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Reduced Cerebral Metabolic Rate of Oxygen in Adults with Sickle Cell Disease
Conclusion:We observed reduced CMRO2 in patients with SCD compared to healthy controls due to low OEF. A reduced CMRO2 could pose a risk for ischemia, despite high flow rate delivering oxygen, because of low OEF. This is supported by the fact that the silent cerebral infarcts are located in regions with the lowest CMRO2. We postulate that patients with SCD have a reduced capacity to increase the OEF in regions with inadequate CBF resulting in local ischemia and local infarction. The pathogenesis of the reduced OEF remains unclear but could be related to arteriovenous shunting whereby there is insufficient time for oxygen t...
Source: Blood - November 21, 2018 Category: Hematology Authors: Vaclavu, L., Petersen, E. T., VanBavel, E. T., Majoie, C. B., Nederveen, A. J., Biemond, B. J. Tags: 114. Hemoglobinopathies, Excluding Thalassemia-Clinical: Organ Damage and Clinical Complications in Sickle Cell Disease Source Type: research

Predictors of Adverse Outcome in Sickle Cell Disease Patients from Oman
Conclusion: Multi-organ failure was mostly associated with the terminal events relating to mortality. Although sepsis played a major role as a cause of death in association with multiorgan failure, low hemoglobin, and low platelet counts with increased WBC counts along with elevated LDH and CRP played a significant role in the terminal event in this cohort of SCD patients.DisclosuresNo relevant conflicts of interest to declare.
Source: Blood - November 21, 2018 Category: Hematology Authors: Alkindi, S., AlJadidi, S., Al Adawi, S., Pathare, A. Tags: 113. Hemoglobinopathies, Excluding Thalassemia-Basic and Translational Science Source Type: research

Comparison between Automated Erythrocytopharesis (AECP) and Manual Exchange Transfusion (M-Ex)) in Reducing Hb-S and in Recovery of Acute Chest Syndrome and Other Acute Presentations of Sickle Cell Disease Patients
Conclusions:Erythrocytopharesis (Automated RBC exchange) is effective, quick and safe procedure that is life saving for many patients with ACS and is associated with less difficulties and complications if compared with the manual exchange. Because SCA is a national problem in Saudi Arabia and acute chest syndrome and other acute major complications comprehensively kill SCD patients, Automated Erythocytopharesis should be available nation-wide like dialysis machines at all large hospitals in all cities and should be distributed according to the prevalence of SCA in the area or location.DisclosuresNo relevant conflicts of interest to declare.
Source: Blood - November 21, 2018 Category: Hematology Authors: Ahmed, S. Y., Saleh, S. M., Hameed, M. S., Ragheb, A. M., Abbas, T. M., Fadel, A., Hassan, S., Baden, H. S., Tayeb, K. I., Bakshi, N., El Yamany, G., Alotaibi, S. B. Tags: 114. Hemoglobinopathies, Excluding Thalassemia-Clinical Source Type: research

A Genetic Variant in GOLGB1 Affects Golgi Function and Stroke Risk in Patients with Sickle Cell Disease
Conclusions: Our study has shown that a coding variant in GOLGB1, identified as protective against risk of stroke in patients with SCD, has significant effects on Golgi function in SCD samples. We observed that having the GOLGB1 Y1212C variant resulted in more compact and less fragmented Golgi apparatus. Proteomic analysis showed that SCD patients with the GOLGB1 variant also had significantly lower levels of proteins involved in platelet activation and Golgi trafficking. Our findings suggest a novel role for the Golgi apparatus in controlling protein flux that modulates risk of stroke in SCD.DisclosuresNo relevant conflic...
Source: Blood - November 21, 2018 Category: Hematology Authors: Kotu, V., Dubrelle, J., Baker, J., Jenson, K., Flanagan, J. M. Tags: 113. Hemoglobinopathies, Excluding Thalassemia-Basic and Translational Science: Poster III Source Type: research

Elevated Levels of CD64 MFI on Monocyte Subsets Are Associated with a History of Stroke in Sickle Cell Disease
Conclusion: Levels of monocyte subsets have been shown to correlate with clinical outcomes in non-SCD stroke, but to our knowledge this is the first study to examine their roles in ischemic stroke in SCD. The pathophysiology of stroke in SCD is unique and the role of monocytes in it deserves separate study from the role of monocytes in non-SCD stroke. We saw that while monocyte subsets were associated with a history of ischemic stroke, CD64 MFI on all monocyte subsets showed a strong association. We wonder if this may be due to CD64 MFI being a marker for cells which are more adherent to endothelium as has been shown in pr...
Source: Blood - November 21, 2018 Category: Hematology Authors: Curtis, S. A., Balbuena-Merle, R., Devine, L., Zelterman, D., Roberts, J. D., Dearborn-Tomazos, J., Sansing, L., Hendrickson, J. E. Tags: 114. Hemoglobinopathies, Excluding Thalassemia-Clinical: Poster I Source Type: research

Comprehensive Sickle Cell Disease Management Is Associated with Superior Grade Attainment and High School Graduation Rates
Discussion: In this cohort, a high percentage of patients (86.7%) graduated or were on track to graduate from high school. In the United States, approximately 90% of patients with SCD are African American. All patients analyzed here were African American. With this in mind, ACHSCDP patients' graduation rate compares favorably to estimated graduation rates across the US general population (84%), the U.S. African America population (76%), the U.S. sickle cell patient population (71%), the Ohio general population (84%), and the Ohio African American population (68%).The higher graduation rate among children at the ACHSCDP is ...
Source: Blood - November 21, 2018 Category: Hematology Authors: Schulte, K., Mikofalvy, K., Beck, L., Bodas, P. V. Tags: 901. Health Services Research-Non-Malignant Conditions: Poster III Source Type: research