过改变晚期疾病中的胆固醇酯化而进一步修饰。
The nature and etiology of plasma lipoprotein abnormalities in patients with varying stages of primary biliary cirrhosis (PBC) were assessed. Two distinct lipoprotein patterns were observed. In patients with early and intermediate histologic stages of PBC, mild elevations of very low density lipoproteins and low density lipoproteins (LDLs), and marked increases in high density lipoproteins (HDLs) were often noted (group 1). In contrast, patients with advanced disease had marked elevations in LDLs with the presence of lipoprotein-X, and a significant decrease in HDLs (group 2). The lipoprotein pattern in group 1 was characterized by a normal ratio of free cholesterol to total cholesterol, whereas in group 2, this ratio was elevated. In plasma from group 1 patients only spherical micelles were observed on electron microscopy, whereas in plasma from group 2 patients bilayered disks could be seen in LDLs and HDLs. The increase in HDLs observed in group 1 subjects was associated with elevations in HDL2, whereas in group 2 subjects only very low amounts of HDL3 were increased in both groups; but apolipoprotein A-I and A-II values were divergent, with group 1 subjects having elevated values, and group 2 subjects having decreased values. Mean postheparin hepatic lipase activity was decreased in both groups of patients with PBC due to the presence of an inhibitor in PBC plasma, while altered cholesterol esterification was only observed in group 2. These data indicate that hepatic lipase inhibition may play an important role in the pathogenesis of the lipoprotein abnormalities seen in early and intermediate PBC, and these abnormalities are then further modified by altered cholesterol esterification in advanced disease.