(精编)【医学课件】 DISORDERS OF LIPID METABOLISM教学课件.pptVIP

抽血前受试者至少应坐位休息5min； （7）静脉穿刺过程中止血带使用不应超过1min； （8）血清或血浆标本均适用于TC、TG和HDL-C测定。 如用EDTA作抗凝剂，分离血浆后应立刻放~40C 保存，测定结果乘以1.03后报告； （9）测定TC血清标本可在40C或冰冻进行转送，-200C 贮存标本适于TC测定。但对于TG和其他脂蛋白/ 载脂蛋白测定的标本必须放在-700C或更低温度下 保存； （10）所有血标本应考虑其潜在传染性，应根据有关操 作规程进行处理。 Specimen collection 血清外观检查 DISORDERS OF LIPID  METABOLISM Department of Laboratory Medicine West China Medical School Sichuan University 一、Plasma Lipoprotein 二、Disorders of Lipoprotein Metabolism 三、Analysis Assay 四、Case analysis 一、Plasma Lipoprotein 分类（classification） ? 根据化学结构将血浆脂肪分为胆固醇(cholesterol)，甘油三酯(triglyceride) ，磷脂(phospholipid)和游离脂肪酸(free lipid)。 脂肪在血浆中以脂蛋白型式存在, 所以高脂血症一定是高脂蛋白血症（hyperlipoproteinemia）。 分类（classification） ? 根据化学结构，脂蛋白电泳或超速离心对血浆脂蛋白进行分类。主要的脂蛋白有高密度脂蛋白（HDL），低密度脂蛋白（LDL），极低密度脂蛋白（VLDL）和乳糜微粒（CM）。血浆脂蛋白分析可有助于明确病因。 超速离心 CM VLDL LDL HDL 电泳 CM 前β-LP β-LP α-LP ? 胆固醇主要存在于HDL和LDL中，而甘油三酯主要存在于VLDL和乳糜微粒中。不同的基因缺陷可能产生同样的脂蛋白异常，只有通过广泛的家族分析予以鉴别。 ? LDL Receptor Pathway LDL受体途径主要功能：通过摄取胆固醇进入细胞内，用于细胞增殖和类固醇激素及胆汁酸盐的合成等。 Lipoprotein Metabolism (summary) Lipoprotein are complexes of lipid and proteins which facilitate lipid transport Their metabolism can be thought of as two interconnected cycles centered on the liver Lipoproteins are defined by their density and differ in composition, structure and function Apolipoproteins have a functional as well as structural importance Cholesterol can only be excreted from the body by way of the liver. 二、Disorders of Lipoprotein  Metabolism Lipoprotein disorders are some of the commonest metabolic diseases seen in clinical practice. They may present with their various sequelae which include: coronary heart disease, CHD acute pancreatitis failure to thrive and weakness cataracts Classification Genatic Pathogeny Primary -- when the disorder is not due to an identifiable underlying disease.