title: Ezetimibe reduces plant sterol accumulation and favorably increases platelet count in sitosterolemia.
abstract: OBJECTIVE
  To assess if ezetimibe (EZE), a sterol-absorption inhibitor, improves platelet (PLT) count and size relative to its effect on plasma plant sterol (PS) in patients with sitosterolemia (STSL).
  
  
  METHODS
  Patients with STSL (5 males, 3 females, 16-56 years of age) receiving EZE intervention as part of their routine care participated in this study. EZE was discontinued for 14 weeks (off) and then resumed for another 14 weeks (on). Hematology variables along with plasma and red blood cells (RBC) PS and total cholesterol (TC) levels were measured at the end of each phase.
  
  
  RESULTS
  EZE increased PLT count (23% ± 9%) and decreased mean PLT volume (MPV; 10% ± 3%, all P < .05). In patients off EZE, PLT counts inversely correlated (r = -0.96 and r = -0.91, all P < .01) with plasma and RBC PS to TC ratio (PS/TC), and MPV positively correlated (r = 0.91, P = .03 and r = 0.93, P = .02) with plasma and RBC PS/TC. EZE reduced plasma and RBC sitosterol (-35% ± 4% and -28% ± 3%), total PS (-37% ± 4% and -28% ± 3%, all P < .0001) levels, and PS/TC (-27% ± 4% and -28% ± 4%, P < .01).
  
  
  CONCLUSIONS
  EZE reduces plasma and RBC PS levels, while increasing PLT count and decreasing MPV, and thereby may reduce the risk for bleeding in STSL. Plasma PS levels and ABCG5/ABCG8 genes should be analyzed in patients with unexplained hematologic abnormalities.
authors.affiliation: Human Nutritional Sciences, University of Manitoba, Winnipeg, Manitoba, Canada; Richardson Center for Functional Foods and Nutraceuticals, University of Manitoba, Winnipeg, Manitoba, Canada.
authors.forename: Rgia A
authors.initials: RA
authors.lastname: Othman
authors.affiliation: Human Nutritional Sciences, University of Manitoba, Winnipeg, Manitoba, Canada; Richardson Center for Functional Foods and Nutraceuticals, University of Manitoba, Winnipeg, Manitoba, Canada.
authors.forename: Semone B
authors.initials: SB
authors.lastname: Myrie
authors.affiliation: Internal Medicine, University of Manitoba, Winnipeg, Manitoba, Canada.
authors.forename: David
authors.initials: D
authors.lastname: Mymin
authors.affiliation: Pediatrics, Institute on Development and Disability/Doernbecher Children's Hospital, Oregon Health and Science University, Portland, OR.
authors.forename: Louise S
authors.initials: LS
authors.lastname: Merkens
authors.affiliation: Pediatrics, Institute on Development and Disability/Doernbecher Children's Hospital, Oregon Health and Science University, Portland, OR.
authors.forename: Jean-Baptiste
authors.initials: JB
authors.lastname: Roullet
authors.affiliation: Pediatrics, Institute on Development and Disability/Doernbecher Children's Hospital, Oregon Health and Science University, Portland, OR; Molecular and Medical Genetics, Institute on Development and Disability/Doernbecher Children's Hospital, Oregon Health and Science University, Portland, OR; Marshfield Clinic Research Foundation, Marshfield, WI and the University of Wisconsin, Madison, WI.
authors.forename: Robert D
authors.initials: RD
authors.lastname: Steiner
authors.affiliation: Human Nutritional Sciences, University of Manitoba, Winnipeg, Manitoba, Canada; Richardson Center for Functional Foods and Nutraceuticals, University of Manitoba, Winnipeg, Manitoba, Canada. Electronic address: peter_jones@umanitoba.ca.
authors.forename: Peter J H
authors.initials: PJ
authors.lastname: Jones
mesh_terms: D000293:Adolescent; D000328:Adult; D000924:Anticholesteremic Agents / Q000008:administration & dosage / Q000627:therapeutic use*; D001384:Azetidines / Q000008:administration & dosage / Q000627:therapeutic use*; D002170:Canada; D002784:Cholesterol / Q000097:blood*; D004906:Erythrocyte Count; D000069438:Ezetimibe; D005260:Female; D006801:Humans; D006937:Hypercholesterolemia / Q000097:blood / Q000188:drug therapy*; D007410:Intestinal Diseases / Q000097:blood / Q000188:drug therapy*; D008052:Lipid Metabolism, Inborn Errors / Q000097:blood / Q000188:drug therapy*; D008297:Male; D008875:Middle Aged; D010840:Phytosterols / Q000009:adverse effects* / Q000097:blood*; D010865:Pilot Projects; D010976:Platelet Count; D016896:Treatment Outcome; D014481:United States; D055815:Young Adult
chemical_list: D000924:Anticholesteremic Agents; D001384:Azetidines; D010840:Phytosterols; D002784:Cholesterol; D000069438:Ezetimibe