scholarly journals Progressive loss of brain volume in children with sickle cell anemia and silent cerebral infarct: A report from the silent cerebral infarct transfusion trial

2018 ◽  
Vol 93 (12) ◽  
pp. E406-E408 ◽  
Author(s):  
Deepika S. Darbari ◽  
Odianosen Eigbire-Molen ◽  
Maria R. Ponisio ◽  
Mikhail V. Milchenko ◽  
Mark J. Rodeghier ◽  
...  
Keyword(s):  
Sickle Cell ◽  
Sickle Cell Anemia ◽  
Brain Volume ◽  
Cerebral Infarct ◽  
Progressive Loss ◽  
Silent Cerebral Infarct

scholarly journals Silent cerebral infarct definitions and full-scale IQ loss in children with sickle cell anemia

Neurology ◽  
2017 ◽  
Vol 90 (3) ◽  
pp. e239-e246 ◽  
Author(s):  
Natasha A. Choudhury ◽  
Michael R. DeBaun ◽  
Mark Rodeghier ◽  
Allison A. King ◽  
John J. Strouse ◽  
...  
Keyword(s):  
Linear Regression ◽  
Confidence Interval ◽  
Sickle Cell ◽  
Sickle Cell Anemia ◽  
Full Scale ◽  
Cerebral Infarct ◽  
Significant Change ◽  
Definition Of ◽  
Silent Cerebral Infarct

ObjectiveTo evaluate whether application of the adult definition of silent cerebral infarct (SCI) (T2-weighted hyperintensity ≥5 mm with corresponding T1-weighted hypointensity on MRI) is associated with full-scale IQ (FSIQ) loss in children with sickle cell anemia (SCA), and if so, whether this loss is greater than that of the reference pediatric definition of SCI (T2-weighted hyperintensity ≥3 mm in children on MRI; change in FSIQ −5.2 points; p = 0.017; 95% confidence interval [CI] −9.48 to −0.93).MethodsAmong children with SCA screened for SCI in the Silent Cerebral Infarct Transfusion trial, ages 5–14 years, a total of 150 participants (107 with SCIs and 43 without SCIs) were administered the Wechsler Abbreviated Scale of Intelligence. A multivariable linear regression was used to model FSIQ in this population, with varying definitions of SCI independently substituted for the SCI covariate.ResultsThe adult definition of SCI applied to 27% of the pediatric participants with SCIs and was not associated with a statistically significant change in FSIQ (unstandardized coefficient −3.9 points; p = 0.114; 95% CI −8.75 to 0.95), with predicted mean FSIQ of 92.1 and 96.0, respectively, for those with and without the adult definition of SCI.ConclusionsThe adult definition of SCI may be too restrictive and was not associated with significant FSIQ decline in children with SCA. Based on these findings, we find no utility in applying the adult definition of SCI to children with SCA and recommend maintaining the current pediatric definition of SCI in this population.

Low Socioeconomic Status, Not Silent Cerebral Infarcts, Is Associated with Grade Failure in Children with Sickle Cell Anemia.

Blood ◽  
2007 ◽  
Vol 110 (11) ◽  
pp. 2254-2254
Author(s):  
Allison A. King ◽  
Keyword(s):  
Socioeconomic Status ◽  
Risk Factor ◽  
Sickle Cell ◽  
Low Socioeconomic Status ◽  
Sickle Cell Anemia ◽  
Cerebral Infarct ◽  
Annual Income ◽  
Cerebral Infarcts ◽  
Grade Failure ◽  
Silent Cerebral Infarct

Abstract Background: Among students with sickle cell anemia (SCA), cerebral infarcts are an established risk factor for poor cognition and academic achievement. We tested the hypothesis that in children with SCA, lower socioeconomic status (SES) is associated with grade failure. Methods: We evaluated the association between the SES as measured by annual income per person in household per year, age of student, gender, medical history and grade failure in children with SCA in 24 sites participating in the Silent Cerebral Infarct Multi-Center Clinical Trial. Results: A total of 404 students were evaluable. The mean age was 9.2 years (range 6–13), 37% had a silent infarct, 15% failed a grade, and 18% had an Individualized Education Plan. SES was divided in tertiles based on annual income/person in the household/year < $5000, $5000–$9999, > $10,000. After adjustment for covariates, the risk of grade failure was 1.4 (95% CI 1.2, 1.6) for each increasing year of age, and 1.9 (95% CI 1.03, 3.5) for male gender. Children from households of the lowest tertile were 4 times more likely to fail a grade than children from the top tertile. A silent cerebral infarct was not associated with grade failure 1.4 (95% CI 0.8, 2.6). No student with a history of grade failure was hospitalized > 3 times per year over the past three years. Conclusion: Among students with SCA, poverty is the greatest risk factor for grade failure; whereas the presence of silent cerebral infarct is not. Targeted strategies to improve educational attainment in students with SCA are needed.

scholarly journals Silent cerebral infarct in sickle cell anemia patients of southern Turkey

2020 ◽  
Vol 50 (8) ◽  
pp. 1887-1893
Author(s):  
Ezgi NAFİLE SAYMAN ◽  
Göksel LEBLEBİSATAN ◽  
Şerife LEBLEBİSATAN ◽  
Kenan BIÇAKÇI ◽  
Yurdanur KILINÇ ◽  
...  
Keyword(s):  
Sickle Cell ◽  
Sickle Cell Anemia ◽  
Cerebral Infarct ◽  
Southern Turkey ◽  
Silent Cerebral Infarct

scholarly journals Thrombospondin-1 and L-selectin are associated with silent cerebral infarct in children with sickle cell anaemia

2013 ◽  
Vol 162 (3) ◽  
pp. 421-424 ◽  
Author(s):  
Lisa M. Faulcon ◽  
Zongming Fu ◽  
Pratima Dulloor ◽  
Emily Barron-Casella ◽  
William Savage ◽  
...  
Keyword(s):  
Sickle Cell ◽  
Sickle Cell Anaemia ◽  
Cerebral Infarct ◽  
Thrombospondin 1 ◽  
Silent Cerebral Infarct

scholarly journals Cognitive Function in Sickle Cell Disease Across Domains, Cerebral Infarct Status, and the Lifespan: A Meta-Analysis

2019 ◽  
Vol 44 (8) ◽  
pp. 948-958 ◽  
Author(s):  
Kemar V Prussien ◽  
Lori C Jordan ◽  
Michael R DeBaun ◽  
Bruce E Compas
Keyword(s):  
Cognitive Function ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Meta Analysis ◽  
Age Groups ◽  
Sampling Bias ◽  
Cerebral Infarct ◽  
Cell Disease ◽  
Perceptual Reasoning ◽  
Silent Cerebral Infarct

Abstract Objective To provide a comprehensive quantitative review of neurocognitive function in sickle cell disease (SCD) across multiple domains, cerebral infarct status, and the lifespan. Methods One hundred and ten studies were identified in PubMed, MedLine, and PsycINFO involving 110 studies of 3,600 participants with SCD and 1,127 sibling or health controls. Results Meta-analytic findings indicate significant deficits across all neurocognitive domains, age groups, and infarct status. Significant deficits relative to the normative mean ranged from Hedges’ g = −.39 to g = −.63 in preschool children, g = −.83 to g = −1.18 in school-aged children and adolescents, and g = −.46 to g = −.86 in adults. Deficits in full scale IQ (FSIQ), verbal reasoning, perceptual reasoning, and executive function increased from preschool to school-aged samples. However, findings also showed that deficits were smaller in adult samples relative to school-aged samples, likely due to sampling bias in adult studies. Findings across infarct status in sickle cell anemia showed that deficits ranged from g = −.54 to g = −.65 in samples without infarcts, g = −.52 to g = −1.03 in samples with silent cerebral infarct, and g = −1.35 to g = −1.82 in samples with stroke. Deficits in each domain increased in magnitude from no infarct or stroke, to silent cerebral infarct, to overt stroke. Conclusion Individuals with SCD are at risk for cognitive deficits across domains, infarct status, and the lifespan. More research is necessary to determine unbiased effects for cognitive function in adults with SCD.

Low Levels of Apolipoprotein A1 Are Associated with Silent Cerebral Infarcts in Children with Sickle Cell Disease.

Blood ◽  
2009 ◽  
Vol 114 (22) ◽  
pp. 259-259 ◽  
Author(s):  
John J. Strouse ◽  
Michael Kraut ◽  
Lisa M. Williams ◽  
Michael R. DeBaun ◽  
James F. Casella
Keyword(s):  
Logistic Regression ◽  
Sickle Cell ◽  
Apolipoprotein B ◽  
Research Funding ◽  
Cerebral Infarct ◽  
Apolipoprotein A1 ◽  
Cell Disease ◽  
Cerebral Infarcts ◽  
Increased Risk ◽  
Silent Cerebral Infarct

Abstract Abstract 259 Background: Cerebral infarct is a frequent complication in children and adults with sickle cell disease (SCD). About 10% of children with sickle cell anemia (HbSS) will have an overt stroke by 18 years of age and another 22% will have “silent” cerebral infarcts by 14 years of age. There is a second peak in the incidence of overt ischemic stroke in adults with SCD. Both silent and overt stroke can be accompanied by cognitive impairment and thereby increased risk of academic failure. Several laboratory parameters have been associated with these complications, including low hematocrit or hemoglobin, high white blood cell count (WBC), and high platelet count, while lactate dehydrogenase (LDH) and apolipoprotein A1 have been associated with pulmonary vasculopathy in adults with SCD. Elevated C-reactive protein (CRP) and the ratio of apolipoprotein B/A1 are associated with increased stroke risk in the general population. We hypothesized that these and other biomarkers might also be important in children with silent cerebral infarction. Methods: We prospectively measured potential biomarkers, including complete blood count, reticulocytes, fetal hemoglobin (HbF), LDH, CRP, and apolipoprotein A1 and B in 73 children with HbSS and 3 with sickle-β0-thalassemia (HbSβ0), in addition to performing brain magnetic resonance imaging (MRI), in a single center ancillary study of the Silent Infarct Transfusion Trial. MRIs were interpreted for the presence or absence of silent cerebral infarcts (FLAIR hyperintensities ≥3 mm in diameter seen in at least two imaging planes without corresponding symptoms or signs of stroke). Groups were compared by Student's t-test or Wilcoxon rank-sum test and associations evaluated by univariate and multivariate logistic regression. Results: Twenty-seven participants (35.5%, 1 with HbSβ0) had silent cerebral infarcts (age 8.9 ± 2.4 years, 59% male) and 49 (65.2%) had normal brain MRIs (age 8.7 ± 2.1, 53% male). The group with SCD and silent cerebral infarcts had significantly lower hemoglobin concentration and apolipoprotein A1 (P=0.01 for both) than children with SCD without silent cerebral infarcts. Apolipoprotein B and the ratio of apolipoprotein B to A1 were similar between groups. By univariate logistic regression, both apolipoprotein A1 [Odds ratio (OR) 0.95 per mg/dl increase, P<0.05] and hemoglobin (OR 0.52 per g/L increase, P<0.05) were associated with silent cerebral infarct; however, in a multivariate model, apolipoprotein A1 (OR 0.96 per mg/dl, P<0.05), but not hemoglobin (OR 0.78 per g/L, P=0.5), was significantly associated with silent cerebral infarct. Discussion and Conclusions: These data suggest a role of apolipoprotein A1 in the pathogenesis of silent cerebral infarct in children with SCD. Apolipoprotein A1 is a plausible marker or risk factor for cerebrovascular disease in SCD, given the association of lower levels with pulmonary hypertension in SCD and increased risk for cererbrovascular and cardiac disease in the general population. This marker should be studied in a larger longitudinal cohort of children at risk for cerebral infarcts. Disclosures: Strouse: General Electric: Equity Ownership. Casella:Boehringer Ingelheim: Honoraria; Cytrex: Research Funding; Ikaria: Research Funding.

The orientation of sickle hemoglobin fibers within fascicles

1988 ◽  
Vol 46 ◽  
pp. 400-401
Author(s):  
Christopher A. Miller ◽  
Bridget Carragher ◽  
William A. McDade ◽  
Robert Josephs
Keyword(s):  
Sickle Cell ◽  
Sickle Cell Anemia ◽  
Relative Orientation ◽  
Unit Cell ◽  
Red Cell ◽  
High Ph ◽  
Sickle Hemoglobin ◽  
Polar Crystal ◽  
Helical Configuration

Highly ordered bundles of deoxyhemoglobin S (HbS) fibers, termed fascicles, are intermediates in the high pH crystallization pathway of HbS. These fibers consist of 7 Wishner-Love double strands in a helical configuration. Since each double strand has a polarity, the odd number of double strands in the fiber imparts a net polarity to the structure. HbS crystals have a unit cell containing two double strands, one of each polarity, resulting in a net polarity of zero. Therefore a rearrangement of the double strands must occur to form a non-polar crystal from the polar fibers. To determine the role of fascicles as an intermediate in the crystallization pathway it is important to understand the relative orientation of fibers within fascicles. Furthermore, an understanding of fascicle structure may have implications for the design of potential sickling inhibitors, since it is bundles of fibers which cause the red cell distortion responsible for the vaso-occlusive complications characteristic of sickle cell anemia.

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