Macnamara B G, Farn K T, Mitra A K, Lloyd J K, Fosbrooke A S
Arch Dis Child. 1970 Aug;45(242):553-60. doi: 10.1136/adc.45.242.553.
A girl with the typical features of progeria died at the age of 10 years from cardiac failure following repeated myocardial infarctions. Serial studies of the serum lipids were made from the age of 2 years 3 months. Variable hyperlipidaemia, increased β- and pre-β-lipoproteins, and impaired clearing of absorbed dietary fat were shown. A polyunsaturated fat diet, which was started at the age of 2 years 5 months and continued throughout the rest of her life, controlled the hyperlipidaemia and the clearing of fat became normal. Pre-β-lipoprotein could still, however, be seen occasionally. The dietary regimen did not delay the development of atherosclerosis, the pathogenesis of which, in progeria, may be due to factors other than hyperlipidaemia.
一名具有典型早老症特征的女孩在10岁时因反复心肌梗死后心力衰竭而死亡。从2岁3个月起对其血脂进行了系列研究。结果显示存在可变的高脂血症、β-脂蛋白和前β-脂蛋白增加以及吸收的膳食脂肪清除受损。从2岁5个月开始并在其余生持续的多不饱和脂肪饮食控制了高脂血症，脂肪清除恢复正常。然而，仍偶尔可见前β-脂蛋白。这种饮食方案并未延缓动脉粥样硬化的发展，早老症中动脉粥样硬化的发病机制可能归因于高脂血症以外的因素。
