title: Plasma kinetics of a cholesterol-rich microemulsion in subjects with heterozygous beta-thalassemia.
abstract: Patients with beta-thalassemia trait have been reported to present lower plasma concentrations of low-density lipoprotein (LDL) and lower frequencies of acute myocardial infarction than normal subjects. In this study, the metabolism of LDL was tested in 12 patients with heterozygous beta-thalassemia trait (HBT) and 13 healthy subjects without the disease by determining the plasma kinetics of an artificially made cholesterol-rich microemulsion (LDE) that mimics the LDL metabolism and binds to LDL receptors. The emulsion was labeled with 14C-cholesterol ester and injected intravenously into the subjects. Blood samples were drawn at regular intervals over 24 hr to determine the plasma decay curve of the emulsion radioactive label and to estimate its plasma fractional clearance rate (FCR, in hr(-1)). FCR of the 14C-cholesterol ester was greater in HBT compared to controls (0.0631 +/- 0.0178 hr(-1) and 0.0501 +/- 0.0094 hr(-1), respectively; mean +/- SD, P = 0.022). No differences were found regarding LDL cholesterol plasma concentration between the two groups, but apolipoprotein B concentration was lower in HBT than in control subjects (80 +/- 44 and 96 +/- 14, respectively; mean +/- SD, P = 0.026). Our results show that LDE FCR is increased in HBT, indicating that LDL clearance is increased in patients with beta-thalassemia trait possibly due to the increased proliferation in the bone marrow of erythroid precursors.
authors.affiliation: Hematology Service, University of São Paulo Medical School, São Paulo, Brazil.
authors.forename: Flávio A
authors.initials: FA
authors.lastname: Naoum
authors.forename: Sandra F M
authors.initials: SF
authors.lastname: Gualandro
authors.forename: Maria da Conceição M
authors.initials: Mda C
authors.lastname: Latrilha
authors.forename: Raul C
authors.initials: RC
authors.lastname: Maranhão
mesh_terms: D000328:Adult; D000368:Aged; D002788:Cholesterol Esters / Q000008:administration & dosage / Q000493:pharmacokinetics*; D008078:Cholesterol, LDL / Q000378:metabolism; D005217:Fat Emulsions, Intravenous / Q000008:administration & dosage / Q000493:pharmacokinetics*; D005260:Female; D006579:Heterozygote; D006801:Humans; D008297:Male; D008657:Metabolic Clearance Rate; D008875:Middle Aged; D008954:Models, Biological; D011973:Receptors, LDL / Q000378:metabolism; D017086:beta-Thalassemia / Q000097:blood* / Q000235:genetics / Q000378:metabolism
chemical_list: D002788:Cholesterol Esters; D008078:Cholesterol, LDL; D005217:Fat Emulsions, Intravenous; D011973:Receptors, LDL