The level of serum lipids, vitamin E and low density lipoprotein oxidationin Wilson's disease patients

The aim of this study was to estimate the level of lipids and of the main s erum antioxidant, alpha-tocopherol (vitamin E), and to evaluate the suscept ibility of low density lipoprotein (LDL) to oxidation in Wilson's disease p atients. It was assumed that enhanced LDL peroxidation caused by high coppe r levels could contribute to the injury of liver and other tissues. The gro up investigated comprised 45 individuals with Wilson's disease treated with penicillamine or zinc salts and a control group of 36 healthy individuals. Lipids were determined by enzymatic methods, alpha-tocopherol by high perf ormance liquid chromatography, the susceptibility of LDL to oxidation in vi tro by absorption changes at 234 nm during 5 h and end-products of LDL lipi d oxidation as thiobarbituric acid reacting substances. In Wilson's disease patients total cholesterol, LDL cholesterol and alpha-tocopherol levels we re significantly lower compared with the control group. No difference in LD L oxidation in vitro between the patients and the controls was stated. Conc lusion: enhanced susceptibility of isolated LDL for lipid peroxidation in v itro was not observed in Wilson's disease patients. One cannot exclude, how ever, that because of low alpha-tocopherol level lipid peroxidation in the tissues can play a role in the pathogenesis of tissue injury in this diseas e.