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Total 308 results found since Jan 2013.

Red cell exchange for patients with sickle cell disease: an international survey of current practices.
CONCLUSIONS: This survey solicited the current practice variations in RCE from a diverse range of practice sites. Many sites reported similar practice patterns and challenges but some variations emerged. To our knowledge, this survey represents the largest and most in-depth investigation of the use of RCE for patients with SCD, and could inform future studies in the field. PMID: 32583456 [PubMed - as supplied by publisher]
Source: Transfusion - June 23, 2020 Category: Hematology Authors: Karafin MS, Hendrickson JE, Kim HC, Kuliya-Gwarzo A, Pagano MB, Perumbeti A, Shi PA, Tanhehco YC, Webb J, Wong E, Eichbaum Q Tags: Transfusion Source Type: research

Transfusion therapy for sickle cell disease
The clinical presentations of sickle cell disease include acute pain, acute chest syndrome, stroke and acute anaemia and blood transfusion therapy can be used effectively in the emergency setting for the treatment of many of these complications. Long‐term blood transfusion therapy is also used for the prevention of disease complications with most evidence for its use in primary stroke prevention. Transfusion can be given as a simple or top‐up transfusion or as an exchange transfusion and donor red cells should be haemoglobin S negative and as a minimum matched for ABO, full Rh and Kell type. Repeated transfusions lead ...
Source: ISBT Science Series - January 29, 2016 Category: Hematology Authors: J. Howard, S. E. Robinson Tags: Invited Review Source Type: research

Blood transfusion services for patients with sickle cell disease in Nigeria
Conclusion Current efforts of Nigerian public hospitals to provide safe blood and CTT fall short of best practice. Provision of apheresis machines, improvement of voluntary non-remunerated donor drive, screening for red cell antigens and antibodies, and availability of iron chelators would significantly improve SCD care in Nigeria.
Source: International Health - September 27, 2016 Category: Global & Universal Authors: Diaku-Akinwumi, I. N., Abubakar, S. B., Adegoke, S. A., Adeleke, S., Adewoye, O., Adeyemo, T., Akinbami, A., Akinola, N. O., Akinsulie, A., Akinyoola, A., Aneke, J., Awwalu, S., Babadoko, A., Brown, B., Ejike, O., Emodi, I., George, I., Girei, A., Hassan, Tags: Original Article Source Type: research

Increased complications of chronic erythrocytapheresis compared with manual exchange transfusions in children and adolescents with sickle cell disease
ConclusionaRBCX may have increased complication risks compared with MET for chronic transfusion therapy in SCD. Risks and benefits of aRBCX and MET should be considered when selecting a chronic transfusion modality. Transfusion therapy modalities should be compared in prospective studies for stroke prevention in children with SCD.
Source: Pediatric Blood and Cancer - May 19, 2017 Category: Cancer & Oncology Authors: Deborah Woods, Robert J. Hayashi, Michael M. Binkley, Gianna W. Sparks, Monica L. Hulbert Tags: RESEARCH ARTICLE Source Type: research

Cerebral vasculopathy in children with sickle cell disease: Key issues and the latest data.
Abstract Cerebral vasculopathy is a common and severe complication of sickle cell disease in children. The pathophysiology consists of progressive damage to the basal intracranial arteries and cerebral microcirculation, while chronic anemia worsens exposure to cerebral hypoxia. It results in stroke and subclinical or poorly symptomatic ischemic lesions. Many clinical, biological, and radiological risk factors have been identified. The prevention strategy through systematic transcranial Doppler screening of large-vessel vasculopathy has revolutionized the management of this disease and has greatly decreased the ris...
Source: Archives de Pediatrie - December 15, 2017 Category: Pediatrics Authors: Corvest V, Blais S, Dahmani B, De Tersant M, Etienney AC, Maroni A, Ormières C, Roussel A, Pondarré C Tags: Arch Pediatr 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

Hydroxyurea reduces cerebral metabolic stress in patients with sickle cell anemia
Chronic transfusion therapy (CTT) prevents stroke in selected patients with sickle cell anemia (SCA). We have shown that CTT mitigates signatures of cerebral metabolic stress, reflected by elevated oxygen extraction fraction (OEF), which likely drives stroke risk reduction. The region of highest OEF falls within the border zone, where cerebral blood flow (CBF) nadirs; OEF in this region was reduced after CTT. The neuroprotective efficacy of hydroxyurea (HU) remains unclear. To test our hypothesis that patients receiving HU therapy have lower cerebral metabolic stress compared with patients not receiving disease-modifying t...
Source: Blood - May 29, 2019 Category: Hematology Authors: Fields, M. E., Guilliams, K. P., Ragan, D., Binkley, M. M., Mirro, A., Fellah, S., Hulbert, M. L., Blinder, M., Eldeniz, C., Vo, K., Shimony, J. S., Chen, Y., McKinstry, R. C., An, H., Lee, J.-M., Ford, A. L. Tags: Sickle Cell Disease, Red Cells, Iron, and Erythropoiesis 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

107. Ultrasound investigations in sickle cell anemia. Initial assessment and follow up
This study was proposed to compare two different equipment: TCD (trans-cranial doppler) and TCCS (transcranial color-coded sonography) with or without using the angular correction, in a group of patients with the sickle cell anemia in order to identify the most suitable technique for the sampling of blood velocity (TAMM). 35 pediatric patients without previus history of neurological disease or transfusion were enrolled. Arteries examined were the middle cerebral artery, the anterior cerebral artery, the posterior cerebral and the basilar arteries. TAMM values greater than 200cm/s identify patients at high risk of stroke wh...
Source: Clinical Neurophysiology - October 21, 2013 Category: Neuroscience Authors: V. Marcon, G. Meneghetti, P. Rampazzo, M. Ermani Tags: Society Proceedings 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

Immunophenotypic Parameters and RBC Alloimmunization in Children with Sickle Cell Disease on Chronic Transfusion
This article is protected by copyright. All rights reserved.
Source: American Journal of Hematology - September 1, 2015 Category: Hematology Authors: Robert S. Nickel, John T. Horan, Ross M. Fasano, Erin Meyer, Cassandra D. Josephson, Anne M. Winkler, Marianne E. M. Yee, Leslie S. Kean, Jeanne E. Hendrickson Tags: Research Article Source Type: research

Organ iron accumulation in chronically transfused children with sickle cell anaemia: baseline results from the TWiTCH trial
We report the baseline findings of abdominal organ iron burden in 121 participants. At enrollment, patients were young (9·8 ± 2·9 years), predominantly female (60:40), and previously treated with transfusions (4·1 ± 2·4 years) and iron chelation (3·1 ± 2·1 years). Liver iron concentration (LIC; 9·0 ± 6·6 mg/g dry weight) and serum ferritin were moderately elevated (2696 ± 1678 μg/l), but transferrin was incompletely saturated (47·2 ± 23·6%). Spleen R2* was 509 ± 399 Hz (splenic iron ~13·9 mg/g) and correlated with LIC (r2 = 0·14, P = 0·0008). Pancreas R2* was increased in 38·3...
Source: British Journal of Haematology - November 2, 2015 Category: Hematology Authors: John C. Wood, Alan R. Cohen, Sara L. Pressel, Banu Aygun, Hamayun Imran, Lori Luchtman‐Jones, Alexis A. Thompson, Beng Fuh, William H. Schultz, Barry R. Davis, Russell E. Ware, Tags: Research Paper Source Type: research

Management of Sickle Cell Disease: Recommendations from the 2014 Expert Panel Report.
Abstract Family physicians are the primary and sometimes only health care resource for families affected by sickle cell disease. Recently published guidelines provide important recommendations for health maintenance, acute care, and monitoring of disease-modifying therapy in persons with this condition. This overview highlights some of the most important clinical activities that can and should be carried out in the community care setting. Children with sickle cell anemia should receive prophylactic penicillin from birth through at least five years of age, and all persons with sickle cell disease require vaccinatio...
Source: American Family Physician - December 15, 2015 Category: Primary Care Authors: Yawn BP, John-Sowah J Tags: Am Fam Physician Source Type: research