MRI abnormalities of the brain in one‐year‐old children with sickle cell anemia

Wang, Winfred C.; Pavlakis, Steven G.; Helton, Kathleen J.; McKinstry, Robert C.; Casella, James F.; Adams, Robert J.; Rees, Renee C.

doi:10.1002/pbc.21612

Cited by 77 publications

(74 citation statements)

References 23 publications

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“…31 The lower hemoglobin levels may explain in part why SCIs have been detected in an infant as young as 7 months 32 and in several infants younger than 15 months. 33 The majority of SCIs occur in children younger than 6 years, 4 as the hemoglobin levels decline continuously until levels reach steady state. 31 In addition to our findings that low hemoglobin levels are associated with SCI, two singlecenter studies of children with SCA have demonstrated similar findings.…”

Section: Org Frommentioning

confidence: 99%

Associated risk factors for silent cerebral infarcts in sickle cell anemia: low baseline hemoglobin, sex, and relative high systolic blood pressure

DeBaun

Sarnaik

Rodeghier³

et al. 2012

Blood

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185

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The most common form of neurologic injury in sickle cell anemia (SCA) is silent cerebral infarction (SCI). In the Silent Cerebral Infarct Multi-Center Clinical Trial, we sought to identify risk factors associated with SCI. In this cross-sectional study, we evaluated the clinical history and baseline laboratory values and performed magnetic resonance imaging of the brain in participants with SCA (HbSS or HbS␤°thalassemia) between the ages of 5 and 15 years with no history of overt stroke or seizures. Neuroradiology and neurology committees adjudicated the presence of SCI. SCIs were diagnosed in 30.8% (251 of 814) participants who completed all evaluations and had valid data on all prespecified demographic and clinical covariates. The mean age of the participants was 9.1 years, with 413 males (50.7%). In a multivariable logistic regression analysis, lower baseline hemoglobin concentration (P < .001), higher baseline systolic blood pressure (P ‫؍‬ .018), and male sex (P ‫؍‬ .030) were statistically significantly associated with an increased risk of an SCI. Hemoglobin concentration and systolic blood pressure are risk factors for SCI in children with SCA and may be therapeutic targets for decreasing the risk of SCI. This study is registered at www.clinicaltrials.gov as #NCT00072761. IntroductionSilent cerebral infarcts (SCIs) have been recognized by neuroimaging in neurologically normal older adult populations since 1981 1 and were documented in sickle cell anemia (SCA) soon afterward. 2 As with overt stroke, SCIs represent a clinical finding that is common in older adults without SCD, but they appear during early childhood in persons with SCA. SCIs are defined as an MRI signal abnormality visible on 2 views on the T2-weighted images (axial and coronal) that must measure at least 3 mm in one dimension; further, the person deemed to have an SCI must have an absence of focal neurologic deficit compatible with the anatomic location of the brain lesion. 3 SCI is the most common form of neurologic injury among children with SCA, occurring in at least 27% before 6 years of life 4 and 37% by 14 years of life. 5 SCIs in children with SCA are associated with increased risk of future overt strokes and new or progressive SCIs. 6,7 In addition, children with SCA and SCI have been found to have poorer cognitive function The publication costs of this article were defrayed in part by page charge payment. Therefore, and solely to indicate this fact, this article is hereby marked ''advertisement'' in accordance with 18 USC section 1734. 3684BLOOD, 19 APRIL 2012 ⅐ VOLUME 119, NUMBER 16For personal use only. on March 28, 2019. by guest www.bloodjournal.org From than children with SCA with normal MRI of the brain 8-10 or sibling controls. 10,11 Clinical and laboratory risk factors for SCI have been evaluated only sparingly. In the most rigorous study to date, the investigators from the Cooperative Study for Sickle Cell Disease (CSSCD) described risk factors associated with SCI in 42 participants, comparing them with 188 controls with ...

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Section: Org Frommentioning

confidence: 99%

Associated risk factors for silent cerebral infarcts in sickle cell anemia: low baseline hemoglobin, sex, and relative high systolic blood pressure

DeBaun

Sarnaik

Rodeghier³

et al. 2012

Blood

Self Cite

185

144

View full text Add to dashboard Cite

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“…The severity of anemia, a history of recurrent acute chest syndrome, obesity, and elevated blood pressure are all considered independent risk factors for the development of stroke (Akingbola et al, 2014;Ohene-Frempong et al, 1998;Wang et al, 2008). Other risk factors include prior transient ischemic attack, low steady-state hemoglobin levels, hypertension, and high leukocyte counts (Ohene-Frempong et al, 1998).…”

Section: Stroke In Scdmentioning

confidence: 99%

Genetics of Sickle Cell-Associated Cardiovascular Disease: An Expert Review with Lessons Learned in Africa

Geard

Pule

Chelo

et al. 2016

OMICS: A Journal of Integrative Biology

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Sickle cell disease (SCD) vastly impacts the African continent and is associated with cardiovascular diseases. Stroke, kidney disease, and pulmonary hypertension are considered as proxies of severity in SCD with several genomic loci implicated in their heritability. The present expert review examined the current data on epidemiology and genetic risk factors of stroke, pulmonary hypertension, and kidney disease associated with SCD, as indexed in PubMed Ò and Google Scholar Ò . Studies collectively show that stroke and kidney disease each affect *10% of SCD patients, with pulmonary hypertension displaying a higher prevalence of 30% among adults with SCD. There is some evidence that these epidemiology figures may be an underestimate in SCD patients living in Africa. A modest number of publications have identified genetic factors involved in pathways regulating inflammation, coagulation, cell adhesion, heme degradation, a-globin and c-globin production, and others, which contribute to the development risk of targeted cardiovascular phenotypes. However, in most cases, these studies have not been validated across populations. There is therefore an urgent need for large-scale genome-wide association, wholeexome and whole-genome studies, and multiomics research on cardiovascular diseases associated with SCD, particularly in Africa, to allow for proportional investment of global research funding on diseases that greatly impact the African continent. Ultimately, this will cultivate socially responsible research investments and identification of at-risk individuals with improved preventive medicine, which should be a cornerstone of global precision medicine.

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“…SCI have been reported in very young children; 4/39 children (10%) with SCA and no history of stroke between 7 and 48 months of age had SCI [9]; 3/23 children (13%) at an average age of 13.7 months had SCI [10]; and 18/65 children (27.7%) with SCA who were asymptomatic had SCI [31]. A French study showed incidence of SCI as 28.2% by 8 years and 37.4% by 14 years [66].…”

Section: Progression Of Scimentioning

confidence: 99%

“…Silent cerebral infarction (SCI) is diagnosed only using magnetic resonance imaging (MRI) in patients with no focal neurological deficit, but is associated with cognitive difficulties [8] that families often report. SCI can develop very early in life, with rates between 11 and 15% in children less than 2 years [9][10][11] and progressive accrual throughout childhood and adolescence [11,12] and into adulthood.…”

Section: Introductionmentioning

confidence: 99%

Neurological Complications and MRI

Kawadler¹,

Kirkham²

2016

Sickle Cell Disease - Pain and Common Chronic Complications

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Cerebrovascular diseases (cerebral infarction, intracranial haemorrhage and vasculopathy) are common manifestations of sickle cell disease (SCD) associated with significant morbidity and mortality. These neurological complications and potential corresponding neuropsychological compromise may have devastating consequences for a child with SCD. This chapter aims to review the neurological complications in SCD using magnetic resonance imaging (MRI) as both a qualitative and a quantitative tool for detecting abnormality. Advanced MRI pulse sequences, such as high-resolution 3D T1-weighted imaging for brain volumetrics, diffusion tensor imaging for white matter integrity and non-invasive perfusion MRI for cerebral blood flow (CBF) measurement, can provide additional information about the structure and function of brain tissue beyond the scope of conventional clinical imaging. These studies have set to establish quantitative biomarkers that relate to disease severity and neuropsychological sequelae.

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MRI abnormalities of the brain in one‐year‐old children with sickle cell anemia

Abstract: Silent brain infarcts occur in a small but significant number of infants with SCA as early as a year of age. This finding indicates a need for thorough evaluation of the CNS very early in life in children with SCA in order to develop timely intervention strategies.

Cited by 77 publications

References 23 publications

Associated risk factors for silent cerebral infarcts in sickle cell anemia: low baseline hemoglobin, sex, and relative high systolic blood pressure

Associated risk factors for silent cerebral infarcts in sickle cell anemia: low baseline hemoglobin, sex, and relative high systolic blood pressure

Genetics of Sickle Cell-Associated Cardiovascular Disease: An Expert Review with Lessons Learned in Africa

Neurological Complications and MRI

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