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Primary stroke prevention in children with sickle cell anemia living in africa: the false choice between patient-oriented research and humanitarian service.
PRIMARY STROKE PREVENTION IN CHILDREN WITH SICKLE CELL ANEMIA LIVING IN AFRICA: THE FALSE CHOICE BETWEEN PATIENT-ORIENTED RESEARCH AND HUMANITARIAN SERVICE. Trans Am Clin Climatol Assoc. 2016;127:17-33 Authors: Debaun MR, Galadanci NA, Kassim AA, Jordan LC, Phillips S, Aliyu MH Abstract In the United States, primary stroke prevention in children with sickle cell anemia (SCA) is now the standard of care and includes annual transcranial Doppler ultrasound evaluation to detect elevated intracranial velocities; and for those at risk, monthly blood transfusion therapy for at least a year followed by the opt...
Source: Transactions of the American Clinical and Climatological Association - January 12, 2017 Category: Journals (General) Tags: Trans Am Clin Climatol Assoc 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

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

Hydroxyurea use in prevention of stroke recurrence in children with sickle cell disease in a developing country: A cost effectiveness analysis
We undertook a cost effectiveness analysis (CEA) of hydroxyurea (HU) in preventing stroke recurrence and/or death. We followed 43 children with sickle cell disease from 2000 to 2009 after having a first clinical stroke, of whom 10 opted for HU therapy. HU use led to decreased stroke recurrence and death without significantly increasing the annual cost of care per patient (J$83,250 vs. J$76,901, P = 0.491). The incremental cost effectiveness ratio (ICER) for prevention of stroke recurrence amounted to J$169,238 (US$1,900), while that for death prevention equalled J$635,843 (US$7,140). HU may be recommended when safe and...
Source: Pediatric Blood and Cancer - April 30, 2015 Category: Cancer & Oncology Authors: Colette Cunningham‐Myrie, Abdullahi Abdulkadri, Andre Waugh, Susanna Bortolusso Ali, Lesley‐Gaye King, Jennifer Knight‐Madden, Marvin Reid Tags: Brief Report 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

Primary stroke prevention in Nigerian children with sickle cell disease (SPIN): Challenges of conducting a feasibility trial
ConclusionA hydroxyurea trial (NCT01801423) for children with SCD is feasible in sub‐Saharan Africa; however, extensive training and resources are needed to build a global patient oriented multi‐disciplinary research team with a common purpose. Pediatr Blood Cancer. © 2014 Wiley Periodicals, Inc.
Source: Pediatric Blood and Cancer - November 14, 2014 Category: Cancer & Oncology Authors: Najibah A. Galadanci, Shehu U. Abdullahi, Musa A. Tabari, Shehi Abubakar, Raymond Belonwu, Auwal Salihu, Kathleen Neville, Fenella Kirkham, Baba Inusa, Yu Shyr, Sharon Phillips, Adetola A. Kassim, Lori C. Jordan, Muktar H. Aliyu, Brittany V. Covert, Micha Tags: Research Article 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

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

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

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

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