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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

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

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

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

Reduction in Overt and Silent Stroke Recurrence Rate Following Cerebral Revascularization Surgery in Children with Sickle Cell Disease and Severe Cerebral Vasculopathy
ConclusionsThe rate of overt and silent infarct recurrence was significantly lower following indirect cerebral revascularization. A prospective study of cerebral revascularization in children with SCD is needed.
Source: Pediatric Blood and Cancer - April 21, 2016 Category: Cancer & Oncology Authors: Erin M. Hall, Jeffrey Leonard, Jodi L. Smith, Kristin P. Guilliams, Michael Binkley, Robert J. Fallon, Monica L. Hulbert Tags: Research Article Source Type: research