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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

Haploidentical bone marrow transplant with post-transplant cyclophosphamide plus thiotepa improves donor engraftment in patients with sickle cell anemia: results of an international learning collaborative
Sickle cell disease (SCD) has evolved from a life-threatening condition in early childhood, to a chronic disease in adults [1,2]. Improved supportive care [3], use of hydroxyurea therapy [4], and regular blood transfusion therapy for primary [5] and secondary [6] stroke prevention, have resulted in improved childhood clinical outcomes and survival. Approximately 99% of all children born in high resource countries with SCD are expected to live to adulthood [7,8]. The most feared complication in children with SCD is stroke [9].
Source: Biology of Blood and Marrow Transplantation - November 27, 2018 Category: Hematology Authors: Josu de la Fuente, Nathalie Dhedin, Tatsuki Koyama, Francoise Bernaudin, Mathieu Kuentz, Leena Karnik, G érard Socié, Kathryn A. Culos, Robert A. Brodsky, Michael R. DeBaun, Adetola A. Kassim Source Type: research

Haploidentical Bone Marrow Transplantation with Post-Transplantation Cyclophosphamide Plus Thiotepa Improves Donor Engraftment in Patients with Sickle Cell Anemia: Results of an International Learning Collaborative
Sickle cell disease (SCD) has evolved from a life-threatening condition in early childhood to a chronic disease in adults [1,2]. Improved supportive care [3], use of hydroxyurea therapy [4], and regular blood transfusion therapy for primary [5] and secondary [6] stroke prevention have resulted in improved childhood clinical outcomes and survival. Approximately 99% of all children born with SCD are expected to live to adulthood [7,8]. The most feared complication in children with SCD is stroke [9].
Source: Biology of Blood and Marrow Transplantation - November 27, 2018 Category: Hematology Authors: Josu de la Fuente, Nathalie Dhedin, Tatsuki Koyama, Francoise Bernaudin, Mathieu Kuentz, Leena Karnik, G érard Socié, Kathryn A. Culos, Robert A. Brodsky, Michael R. DeBaun, Adetola A. Kassim Source Type: research

Blood Transfusion Frequency and Indications in Yemeni Children with Sickle Cell Disease.
Conclusion: Intermittent blood transfusion remains a common practice for the management of children with acute SCD complications. Main indications were acute anemic crises, severe pain crises, ACS, and stroke. In limited resource settings, such as Yemen, conservative transfusion policy appears to be appropriate. PMID: 32908695 [PubMed]
Source: Anemia - September 13, 2020 Category: Hematology Tags: Anemia Source Type: research

Challenges in transfusion of patients with sickle cell disease
SCD is an inherited disorder of hemoglobin with the highest incidence in people of African ancestry. Worldwide, 275.000 infants with this disease are born each year. Complications are caused by chronic hemolytic anemia and vascular occlusion. Patients suffer from recurrent painful vaso-occlusive crises and develop irreversible damage of the brain, lungs, heart and other organs. They receive frequent blood transfusions, both for acute indications and in monthly schedules, for the prevention of symptomatic stroke or acute chest syndrome.
Source: Transfusion and Apheresis Science - March 15, 2013 Category: Hematology Authors: Karin Fijn van Draat Tags: Guest Editorial Source Type: research

The role of blood transfusion in Sickle Cell Disease
Blood transfusion is used in the treatment and prevention of acute and chronic complications of Sickle Cell Disease. Blood may be administered in the emergency situation as a simple top‐up transfusion or as an automated or manual exchange procedure. Elective transfusions may be offered as an one‐off procedure, often pre‐operatively or as a long‐term regime, usually for primary or secondary stroke prevention. The main complications of transfusion in this patient population are alloimmunization, hyperhaemolytic transfusion reactions and iron overload.
Source: ISBT Science Series - May 31, 2013 Category: Hematology Authors: J. Howard Tags: Supplement Article Source Type: research

Cerebral Artery Velocity Determined by Transcranial Doppler Ultrasonography in Patients With {beta}-Thalassemia Intermedia Compared to {beta}-Thalassemia Major
We aimed to compare cerebral artery velocity and relevant clinical factors in patients with β-thalassemia intermedia (β-TI), β-thalassemia major (β-TM), and healthy individuals. For this study, 60 patients with β-TM and 64 with β-TI were randomly selected. Sixty healthy adults comprised the control group. Time average maximum mean velocity (TAMMV) was measured in large cerebral arteries with transcranial Doppler (TCD) ultrasonography. In all arteries, TAMMV was significantly higher in the β-TI group than that of β-TM or controls (P < .001). Patients with β-TM had significantl...
Source: Clinical and Applied Thrombosis/Hemostasis - July 3, 2013 Category: Hematology Authors: Karimi, M., Haghpanah, S., Ashjazadeh, N., Shariat, A., Nazeri, M., Emami, S., Forough, A. A., Petramfar, P., Yaghoubi, E. Tags: Original Articles Source Type: research

Life-threatening epistaxis and red blood cell polyagglutination under dabigatran
Dabigatran is an oral thrombin inhibitor which has been approved for prevention of stroke or embolism in atrial fibrillation patients as an alternative to vitamin K antagonists. Dabigatran has been introduced into clinical practice, although issues like laboratory monitoring, its use in elderly patients, drug and food interactions, and an antidote have not been completely clarified. Severe epistaxis leading to haemorrhagic shock occurred in an 89-year-old woman with atrial fibrillation and moderate renal insufficiency after 10 months of dabigatran 110 mg/b.i.d. Correction of the anaemia with blood transfusions became dif...
Source: Blood Coagulation and Fibrinolysis - May 1, 2014 Category: Hematology Tags: Case Reports Source Type: research

The Effects of Lysine Analogs During Pelvic Surgery: A Systematic Review and Meta-Analysis
The objective of this study was to assess the safety and efficacy of lysine analogs during pelvic surgery. A systematic literature search was performed using Medline, Cochrane Register of Clinical Trials, Embase, and the reference lists of relevant articles. Randomized controlled trials or observational cohort studies comparing a lysine analog to placebo or standard care were included. Outcomes collected were blood transfusion, blood loss, thromboembolic adverse events (myocardial infarction, stroke, deep vein thrombosis, and pulmonary embolism), nonthromboembolic adverse events, and death. There were no language limitatio...
Source: Transfusion Medicine Reviews - May 20, 2014 Category: Hematology Authors: Rodney H. Breau, Madzouka B. Kokolo, Nahid Punjani, Ilias Cagiannos, Andrew Beck, Naomi Niznick, Chieny Buenaventura, Juthaporn Cowan, Gregory Knoll, Franco Momoli, Chris Morash, Marcel Ruzicka, Svetlana Schachkina, Alan Tinmouth, Hai Yan Xie, Dean A. Fer Tags: Original Articles Source Type: research

Hypertransfusion therapy in sickle cell disease in Nigeria.
Conclusion. Improved knowledge of the benefits and practice of hypertransfusion will effectively translate into improved health status even among Nigerian sickle cell disease patients. PMID: 25177350 [PubMed]
Source: Advances in Hematology - November 16, 2014 Category: Hematology Tags: Adv Hematol Source Type: research

How I treat and manage strokes in sickle cell disease
Neurologic complications are a major cause of morbidity and mortality in sickle cell disease (SCD). In children with sickle cell anemia, routine use of transcranial Doppler screening, coupled with regular blood transfusion therapy, has decreased the prevalence of overt stroke from ~11% to 1%. Limited evidence is available to guide acute and chronic management of individuals with SCD and strokes. Current management strategies are based primarily on single arm clinical trials and observational studies, coupled with principles of neurology and hematology. Initial management of a focal neurologic deficit includes evaluation by...
Source: Blood - May 28, 2015 Category: Hematology Authors: Kassim, A. A., Galadanci, N. A., Pruthi, S., DeBaun, M. R. Tags: Sickle Cell Disease, How I Treat, Free Research Articles, Red Cells, Iron, and Erythropoiesis, Clinical Trials and Observations Source Type: research

Liver iron concentration measurements by MRI in chronically transfused children with sickle cell anemia: Baseline results from the TWiTCH trial
This article is protected by copyright. All rights reserved.
Source: American Journal of Hematology - June 19, 2015 Category: Hematology Authors: John C. Wood, Sara Pressel, Zora R. Rogers, Isaac Odame, Janet L. Kwiatkowski, Margaret T. Lee, William C. Owen, Alan R. Cohen, Timothy St. Pierre, Matthew M. Heeney, William H. Schultz, Barry R. Davis, Russell E. Ware, Tags: Research Article Source Type: research

Blood transfusion and 30-day readmission rate in adult patients hospitalized with sickle cell disease crisis.
CONCLUSION: Our findings point to blood transfusion as a potential means to reduce the 30-day readmission rate among Medicaid patients hospitalized with sickle cell crisis. There is a need for a prospective study to examine the potential benefit and safety of simple blood transfusion for this purpose. PMID: 26126756 [PubMed - as supplied by publisher]
Source: Transfusion - June 30, 2015 Category: Hematology Authors: Nouraie M, Gordeuk VR Tags: Transfusion Source Type: research

The role of the carotid doppler examination in the evaluation of atherosclerotic changes in beta thalassemia patients
Conclusion:  Carotid  Doppler is very useful method in measurement of cIMT that increased in thalassemic patients which  shows a strong relationship with features of iron overload. We recommend the routine use of Doppler measurement of cIMT in these patients to predict early atherosclerotic changes as well as in the follow-up to prevent progression of atherosclerosis. 
Source: Mediterranean Journal of Hematology and Infectious Diseases - February 16, 2015 Category: Hematology Source Type: research

Outcome of Jehovah's Witnesses after adult cardiac surgery: systematic review and meta-analysis of comparative studies.
CONCLUSION: JWs undergoing adult cardiac surgery have a nonsignificant trend toward better early outcome than controls receiving or not blood transfusions. The suboptimal quality of available studies prevents conclusive results on the possible benefits of a transfusion-free strategy in patients not refusing blood transfusion. PMID: 27197962 [PubMed - as supplied by publisher]
Source: Transfusion - May 19, 2016 Category: Hematology Authors: Vasques F, Kinnunen EM, Pol M, Mariscalco G, Onorati F, Biancari F Tags: Transfusion Source Type: research

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