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Condition: Anemia
Therapy: Transfusion Therapy

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Total 252 results found since Jan 2013.

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

Central nervous system complications and management in sickle cell disease
With advances in brain imaging and completion of randomized clinical trials (RCTs) for primary and secondary stroke prevention, the natural history of central nervous system (CNS) complications in sickle cell disease (SCD) is evolving. In order of current prevalence, the primary CNS complications include silent cerebral infarcts (39% by 18 years), headache (both acute and chronic: 36% in children with sickle cell anemia [SCA]), ischemic stroke (as low as 1% in children with SCA with effective screening and prophylaxis, but ~11% in children with SCA without screening), and hemorrhagic stroke in children and adults with SCA ...
Source: Blood - February 18, 2016 Category: Hematology Authors: DeBaun, M. R., Kirkham, F. J. Tags: Sickle Cell Disease, Free Research Articles, Red Cells, Iron, and Erythropoiesis, Review Articles, Review Series, Clinical Trials and Observations 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

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

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

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

In Context News in brief
Stroke and silent cerebral infarcts are common in children with sickle cell anaemia. Although the incidence of silent cerebral infarcts can be reduced with regular blood transfusions, it is unknown whether regular transfusions can prevent the recurrence of an infarct (stroke or new or enlarged silent cerebral infarction). In a multicentre, randomised, single-blind, controlled trial of 196 children aged 5 to 15 years with sickle cell anaemia who had silent cerebral infarcts, 6 of 99 (6%) children who received transfusion therapy had an end-point event (one had stroke and five had new or enlarged silent cerebral infarcts) ov...
Source: Lancet Neurology - September 16, 2014 Category: Neurology Authors: The Lancet Neurology Tags: In Context 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

Pial synangiosis for moyamoya syndrome in children with sickle cell anemia: a comprehensive review of reported cases.
Conclusions Pial synangiosis in patients with SCA, MMS, and brain ischemia appears to be a safe and effective treatment option. Transcranial Doppler and/or MRI screening in asymptomatic patients with SCA is recommended for the diagnosis of MMS. PMID: 24380478 [PubMed - in process]
Source: Neurosurgical Focus - January 1, 2014 Category: Neurosurgery Authors: Kennedy BC, McDowell MM, Yang PH, Wilson CM, Li S, Hankinson TC, Feldstein NA, Anderson RC Tags: Neurosurg Focus 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

Transcranial Doppler Velocity and Brain MRI/MRA Changes in Children With Sickle Cell Anemia on Chronic Transfusions to Prevent Primary Stroke
ConclusionsFor children with SCA and abnormal TCD velocities, transfusions lower TCD velocities and help prevent stroke, but do not always result in normal velocities or protect against progression of cerebral vasculopathy. Improved adherence to transfusion goals may improve on‐treatment TCD velocities. Pediatr Blood Cancer 2013;9999:XX–XX. © 2013 Wiley Periodicals, Inc.
Source: Pediatric Blood and Cancer - April 26, 2013 Category: Cancer & Oncology Authors: Vivien A. Sheehan, Eileen N. Hansbury, Matthew P. Smeltzer, Gail Fortner, M. Beth McCarville, Banu Aygun Tags: Research Article 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