Daak, Ahmed A. and Ghebremeskel, Kebreab (2015) Blood Cell Membrane Omega-3 (n-3) Fatty Acid Abnormality and Supplementation in Patients with Sickle Cell Anemia. In: Handbook of Lipids in Human Function: Fatty Acids. Elsevier Publishing Company, Oxford, pp. 711-730. ISBN 9781630670368
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Sickle cell disease (SCD) is a group of autosomal recessive genetic blood disorders caused by a mutation in the sixth codon of the β goblin gene that results in abnormal hemoglobin (Hemoglobin S, HbS) (Knight-Perry et al., 2009; Rees et al., 2010; Serjeant and Serjeant, 2001). The principal phenotypes are homozygous sickle cell (HbSS) disease, sickle cell-hemoglobin C , sickle cell-β0 thalassemia, sickle cell-β1 thalassemia, HbSOArab and HbSDPunjab and HbSLepore Boston SCD (Nagel et al., 2003; Serjeant and Serjeant, 2001). Deoxygenated HbS forms insoluble rigid polymers (sickle) under hypoxic conditions and reverts back to normal on re-oxygenation. However, with repeated cycles of sickling and unsickling, erythrocytes become irreversibly sickled and lose their biconcave shape and fluidity.
The primary pathological process in SCD, namely vasoocclusive crisis is a recurrent occlusion of blood vessels which causes ischemia, severe pain episodes (painful crisis), and damage to the brain, eyes, lungs, spleen, liver, and other vital organs (Ballas et al., 2010; Serjeant and Serjeant, 2001). Despite the apparent genetic simplicity, patients with SCD display a remarkable diversity in clinical manifestations and disease severity (Chui and Dover, 2001; Fertrin and Costa, 2010). The Cooperative Study of Sickle Cell Disease (Platt et al., 1991) found that 39% of 3578 patients with SCD did not have painful episodes, whereas 1% had more than six per year. It appears that type and severity of the complication of the disease are modulated by genetic, environmental, and other factors (Sebastiani et al., 2005; Steinberg, 2005).
|Item Type:||Book Section|
|Uncontrolled Keywords:||Sickle cell disease; SCD; abnormal hemoglobin; Hemoglobin S, HbS; blood cell membrane Omega-3 (n-3); fatty acids|
|Subjects:||600 Technology > 610 Medicine & health|
|Department:||School of Human Sciences|
|Depositing User:||Kebreab Ghebremeskel|
|Date Deposited:||14 Jan 2016 17:53|
|Last Modified:||14 Jan 2016 17:53|
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