scholarly journals Vascular complications of sickle cell disease

2018 ◽  
Vol 68 (2-3) ◽  
pp. 205-221 ◽  
Author(s):  
Ashar Usmani ◽  
Roberto F. Machado
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Vascular Complications ◽  
Cell Disease

Growth differentiation factor-15 in young sickle cell disease patients: Relation to hemolysis, iron overload and vascular complications

2014 ◽  
Vol 53 (4) ◽  
pp. 189-193 ◽  
Author(s):  
Azza Abdel Gawad Tantawy ◽  
Amira Abdel Moneam Adly ◽  
Eman Abdel Rahman Ismail ◽  
Yasser Wagih Darwish ◽  
Marwa Ali Zedan
Keyword(s):  
Sickle Cell Disease ◽  
Iron Overload ◽  
Sickle Cell ◽  
Vascular Complications ◽  
Cell Disease ◽  
Growth Differentiation Factor 15 ◽  
Differentiation Factor

scholarly journals Priapism in Sickle Cell Anemia: Emerging Mechanistic Understanding and Better Preventative Strategies

Anemia ◽  
2011 ◽  
Vol 2011 ◽  
pp. 1-6 ◽  
Author(s):  
Genevieve M. Crane ◽  
Nelson E. Bennett
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Sickle Cell Anemia ◽  
Treatment Strategies ◽  
Vascular Complications ◽  
Cell Disease ◽  
Nitric Oxide Signaling ◽  
Risk Of Death ◽  
Ischemic Priapism

Sickle cell anemia is a common and disabling disorder profoundly affecting mortality as well as quality of life. Up to 35% of men with sickle cell disease are affected by painful, prolonged erections termed ischemic priapism. A priapic episode may result in fibrosis and permanent erectile dysfunction. The severity of sickle cell disease manifestations is variable dependent on a number of contributing genetic factors; however, priapism tends to cluster with other severe vascular complications including pulmonary hypertension, leg ulceration, and overall risk of death. The mechanisms underlying priapism in sickle cell disease have begun to be elucidated including hemolysis-mediated dysregulation of the nitric oxide signaling pathway and dysregulation of adenosine-mediated vasodilation. A better understanding of these mechanisms is leading toward novel preventative strategies. This paper will focus on the mechanisms underlying development of ischemic priapism in sickle cell disease, current acute and preventative treatment strategies, and future directions for improved management of this disorder.

Skin Blood Flow Measured By LSCI Demonstrates Treatment Effect of Chronic Transfusion Protocol in Sickle Cell Disease

Blood ◽  
2016 ◽  
Vol 128 (22) ◽  
pp. 4871-4871
Author(s):  
Nicolas V Currier ◽  
Gersham Dent ◽  
Paul S. Swerdlow ◽  
Willem Birkhoff ◽  
Jacobus Burggraaf ◽  
...  
Keyword(s):  
Blood Flow ◽  
Sickle Cell Disease ◽  
Healthy Volunteers ◽  
Sickle Cell ◽  
Skin Blood Flow ◽  
Vascular Complications ◽  
Microvascular Blood Flow ◽  
Cell Disease ◽  
Blood Flow Patterns ◽  
Transfusion Protocol

Abstract Background: Vascular complications such as stroke and pulmonary hypertension are central features of sickle cell disease (SCD) pathophysiology and are associated with early mortality among patients with SCD. Better understanding of the abnormal blood flow patterns in sickle cell disease is critical to assessing the therapeutic benefit of emerging therapies. Previous studies have shown abnormal blood flow patterns in sickle cell patients using laser speckle contrast imaging (LSCI) (Ikeda et al. poster 1080 ASH annual meeting, December 8, 2012), however, no test re-test variability or changes in response to therapeutic intervention were assessed. As part of a larger study using multiple imaging technologies to concurrently evaluate blood flow and oxygenation in several organs of healthy subjects and subjects with SCD, the present study has analyzed skin blood flow by LSCI in these patients longitudinally using a multiple visit protocol. Furthermore, we analyzed the effect of Chronic Transfusion Protocol, the most effective intervention against vascular complications on skin blood flow. Methods: We enrolled 9 SCD patients (age 30.2 ± 8.6 years 5 men / 4 women) and 4 age and ethnically matched healthy controls (age 24.5 ± 4.5 years, 2 men / 2 women). 5 of the 9 SCD patients were on Chronic Transfusion Protocol. Following a screening visit where patients consented for the study, had basic labs, 12-lead EKG and physical exam, cutaneous blood flow was directly measured using LCSI at baseline, during and after a standard brachial artery occlusion-reperfusion maneuver (inflation of an occlusive pneumatic cuff for 3-5 min depending on patient tolerance). For assessment of test, re-test reliability this visit protocol was repeated within 1-10 days for patients not on transfusion protocols and healthy volunteers. Three visits occurred for transfusion patients, the first 2-4 days prior to transfusion, the second 1-2 days post transfusion and the third 6-14 days after the first post-transfusion imaging visit. Results: Baseline microvascular blood flow measured by LSCI was greater in patients with sickle cell disease compared to healthy controls (47 + 11 vs. 27 + 3 arbitrary units (AU); p=0.002) with sickle cell patients on chronic transfusion protocols exhibiting an intermediate blood flow phenotype (32 + 7 AU p= 0.001 vs. non-transfused patients and p= 0.039 vs. HV). Surprisingly, transfusion had no impact on baseline blood flow both 1-2 days and 6-14 days following transfusions (34 + 9 AU pre-transfusion, 32 + 7 AU first post-transfusion, 33 + 6 AU second post-transfusion). Maximal microvascular blood flow was similar across all groups (86 + 14 AU sickle cell patients vs. 82 + 12 AU healthy volunteers vs. 75 + 15 AU sickle cell patients on chronic transfusion). All measured values exhibited significant longitudinal stability across visits with r2= 0.865 for baseline blood flow measurements for non-transfusion sickle cell patients and healthy volunteers between visit 1 and 2. For transfusion patients r2= 0.927 and r2= 0.866 between visits 1 and 2 and 1 and 3 respectively. No statistically significant differences were observed in our analysis of the time from half-maximum to maximum blood flow or time from maximum to half-maximum blood flow during the recovery period after occlusion across all subjects. Conclusion: Compared to healthy individuals, patients with SCD have greater baseline microvascular blood flow in skin and these numbers are stable over a multi-visit protocol. Furthermore, patients on chronic transfusion protocols exhibit lower, but not normal skin blood flow parameters. Surprisingly, these values are not impacted acutely by transfusion. These results not only further validate that LSCI could function as a non-invasive disease biomarker for vascular dysfunction in sickle cell disease but that it is sensitive enough to detect vascular changes that occur in response to effective therapy. Finally, these results suggest that the therapeutic changes in vascular function provided by Chronic Transfusion Protocols occur over the long term versus acutely. Disclosures Currier: Biogen: Employment, Other: shareholder. Dent:Biogen: Employment, Other: shareholder. Birkhoff:CHDR: Employment. Burggraaf:CHDR: Employment. de Vries:CHDR: Employment. Hobbs:Biogen: Employment, Equity Ownership, Other: shareholder. Verma:Biogen: Employment, Other: shareholder.

Endothelial dysfunction in patients with sickle cell disease is related to selective impairment of shear stress–mediated vasodilation

Blood ◽  
2001 ◽  
Vol 97 (6) ◽  
pp. 1584-1589 ◽  
Author(s):  
Laurent Belhassen ◽  
Gabriel Pelle ◽  
Saı̈d Sediame ◽  
Dora Bachir ◽  
Claudine Carville ◽  
...  
Keyword(s):  
Shear Stress ◽  
Steady State ◽  
Endothelial Dysfunction ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Vascular Complications ◽  
No Production ◽  
Cell Disease ◽  
Arterial Diameter ◽  
Oxygen Inhalation

Interactions between the endothelium and erythrocytes may contribute to the vascular complications of sickle cell disease (SCD). Endothelium-derived nitric oxide (NO) plays a major role in the regulation of vasomotor tone in response to wall shear stress (WSS) variations and pharmacologic stimuli. However, little is known about endothelial NO production in patients with steady-state SCD. We investigated endothelial NO production in response to flow or vasoactive agonists in 16 homozygous patients with steady-state SCD and 15 controls. Flow-mediated dilation (FMD), arterial diameter changes in response to 100% oxygen inhalation, blood viscosity, and calculated WSS were determined in all patients and controls. At baseline, WSS was higher in SCD patients than in controls, whereas arterial diameter was similar. In patients with SCD, FMD was impaired (1.73% ± 0.44% vs 3.97% ± 0.24% in the controls, P < .001) and vasoconstriction in response to 100% oxygen was abolished. Using venous occlusion plethysmography, forearm blood flow (FBF) was evaluated in response to acetylcholine, nitro-monomethyl-L-arginine (L-NMMA), and sodium nitroprusside (SNP) in subgroups of 9 controls and 7 patients with SCD. Acetylcholine induced a significantly greater FBF increase in the patients (9.7 ± 2.9 mL/min/100 mL of forearm volume vs 2.5 ± 1.5 mL/min/100 mL in the controls,P < .001), whereas responses to L-NMMA and SNP were similar. These results suggest that endothelial dysfunction may prevent the arterial diameter of patients with SCD from adapting to chronic or acute shear stress elevations. This may contribute to the pathophysiology of vaso-occlusive crisis in patients with SCD.

scholarly journals CO‑PRESENCE OF FACTOR V LEIDEN G1691A, MTHFR C677T AND XMN‑1 − 158 GΓ (C → T) IN SICKLE CELL PATIENTS

2016 ◽  
Vol 68 (1) ◽  
pp. 73
Author(s):  
Sanjay Pandey ◽  
Rahasya Mani Mishra ◽  
Sweta Pandey ◽  
Renu Saxena
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Sickle Cell Anemia ◽  
Vascular Complications ◽  
Factor V Leiden ◽  
Mthfr C677t ◽  
Factor V ◽  
Cell Disease ◽  
Platelet Activity ◽  
Coagulation Inhibitors

<p>Vascular complications are an important and perplexing aspect of the clinical spectrum of sickle cell anemia. Patients with sickle cell disease show activation of the blood coagulation, fibrinolytic systems, increased platelet activity and consumption of coagulation inhibitors during vaso‑occlusive crises.</p>

scholarly journals Spontaneous development of cerebrovascular pathology and microinfarcts in a mouse model of sickle cell disease

2017 ◽  
Author(s):  
Hyacinth I. Hyacinth ◽  
Cortney L. Sugihara ◽  
Thomas L. Spencer ◽  
David R. Archer ◽  
Andy Y. Shih
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Vascular Complications ◽  
Vascular Occlusion ◽  
Vessel Diameter ◽  
Cell Disease ◽  
Two Photon Microscopy ◽  
Two Photon ◽  
Vessel Tortuosity

ABSTRACTStroke is a dramatic complication of sickle cell disease (SCD) and is associated with aneurysms, moya moya, intravascular thrombi, cerebral hyperemia and increased vessel tortuosity. We show that aged SCD mice spontaneously develop the characteristics features of cerebral vasculopathy seen in human SCD. Thirteen month old Townes SCD mice and age-matched controls had a cranial windows implanted over the somatosensory cortex. Cortical capillaries were imaged using in vivo two-photon microscopy after the blood plasma was labeled with a fluorescent dye. Results showed that SCD mice compared to controls, had significantly higher red blood cell (RBC) velocity and capillary vessel diameter. SCD mice also had a significantly higher number of occlusive events in the capillary bed, resulting in more stalling of RBC flow. Microvascular topology was also altered, as SCD mice had significantly higher vessel tortuosity and shorter capillary branch lengths. Finally, post-mortem analyses revealed a greater number of cortical microinfarcts, likely caused by vascular occlusion since local tissue hypoxia and blood-brain barrier leakage was prominent. We concluded that aged Townes sickle cell mice spontaneously develop SCD-associated cerebral vasculopathy, and that in vivo two-photon imaging is a powerful approach to investigate the mechanisms of vascular complications in SCD.

MTHFRPolymorphic Variant C677T Is Associated to Vascular Complications in Sickle-Cell Disease

2012 ◽  
Vol 16 (9) ◽  
pp. 1038-1043 ◽  
Author(s):  
Betânia L. D. Hatzlhofer ◽  
Marcos André C. Bezerra ◽  
Magnun N.N. Santos ◽  
Dulcinéia M. Albuquerque ◽  
Elizabete M. Freitas ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Vascular Complications ◽  
Cell Disease

scholarly journals Elevated Proangiogenic Markers are Associated with Vascular Complications within Ghanaian Sickle Cell Disease Patients

2018 ◽  
Vol 6 (3) ◽  
pp. 53 ◽  
Author(s):  
Charles Antwi-Boasiako ◽  
Emmanuel Frimpong ◽  
Ben Gyan ◽  
Eric Kyei-Baafour ◽  
Fredericka Sey ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Vascular Complications ◽  
Cell Disease
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