Takeshita Jun, Arai Yasumichi, Hirose Nobuyoshi, Tsukamoto Hideko, Shirahase Jyoichiro
Department of Internal Medicine, Keio University School of Medicine, Tokyo, Japan.
Am J Med Sci. 2002 Nov;324(5):281-4. doi: 10.1097/00000441-200211000-00009.
We report the case of a 17-year-old woman with anorexia nervosa (AN) who developed an abetalipoproteinemia-like lipid profile and acanthocytosis. These abnormalities resolved slowly as her nutritional status improved. We considered 3 possible causes of an abetalipoproteinemia-like lipid profile in AN: (1) depletion of hepatic substrate for apolipoprotein B synthesis, (2) lack of exogenous fatty acids with exhaustion of endogenous stores of triglycerides in adipose tissue, and (3) preservation of the lipoprotein lipase (LPL) mass. This unusual case provides important clues that enhance our understanding of lipid metabolism under exogenous and endogenous fat deprivation and highlights the pivotal role of LPL as a gatekeeper of the energy source.
我们报告了一名17岁神经性厌食症(AN)女性患者的病例,该患者出现了无β脂蛋白血症样血脂谱和棘红细胞增多症。随着她营养状况的改善,这些异常情况逐渐得到缓解。我们考虑了AN患者出现无β脂蛋白血症样血脂谱的3种可能原因:(1)载脂蛋白B合成的肝脏底物耗竭;(2)外源性脂肪酸缺乏,同时脂肪组织内源性甘油三酯储备耗尽;(3)脂蛋白脂肪酶(LPL)量的维持。这一罕见病例提供了重要线索,增进了我们对外源性和内源性脂肪缺乏情况下脂质代谢的理解,并突出了LPL作为能量来源守门人的关键作用。