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1.
Article in English | MEDLINE | ID: mdl-31186149

ABSTRACT

BACKGROUND: Inflammation and vaso-occlusion play key roles in Sickle Cell Disease (SCD) pathophysiology. Lipoxygenase products of the omega-3 fatty acids (O3FAs), docosahexaenoic (DHA) and eicosapentaenoic (EPA) acids, are potent anti-inflammatory mediators modulating pain. O3FAs decrease episodes of vaso-occlusion in SCD. METHODS: We assessed erythrocyte fatty acid composition in two major cell membrane phospholipids, phosphatidylcholine and phosphatidylethanolamine, in children with SCD HbSS-disease (n = 38) and age/race-matched HbAA-controls (n = 18). Ratio of pro-inflammatory arachidonic acid (AA) to anti-inflammatory DHA and EPA (FA-Ratio), and its relationship to hs-CRP were evaluated. RESULTS: FA-Ratios were increased in both phosphatidylcholine and phosphatidylethanolamine in HbSS compared to controls. Correlations were noted in HbSS subjects between hs-CRP and FA-Ratios (p = 0.011). FA-Ratios increased with age (p = 0.0007) due to an increase in pro-inflammatory AA with a concomitant decrease in anti-inflammatory DHA. CONCLUSIONS: Findings demonstrate relative deficiencies in HbSS of the anti-inflammatory precursor fatty acids DHA and EPA, which correlates positively with hs-CRP.


Subject(s)
Anemia, Sickle Cell/blood , Biomarkers/blood , C-Reactive Protein/metabolism , Docosahexaenoic Acids/blood , Eicosapentaenoic Acid/blood , Fatty Acids, Omega-3/blood , Inflammation/blood , Adolescent , Anemia, Sickle Cell/diagnosis , Arachidonic Acid/blood , Child , Child, Preschool , Erythrocytes/metabolism , Humans , Phosphatidylcholines/blood , Phosphatidylethanolamines/blood , Risk Factors
2.
Pediatr Hematol Oncol ; 26(8): 589-96, 2009 Nov.
Article in English | MEDLINE | ID: mdl-19954369

ABSTRACT

BACKGROUND: SCD is characterized by hemolysis and oxidative stress, resulting in endothelial dysfunction (EDF). Peripheral arterial tonometry (PAT), a noninvasive technology for measuring EDF, utilizes reactive hyperemia following mini-ischemic stress (reactive hyperemia index or RHI). METHODS: The authors studied PAT in 36 SCD children to determine the influence of hemoglobin genotype and treatment on EDF. RESULTS AND CONCLUSIONS: Blunted RHI was seen in the majority of children with SCD, especially with increased symptomatology (1.53 and 1.71; p value .032). RHI was not normal in children on chronic transfusion or hydroxyurea. RHI correlated with reticulocyte fraction (Spearman r = -.47, p = .037). PAT merits further exploration as a measure of EDF in SCD.


Subject(s)
Anemia, Sickle Cell/diagnosis , Endothelium, Vascular/physiopathology , Hemoglobins/genetics , Manometry/methods , Peripheral Vascular Diseases/diagnosis , Adolescent , Anemia, Sickle Cell/physiopathology , Arteries/physiopathology , Child , Genotype , Humans , Hyperemia , Oxidative Stress , Peripheral Vascular Diseases/physiopathology , Reticulocytes , Young Adult
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