Evaluation of risk for atherosclerosis in Alagille syndrome and progressive familial intrahepatic cholestasis: Two congenital cholestatic diseases with different lipoprotein metabolisms
Evaluation of risk for atherosclerosis in Alagille syndrome and progressive familial intrahepatic cholestasis: Two congenital cholestatic diseases with different lipoprotein metabolisms
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DOI：
10.1016/j.jpeds.2004.10.047
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发表时间：
2005-03-01
影响因子：
5.1
通讯作者：
Kobayashi, K
中科院分区：
文献类型：
--
作者：
Nagasaka, H;Yorifuji, T;Kobayashi, K
目的以脂蛋白代谢为基础，通过超声检查评估Alagille综合征（AGS）和进行性家族性肝内胆汁淤积症（PFIC）患者发生动脉粥样硬化的风险。超声检查颈总动脉内膜中层厚度和管壁硬度。测定血清脂质和脂蛋白水平。此外，LDL的化学成分和其将巨噬细胞转化为泡沫细胞的能力进行了determined.Results内膜-中层厚度和壁刚度增加PFIC患者，但没有生病的患者与AGS。AGS患者的总胆固醇、LDL胆固醇、HDL胆固醇和脂蛋白X显著升高，而PFIC患者的显著结果是甘油三酯升高和HDL胆固醇降低。然而，尽管LDL胆固醇水平正常，氧化LDL水平在PFIC患者中显著升高。PFIC患者的LDL中TG含量高，并具有较高的将巨噬细胞转化为泡沫细胞的能力。结论PFIC患者是动脉粥样硬化性心血管疾病的高危人群。
Objectives To evaluate the risk for atherosclerosis in Alagille syndrome (AGS) and progressive familial intrahepatic cholestasis (PFIC) oil the basis of lipoprotein metabolism and by ultrasonography.Study design Five patients with AGS and 5 with PFIC, ages 3 to 4 years, were enrolled. Intimal-medial thickness and wall stiffness of the common carotid artery were examined by ultrasonography. Serum levels of lipids and lipoproteins were determined. Further, the chemical composition of LDL and its ability to transform macrophages into foam cells were determined.Results Intimal-medial thickness and wall stiffness were increased in patients with PFIC but not ill patients with AGS. Total cholesterol, LDL cholesterol, HDL cholesterol, and lipoprotein X were remarkably increased in patients with AGS, whereas in patients with PFIC, an increase in triglyceride and a decrease in HDL cholesterol were the prominent findings. However, despite the normal LDL cholesterol level, oxidized LDL level was strikingly high in patients with PFIC. LDLs from patients with PFIC had high TG contents and exhibited high abilities to transform macrophages into foam cells.Conclusions These findings suggest that patients with PFIC are at high risk for cardiovascular disorders involving atherosclerosis.