title: Supplementation of patients with homozygous sickle cell disease with zinc, alpha-tocopherol, vitamin C, soybean oil, and fish oil.
abstract: Thirteen patients (aged 0.7-17.9 y) with homozygous sickle cell disease were supplemented with alpha-tocopherol, vitamin C, zinc, and soybean oil (suppl 1; for 8 mo) and alpha-tocopherol, vitamin C, and fish oil (suppl 2; for 7 mo). Urinary zinc (suppl 1), plasma vitamin C, plasma cholesterol ester and erythrocyte (RBC) omega 3 fatty acids (suppl 2), and plasma and RBC alpha-tocopherol (suppl 1 and 2) increased. Suppl 1 decreased irreversibly sickled cells by 37.5%, decreased RBC protoporphyrin and urinary porphyrins, and increased the RBC total fatty acid-cholesterol ratio. Suppl 2 decreased plasma triglycerides, further increased the RBC alpha-tocopherol, moderately increased the RBC double-bond index, but decreased the RBC total fatty acid-cholesterol ratio. Zinc, copper, and porphyrins showed prolonged changes. The supplements did not change hemoglobin concentrations, RBC age (reticulocytes, polyamines), or number of aplastic and vasoocclusive crises. Zinc reduces irreversibly sickled cells. Augmentation of RBC antioxidant status by alpha-tocopherol and vitamin C and incorporation of omega 3 fatty acids into RBCs do not affect hemolytic component. Effects on vasoocclusive component are unclear.
authors.affiliation: Central Laboratory for Clinical Chemistry, University Hospital, Groningen, The Netherlands.
authors.forename: F A
authors.initials: FA
authors.lastname: Muskiet
authors.forename: F D
authors.initials: FD
authors.lastname: Muskiet
authors.forename: G
authors.initials: G
authors.lastname: Meiborg
authors.forename: J G
authors.initials: JG
authors.lastname: Schermer
mesh_terms: D000293:Adolescent; D000755:Anemia, Sickle Cell / Q000178:diet therapy* / Q000188:drug therapy; D001205:Ascorbic Acid / Q000097:blood / Q000627:therapeutic use*; D002648:Child; D002675:Child, Preschool; D002788:Cholesterol Esters / Q000097:blood; D004042:Dietary Fats, Unsaturated / Q000627:therapeutic use*; D004906:Erythrocyte Count; D004912:Erythrocytes / Q000737:chemistry; D005227:Fatty Acids / Q000097:blood; D005260:Female; D005319:Fetal Hemoglobin / Q000032:analysis; D005395:Fish Oils / Q000627:therapeutic use; D006451:Hemoglobin, Sickle / Q000032:analysis; D006801:Humans; D007223:Infant; D008297:Male; D013024:Soybean Oil / Q000627:therapeutic use; D014810:Vitamin E / Q000097:blood / Q000627:therapeutic use*; D015032:Zinc / Q000627:therapeutic use* / Q000652:urine
chemical_list: D002788:Cholesterol Esters; D004042:Dietary Fats, Unsaturated; D005227:Fatty Acids; D005395:Fish Oils; D006451:Hemoglobin, Sickle; D014810:Vitamin E; D013024:Soybean Oil; D005319:Fetal Hemoglobin; D015032:Zinc; D001205:Ascorbic Acid