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Effectiveness of red blood cell exchange, partial manual exchange, and simple transfusion concurrently with iron chelation therapy in reducing iron overload in chronically transfused sickle cell anemia patients.
CONCLUSION: We recommend RCE plus chelation as an effective method for reducing iron overload, while maintaining HbS at 30% to 35%. PMID: 26997031 [PubMed - as supplied by publisher]
Source: Transfusion - March 19, 2016 Category: Hematology Authors: Fasano RM, Leong T, Kaushal M, Sagiv E, Luban NL, Meier ER Tags: Transfusion 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

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

Cerebral vasculopathy in pediatric sickle-cell anemia.
Abstract In children with sickle-cell anemia, cerebral vasculopathy is a frequent and severe complication. It is attributed not only to erythrocyte sickling but also to multiple physiological modifications associated with sickle-cell anemia: platelet and leukocyte activation, endothelial injury and remodeling, coagulation activation, hemolysis and subsequent chronic inflammation, impaired vasomotricity, etc. Intracranial large-vessel remodeling leads to clinical cerebral infarction, whereas microvascular injury and impaired vasoreactivity lead to so-called silent infarcts, which are actually associated with impair...
Source: Archives de Pediatrie - March 12, 2014 Category: Pediatrics Authors: Kossorotoff M, Grevent D, de Montalembert M Tags: Arch Pediatr 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

Patients with sickle cell anemia on simple chronic transfusion protocol show sex differences for hemodynamic and hematologic responses to transfusion.
CONCLUSION: In our sample population, males exhibit a paradoxical reduction in oxygen delivery in response to transfusion because the increase in vascular resistance is larger than the increase in oxygen capacity. This may result from an inability to adequately suppress their HbS% between transfusion cycles. PMID: 23176402 [PubMed - as supplied by publisher]
Source: Transfusion - November 26, 2012 Category: Hematology Authors: Detterich JA, Sangkatumvong S, Kato R, Dongelyan A, Bush A, Khoo M, Meiselman HJ, Coates TD, Wood JC Tags: Transfusion Source Type: research

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