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• W2018173037 abstract "Background Familial homozygous hypercholesterolemia is an inherited disease which without therapy, leads to a lethal outcome before the age of 20 due to accelerated atherosclerosis. Therapy is either “gene therapy”, liver transplantation, or chronic low density lipoprotein (LDL) apheresis. The aim of the study is to asses the efficacy of LDL apheresis in two patients with familial homozygous hypercholesterolemia, who have been treated with chronic LDL apheresis in our Dialysis center for more than 10 years. Methods A girl, born in 1977 entered a chronic LDL apheresis program in 1990, aged 13 years. Apheresis sessions have been carried out every two to three weeks. Vascular access was via cubital peripheral vein, and the anticoagulant used was heparin. LDL apheresis monitor was Kaneka, Japan, with dextran sulphate column. Three volumes of plasma (each 8000 mL) were processed per session, which lasted for about 4 hours. A boy, born in 1988 entered an LDL apheresis programe in 1994, aged 6 years. Vascular access was at first via double lumen hemodialysis catheter, but at age 8, AV fistula was created because of serious access problems. LDL apheresis was performed as membrane plasma exchange up to the age of 10. Since then a monitor Kaneka with dextran sulphate column was used. LDL apheresis was performed every 2 weeks, 3 volumes of plasma being processed, each 6000 mL. Serum concentration of total cholesterol, LDL, high density lipoprotein (HDL), and triglycerides were monitored at the start and end of the LDL apheresis. Results The girl has been treated for 14 years. In total 302 LDL procedures have been performed. In the year 2004 average concentrations of total cholesterol at start (12.7 mmol/L) and at the end (2.1 mmol/L), as well as time average concentration (TAC; 7.0 mmol/L) were stable and comparable with concentrations in the past. The same was true for the average concentration of LDL cholesterol at the start (10.7 mmol/L), at the end (1.2 mmol/L), and for TAC (5.6 mmol/L). The boy has been treated for 10 years, a total of 135 LDL apheresis procedures being carried out. Average concentration (at start, after LDL apheresis, and TAC) of total cholesterol and LDL cholesterol were 11.0 mmol/L, 2.3 mmol/L, 6.6 mmol/L and 9.4 mmol/L, 1.3 mmol/L, 5.1 mmol/L, respectively. Conclusion Chronic LDL apheresis performed every 2–3 weeks provides stable metabolism of cholesterol and acceptable concentration of lipids. Up to now there has been no sign of accelerated atherosclerosis in the patients." @default.
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• W2018173037 date "2005-02-01" @default.
• W2018173037 modified "2023-10-17" @default.
• W2018173037 title "32 Chronic LDL Apheresis in Children and Young Adults" @default.
• W2018173037 doi "https://doi.org/10.1111/j.1526-0968.2005.222_32_32.x" @default.
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