Enzi G, Digito M, Baldo-Enzi G, Cominacini L, Dodi G, Carraro R, Zurlo F
Department of Internal Medicine, University of Padua, Italy.
Horm Metab Res. 1988 Sep;20(9):587-91. doi: 10.1055/s-2007-1010891.
Some aspects of lipid metabolism were studied in 4 patients with a congenital lipoatrophic diabetes (LAD) associated to a type IV hyperlipoproteinemia. The analysis of lipoprotein composition, expressed as mg/dl, demonstrates a significant increase of VLDL mass and a significant reduction of HDL mass. The analysis of lipoprotein composition, expressed as per cent of total mass demonstrates an increase of the triglyceride content in all fractions and a significant reduction of the cholesterol and phospholipid content in HDL2 particles. Apo C-II, C-III0, C-III1 and C-III2 levels in lipoprotein fractions were normal in LAD patients. Lipoprotein lipase activity in omental adipose tissue, collected during laparoscopy in one patient was undetectable. The serum of this patient did not fully activate the lipoprotein lipase eluted from normal adipose tissue. In all patients the adipose tissue lipoprotein lipase activity in post-heparin plasma was blunted or near absent. Thus a reduced peripheral clearance of triglyceride-rich lipoprotein could be an important determinant of lipoprotein abnormalities in lipoatrophic diabetes.
对4例伴有IV型高脂蛋白血症的先天性脂肪萎缩性糖尿病（LAD）患者的脂质代谢某些方面进行了研究。以mg/dl表示的脂蛋白成分分析显示，极低密度脂蛋白（VLDL）质量显著增加，高密度脂蛋白（HDL）质量显著降低。以占总质量的百分比表示的脂蛋白成分分析显示，所有组分中甘油三酯含量增加，HDL2颗粒中的胆固醇和磷脂含量显著降低。LAD患者脂蛋白组分中的载脂蛋白C-II、C-III0、C-III1和C-III2水平正常。在1例患者腹腔镜检查期间采集的网膜脂肪组织中的脂蛋白脂肪酶活性未检测到。该患者的血清不能完全激活从正常脂肪组织洗脱的脂蛋白脂肪酶。所有患者肝素后血浆中的脂肪组织脂蛋白脂肪酶活性均减弱或几乎缺失。因此，富含甘油三酯脂蛋白的外周清除率降低可能是脂肪萎缩性糖尿病患者脂蛋白异常的一个重要决定因素。
