Goebel K M, Goebel F D, Schubotz R, Schneider J
Br J Haematol. 1977 Apr;35(4):573-85. doi: 10.1111/j.1365-2141.1977.tb00622.x.
Zieve's syndrome (ZS), which consists of transient haemolytic anaemia, jaundice, hyperlipoproteinaemia, and alcohol-induced liver disease, was studied in male patients during the acute (n = 20) and the remittent (n = 10) phase. Chronic alcoholics (n = 10) without haemolysis and healthy male persons (n = 10) served as controls. Erythrocytes were separated into old and young cells by means of density-layer centrifugation. Those fractions which contained older red cells disclosed a pyruvate-kinase instability which resulted in impaired metabolism. Changes in membrane lipid composition as indicated by increased cholesterol and polyunsaturated fatty acids (PUFA) were also detected in patients during the acute phase of ZS. Alcohol-induced red-cell vitamin-E deficiency with a decrease in PUFA levels may provoke an oxidation of reduced red-cell glutathione which in turn results in the enzyme instability. This study lends further support to the hypothesis that the putative role of the red-cell metabolic injury in the origin of haemolysis in ZS cannot be envisaged without introducing membrane-linked and extracellular cofactors.
齐夫综合征(ZS)由短暂性溶血性贫血、黄疸、高脂蛋白血症和酒精性肝病组成,我们对男性患者在急性期(n = 20)和缓解期(n = 10)进行了研究。无溶血的慢性酒精中毒患者(n = 10)和健康男性(n = 10)作为对照。通过密度梯度离心将红细胞分为老细胞和年轻细胞。含有较老红细胞的那些组分显示丙酮酸激酶不稳定,导致代谢受损。在ZS急性期患者中还检测到膜脂质组成的变化,表现为胆固醇和多不饱和脂肪酸(PUFA)增加。酒精诱导的红细胞维生素E缺乏伴PUFA水平降低可能会引发还原型红细胞谷胱甘肽的氧化,进而导致酶不稳定。这项研究进一步支持了这样一种假设,即如果不引入膜相关和细胞外辅助因子,就无法设想红细胞代谢损伤在ZS溶血起源中的假定作用。