Pilot assessment of omega-3 fatty acids and potassium thiocyanate in sickle cell anemia patients with conditional peak systolic cerebral artery blood velocity

Ugwu, Angela Ogechukwu; Iloanusi, Nneka; Ugwu, Ngozi Immaculata; Chukwu, BF; Ezenwosu, Osita U; Modebe, Emmanuel; Duru, Augustine Nwakuche; Madu, Anazoeze Jude; Chibueze, Ezinne C; Igboke, Michael; Ejiofor, Obiora Shedrack; Efobi, Chilota Chibuife; E, Uchendu; Okpala, Iheanyi

doi:10.1016/j.bcmd.2021.102564

Cited by 3 publications

(10 citation statements)

References 22 publications

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“…The age range for all participants was 2–16 years, the mean age was 8.86 years and the SD 3.98 years. Since two types of TCD machines are used in Nigeria,10–12 PSV and TAMMV were measured in separate cohorts used to make the findings clinically relevant and useful to hospitals with either PSV or TAMMV equipment. The TAMMV cohort had 115 children (50 girls, 65 boys) with an age range of 3–16 years, mean±SD 8.78±3.37 years.…”

Section: Resultsmentioning

confidence: 99%

“…The main outcome measure was the proportion of children with SCD who have normal, high or conditional PSV or TAMMV in the cerebral or internal carotid arteries. Although it was not possible to calculate the minimum sample size required for this study because the prevalence in Nigeria of cerebral artery conditional blood velocity in childhood SCD is not certain, a previous study in this country recruited 232 children 12. Guided by the previous report, the minimum sample size for this study was increased to 300.…”

Section: Methodsmentioning

confidence: 99%

“…Conditional TAMMV in the ICA/MCA is associated with a stroke risk of 3%–4% per year in childhood SCD 4 9. Some TCD machines determine peak systolic blood velocity (PSV) in the cerebral arteries 10–12. The normal PSV is <200 cm/s, high >250 cm/s and conditional 200–249 cm/s.…”

Section: Introductionmentioning

confidence: 99%

“…The standard of care for high blood velocity in the ICA/MCA is regular blood transfusion to prevent stroke,1–9 but active intervention is not generally considered the standard treatment for conditional blood flow velocity. Conditional blood flow velocity does progress in a significant proportion of children to abnormal values that are associated with increased risk of ischaemic stroke 11 12. In a previous evaluation of the benefits of active intervention with 3 months of multimodal therapy comprising omega-3 fatty acids and potassium thiocyanate, progression of conditional blood velocity to abnormal values occurred in none (0%) of treated children relative to 14% of the control group on standard care of SCD only, and PSV reduced to normal values in 79% of the treatment compared with 43% of the control group 12.…”

Section: Introductionmentioning

confidence: 99%

“…Conditional blood flow velocity does progress in a significant proportion of children to abnormal values that are associated with increased risk of ischaemic stroke 11 12. In a previous evaluation of the benefits of active intervention with 3 months of multimodal therapy comprising omega-3 fatty acids and potassium thiocyanate, progression of conditional blood velocity to abnormal values occurred in none (0%) of treated children relative to 14% of the control group on standard care of SCD only, and PSV reduced to normal values in 79% of the treatment compared with 43% of the control group 12. Two other studies showed reduction of conditional blood velocity to normal values following hydroxycarbamide therapy 13 14.…”

Section: Introductionmentioning

confidence: 99%

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Cerebral artery conditional blood velocity in sickle cell disease: a multicentre study and evidence for active treatment

et al. 2023

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ObjectiveTo obtain multicentre data on the prevalence of normal, high or conditional (intermediate) blood velocity in the cerebral arteries among children with sickle cell disease (SCD) in Nigeria.DesignA prospective observational study in five tertiary healthcare institutions. By transcranial Doppler (TCD) ultrasonography, cerebral artery peak systolic blood velocity (PSV) was determined in 193 children with SCD and time averaged mean of the maximum blood velocity (TAMMV) in a different cohort of 115 children. This design was to make the findings relevant to hospitals with TCD equipment that measure either PSV or TAMMV.SettingNigeria.Participants308 children (126 girls, 182 boys; age 2–16 years).Main outcome measuresPercentage of children with SCD who have normal, high or intermediate (often termed conditional) PSV or TAMMV.ResultsIn the cohort of 193 children, PSV was normal in 150 (77.7%), high in 7 (3.6%) and conditional in 36 (18.7%). In the cohort of 115 children, TAMMV was normal in 96 (84%), high in 7 (6%) and conditional in 12 (10%). There were no significant differences in gender or age distribution between the PSV and TAMMV cohorts. Altogether, cerebral artery blood velocity was normal in 246/308 children (80%), high in 14 (4.5%) and conditional in 48 (15.5%).ConclusionSince conditional blood velocity in cerebral arteries can progress to high values and predispose to stroke, the proportion of children with SCD who are affected (15.5%) raises the question of whether regular monitoring and proactive intervention ought to be the standard of care.

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Section: Resultsmentioning

confidence: 99%

Section: Methodsmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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Cerebral artery conditional blood velocity in sickle cell disease: a multicentre study and evidence for active treatment

et al. 2023

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Epeleuton, a novel synthetic ω-3 fatty acid, reduces the hypoxia/reperfusion stress in a mouse model of sickle cell disease

Mattè,

Federti,

Recchiuti

et al. 2023

haematol

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Inflammatory vasculopathy is critical in sickle cell disease (SCD)-associated organ damage. An imbalance between pro-inflammatory and pro-resolving mechanisms in response to different triggers such as hypoxia/reoxygenation or infections has been proposed to contribute to SCD disease progression. Administration of specialized pro-resolving lipid mediators may provide an effective therapeutic strategy to target inflammatory vasculopathy and to modulate inflammatory response. Epeleuton (15 hydroxy eicosapentaenoic acid ethyl ester) is a novel orally administered second-generation ω-3 fatty acid with a favorable clinical safety profile. In this study we show that epeleuton re-programs the lipidomic pattern of target organs for SCD towards a pro-resolving pattern. This protects against systemic and local inflammatory response and improves red cell features, resulting in reduced hemolysis and sickling compared with vehicle treated SCD mice. In addition, epeleuton prevents the hypoxia/reoxygenation induced activation of NF-kB with downregulation of NLRP3 inflammasome in lung, kidney, and liver. This was associated with down-regulation of vascular activation markers in epeleuton treated SCD mice when compared to vehicle treated animals. Collectively our data support the potential therapeutic utility of epeleuton and provide the rationale for the design of clinical trials to evaluate the efficacy of epeleuton in patients with SCD.

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Palatability and Acceptability of Flaxseed-Supplemented Foods in Children with Sickle Cell Disease

et al. 2023

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Omega-3 fatty acids (w3FAs) have demonstrated benefits in several inflammatory disease states; however, limited research has been conducted in sickle cell disease (SCD). While marine-based w3FAs are used, their strong odor and taste are a barrier to long-term use. Plant-based sources, especially those in whole foods, may circumvent this barrier. We tested whether flaxseed (rich source of w3FAs) was acceptable to children with SCD. A cross-sectional tasting trial of flaxseed added to baked products (cookies, pancakes, brownies) or to readily available foods (applesauce, pudding, yogurt) was conducted among 30 children (median age = 13 years) reporting to a clinic for routine follow-up, sick visits, or transfusion for SCD to determine acceptability. A food preference rank scale (1–7) was used to rank products based on taste, sight, smell, and texture. An average score for each product was computed. Children were also asked to rank their top three products. The top-ranked products were flaxseed baked in brownies and cookies and ground flaxseed added to yogurt. More than 80% of participants indicated willingness to be contacted for a follow-up study in which a flaxseed-supplemented diet would be evaluated for mitigation of SCD-associated pain. In conclusion, flaxseed-enriched products are palatable and acceptable in children with SCD.

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Pilot assessment of omega-3 fatty acids and potassium thiocyanate in sickle cell anemia patients with conditional peak systolic cerebral artery blood velocity

Cited by 3 publications

References 22 publications

Cerebral artery conditional blood velocity in sickle cell disease: a multicentre study and evidence for active treatment

Cerebral artery conditional blood velocity in sickle cell disease: a multicentre study and evidence for active treatment

Epeleuton, a novel synthetic ω-3 fatty acid, reduces the hypoxia/reperfusion stress in a mouse model of sickle cell disease

Palatability and Acceptability of Flaxseed-Supplemented Foods in Children with Sickle Cell Disease

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