Abstract
One hundred and eighteen (118) subjects aged 15 to 36 years divided into control subjects (AA n = 42), heterozygous sickle cell patients (AS n = 33) and homozygous sickle cell patients (SS n = 43) were investigated for a lipid profile including the measurement of oxidized low density lipoprotein (LDL) to assess the risk of early atherosclerosis in sickle cell disease. The results show that total, high density lipoprotein (HDL) and LDL plasma cholesterol levels are significantly lower in the sickle cell patients than in control group (p < 0.05). In contrast, the triglyceride levels, the ratio of triglycerides to HDL-cholesterol and the oxidized LDL fraction are higher in patients (p < 0.05). These lipid abnormalities could represent a cardiovascular risk for sickle cell disease patients. Key words: Atherosclerosis, dyslipidemia, oxidized low density lipoprotein (LDL), sickle cell disease.
Highlights
Sickle cell disease (SCD) is a genetic disorder caused by a single substitution (GTG for GAG) at the beta globin gene on chromosome 11
This inherited affection called Sickle cell anemia (SCA) is a condition characterized by defect in plasma and erythrocyte lipids associated with a chronic oxidative stress (Oztaz et al, 2011)
They promote a defect in lipid metabolism and abnormalities in lipids homeostasis by compromising the Lecithin cholesterol acyltransferase and the paraoxonase activity (Bielicki and Forte, 1999; Aviram et al, 1999). These two enzymes have been shown to play a key role in esterification of cholesterol on high density lipoprotein (Santamarina-Fojo et al, 2000) and in the lipid oxidation (Hine et al, 2012). Consistent with these data involved in atherogenesis, the aim of this study is to evaluate plasma lipids and oxidized low density lipoprotein (LDL) concentrations in SCA patients in order to verify the presence of an atherogenic lipid profile
Summary
Sickle cell disease (SCD) is a genetic disorder caused by a single substitution (GTG for GAG) at the beta globin gene on chromosome 11. This gene defect codes for the sickle beta-hemoglobin characterized by the substitution of valine for glutamic acid at the sixth position of the betachain (Pauling et al, 1949; Ingram, 1957) This inherited affection called Sickle cell anemia (SCA) is a condition characterized by defect in plasma and erythrocyte lipids associated with a chronic oxidative stress (Oztaz et al, 2011). The generated hydroperoxides and oxidized low density lipoproteins (oxLDL) induce an atherogenic process and serial deleterious effects (Peluso et al, 2012; Itabe, 2009) They promote a defect in lipid metabolism and abnormalities in lipids homeostasis by compromising the Lecithin cholesterol acyltransferase and the paraoxonase activity (Bielicki and Forte, 1999; Aviram et al, 1999). These two enzymes have been shown to play a key role in esterification of cholesterol on high density lipoprotein (Santamarina-Fojo et al, 2000) and in the lipid oxidation (Hine et al, 2012)
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