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Effect of chronic transfusion therapy on progression of neurovascular pathology in pediatric patients with sickle cell anemia

Show simple item record Bishop S. Matheus M.G. Abboud M.R. Cane I.D. Adams R.J. Jackson S.M. Kalpatthi R.
dc.contributor.editor Aug-2011 2017-10-05T16:01:13Z 2017-10-05T16:01:13Z 2011
dc.identifier 10.1016/j.bcmd.2011.06.002
dc.identifier.issn 10799796
dc.description.abstract Background: Chronic blood transfusion (CBT) is currently the standard of care for primary and secondary stroke prevention in children with sickle cell anemia (SCA). However, the effect of CBT on cerebrovascular pathology is not well known. Methods: We reviewed children with SCA receiving CBT for abnormal transcranial Doppler (TCD) [n = 12] or cerebrovascular accident (CVA) [n = 22]. Baseline cerebral magnetic resonance imaging (MRI) and magnetic resonance angiogram (MRA) were compared with the most recent scans available for each patient and independently scored by a neuroradiologist. Results: Thirty-four patients with a mean age of 6.5. years at the time of baseline MRI-MRA were studied. Average elapsed time from baseline to most recent scans was 7.3 years. Overall, patients experienced worsening vasculopathy, as measured by mean increases in their baseline MRI and MRA scores of +. 0.76 and +. 1.03. There was a significant difference in the mean change of MRI-MRA scores between patients who had CVA and abnormal TCD (MRI; + 1.23 vs - 0.08, p = 0.001 and MRA; + 1.54 vs + 0.08, p = 0.02). Patients with abnormal baseline MRA had worsening scores compared to those with normal baseline MRA (54percent vs. 9.5percent, p = 0.01). Also, patients who had CVA were more likely to have an abnormal baseline MRA and worsening scores compared to abnormal TCD patients. Conclusion: We show that children with CVA experience progression of cerebral vasculopathy despite CBT. In contrast, CBT for abnormal TCD confers protection against the development and-or progression of cerebral vasculopathy. This effect appears to be real given our large cohort of patients with longer follow up as compared to previous studies. © 2011 Elsevier Inc.
dc.format.extent Pages: (125-128)
dc.language English
dc.publisher SAN DIEGO
dc.relation.ispartof Publication Name: Blood Cells, Molecules, and Diseases; Publication Year: 2011; Volume: 47; no. 2; Pages: (125-128);
dc.source Scopus
dc.title Effect of chronic transfusion therapy on progression of neurovascular pathology in pediatric patients with sickle cell anemia
dc.type Article
dc.contributor.affiliation Bishop, S., Department of Pediatrics, Medical University of South Carolina, Charleston, SC, United States
dc.contributor.affiliation Matheus, M.G., Department of Radiology, Medical University of South Carolina, Charleston, SC, United States
dc.contributor.affiliation Abboud, M.R., Children's Cancer Center of Lebanon, American University of Beirut Medical Center, Beirut, Lebanon
dc.contributor.affiliation Cane, I.D., Department of Pediatrics, Medical University of South Carolina, Charleston, SC, United States
dc.contributor.affiliation Adams, R.J., Department of Neurology, Medical University of South Carolina, Charleston, SC, United States
dc.contributor.affiliation Jackson, S.M., Department of Pediatrics, Medical University of South Carolina, Charleston, SC, United States
dc.contributor.affiliation Kalpatthi, R., Division of Pediatric Hematology and Oncology, Children's Mercy Hospital, Kansas City, MO, United States
dc.contributor.authorAddress Kalpatthi, R.; Division of Pediatric Hematology and Oncology, Children's Mercy Hospital, 2401 Gillham Road, Kansas City, MO 64108, United States; email:
dc.contributor.authorCorporate University: American University of Beirut Medical Center; Faculty: Faculty of Medicine; Department: Pediatrics and Adolescent Medicine;
dc.contributor.authorDepartment Pediatrics and Adolescent Medicine
dc.contributor.faculty Faculty of Medicine
dc.contributor.authorInitials Bishop, S
dc.contributor.authorInitials Matheus, MG
dc.contributor.authorInitials Abboud, MR
dc.contributor.authorInitials Cane, ID
dc.contributor.authorInitials Adams, RJ
dc.contributor.authorInitials Jackson, SM
dc.contributor.authorInitials Kalpatthi, R
dc.contributor.authorReprintAddress Kalpatthi, R (reprint author), Childrens Mercy Hosp, Div Pediat Hematol and Oncol, 2401 Gillham Rd, Kansas City, MO 64108 USA.
dc.contributor.authorUniversity American University of Beirut Medical Center
dc.description.cited Abboud MR, 2004, BLOOD, V103, P2822, DOI 10.1182-blood-2003-06-1972; Adams RJ, 1998, NEW ENGL J MED, V339, P5, DOI 10.1056-NEJM199807023390102; Adams RJ, 2005, NEW ENGL J MED, V353, P2769; Bader-Meunier B, 2009, HAEMATOL-HEMATOL J, V94, P123, DOI 10.3324-haematol.13610; Brousse V, 2009, ANN HEMATOL, V88, P785, DOI 10.1007-s00277-008-0670-x; COHEN AR, 1992, BLOOD, V79, P1657; Helton KJ, 2002, AM J NEURORADIOL, V23, P1692; Hulbert ML, 2006, J PEDIATR, V149, P710, DOI 10.1016-j.jpeds.2006.06.037; Ohene-Frempong K, 1998, BLOOD, V91, P288; RUSSELL MO, 1984, BLOOD, V63, P162; Sumoza A, 2002, AM J HEMATOL, V71, P161, DOI 10.1002-ajh.10205; Switzer JA, 2006, LANCET NEUROL, V5, P501, DOI 10.1016-S1474-4422(06)70469-0; Verduzco LA, 2009, BLOOD, V114, P5117, DOI 10.1182-blood-2009-05-220921; Walters MC, 2010, BIOL BLOOD MARROW TR, V16, P263, DOI 10.1016-j.bbmt.2009.10.005; Wang WC, 2007, CURR OPIN HEMATOL, V14, P191, DOI 10.1097-MOH.0b013e3280ec5243; Ware RE, 2004, J PEDIATR-US, V145, P346, DOI 10.1016-j.jpeds.2004.04.058
dc.description.citedCount 9
dc.description.citedTotWOSCount 10
dc.description.citedWOSCount 10
dc.format.extentCount 4
dc.identifier.coden BCMDF
dc.identifier.pubmedID 21724428
dc.identifier.scopusID 79960839271
dc.publisher.address 525 B ST, STE 1900, SAN DIEGO, CA 92101-4495 USA
dc.relation.ispartOfISOAbbr Blood Cells Mol. Dis.
dc.relation.ispartOfIssue 2
dc.relation.ispartofPubTitle Blood Cells, Molecules, and Diseases
dc.relation.ispartofPubTitleAbbr Blood Cells Mol. Dis.
dc.relation.ispartOfVolume 47
dc.source.ID WOS:000293675000007
dc.type.publication Journal
dc.subject.otherAuthKeyword Children
dc.subject.otherAuthKeyword MRI
dc.subject.otherAuthKeyword Stroke
dc.subject.otherAuthKeyword Transcranial Doppler
dc.subject.otherAuthKeyword Transfusion
dc.subject.otherChemCAS Hemoglobin, Sickle
dc.subject.otherIndex article
dc.subject.otherIndex blood transfusion
dc.subject.otherIndex cerebrovascular accident
dc.subject.otherIndex cerebrovascular disease
dc.subject.otherIndex child
dc.subject.otherIndex clinical article
dc.subject.otherIndex cohort analysis
dc.subject.otherIndex controlled study
dc.subject.otherIndex disease course
dc.subject.otherIndex Doppler echography
dc.subject.otherIndex female
dc.subject.otherIndex follow up
dc.subject.otherIndex human
dc.subject.otherIndex long term care
dc.subject.otherIndex magnetic resonance angiography
dc.subject.otherIndex male
dc.subject.otherIndex nuclear magnetic resonance imaging
dc.subject.otherIndex outcome assessment
dc.subject.otherIndex preschool child
dc.subject.otherIndex priority journal
dc.subject.otherIndex radiologist
dc.subject.otherIndex school child
dc.subject.otherIndex sickle cell anemia
dc.subject.otherIndex Adolescent
dc.subject.otherIndex Anemia, Sickle Cell
dc.subject.otherIndex Blood Transfusion
dc.subject.otherIndex Brain
dc.subject.otherIndex Cerebrovascular Circulation
dc.subject.otherIndex Child
dc.subject.otherIndex Child, Preschool
dc.subject.otherIndex Cohort Studies
dc.subject.otherIndex Disease Progression
dc.subject.otherIndex Female
dc.subject.otherIndex Follow-Up Studies
dc.subject.otherIndex Hemoglobin, Sickle
dc.subject.otherIndex Humans
dc.subject.otherIndex Magnetic Resonance Angiography
dc.subject.otherIndex Magnetic Resonance Imaging
dc.subject.otherIndex Male
dc.subject.otherIndex Pediatrics
dc.subject.otherIndex Retrospective Studies
dc.subject.otherIndex Stroke
dc.subject.otherIndex Ultrasonography, Doppler, Transcranial
dc.subject.otherKeywordPlus DISEASE
dc.subject.otherKeywordPlus STROKE
dc.subject.otherKeywordPlus CHILDREN
dc.subject.otherKeywordPlus PREVENTION
dc.subject.otherKeywordPlus HYDROXYUREA
dc.subject.otherKeywordPlus PATHOPHYSIOLOGY
dc.subject.otherKeywordPlus VASCULOPATHY
dc.subject.otherKeywordPlus RISK
dc.subject.otherWOS Hematology

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