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Query: UMLS:C0002895 (sickle cell disease)
 11,747 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The most devastating complication of sickle cell anemia is cerebral infarction, affecting approximately 30% of all individuals with sickle cell anemia. Despite being one of the most common causes of stroke in infants and children, the mechanism of cerebral infarction in this population has not been extensively studied and is poorly understood. Multiple, synergistic factors are important in the pathogenesis of stroke including the hemodynamic effects of cerebral arterial occlusive disease, viscosity, chronic and acute anemia and acute medical events. This review focuses on the relationship between these factors in order to provide a foundation for further study of the etiology of strokes in this high-risk population.
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PMID:Etiology of strokes in children with sickle cell anemia. 1706 Dec 88

Sickle cell disease (SCD) affects over 30,000 students in the United States. Central nervous system complications are widespread among students with SCD and include stroke, silent cerebral infarction, and cognitive impairment. The effects of these complications may lead to academic failure, limited career options, and for some, total disability. Despite studies describing the significant academic and cognitive impact of sickle cell disease, reports describing interventions are limited. There is a lack of awareness among educators of the academic risks associated with sickle cell disease and a lack of appropriate resource allocation. The school nurse, as community health advocate, will be called upon to bridge the gap among healthcare providers, parents, students, and educators. This article provides a review of both recent and landmark studies describing the cognitive and academic impact of sickle cell disease and discusses the role of the school nurse as an advocate, liaison, and educator.
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PMID:The cognitive and academic impact of sickle cell disease. 1712 43

Evidence from past studies indicates that children with traumatic brain injury experience difficulties with inhibitory control. Less is known about inhibitory control in children with frontal brain injury related to cerebral infarction. We compared the inhibitory performance of children with frontal infarcts related to sickle cell disease with that of a control group of children with sickle cell disease but no history of cerebral infarction. On a stimulus-response reversal task, children with frontal infarcts made significantly more accuracy errors in the inhibitory condition than controls. Findings from this study and from previous research suggest that impairments in inhibitory control are common following frontal injury in a range of pediatric populations.
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PMID:Inhibitory control in children with frontal infarcts related to sickle cell disease. 1736 70

Cerebral vasculopathy in sickle cell anemia (HbSS) is manifest clinically as cerebral infarction and intracranial hemorrhage. The type of stroke, ischemic or hemorrhagic, is age specific with distinct differences in outcomes. Cerebral infarction with or without clinical stroke begins during early childhood and rarely causes death immediately.
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PMID:Overt and incomplete (silent) cerebral infarction in sickle cell anemia: diagnosis and management. 1764 76

A patient with homozygous hemoglobin SS disease presented with an intracerebral hemorrhage complicating reversible posterior leucoencephalopathy syndrome (RPLS), secondary to hypertension associated with acute post-streptococcal glomerulonephritis (APSGN). Distinguishing potentially reversible causes of central nervous system events from primary cerebral infarction or hemorrhage in patients with sickle cell disease is important because the management and prognosis of these complications is very different. Similarly, because of the difference in prognosis between APSGN and other forms of sickle cell nephropathy, it is also important to differentiate these conditions.
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PMID:Sickle cell disease complicated by post-streptococcal glomerulonephritis, cerebral hemorrhage and reversible posterior leucoencephalopathy syndrome. 1797 21

An 18-year-old boy was admitted to the hospital for a right leg ulcer, which appeared 1 year ago. After physical and hematologic examinations, he was referred for neurologic and cardiologic examination because his hemoglobin analysis and hematologic findings were interpreted as being consistent with sickle cell anemia. Although he had no neurologic symptoms, MRI and Tc-99m ethyl cysteinate dimer cerebral perfusion single photon emission computer tomography were performed to rule out a silent cerebral infarction. Changes secondary to an infarct were seen in the region of the caudate nucleus.
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PMID:Sickle cell disease with regional silent cerebral infarction detected by SPECT. 1807 16

Arterial spin labeling is a fully noninvasive magnetic resonance perfusion imaging method, ideally suited to pediatric perfusion imaging. We describe an 8-year-old boy with sickle cell disease, extensive right-hemispheric cerebral infarction, and crossed-cerebellar diaschisis apparent on arterial spin labeling perfusion magnetic resonance imaging. To our knowledge, this is the first case of crossed-cerebellar diaschisis demonstrated with arterial spin labeling, highlighting the potential value of perfusion magnetic resonance imaging in the clinical evaluation and follow-up of crossed-cerebellar diaschisis, and the suitability of arterial spin labeling methods for routine perfusion imaging in pediatric patients.
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PMID:Perfusion MRI demonstrates crossed-cerebellar diaschisis in sickle cell disease. 2047 98

Children with sickle cell disease (SCD) and strokes receive blood transfusion therapy for secondary stroke prevention; despite this, approximately 20% experience second overt strokes. Given this rate of second overt strokes and the clinical significance of silent cerebral infarcts, we tested the hypothesis that silent cerebral infarcts occur among children with SCD being transfused for secondary stroke prevention. A prospective cohort enrolled children with SCD and overt strokes at 7 academic centers. Magnetic resonance imaging and magnetic resonance angiography of the brain were scheduled approximately every 1 to 2 years; studies were reviewed by a panel of neuroradiologists. Eligibility criteria included regularly scheduled blood transfusion therapy. Forty children were included; mean pretransfusion hemoglobin S concentration was 29%. Progressive cerebral infarcts occurred in 45% (18 of 40 children) while receiving chronic blood transfusion therapy; 7 had second overt strokes and 11 had new silent cerebral infarcts. Worsening cerebral vasculopathy was associated with new cerebral infarction (overt or silent; relative risk = 12.7; 95% confidence interval, 2.65-60.5, P = .001). Children with SCD and overt strokes receiving regular blood transfusion therapy experience silent cerebral infarcts at a higher rate than previously recognized. Additional therapies are needed for secondary stroke prevention in children with SCD.
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PMID:Silent cerebral infarcts occur despite regular blood transfusion therapy after first strokes in children with sickle cell disease. 2125 94

Sickle cell disease is hereditary hemoglobinopathy which causes haemolytic anemia, vaso-occlusive crisis, ischemic injuries and many other morbidities like cerebral infarction. In this report, we describe a case of a young patient with sickle cell disease presenting with right-sided weakness and slurring of speech with examination confirming right-sided hemiparesis with motor aphasia. On further investigation, she was found to have frontotemporal infarction. On magnetic resonance imaging with angiography, she was found to have absent circulation in left internal carotid artery probably secondary to sickle cell disease. Major vessel occlusion is rare complication of sickle cell disease that one must bear in mind.
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PMID:Major artery occlusion: a rare complication of sickle cell disease. 2141 49

Cerebral infarction is a common complication in sickle cell disease. Both overt and silent infarcts evident on neuroimaging have been described. In this article we overview the current knowledge of cerebral infarction in this patient population and discuss recent updates on the role of preventive intervention.
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PMID:Cerebral infarction in children with sickle cell disease: a concise overview. 2196 73


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