Lipids in Children and Links to Adult Vascular Disease.

Q1 Biochemistry, Genetics and Molecular Biology
Clinical Biochemist Reviews Pub Date : 2018-08-01
John C Coakley
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Abstract

Atherosclerosis often begins in childhood or adolescence. Post-mortem studies in children have shown the presence of coronary atheroma, and there are hereditary conditions associated with hyperlipidaemia in childhood which lead to premature cardiovascular disease. Detection of hyperlipidaemia early in life can be crucial in the prevention of premature death from atherosclerosis. The circulating lipoproteins are in a constant state of flux, with passage of apolipoproteins and lipids between the various particles. Genetic variants of apolipoproteins can cause both hypercholesterolaemia and hypertriglyceridaemia. Elevated concentrations of lipoprotein(a) predispose to coronary artery disease. Another important molecule in lipid metabolism, proprotein convertase subtilisin/kexin type 9 (PCSK9), plays a crucial role in the removal of low-density lipoprotein (LDL) receptors. Reference intervals for the various lipid subfractions are now available for children, and there are guidelines regarding when to take action regarding paediatric hyperlipidaemia. The most important genetic condition in children which may lead to premature death from coronary heart disease is familial hypercholesterolaemia (FH). FH is best diagnosed and treated early in life. Most cases are due to defects in the LDL receptor. Pharmacotherapy for FH usually involves the statin group of drugs, although newer medications are now available, especially for the treatment of homozygous FH. Statin therapy has been demonstrated to be successful in preventing cardiac events in FH. Secondary dyslipidaemia in childhood can be associated with numerous diseases including diabetes, lifestyle disorders such as obesity, and drugs. Treatment of the underlying condition usually resolves the hyperlipidaemia.

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儿童血脂与成人血管疾病的关系
动脉粥样硬化通常开始于儿童或青少年时期。对儿童的尸检研究表明存在冠状动脉粥样硬化,并且存在与儿童期高脂血症相关的遗传条件,导致过早的心血管疾病。在生命早期检测高脂血症对于预防动脉粥样硬化导致的过早死亡至关重要。随着载脂蛋白和脂质在不同颗粒之间的传递,循环脂蛋白处于恒定的流动状态。载脂蛋白的遗传变异可引起高胆固醇血症和高甘油三酯血症。脂蛋白(a)浓度升高易患冠状动脉疾病。脂质代谢的另一个重要分子是蛋白转化酶枯草素/酮素9型(PCSK9),它在低密度脂蛋白(LDL)受体的去除中起着至关重要的作用。各种脂质亚组分的参考区间现在可用于儿童,并有关于何时采取行动,针对儿科高脂血症的指导方针。家族性高胆固醇血症(FH)是可能导致儿童因冠心病过早死亡的最重要的遗传疾病。FH最好在生命早期得到诊断和治疗。大多数病例是由于LDL受体的缺陷。FH的药物治疗通常包括他汀类药物,尽管现在有新的药物可用,特别是纯合子FH的治疗。他汀类药物治疗已被证明可成功预防FH患者的心脏事件。儿童期继发性血脂异常可能与许多疾病有关,包括糖尿病、生活方式紊乱(如肥胖)和药物。治疗基础疾病通常能解决高脂血症。
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来源期刊
Clinical Biochemist Reviews
Clinical Biochemist Reviews Biochemistry, Genetics and Molecular Biology-Clinical Biochemistry
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