Large-Vessel Vasculopathy in Children With Sickle Cell Disease: A Magnetic Resonance Imaging Study of Infarct Topography and Focal Atrophy.
Kristin P. Guilliams,Melanie E. Fields,Dustin K. Ragan,Yasheng Chen,Cihat Eldeniz,Monica L. Hulbert,Michael M. Binkley,James Rhodes,Joshua S. Shimony,Robert C. McKinstry,Katie D. Vo,Hongyu An,Jin-Moo Lee,Andria L. Ford +13 more
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TLDR
Large-vessel vasculopathy in sickle cell disease appears to increase ischemic vulnerability in the borderzone region, as demonstrated by the increased frequency and extent of infarction within deep white matter, and increased risk of focal atrophy.About:
This article is published in Pediatric Neurology.The article was published on 2017-04-01 and is currently open access. It has received 35 citations till now. The article focuses on the topics: Cerebral atrophy & Stroke.read more
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Journal ArticleDOI
Pathophysiology of Sickle Cell Disease.
TL;DR: This review provides an exhaustive overview of the current understanding of the molecular pathophysiology of SCD, how this pathophysiological contributes to complications of the central nervous and cardiopulmonary systems, and how this knowledge is being harnessed to develop current and potential therapies.
Journal ArticleDOI
Regional oxygen extraction predicts border zone vulnerability to stroke in sickle cell disease
Melanie E. Fields,Kristin P. Guilliams,Dustin K. Ragan,Michael M. Binkley,Cihat Eldeniz,Yasheng Chen,Monica L. Hulbert,Robert C. McKinstry,Joshua S. Shimony,Katie D. Vo,Allan Doctor,Hongyu An,Andria L. Ford,Jin-Moo Lee +13 more
TL;DR: Elevated OEF in the deep white matter identifies a signature of metabolically stressed brain tissue at increased stroke risk in pediatric patients with SCD, and it is proposed that border zone physiology, exacerbated by chronic anemic hypoxia, explains the high risk in this region.
Journal ArticleDOI
Silent infarcts in sickle cell disease occur in the border zone region and are associated with low cerebral blood flow.
Andria L. Ford,Dustin K. Ragan,Slim Fellah,Michael M. Binkley,Melanie E. Fields,Kristin P. Guilliams,Hongyu An,Lori C. Jordan,Robert C. McKinstry,Jin-Moo Lee,Michael R. DeBaun +10 more
TL;DR: Using the largest prospective study evaluating SCIs in pediatric SCA, brain regions with increased SCI density are identified, testing the hypothesis that infarct density is greatest within regions in which cerebral blood flow is lowest, further restricting cerebral oxygen delivery in the setting of chronic anemia.
Journal ArticleDOI
Red cell exchange transfusions lower cerebral blood flow and oxygen extraction fraction in pediatric sickle cell anemia
Kristin P. Guilliams,Melanie E. Fields,Dustin K. Ragan,Cihat Eldeniz,Michael M. Binkley,Yasheng Chen,Liam S. Comiskey,Allan Doctor,Monica L. Hulbert,Joshua S. Shimony,Katie D. Vo,Robert C. McKinstry,Hongyu An,Jin-Moo Lee,Andria L. Ford +14 more
TL;DR: Reduction of elevated CBF and OEF, both globally and regionally, suggests that CTT mitigates infarct risk in pediatric SCA by relieving cerebral metabolic stress at patient- and tissue-specific levels.
Journal ArticleDOI
Hydroxyurea reduces cerebral metabolic stress in patients with sickle cell anemia
Melanie E. Fields,Kristin P. Guilliams,Dustin K. Ragan,Michael M. Binkley,Amy E. Mirro,Slim Fellah,Monica L. Hulbert,Morey A. Blinder,Cihat Eldeniz,Katie D. Vo,Joshua S. Shimony,Yasheng Chen,Robert C. McKinstry,Hongyu An,Jin-Moo Lee,Andria L. Ford +15 more
TL;DR: It is concluded that HU may offer neuroprotection by mitigating cerebral metabolic stress in patients with SCA, but not to the same degree as CTT.
References
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