title: Early-onset plasmapheresis and LDL-apheresis provide better disease control for pediatric homozygous familial hypercholesterolemia than HMG-CoA reductase inhibitors and ameliorate atherosclerosis.
abstract: Plasmapheresis (PA) and low-density lipoprotein apheresis (LDL-A) were assessed in five children with homozygous familial hypercholesterolemia (HFH) previously receiving statins. LDL-A required smaller extracorporeal blood volumes. Mean HDL-cholesterol reduction post-procedure was 32% and 60% with LDL-A and PA, respectively. Non-HDL-C reduction was 64% and 69%, respectively. Pre-procedural LDL-C decreased significantly with weekly versus biweekly LDL-A. Carotid intimal media thickness (IMT) studies demonstrated disappearance of atheromatous lesions and normal ITM in 4/5 patients. Echocardiography revealed normal aortic valves, coronary orifices and supra-valvular area in all patients. Apheresis is effective in pediatric HFH. It may be started in patients weighing 14 kg.
authors.affiliation: Blood Bank and Apheresis Unit, Rambam Health Care Campus, Haifa, Israel; Bruce Rappaport Faculty of Medicine, Technion, Israel Institute of Technology, Haifa, Israel. Electronic address: e_dann@rambam.health.gov.il.
authors.forename: Eldad J
authors.initials: EJ
authors.lastname: Dann
authors.forename: Raanan
authors.initials: R
authors.lastname: Shamir
authors.forename: Tatiana
authors.initials: T
authors.lastname: Mashiach
authors.forename: Ron
authors.initials: R
authors.lastname: Shaoul
authors.forename: Alla
authors.initials: A
authors.lastname: Badian
authors.forename: Tselestina
authors.initials: T
authors.lastname: Stravets
authors.forename: Yelena
authors.initials: Y
authors.lastname: Kerzman
authors.forename: Svetlana
authors.initials: S
authors.lastname: Finkelbaum
authors.forename: Diana
authors.initials: D
authors.lastname: Gaitini
authors.forename: Avraham
authors.initials: A
authors.lastname: Lorber
authors.forename: Lilach
authors.initials: L
authors.lastname: Bonstein
mesh_terms: D050197:Atherosclerosis / Q000097:blood / Q000503:physiopathology / Q000517:prevention & control*; D059168:Carotid Intima-Media Thickness; D002648:Child; D002675:Child, Preschool; D008076:Cholesterol, HDL / Q000097:blood; D005260:Female; D006801:Humans; D019161:Hydroxymethylglutaryl-CoA Reductase Inhibitors / Q000627:therapeutic use; D006938:Hyperlipoproteinemia Type II / Q000097:blood / Q000150:complications / Q000503:physiopathology / Q000628:therapy*; D008077:Lipoproteins, LDL; D008297:Male; D010956:Plasmapheresis
chemical_list: D008076:Cholesterol, HDL; D019161:Hydroxymethylglutaryl-CoA Reductase Inhibitors; D008077:Lipoproteins, LDL
keywords: Children; Familial hypercholesterolemia; LDL-apheresis; Plasmapheresis