The biochemical, clinical, and genetic features of type III hyperlipoproteinemia.
The biochemical, clinical, and genetic features of type III hyperlipoproteinemia.
复制标题
III 型高脂蛋白血症的生化、临床和遗传特征。
DOI：
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发表时间：
1975
影响因子：
39.2
通讯作者：
D. Fredrickson
中科院分区：
文献类型：
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作者：
J. Morganroth;R. Levy;D. Fredrickson
III型高脂蛋白血症的临床和生化特征描述了49例患者，年龄从23岁至70岁。异常化学成分的极低密度脂蛋白（VLDL）的增加是诊断的基础。未经治疗的患者均患有高胆固醇血症和高甘油血症，平均而言，低密度脂蛋白和高密度脂蛋白的浓度均降低。74%的人患有黄色瘤，超过一半的人发现了经典的“掌纹状黄色瘤”。27%的人患有缺血性心脏病，男性比女性更早发现。27%的患者患有外周血管疾病（相比之下，II型高脂蛋白血症患者为4%）。35例受试者中有25例仅通过饮食治疗达到正常血脂水平。29个亲属的分析表明，高脂血症的一半，成年血液亲属，其中一半有III型，其余通常有样品内源性高甘油血症（IV型）。55名20岁以下的儿童中只有2人受到影响，均为IV型。
The clinical and biochemical features of type III hyperlipoproteinemia are described in 49 patients from 23 to 70 years of age. An increase in very low-density lipoproteins (VLDL) of abnormal chemical composition was the basis for diagnosis. The untreated patients all had hypercholesterolemia and hyperglyceridemia, and, on the average, decreased concentrations of both low- and high-density lipoproteins. Seventy-four percent had xanthomas, and classic "xanthoma striata palmaris" was found in more than half. Twenty-seven percent had ischemic heart disease, detected earlier in men than in women. Twenty-seven percent had peripheral vascular disease (compared to 4% of subjects with type II hyperlipoproteinemia). Twenty-five of 35 subjects achieved normal lipid levels with dietary therapy alone. Analysis of 29 kindred showed hyperlipidemia in half of adult blood relatives; half of these had type III, the remainder usually had sample endogenous hyperglyceridemia (type IV). Only 2 of 55 children less than 20 years of age were affected, both with type IV.