scholarly journals Oxidative stress and phosphatidylserine exposure in red cells from patients with sickle cell anaemia

2018 ◽  
Vol 182 (4) ◽  
pp. 567-578 ◽  
Author(s):  
Anke Hannemann ◽  
David C. Rees ◽  
John N. Brewin ◽  
Andreas Noe ◽  
Ben Low ◽  
...  
Keyword(s):  
Oxidative Stress ◽  
Sickle Cell ◽  
Sickle Cell Anaemia ◽  
Red Cells ◽  
Phosphatidylserine Exposure

scholarly journals The Effect of a Selective Inhibitor of Phosphodiesterase-9 on Oxidative Stress, Inflammation and Cytotoxicity in Neutrophils from Patients with Sickle Cell Anaemia

2015 ◽  
Vol 118 (4) ◽  
pp. 271-278 ◽  
Author(s):  
Maritza Cavalcante Barbosa ◽  
Talyta Ellen de Jesus dos Santos ◽  
Thayna Nogueira dos Santos ◽  
Alano Martins Pedrosa ◽  
Darcielle Bruna Dias Elias ◽  
...  
Keyword(s):  
Oxidative Stress ◽  
Sickle Cell ◽  
Sickle Cell Anaemia ◽  
Selective Inhibitor

scholarly journals Relationship of oxidative stress and antioxidant response with vaso-occlusive crisis in sickle cell anaemia

2021 ◽  
Vol 21 (1) ◽  
pp. 150-8
Author(s):  
Godwill Azeh Engwa ◽  
Amanda Okolie ◽  
John Paul Chinedu Chidili ◽  
Precious Amara Okore ◽  
Paul Chigozie Onu ◽  
...  
Keyword(s):  
Oxidative Stress ◽  
Sickle Cell ◽  
Sickle Cell Anaemia ◽  
Antioxidant Response ◽  
Cross Sectional ◽  
Baseline Information ◽  
Nigeria Teaching Hospital ◽  
Relationship Of ◽  
Cell Carriers ◽  
The Relationship

Background: Though sickle cell anaemia (SCA) is known to promote oxidative stress, there is paucity of information on the relationship between oxidative stress and vaso-occlusive crisis (VOC). Objective: This study was undertaken to evaluate the relationship of oxidative stress and antioxidant response with VOC in SCA. Methods: A cross-sectional case-control study was carried out at University of Nigeria Teaching Hospital (UNTH), Itu- ku-Ozalla, Enugu Nigeria involving 116 individuals which included 36 SCA subject, 40 sickle cell carriers (AS) and 40 healthy individuals (AA). Baseline information as well as the frequency of VOC was obtained from the participants and anaemia as well as oxidative stress and antioxidant indices were assessed in blood. Results: Anaemia was prevalent (88.9 %) in SCA individuals compared to AS (52.5%) and AA (47.5 %) individuals. Nitric oxide scavenging (NOS) and superoxide dismutase (SOD) activities as well as glutathione level were significantly (p<0.005) lower while catalase activity was higher in SCA individuals compared to controls (AA and AS). Higher malondialdehyde (MDA) level was associated with very severe VOC while low level of NOS activity was associated with severe VOC in SCA individuals. Conclusion: Sickle cell anaemia exhibited oxidative stress and alteration in the levels of antioxidant indices which was pos- sibly associated with vaso-occlusive crisis. Keywords: Sickle cell anaemia; oxidative stress; antioxidant; vaso-occlusive crisis.

scholarly journals Role of Emergency Automated Red Cell Exchange in Sickle Cell Crisis: A Case Report

2020 ◽  
Vol 13 ◽  
pp. 117954762097020
Author(s):  
Anubhav Gupta ◽  
Kiran Chaudhary ◽  
Rajnish Kaushik
Keyword(s):  
Sickle Cell ◽  
Sickle Cell Anaemia ◽  
Symptomatic Improvement ◽  
Red Cells ◽  
Acute Chest Syndrome ◽  
Compound Heterozygous ◽  
Red Cell ◽  
Transfusion Therapy ◽  
Sickle Cell Crisis ◽  
Patient At Risk

For many years main stay of treatment for sickle cell anaemia was transfusion therapy. But repeated transfusions put the patient at risk of iron overload. Automated red cell exchange is an evolving and newer technique which rapidly removes the sickle cells and has benefit in decreasing sickle cell load and related complications. Red cell exchange is a therapeutic procedure in which the patient’s whole blood is processed centrifugally in cell separator. Patient’s red cells are separated from other blood components and removed and replaced with donor red cells and colloids. We report our first experience of automated red cell exchange in 24-year-old female diagnosed case of sickle cell anaemia presented to us with acute chest syndrome with septic shock. Red cell exchange was planned to tide over the acute sickle cell crisis and provide symptomatic improvement. We also highlight that compound heterozygous thalassaemia could be associated with sickle cell disease which could make the diagnosis difficult. New generation automated Apheresis equipment’s provides better monitoring of the procedure that can be useful in severely ill patients also.

scholarly journals Yoda1 and phosphatidylserine exposure in red cells from patients with sickle cell anaemia

2020 ◽  
Vol 10 (1) ◽  
Author(s):  
R. Wadud ◽  
A. Hannemann ◽  
D. C. Rees ◽  
J. N. Brewin ◽  
J. S. Gibson
Keyword(s):  
Sickle Cell ◽  
Sickle Cell Anaemia ◽  
Red Cells ◽  
Mechanosensitive Channel ◽  
Sickle Cells ◽  
Molecular Identity ◽  
Calphostin C ◽  
Intracellular Ca ◽  
Pkc Inhibitors ◽  
Low Levels

AbstractPhosphatidylserine (PS) exposure is increased in red cells from sickle cell anaemia (SCA) patients. Externalised PS is prothrombotic and attractive to phagocytes and activated endothelial cells and thus contributes to the anaemic and ischaemic complications of SCA. The mechanism of PS exposure remains uncertain but it can follow increased intracellular Ca2+ concentration ([Ca2+]i). Normally, [Ca2+]i is maintained at very low levels but in sickle cells, Ca2+ permeability is increased, especially following deoxygenation and sickling, mediated by a pathway sometimes called Psickle. The molecular identity of Psickle is also unclear but recent work has implicated the mechanosensitive channel, PIEZO1. We used Yoda1, an PIEZO1 agonist, to investigate its role in sickle cells. Yoda1 caused an increase in [Ca2+]i and PS exposure, which was inhibited by its antagonist Dooku1 and the PIEZO1 inhibitor GsMTx4, consistent with functional PIEZO1. However, PS exposure did not necessitate an increase in [Ca2+]i. Two PKC inhibitors were also tested, chelerytherine chloride and calphostin C. Both reduced PS exposure whilst chelerytherine chloride also reduced Yoda1-induced increases in [Ca2+]i. Findings are therefore consistent with the presence of PIEZO1 in sickle cells, able to mediate Ca2+ entry but that PKC was also involved in both Ca2+ entry and PS exposure.

scholarly journals Oxidative stress markers and disease severity among children with Sickle Cell Anaemia

2019 ◽  
pp. 193-202
Author(s):  
OS Smith ◽  
SA Adegoke ◽  
MA Akinlosotu ◽  
OA Ajose
Keyword(s):  
Oxidative Stress ◽  
Disease Severity ◽  
Sickle Cell ◽  
Sickle Cell Anaemia ◽  
Stress Index ◽  
Oxidative Stress Markers ◽  
Mild Disease ◽  
Stress Markers ◽  
Moderate Disease ◽  
The Mean

Background: Sickle cell anaemia has been associated with oxidative stress. Total Antioxidant Capacity (TAC), Total Oxidant Status (TOS) and Oxidative Stress Index (OSI) are cumulative markers of oxidative stress. Objective: To evaluate the serum levels of oxidative stress markers in children with sickle cell anaemia (SCA) and determine the relationship between these markers and disease severity. Method: One hundred and fifty-six children, comprising 78 with SCA, aged 1 - 15 years and 78 age- and sex-matched Haemoglobin AA controls were studied. Serum TOS, OSI, and TAC were determined using ELISA kits. The severity of the SCA was determined using clinical and laboratory parameters. Result: Children with SCA had lower mean serum TAC (0.83±0.31UAE) than controls (1.19±0.24UAE) with p<0.001. However, the mean serum TOS and OSI of children with SCA was higher than among the controls (13.33±4.64U/ml vs. 9.70±2.72U/ml and 20.95±16.75 vs. 8.68±3.76 respectively) with p<0.001. SCA subjects with mild disease had higher mean serum TAC (0.91 ± 0.27UAE) than those with moderate disease (0.54±0.27UAE) (p<0.001). On the other hand, the mean TOS and OSI were lower in children with mild disease compared to those with moderate disease (12.64±4.32U/ml vs. 15.63±5.07U/ml, p = 0.016 and 16.26±10.25 vs. 36.61±23.89 p<0.001 respectively). Sickle cell disease severity score had negative correlation with TAC (r = -0.60, p < 0.001) but positive correlation with TOS (r = 0.3, p = 0.008) and OSI (r = 0.6, p < 0.001). Conclusion: Children with SCA had lower TAC but higher TOS and OSI than matched controls. Oxidative stress markers had a significant relationship with SCD severity.

scholarly journals Moderate endurance exercise in patients with sickle cell anaemia: effects on oxidative stress and endothelial activation

2013 ◽  
Vol 164 (1) ◽  
pp. 124-130 ◽  
Author(s):  
Camille Faes ◽  
Edwige Balayssac-Siransy ◽  
Philippe Connes ◽  
Ludovic Hivert ◽  
Clotaire Danho ◽  
...  
Keyword(s):  
Oxidative Stress ◽  
Sickle Cell ◽  
Endurance Exercise ◽  
Sickle Cell Anaemia ◽  
Endothelial Activation

scholarly journals The role of WNK in modulation of KCl cotransport activity in red cells from normal individuals and patients with sickle cell anaemia

2019 ◽  
Vol 471 (11-12) ◽  
pp. 1539-1549
Author(s):  
David C.-Y. Lu ◽  
Anke Hannemann ◽  
Rasiqh Wadud ◽  
David C. Rees ◽  
John N. Brewin ◽  
...  
Keyword(s):  
Oxygen Tension ◽  
Sickle Cell ◽  
Sickle Cell Anaemia ◽  
Kinase Inhibitors ◽  
Red Cells ◽  
Red Cell ◽  
Sickle Cells ◽  
Kcl Cotransport ◽  
Pre Treatment

AbstractAbnormal activity of red cell KCl cotransport (KCC) is involved in pathogenesis of sickle cell anaemia (SCA). KCC-mediated solute loss causes shrinkage, concentrates HbS, and promotes HbS polymerisation. Red cell KCC also responds to various stimuli including pH, volume, urea, and oxygen tension, and regulation involves protein phosphorylation. The main aim of this study was to investigate the role of the WNK/SPAK/OSR1 pathway in sickle cells. The pan WNK inhibitor WNK463 stimulated KCC with an EC50 of 10.9 ± 1.1 nM and 7.9 ± 1.2 nM in sickle and normal red cells, respectively. SPAK/OSR1 inhibitors had little effect. The action of WNK463 was not additive with other kinase inhibitors (staurosporine and N-ethylmaleimide). Its effects were largely abrogated by pre-treatment with the phosphatase inhibitor calyculin A. WNK463 also reduced the effects of physiological KCC stimuli (pH, volume, urea) and abolished any response of KCC to changes in oxygen tension. Finally, although protein kinases have been implicated in regulation of phosphatidylserine exposure, WNK463 had no effect. Findings indicate a predominant role for WNKs in control of KCC in sickle cells but an apparent absence of downstream involvement of SPAK/OSR1. A more complete understanding of the mechanisms will inform pathogenesis whilst manipulation of WNK activity represents a potential therapeutic approach.

Reduction in Band 3 Protein of Red Cells in Sickle Cell Anaemia

1991 ◽  
Vol 96 (1) ◽  
pp. 23-33 ◽  
Author(s):  
Abdelrahim Osman Mohamed ◽  
Gunnar Ronquist
Keyword(s):  
Sickle Cell ◽  
Sickle Cell Anaemia ◽  
Band 3 ◽  
Red Cells ◽  
Band 3 Protein
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