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Total 21 results found since Jan 2013.

Practice Patterns in the Use of MRI/MRA and Chronic Transfusion Therapy for Monitoring and Treatment of Stroke in Pediatric Patients with Sickle Cell Anemia
Conclusions: The results of the DISPLACE practice patterns survey suggested nearly universal adoption of CRCT for children with abnormal TCD and prior overt stroke across sites. The use of MRI/MRA in detecting potential cerebrovascular abnormalities in the absence of clinical neurologic symptoms and for monitoring patients on CRCT was much more variable, which likely reflects the minimal amount of guidance for the use of MRI/MRA in SCA. Notably, a number of sites were using MRI/MRA to screen pediatric patients for cerebrovascular abnormalities and to monitor patients on CRCT. The variability in responses for MRI/MRA highli...
Source: Blood - November 21, 2018 Category: Hematology Authors: Schlenz, A. M., Mueller, M., Phillips, S., Melvin, C., Adams, R. J., Kanter, J. Tags: 901. Health Services Research-Non-Malignant Conditions: Poster III Source Type: research

Middle Cerebral Artery Velocities Are Inversely Related to Hemoglobin Levels and Acutely Drop in Response to RBC Transfusion: Implications for Stroke Screening in SCD
DiscussionThese data show a striking relationship between anemia and TCD velocity, and that RBC transfusion can alter MCA velocity. Even an 11% increase in hemoglobin such as that seen in the low-responder in Figure 3 could change a patient classification from high-risk to conditional TCD. These data also show the significant sensitivity of MCA velocity to the depth of measurement (Figure 2 & 3). The investigators in the STOP study went to extreme ends to standardize these measurements. Nonetheless, TCD measures may not be done with such rigor in clinical settings. It is clear that standard TCD screening has been of tr...
Source: Blood - November 21, 2018 Category: Hematology Authors: Perumbeti, A., Carreras, O., Detterich, J. A., Shah, P., Sunwoo, J., Khoo, M., Wood, J. C., Coates, T. D. Tags: 114. Hemoglobinopathies, Excluding Thalassemia-Clinical: Poster II Source Type: research

Dilemma in approach to stroke in sickle cell disease patient: A case report
Conclusion: Recommendations need to be put in place regarding screening for PFO in patients with SCD, in addition to highlighting issues of whether screening needs to be done in patients who have not developed stroke, and if PFO were to be found, what would be the best management approach and how will prognosis be affected.
Source: Medicine - July 15, 2022 Category: Internal Medicine Tags: Research Article: Clinical Case Report Source Type: research

Results from the Displace Consortium: Practice Patterns on the Use of Transcranial Doppler Screening for Risk of Stroke in Children with Sickle Cell Anemia
Conclusions: Nearly all DISPLACE sites order TCD screening annually, as recommended in the guidelines, with some ordering screening more frequently. A few sites did not report initiation of CRCT per STOP protocol for abnormal TCD results; however, over half of the sites reported following up with an MRI/MRA, which may suggest evaluating for vasculopathy prior to CRCT. Some sites reported beginning HU therapy for abnormal results; this may reflect consideration of patients for whom CRCT is not possible, but data were not collected for confirmation. Interestingly, results suggest a reliance on MRI/MRA since sites commonly re...
Source: Blood - November 21, 2018 Category: Hematology Authors: Phillips, S., Mueller, M., Schlenz, A. M., Melvin, C., Adams, R. J., Kanter, J. Tags: 901. Health Services Research-Non-Malignant Conditions: Poster III 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

How I manage sickle cell patients with high transcranial doppler results
Summary Stroke is one of the most severe complications to affect children with sickle cell anaemia (SCA). Transcranial doppler (TCD) is an accurate and non‐invasive method to determine stroke risk. Randomised controlled trials have demonstrated the efficacy of chronic transfusion therapy in stroke prevention based on risk stratification determined by TCD velocities. This has led to the regular use of TCD monitoring for children with SCA in order to determine stroke risk. Significant resource allocation is necessary to facilitate training, quality assurance and failsafe arrangements for non‐attenders. In a subgroup of p...
Source: British Journal of Haematology - August 2, 2017 Category: Hematology Authors: John Brewin, Banu Kaya, Subarna Chakravorty Tags: Review Source Type: research

Sickle Cell Disease Subjects Have a Distinct Abnormal Autonomic Phenotype Characterized by Peripheral Vasoconstriction With Blunted Cardiac Response to Head-Up Tilt
Conclusion We have shown that SCD subjects are much more likely than non-SCD subjects to have impaired cardiac, but intact peripheral responses to orthostatic stress induced by HUT. These abnormal responses are associated with low baseline cardiac parasympathetic activity, independent of hemoglobin level. The classification of autonomic phenotypes based on HUT response may have potential use for predicting disease severity, guiding and targeting treatments/interventions to alleviate the risk of adverse outcomes in SCD. Ethics Statement All experiments were conducted at Children’s Hospital Los Angeles (CHLA). The ...
Source: Frontiers in Physiology - April 10, 2019 Category: Physiology 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

Transfusion Therapy in a Multi-Ethnic Sickle Cell Population Real-World Practice. a Preliminary Data Analysis of Multicentre Survey
Conclusion. The transfusional approach is similar in HbSS, b°-thal/HbS and b+-thal/HbS patients with similar indications, prevalently VOCs and anemia. The significant higher age in Caucasian cohort and the consequent long term follow up could be the cause of variable therapeutic approach observed, however Hydroxycarbamide seemed to be the therapy more frequently used and finally suggested to manage chronic manifestations.Figure.DisclosuresOriga: Apopharma: Honoraria; Novartis: Honoraria; Bluebird Bio: Consultancy; Cerus Corporation: Research Funding. Forni: Apopharma: Other: DSM Board; Celgene: Research Funding; Novart...
Source: Blood - November 21, 2018 Category: Hematology Authors: Graziadei, G., Sainati, L., Bonomo, P., Venturelli, D., Masera, N., Casale, M., Vassanelli, A., Lodi, G., Piel, F. B., Voi, V., De Franceschi, L., Rigano, P., Quota, A., Notarangelo, L. D., Russo, G., Rosso, R., Allo, M., D'Ascola, D., Facchini, E., Macch Tags: 114. Hemoglobinopathies, Excluding Thalassemia-Clinical: Poster II Source Type: research

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

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

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

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