Lipids, fatty acids and trace elements in plasma and erythrocytes of pediatric patients with homozygous sickle cell disease.
Plasma and erythrocyte levels of zinc, copper, fatty acids, total cholesterol and cholesterol sulfate, and plasma vitamin E and free cholesterol were measured in six pediatric patients with HbSS sickle cell anemia, one adult patient in crisis and six age-, sex- and race-matched pediatric controls. Patient plasma zinc levels were significantly decreased, while erythrocyte zinc levels were normal. Although subject to a large range, plasma vitamin E levels were not significantly altered. For patients an increased plasma percentage of free cholesterol, increased levels of oleic acid and vaccenic acid, and decreased values for total cholesterol were found. Erythrocyte fatty acid analyses revealed a significant decrease in total polyunsaturated fatty acids which was 'compensated' for by increased total monounsaturated and saturated fatty acids. The low amounts of total polyunsaturated fatty acids could completely be ascribed to decreased levels of linoleic acid. Erythrocyte cholesterol levels were significantly increased, while the total fatty acid/cholesterol molar ratio was found to be subject to a relatively large range. Cholesterol sulfate determinations showed that patients had relatively low plasma or erythrocyte levels, or both. The present results are suggestive of a lipid peroxidation mediated, and hepatic and/or splenic dysfunction mediated lipid component in the rigidification of the sickle cell membrane.
D000293:Adolescent; D000328:Adult; D000755:Anemia, Sickle Cell / Q000097:blood* / Q000235:genetics; D002648:Child; D002675:Child, Preschool; D002784:Cholesterol / Q000097:blood; D002788:Cholesterol Esters / Q000097:blood; D003300:Copper / Q000097:blood*; D004912:Erythrocytes / Q000032:analysis*; D005227:Fatty Acids / Q000097:blood*; D005230:Fatty Acids, Nonesterified / Q000097:blood; D005260:Female; D006720:Homozygote; D006801:Humans; D007223:Infant; D008055:Lipids / Q000097:blood*; D008297:Male; D014810:Vitamin E / Q000097:blood; D015032:Zinc / Q000097:blood*
D002788:Cholesterol Esters; D005227:Fatty Acids; D005230:Fatty Acids, Nonesterified; D008055:Lipids; D014810:Vitamin E; D003300:Copper; D002784:Cholesterol; D015032:Zinc; C007045:cholesteryl sulfate