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儿童家族性混合性高脂血症:临床表现、代谢缺陷及管理

Familial combined hyperlipidemia in children: clinical expression, metabolic defects, and management.

作者信息

Cortner J A, Coates P M, Liacouras C A, Jarvik G P

机构信息

Lipid-Heart Research Center, Children's Hospital of Philadelphia, Pennsylvania.

出版信息

Curr Probl Pediatr. 1994 Oct;24(9):295-305. doi: 10.1016/0045-9380(94)90010-8.

DOI:10.1016/0045-9380(94)90010-8
PMID:7859485
Abstract

The first evidence that elevation of plasma levels of cholesterol is a risk factor for the development of atherosclerosis in children came from the Bogalusa Heart Study in 1986, which reported an association between aortic fatty streaks in 3- to 26-year-old subjects and increased plasma levels of low-density lipoprotein cholesterol (LDL-C). The most compelling evidence of a cause-and-effect relationship has come from the multicenter cooperative study called the Pathobiological Determinants of Atherosclerosis in Youth. When the investigators examined the abdominal aorta and the right coronary artery of adolescents and young adults who had died of trauma, they found a significant relationship between the sum of the very low density lipoprotein (VLDL) plus LDL-C level and both fatty streaks and raised atherosclerotic lesions. They also found an inverse relationship between those lesions and increased high-density lipoprotein cholesterol (HDL-C) levels. In addition, their studies showed that smoking (as assessed by the serum thiocyanate level) promotes atherogenesis in children as young as age 15 years. Thus many pediatricians have now accepted the importance of identifying children with significant hypercholesterolemia so that appropriate dietary and life-style modifications can be recommended. This is especially important because there is often a major genetic component to the hyperlipidemia seen in children.

摘要

血浆胆固醇水平升高是儿童动脉粥样硬化发展的危险因素,这一最早的证据来自1986年的博加卢萨心脏研究,该研究报告了3至26岁受试者的主动脉脂肪条纹与血浆低密度脂蛋白胆固醇(LDL-C)水平升高之间的关联。因果关系最有力的证据来自一项名为“青少年动脉粥样硬化的病理生物学决定因素”的多中心合作研究。当研究人员检查死于创伤的青少年和年轻人的腹主动脉和右冠状动脉时,他们发现极低密度脂蛋白(VLDL)与LDL-C水平之和与脂肪条纹和动脉粥样硬化病变增加之间存在显著关系。他们还发现这些病变与高密度脂蛋白胆固醇(HDL-C)水平升高呈负相关。此外,他们的研究表明,吸烟(通过血清硫氰酸盐水平评估)在15岁的儿童中也会促进动脉粥样硬化的发生。因此,许多儿科医生现在已经认识到识别严重高胆固醇血症儿童的重要性,以便能够推荐适当的饮食和生活方式改变。这一点尤为重要,因为儿童高脂血症往往有很大的遗传因素。

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Familial combined hyperlipidemia in children: clinical expression, metabolic defects, and management.儿童家族性混合性高脂血症:临床表现、代谢缺陷及管理
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