title: Treatment of homozygous familial hypercholesterolemia with low density lipoprotein apheresis: a 4 year follow-up study.
abstract: Hypercholesterolemia and elevated lipoprotein (a) (Lp[a]) levels are considered to be risk factors for the development and progression of premature atherosclerosis. The purpose of our report is to describe the effects of low density lipoprotein (LDL) apheresis (Liposorber system, Kanegafuchi Chemical Industrial Company LTD, Osaka, Japan) on serum lipoprotein concentrations and the clinical status in 2 male patients with homozygous familial hypercholesterolemia. Compared with pretreatment values, the posttreatment concentrations of total cholesterol, LDL cholesterol, and Lp(a) were significantly reduced by 50-60% (p < 0.0001). The concentration of high density lipoprotein (HDL) cholesterol was slightly affected. After one treatment session, LDL cholesterol and Lp(a) were decreased on average by 65% and then increased to reach about 70-75% of the pretreatment values before the next session. Prior to the treatment with LDL apheresis, each patient had suffered one myocardial infarction and had had 2 coronary angiographies. After treatment with LDL apheresis, neither cardiac complaints nor myocardial infarction were observed. The xanthomas were much decreased during the treatment or disappeared. We conclude that LDL apheresis can be continued safely and without major technical problems for several years. Apheresis effectively lowers the serum levels of total and LDL cholesterol. Furthermore, it reduces Lp(a), which is not influenced by lipid-lowering drugs. The reduction of LDL cholesterol and Lp(a) may delay the progression of the atherosclerotic process, thereby helping to reduce the risk of new episodes of coronary heart disease and thus extending the life expectancy in these patients.
authors.affiliation: Department of Internal Medicine, University Hospital, Uppsala, Sweden.
authors.forename: J E
authors.initials: JE
authors.lastname: Fadul
authors.forename: B
authors.initials: B
authors.lastname: Vessby
authors.forename: B
authors.initials: B
authors.lastname: Wikström
authors.forename: B G
authors.initials: BG
authors.lastname: Danielson
mesh_terms: D000328:Adult; D001781:Blood Component Removal; D002784:Cholesterol / Q000097:blood; D003327:Coronary Disease / Q000150:complications; D016264:Dextran Sulfate / Q000378:metabolism; D005500:Follow-Up Studies; D006720:Homozygote; D006801:Humans; D006938:Hyperlipoproteinemia Type II / Q000150:complications / Q000628:therapy*; D008017:Life Expectancy; D008077:Lipoproteins, LDL / Q000097:blood* / Q000302:isolation & purification; D008137:Longitudinal Studies; D008297:Male; D011788:Quality of Life; D012307:Risk Factors; D016896:Treatment Outcome; D014280:Triglycerides / Q000097:blood
chemical_list: D008077:Lipoproteins, LDL; D014280:Triglycerides; D016264:Dextran Sulfate; D002784:Cholesterol