title: Impaired efficacy of selective LDL-apheresis in primary biliary cirrhosis.
abstract: Low-density lipoprotein apheresis (LDL-apheresis) was done with either cascade filtration (DF) or dextran sulfate cellulose adsorption (DSC) in a patient with primary biliary cirrhosis who developed severe dyslipidemia associated with cholestasis and accumulation of lipoprotein-X (LP-X). The extracorporeal treatment was initially performed weekly, and resulted in a sharp drop in total cholesterol from 1038 to 430 mg/dl. During the next four months the patient was treated every 10-15 days, and pre-apheresis cholesterol levels were maintained between 438 and 505 mg/dl, until an orthotopic liver transplantation was successfully performed. With semi-selective DF a mean 47.1% of total cholesterol was removed per procedure compared to 30.0% with DSC, although the volume of treated plasma was 38.0 vs 49.9 ml/kg body weight. The changes in plasma cholesterol levels during DSC and DF showed that the kinetics of cholesterol removal were similar with both techniques, but the efficacy differed; DF removed both LDL and LP-X from plasma, whereas DSC selectively lowered the LDL content. Cascade filtration may therefore be considered as a first-choice treatment for patients with LP-X accumulation due to cholestasis.
authors.affiliation: Department of Nephrology, Niguarda Ca Granda Hospital, Milano, Italy.
authors.forename: G
authors.initials: G
authors.lastname: Busnach
authors.forename: G
authors.initials: G
authors.lastname: Franceschini
authors.forename: G
authors.initials: G
authors.lastname: Chiesa
authors.forename: B
authors.initials: B
authors.lastname: Brando
authors.forename: A
authors.initials: A
authors.lastname: Cappelleri
authors.forename: L
authors.initials: L
authors.lastname: Isa
authors.forename: L
authors.initials: L
authors.lastname: Minetti
mesh_terms: D000328:Adult; D001781:Blood Component Removal; D002784:Cholesterol / Q000097:blood; D016264:Dextran Sulfate; D005374:Filtration / Q000295:instrumentation; D006801:Humans; D008077:Lipoproteins, LDL / Q000097:blood*; D008105:Liver Cirrhosis, Biliary / Q000628:therapy*; D008297:Male
chemical_list: D008077:Lipoproteins, LDL; D016264:Dextran Sulfate; D002784:Cholesterol