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Pain and other non‐neurological adverse events in children with sickle cell anemia and previous stroke who received hydroxyurea and phlebotomy or chronic transfusions and chelation: Results from the SWiTCH clinical trial
Conclusions: In children with SCA and prior stroke, monthly transfusions and daily iron chelation provided superior protection against acute vaso‐occlusive pain SAEs when compared to hydroxyurea and monthly phlebotomy.
Source: American Journal of Hematology - July 16, 2013 Category: Hematology Authors: Ofelia Alvarez, Nancy A. Yovetich, J. Paul Scott, William Owen, Scott T. Miller, William Schultz, Alexandre Lockhart, Banu Aygun, Jonathan Flanagan, Melanie Bonner, Brigitta U. Mueller, Russell E. Ware, Tags: Research Article Source Type: research

Stroke and presence of patent foramen ovale in sickle cell disease
AbstractSickle cell disease (SCD) is an inherited monogenic hemoglobinopathy characterized by formation of sickle erythrocytes under conditions of deoxygenation. Sickle erythrocytes can lead to thrombus formation and vaso-occlusive episodes that may result in hemolytic anemia, pain crisis and multiple organ damage. Moreover, SCD is characterized by endothelial damage, increased inflammatory response, platelet activation and aggravation, and activation of both the intrinsic and the extrinsic coagulation pathways. Cerebrovascular events constitute an important clinical complication of SCD. Children with SCD have a 300-fold h...
Source: Journal of Thrombosis and Thrombolysis - February 26, 2021 Category: Hematology 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

Correlates of ADL difficulty in a large hemodialysis cohort
Abstract Needing assistance with activities of daily living (ADL) is an early indicator of functional decline and has important implications for individuals' quality of life. However, correlates of need for ADL assistance have received limited attention among patients undergoing maintenance hemodialysis (HD). A multicenter cohort of 742 prevalent HD patients was assessed in 2009–2011 and classified as frail, prefrail and nonfrail by the Fried frailty index (recent unintentional weight loss, reported exhaustion, low grip strength, slow walk speed, low physical activity). Patients reported need for assistance with 4 ADL ta...
Source: Hemodialysis International - October 9, 2013 Category: Hematology Authors: Nancy G. Kutner, Rebecca Zhang, Richard M. Allman, C. Barrett Bowling Tags: Original Article Source Type: research

Sickle cell disease: when and how to transfuse.
Authors: Howard J Abstract Blood transfusion remains an important therapeutic intervention in patients with sickle cell disease (SCD), aiming to both increase the oxygen carrying capacity of blood and to reduce the complications of vaso-occlusion. Simple, manual exchange and automated exchange can be effective in reducing the acute and chronic complications of SCD, and the advantages and disadvantages of each methodology mean they all have a role in different situations. Evidence for the role of emergency transfusion in the management of the acute complications of SCD, including acute pain and acute chest syndrome,...
Source: Hematology ASH Education Program - December 4, 2016 Category: Hematology Tags: Hematology Am Soc Hematol Educ Program Source Type: research

Biomarker signatures of sickle cell disease severity.
Abstract Identifying sickle cell disease patients at high risk of complications could lead to personalized treatment and better prognosis but despite many advances prediction of the clinical course of these patients remains elusive. We propose a system-type approach to discover profiles of multiple, common biomarkers that correlate with morbidity and mortality in sickle cell disease. We used cluster analysis to discover 17 signatures of 17 common circulating biomarkers in 2320 participants of the Cooperative Study of Sickle Cell Disease, and evaluated the association of these signatures with risk for stroke, pain,...
Source: Blood Cells, Molecules and Diseases - May 16, 2018 Category: Hematology Authors: Du M, Van Ness S, Gordeuk V, Nouraie SM, Nekhai S, Gladwin M, Steinberg MH, Sebastiani P Tags: Blood Cells Mol Dis Source Type: research

Biomarker signatures of sickle cell disease severity
Publication date: Available online 16 May 2018 Source:Blood Cells, Molecules, and Diseases Author(s): Mengtian Du, Sarah Van Ness, Victor Gordeuk, Sayed M. Nouraie, Sergei Nekhai, Mark Gladwin, Martin H. Steinberg, Paola Sebastiani Identifying sickle cell disease patients at high risk of complications could lead to personalized treatment and better prognosis but despite many advances prediction of the clinical course of these patients remains elusive. We propose a system-type approach to discover profiles of multiple, common biomarkers that correlate with morbidity and mortality in sickle cell disease. We used cluster ana...
Source: Blood Cells, Molecules, and Diseases - June 15, 2018 Category: Hematology Source Type: research

Tobacco Use in the Myeloproliferative Neoplasms: Patient Behavior, Opinions, and Care
ConclusionsMPN patients with current or previous tobacco use demonstrate significantly higher symptom burden than non-smoking counterparts. In terms of patient care, less than half of patients who are current or previous smokers recall having a physician discuss their smoking habits with them. These results highlight the need for enhanced MPN patient counseling by health care providers, both regarding the risks of smoking and available methods to aid cessation.DisclosuresScherber: Orphan Pharmaceuticals: Honoraria; Incyte: Consultancy. Dueck: Phytogine: Employment; Pfizer: Honoraria; Bayer: Employment. Palmer: Novartis: Re...
Source: Blood - November 21, 2018 Category: Hematology Authors: Scherber, R. M., Geyer, H. L., Mazza, G., Langlais, B. T., Dueck, A. C., Palmer, J., Padrnos, L., Fleischman, A., Mesa, R. A. Tags: 902. Health Services Research-Malignant Diseases Source Type: research

Curing hemoglobinopathies: challenges and advances of conventional and new gene therapy approaches.
Inherited hemoglobin disorders, including beta-thalassemia (BT) and sickle-cell disease (SCD) are the most common monogenic diseases worldwide, with a global carrier frequency of over 5%. With migration they are becoming more common worldwide, making their management and care an increasing concern for health care systems.BT is characterized by an imbalance in the α/β-globin chain ratio, ineffective erythropoiesis, chronic hemolytic anemia, and compensatory haemopoietic expansion. Globally, there are over 25,000 births each year with transfusion-dependent thalassemia (TDT). The current available treatment f...
Source: Mediterranean Journal of Hematology and Infectious Diseases - October 30, 2019 Category: Hematology Source Type: research