Lipoprotein lipase and postheparin esterase activity in primary hyperlipoproteinaemia type IV and V.

L Kucerová, E Fabian, J Fabianová
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Abstract

In a study of the aetiopathogenesis of primary hyperlipoproteinaemias, which has not yet been elucidated, we paid attention to the heparin-activated lipolytic enzymes. Lipoprotein lipase (LPL) and postheparin esterase (PHE) activity was determined in 35 patients with primary hyperlipoproteinaemia (HLP) type IV (average age 50 years), 28 with type V (average age 48 years) and 2 with type III (57 and 62 years). Since PHE activity is correlated to sex and in women also to age, these factors had to be taken into account. The average activity values for the given enzymes in the patients group did not differ from the results in the control group. From these enzymes activities we tried to analyse the findings in individual cases in which the values were lower or higher than the control group range. The low PHE activity in some patients with type IV and V was evidently secondary and due to hepatobiliary disorder (most frequently liver steatosis). The simultaneous elevation of LPL and PHE activity in type IV and V patients with a high serum lipoprotein concentration shows that the response of patients with extreme HLP to heparin is more pronounced. The low PHE activity in type III (tested in only 2 patients) could possibly indicate the liver disorder on which this metabolic disease may be based.

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原发性高脂蛋白血症IV型和V型的脂蛋白脂肪酶和肝素后酯酶活性。
在原发性高脂蛋白血症的发病机制研究中,我们注意到肝素激活的脂溶酶,这一研究尚未阐明。测定35例原发性高脂蛋白血症(HLP) IV型(平均年龄50岁)、28例V型(平均年龄48岁)和2例III型(57岁和62岁)患者的脂蛋白脂肪酶(LPL)和肝素后酯酶(PHE)活性。由于PHE活性与性别有关,女性也与年龄有关,因此必须考虑到这些因素。患者组中给定酶的平均活性值与对照组的结果没有差异。从这些酶的活性,我们试图分析的结果在个别情况下,其值低于或高于对照组的范围。一些IV型和V型患者的PHE活性低显然是继发性的,是由于肝胆功能紊乱(最常见的是肝脂肪变性)。血清脂蛋白浓度高的IV型和V型患者LPL和PHE活性同时升高,说明极端HLP患者对肝素的反应更为明显。III型患者的低PHE活性(仅在2例患者中检测)可能表明这种代谢疾病可能基于肝脏疾病。
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