Cerebral Infarction In Children Research Articles

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

Thrombospondin‐1 and <scp>L</scp>‐selectin are associated with silent cerebral infarct in children with sickle cell anaemia

儿童嗜酸性粒细胞增多性心内膜心肌病伴脑梗塞1例报告

特发性嗜酸性粒细胞增多综合征是临床少见疾病，最常见于热带成年男性病人，主要累及皮肤、心脏、中枢神经系统等脏器，儿童少见。累及心脏可引起嗜酸性粒细胞增多性心内膜心肌病，累及中枢神经系统可引起神经行为改变及脑梗塞等。本例患儿为7岁男孩，入院时症状为流涕、喷嚏20天，发热2周，头痛、腹痛3天。入院后查血片嗜酸性粒细胞占80%，超声心动图显示考虑嗜酸细胞增多综合征(loffler心内膜炎)。颅脑MRI：双侧大脑半球多发异常信号，考虑多发微小缺血梗死灶或炎性改变。给予口服泼尼松治疗4周后好转。该病治疗目前无特殊方法，主要依靠糖皮质激素和其他免疫抑制剂。 Idiopathic hypereosinophilic syndrome is minority in clinical. And more cases were found in male adults while only less cases were found in children. This disease could destroy skin, heart and central never system etc. When the heart was destroyed, it could be named hypereosinophilic carditis. And it also could distroy brain and caused cerebral infarction. As following case, we reported a case of hypereosinophilic carditis combined cerebral infarction in child. The case was a 7 years old boy. Its clinical symptoms were shed tears and sneezing for 20 days, fever for 2 weeks, headache and abdominal pain for 3 days. Eosinophilic cells were 80% of all WBC. Heart echo displaied loffler carditis. Head MRI displaied cerebral infarction. After oral prednison for 4 weeks, the boy came through. This disease had no very effective treatment up to now. The sugar cortical hormone and other immunosuppressant might be effective drugs.

Selection of a stroke risk model based on transcranial Doppler ultrasound velocity

Increased transcranial Doppler ultrasound (TCD) velocity is an indicator of cerebral infarction in children with sickle cell disease (SCD). In this article, the parallel genetic algorithm (PGA) is used to select a stroke risk model with TCD velocity as the response variable. Development of such a stroke risk model leads to the identification of children with SCD who are at a higher risk of stroke and their treatment in the early stages. Using blood velocity data from SCD patients, it is shown that the PGA is an easy-to-use computationally variable selection tool. The results of the PGA are also compared with those obtained from the stochastic search variable selection method, the Dantzig selector and conventional techniques such as stepwise selection and best subset selection.

Silent Cerebral Infarctions in Children and Adolescents with Hemoglobin SC Disease

AbstractAbstract 1011▪▪This icon denotes a clinically relevant abstract BackgroundChildren with sickle cell disease (SCD), especially sickle cell anemia, are at high risk of overt and silent cerebral infarctions, leading to physical and cognitive deficits. Less is known about the risks of cerebral infarction in children with Hemoglobin SC (Hb SC). Prior studies have found a prevalence of silent cerebral infarction of 5.8%1 to 46%2 in children with Hb SC disease. We sought to define the prevalence of cerebral infarctions in a population of children and adolescents with Hb SC disease, and to identify medical risk factors for cerebral infarctions. MethodsSince 2006, screening brain magnetic resonance imaging (MRI) exams have been performed on all children with SCD followed at St. Louis Children's Hospital at approximately age 6 years. Furthermore, brain MRI is performed if patients present with possible stroke symptoms, such as severe headache, visual changes, weakness, or seizures. Cerebral infarctions were defined as T2- or FLAIR-weighted hyperintensities visible in at least 2 planes; silent infarcts were diagnosed when the patient had no neurological symptoms that correlated with the infarct lesions. Human Studies Committee approval and waiver of consent was obtained prior to reviewing all brain MRIs from children with Hb SC disease. SPSS version 20 was used for statistical analysis. ResultsBetween January 2004 and May 2012, 95 children and adolescents with Hb SC disease underwent brain MRI; 54% were male. Forty-nine children (51.6%) had no neurological symptoms at the time of the initial MRI; of the 46 children with neurological symptoms, poor school performance (16 children, 16.8%) and headaches (15 children, 15.8%) were cited most commonly. Neurological symptoms provoking MRI included unilateral hearing loss (2 children) and Bell's palsy (3 children).Prevalence of silent infarctions was 14.7% (14/95 children). Seven (50%) of subjects with silent infarctions were male. The mean age at identification of cerebral infarction was 11.9 years (range, 6.2–19.3 years). Five of the infarctions were identified by screening asymptomatic children. Nine children were found to have infarctions while experiencing neurological symptoms; in all cases, the infarct lesions did not explain the presenting neurological symptoms. Among 84 children with initial MRIs that were free of infarctions, 3 developed silent cerebral infarctions subsequently. There was no association between silent cerebral infarctions and a history of asthma, headaches, school difficulties, or school failure.In all cases, the silent cerebral infarctions were located in periventricular, frontal, or parietal white matter; there were no lobar strokes identified. Infarcts ranged in size from 1 mm to 1 cm. All children with silent cerebral infarctions were referred for neurocognitive testing and evaluation for an individualized educational plan. Ten of the 14 children with silent infarctions have had followup MRIs, ranging from 0.1 to 6.4 years following the initial MRI. None have had progressive silent infarct lesions or overt strokes.Magnetic resonance angiography (MRA) was performed in 83 subjects. None of the children had arterial stenosis or occlusion, moyamoya, or aneurysms. Five subjects had subtle irregularities of cerebral arteries noted on MRA, but none progressed to more severe abnormalities. ConclusionApproximately 15% of children and adolescents with Hb SC disease in this retrospective cohort have silent cerebral infarctions, a much higher prevalence than was found in the Cooperative Study of Sickle Cell Disease.1 The prevalence is lower than that of Steen et al's cohort,2 perhaps due to the fact that our center screens all school-aged children. Clinically significant angiographic abnormalities were not identified in this cohort. Children with silent cerebral infarctions should be referred for neurocognitive testing. Further work is needed to define risk factors and treatments for children with silent cerebral infarctions in Hb SC disease. Disclosures:No relevant conflicts of interest to declare.

Silent Cerebral Infarcts in Children With Sickle Cell Anemia

Source: Debaun MR, Sarnaik SA, Rodeghier MJ, et al. Associated risk factors for silent cerebral infarcts in sickle cell anemia: low baseline hemoglobin and relative high systolic blood pressure. Blood. Epub 2011 Nov 17; doi: 10.1182/blood-2011-05-349621Investigators from 29 clinical sites in the United States conducted the Silent Cerebral Infarct Transfusion (SIT) Trial to assess the risk factors for silent cerebral infarcts (SCI). In this cross-sectional study, children with sickle cell anemia (SCA) between the ages of 5 and 15 years with no history of overt stroke or seizures were evaluated with magnetic resonance imaging (MRI) of the brain to screen for SCI. SCI was radiographically defined as MRI signal abnormality measuring at least 3 mm on the T-2 weighted images judged by two of three neuroradiologists on a central review committee. SCI was determined to be truly silent by a central neurology committee’s review of the local neurologist’s examination and the patient’s MRI findings. Clinical history and baseline laboratory values were examined to determine potential risk factors for SCI.Study data were analyzed on 814 participants; the mean age of enrolled children was 9.1 years and 50.7% were male. SCIs were diagnosed in 30.8% (n=251) of study patients. In a multivariable logistic regression analysis, lower baseline hemoglobin concentration (P<.001), higher baseline systolic blood pressure (SBP) (P=.013), and male gender (P=.026) were all associated with a significantly increased risk of a SCI. The odds ratio for SCIs for patients with a hemoglobin less than 7.6 g/dl or SBP greater than 112 mm/Hg were 2.12 and 1.73, respectively. Those individuals who had both a hemoglobin less than 7.6 and SBP greater than 104 had the highest odds of a SCI. In addition, 83% (5 of 6) of patients who carried a diagnosis of hypertension had a higher rate of SCIs than the other study participants (P=.012).The investigators conclude that lower hemoglobin concentration and higher SBP are risk factors for SCI in children with SCA. Further research is needed to determine if interventions targeting these risk factors, such as hydroxyurea, blood transfusion therapy, or antihypertensive medications, can be effective in preventing SCIs.Dr Villella has disclosed no financial relationship relevant to this commentary. This commentary does not contain a discussion of an unapproved/investigative use of a commercial product/device.Although previous investigators have also identified SCIs in approximately one third of patients with SCA, this study is the first to identify a lower baseline hemoglobin and higher SBP as risk factors for SCI in a large multicenter setting.1–3 SCIs may be silent, but they are not benign. SCIs in children with SCA are associated with an increased risk of overt stroke as well as progressive SCIs.4 Additionally, SCIs are associated with poorer cognitive function and school performance as compared to children with SCA without SCIs as well as sibling controls.5–6 Even more concerning is the finding that cognitive function appears to worsen over time in children with SCA and SCIs.7 As children mature and develop into young adults, they may be silently losing IQ points. Indeed, a recent study showed that asymptomatic adults with SCA have significantly lower performance IQs as compared to matched controls.8These findings have important implications for all pediatric practitioners. Many children do not live in close proximity to a sickle cell center and therefore the responsibility of providing health maintenance for these children can fall onto the shoulders of the primary care giver. The American Academy of Pediatrics recommends that all children with SCA receive a thorough assessment of school attendance and performance with consideration for formal neurocognitive testing.9 MRI of the brain should also be considered for children struggling in school, although this remains controversial since a proven intervention to prevent or limit SCIs is lacking. Neurocognitive testing, however, can be extremely helpful in identifying cognitive deficiencies and helping the family and school to optimize the child’s learning environment.There is strong evidence that SCIs in children with SCA are common and associated with significant morbidity. Studies evaluating potential interventions are desperately needed. By identifying risk factors for SCI in this study, the investigators have uncovered potential therapeutic targets which may respond to interventions to decrease the proportion of sickle cells and thereby increase cerebral blood flow, such as hydroxyurea and chronic blood transfusion therapy, or decrease blood pressure through the use of antihypertensive medications. Thankfully, the investigators of the SIT trial are addressing this as well. Patients enrolled into SIT are randomized to receive chronic blood transfusion therapy or standard observation. Follow-up imaging and neurocognitive testing is then performed to ascertain whether chronic blood transfusion therapy can prevent progression of SCIs and stabilize cognitive function. We anxiously await the results.

Cerebral Infarction in Children with Sickle Cell Disease: A Concise Overview

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.

Stroke in the Young

Stroke in the Young

Posttraumatic Cerebral infarction: A Rare Complication in a pediatric patient after mild head injury

Because the cases of post-traumatic cerebral infarction in children are uncommon, little research has been done on this subject. The case of a 14-month-old child who had cerebral infarction after mild head injury is discussed. He fell from a height of approximately 70 cm 12 hours before. He did not use his left arm after the injury. His parents took him to the public hospital, where he was investigated in terms of mechanical complication and was observed for six hours, but no computed tomography (CT) scan was performed at that time. The patient was then presented to our department by his parents. He was not able to walk and was sitting. His neurological examination revealed right hemihypoesthesia, hemiparesis and mild left facial paresis. CT showed a hypodense region in the right basal ganglia location. The right lateral ventricle seemed mildly compressed due to edematous changes. The magnetic resonance imaging revealed hyperintense signal changes that affected the right lentiform nucleus and the head of the caudate nucleus. The aim of the case is to remind emergency physicians that post-traumatic ischemic stroke is uncommon but may be the cause of disability in pediatric patients, and a systematic physical examination must be performed in all ages even if the patients appear quite well.

Do Transfusions Prevent Recurrent Strokes in Children with Sickle Cell Disease

Overt strokes and silent cerebral infarcts in children with sickle cell disease (SCD) result in considerable physical and cognitive disability. Chronic transfusion therapy (TT) reduces the risk for recurrent overt strokes, but whether it also lowers the frequency of silent brain infarcts is unclear. To investigate this issue, a consortium of researchers from seven academic SCD centers conducted prospective magnetic resonance imaging (MRI) studies of the brain in 40 children …

Identification of Thrombospondin-1 and L-Selectin as Potential Plasma Biomarkers of Silent Cerebral Infarct In Children with Sickle Cell Disease Using a Proteomic-Based Approach

AbstractAbstract 259 Objectives:Silent cerebral infarction (SCI) occurs in approximately 27% of children with sickle cell disease (SCD) by age 6 years, and is associated with decreased neurocognitive function and a 14-fold increased risk of progression to overt stroke. While several clinical parameters, such as increased white blood cell (WBC) and platelet counts and decreased hemoglobin (Hb) or hematocrit, have been reported in the literature to be associated with SCI, to date no validated biomarkers exist to predict SCI in patients with SCD. Furthermore, recent unpublished data from the Silent Infarct Transfusion (SIT) Trial has identified systolic blood pressure and total hemoglobin as risk factors. The aim of this study was to identify candidate biomarker plasma proteins that correlate with SCI in patients with SCD. Methods:We used a proteomic discovery approach involving three sequential separation steps to compare the plasma proteomes of 15 children with SCD (7 with SCI and 8 without SCI), aged 5–15 years. Baseline steady-state plasma samples were obtained from the SIT Trial Biologic Repository and matched for age, Hb and WBC. Plasma samples were Hb depleted in the first dimension, separated using immunoaffinity depletion and reverse phase liquid chromatography fractionation, and then trypsin-digested for characterization using label-free quantification on a LTQ-Orbitrap (Thermo) mass spectrometer. The resulting MS/MS data were analyzed using PASS (Integrated Analysis, Bethesda, MD) with X! Tandem searches (www.thegpm.org; version 2008.12.01) of the International Protein Index peptide database (human, 3.19). We measured candidate proteins in a validation cohort of 116 children with SCD (n=65 SCI, 51 non-SCI) and 24 age-matched, healthy African American control subjects using enzyme-linked immunosorbent assays (thrombospondin 1 [TSP1], L-selectin, RandD Systems, Minneapolis, MN) and immunoassays (E- and P-selectin, Mesoscale Discovery, Maryland). All samples were run in duplicate according to the manufacturers' protocols. Statistical differences in biomarker plasma concentrations between groups were compared by the Mann-Whitney U test. Results:TSP1 (5 peptides) and L-selectin (3 peptides) were among 335 proteins that showed differential detection between the SCI and non-SCI groups based on the spectral counts. TSP1 is an extracellular matrix glycoprotein that is involved with platelet aggregation, inhibition of neovascularization and tumorigenesis and has been shown to promote the adherence of sickle erythrocytes to the vascular endothelium. L-selectin is an adhesion molecule that mediates leukocyte interaction with the vascular endothelium. In a validation cohort of 116 children with SCD (n=65 SCI, 51 non-SCI) and 24 age matched, healthy African American control subjects, TSP1 and L-selectin were both significantly increased in SCI vs. non-SCI groups (median 8.5 vs. 6.2 μ g/ml for TSP1, P =0.03; 1.5 vs. 1.4 μ g/ml for L-selectin, P =0.03). As expected, neither TSP nor L-selectin were elevated in the age-matched normal controls (median=4.6 μ g/ml for TSP1, P =0.10, 1.2 μ g/ml for L-selectin, P =0.10). The specificity of the L-selectin results was verified by demonstrating that E-selectin and P-selectin were not increased in the SCI group. TSP1 was correlated with baseline oxygen saturation in both the SCI and non-SCI groups (r=-0.51, and r=-0.35, P<0.001). L-selectin correlated with systolic blood pressure in the SCI group only (r=0.3, P<0.02). Conclusions:TSP1 and L-selectin may represent the first two plasma biomarkers of SCI in children with SCD. Although further studies are needed, these and other potential biomarkers may provide insight into the pathophysiology of SCI, and may fill an important clinical need in identifying children with SCD who are at risk for SCI. [Display omitted] Disclosures:No relevant conflicts of interest to declare.

Acute Silent Cerebral Ischemia Occurs More Frequently Than Silent Cerebral Infarction In Children with Sickle Cell Anemia

AbstractAbstract 268 Background:Children with sickle cell anemia (HbSS) are at high risk of overt stroke and clinically silent cerebral infarction (SCI). SCI is an infarct-like lesion visualized on magnetic resonance imaging (MRI) of the brain that produces no corresponding motor or sensory deficits. The prevalence of SCI in HbSS is approximately 20 – 30% by 16 years of age, but less is known about its incidence. The Cooperative Study of Sickle Cell Disease (CSSCD) found the incidence of new or more extensive SCI in children with HbSS to be 7 events per 100 patient-years. Given that SCI is clinically silent, the only way to determine its incidence (the number of new events occurring in a specific time-period) is to screen with two sequential MRIs of the brain. MRI can also detect acute cerebral ischemia in asymptomatic patients using diffusion-weighted imaging (DWI). The incidence of acute silent cerebral ischemic events (ASCIEs) is not known. A clinical trial setting provides a unique opportunity to determine the incidence of ASCIEs and SCI in children with HbSS. Objectives:To determine the incidence rates of (1) ASCIEs in children with HbSS without prior evidence of focal neurological deficits and (2) new, recurrent SCI in children with HbSS who have pre-existing SCI. Methods:We studied a cohort of children with HbSS and sickle-β0-thalassemia who had brain MRIs for the Silent Infarct Transfusion Trial. All participants had no prior history of overt stroke, seizures, or transient ischemic attacks. ASCIE was defined as an infarct-like lesion on brain MRI without corresponding motor or sensory deficits that appeared as a focus of T2 hyperintensity with restricted diffusion on DWI sequences. SCI was defined an infarct-like lesion without corresponding motor or sensory deficits that appeared as a focus of T2 hyperintensity without restricted diffusion. Given that acute cerebral ischemia appears as a focus of restricted diffusion on DWI for 10 days, we assumed that each MRI scan provided 10 patient-days of observation for detecting ASCIE. Therefore, the incidence of ASCIEs was calculated using a single MRI per patient. The incidence of new or more extensive SCI in children with pre-existing SCI was determined in those who had two MRIs each (screening and pre-randomization). We statistically compared the incidence rates of ASCIEs and SCI obtained by these two different methods. For all ASCIEs and new SCI events, a medical history tool was completed at the local site at the time of MRI of the brain. Results:In total, 972 MRIs were studied (745 screening, 227 pre-randomization). There were 844 MRIs with DWI sequences, providing 23.1 patient-years of observation in 640 children (52% male; mean age 9.7 years). ASCIEs were detected on 1.2% (10 of 844 MRIs), corresponding to an incidence of 43.3 (95% CI: 20.7 – 79.6) events per 100 patient-years. Nine of the 10 ASCIEs were detected incidentally; 1 ASCIE occurred in a participant who was recovering from a recent episode of acute chest syndrome (onset 5 days before MRI) complicated by severe anemia and hypertension. Standard neurological examination was normal in all cases. Two of the 10 participants with ASCIEs had follow-up MRIs of the brain 4 to 10 months later; one had SCI in the same location as the previously detected acute ischemia, but the other had no residual lesion in the same location. Thus, not all ASCIEs produce detectable SCI. A total of 220 participants (55% male; mean age 10.0 years) had both screening and pre-randomization MRIs. The mean interval between the two MRIs was 124.3 days (range: 14 – 645), providing 74.9 patient-years of observation. All screening MRIs showed baseline SCI. New, recurrent SCI was detected on pre-randomization MRI in 8 participants, corresponding to an incidence of 10.7 events per 100 patient-years (95% CI: 4.6 – 21.0). The incidence of ASCIEs was 4-fold higher than recurrent SCI (43.2 vs. 10.7 events per 100 patient-years; P=0.001). Conclusions:The incidence of recurrent SCI was similar to CSSCD findings. However, we show that children with HbSS experience acute cerebral ischemic events far more frequently than previously recognized. These acute ischemic events are mostly clinically silent, potentially reversible radiographically, and not associated with antecedent medical events. Disclosures:No relevant conflicts of interest to declare.

Two young patients with stroke in conjunction with migraineus headache.

In adults, migraine with aura is considered to be a risk factor for stroke, especially when other risk factors exist. However, the relationship between migraine with aura and risk of cerebral infarct in children has not been well established and requires further study. We describe four young patients who suffered from migraine with aura and focal neurological deficits. All four patients had migraine with aura and focal neurological deficits associated with headaches. Two of the four young patients had cerebral infarct during their migraine headaches. Detailed investigation did not reveal the etiology of the cerebral infarct in these two adolescents. No risk factor for stroke was apparent in any of the four patients and no specific risk factor or reliable clinical feature could be used to identify these young patients at risk for irreversible cerebral ischemia. Thus, our observations provide support that there may be an association between migraine with aura and risk of stroke in children. We, therefore, advocate aggressive treatment using prophylactic agents to prevent migraine attacks in children suffering from migraine with aura, especially when auras are prolonged.

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

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.Table:Potential Biomarkers by Group [Mean ± Standard Deviation Compared by T-Test or Median (IQR) by Rank-Sum Test]MarkerSCD: InfarctSCD: No InfarctP-ValueHemoglobin (g/dl)7.7±1.08.3±1.00.01Hemoglobin F (%)8.4 (5.5, 11.5)10.6 (5.1, 16.5)0.18WBC (per ul)12,514±346912,076±35990.60Reticulocytes (%)12.6±6.511.6±4.80.46CRP (mg/L)2.2 (1.6, 5)2 (1.2, 4.3)0.5Apo A1 (mg/dl)114±16124±190.01Apo B (mg/dl)57±1560±200.72Apo B/Apo A10.50±0.130.49±0.180.49LDH (U/L)573±140631±1780.21Apo indicates apolipoprotein [Display omitted] Disclosures:Strouse:General Electric: Equity Ownership. Casella:Boehringer Ingelheim: Honoraria; Cytrex: Research Funding; Ikaria: Research Funding.

Elevated Systolic Blood Pressure and Low Fetal Hemoglobin Are Risk Factors for Silent Cerebral Infarcts in Children with Sickle Cell Anemia.

Abstract 262 Introduction:The most common cause of neurological injury in sickle cell anemia is silent cerebral infarcts (SCI). In the Silent Cerebral Infarct Multi-Center Clinical Trial (SIT Trial) cohort, we sought to identify risk factors associated with SCI. Patients and Methods:In this cross-sectional study, we evaluated the clinical history, baseline laboratory values and performed magnetic resonance imaging of the brain. For those children with SCI-like lesions, a pediatric neurologist examined the child and neuroradiology and neurology committees adjudicated the presence of SCI. Children between the ages of 5 and 15 years with hemoglobin SS or S-beta° thalassemia and no history of overt strokes or seizure were evaluated. Results:A total of 542 children were evaluated; 173 (31.9%) had SCI. The mean age of the children was 9.3 years, with 280 males (51.7%). In a multivariate logistic analysis, two covariates were significant: a single systolic blood pressure (SBP) obtained during a baseline well-visit, p = 0.015 and hemoglobin F (Hgb F) level obtained after three years of age, p = 0.038. Higher values of SBP and lower values of Hgb F increased the odds of SCI; Figure. Baseline values of white blood cell count, hemoglobin level, oxygen saturation, reticulocytes, pain, or ACS event rates were not associated with SCI. [Display omitted] Conclusion:SBP and Hgb F level are two previously unidentified risk factors for SCI in children with sickle cell disease. Modulation of SBP and Hgb F levels might decrease the risk of SCI. Disclosures:No relevant conflicts of interest to declare.

Acute silent cerebral infarction in children with sickle cell anemia

Silent cerebral infarctions (SCI) occur in up to 35% of children with sickle cell anemia (HbSS) but are rarely recognized during the initial 10-14 days when diffusion weighted magnetic resonance imaging (MRI) can differentiate acute infarctions from remote events. We report acute SCI in seven children with HbSS who had areas of restricted diffusion on MRI without persistent focal neurologic deficits. Four had acute SCI identified following acute anemic events. Our observations suggest that SCI are detectible in the acute phase, present with subtle neurologic symptoms, result in permanent neurologic injury, and may be caused by acute anemic events.

Trombolytisk behandling ved arterielt hjerneinfarkt hos barn

After the neonatal period, the incidence of arterial cerebral infarction is 1-2/100 000 children/year. Thrombolysis in cerebral stroke is recommended for adults, but is still controversial for children. The aim of this paper was to provide an overview of documentation on treatment with thrombolysis after arterial cerebral infarction in children. The article is based on literature identified through a non-systematic search in PubMed and own clinical experience in treating young adults with cerebral infarction. In the western world cardiac disease, cardiac interventions and infections are the most important causes of cerebral infarction in children. Children with arterial cerebral infarction should initially be treated as adults, i.e. rapid admission to hospital and immediate imaging, preferably magnetic resonance imaging with diffusion and intracranial angiography. There are no randomized controlled trials of efficacy and safety of thrombolysis in cerebral infarction in children. Thrombolysis is normally not recommended for children because of the lack of scientific evidence. Nevertheless, thrombolysis is used in children with cerebral infarction and case reports are available. The prospect of severe disability should lead to consideration of thrombolysis if age below 18 years is the only contraindication. Treatment of children with thrombolysis should be recorded in an international registry.

Progress in diagnosis and treatment of cerebral infarction in children

脑血栓形成和脑栓塞都是缺血性脑血管病,临床上统称为脑梗死.儿童脑梗死的发病率为8～13/10万[1-2].随着诊断技术的提高,脑梗死病例在婴儿及儿童中检出率越来越高.脑血栓形成多起病缓慢,常于数十小时或数日内病情达到高峰。

Hemorrhage Predisposing to Cerebral Infarction in Children With Moyamoya Disease

Two children with a new diagnosis of hemorrhagic type moyamoya disease experienced cerebral infarction after intraventricular hemorrhage. The patients developed ischemia 15 and 2 days after the initial diagnosis of hemorrhage. No provocative factor such as hypoperfusion was identified, but both patients exhibited signs of increased intracranial pressure on computed tomographic scan before infarction. Increased intracranial pressure in the setting of hemorrhage may be a risk factor for ischemic complications in patients with moyamoya disease. The optimal acute management of these patients requires further study.

Cognitive Screening for Silent Cerebral Infarction in Children With Sickle Cell Disease

Silent cerebral infarctions have been shown to cause major morbidity in children with sickle cell disease, suggesting that silent infarctions are not as "silent" as once thought. The current definition of silent infarction includes signal changes on magnetic resonance imaging, the absence of overt abnormalities on neurologic examination, and no history of focal neurologic event. Using a decision tree algorithm, we identified a cognitive profile distinguishing children with (n=16) and without (n=49) silent infarctions. The best model combined learning slope from the California Verbal Learning Test-Children's Version and Block Design from the Wechsler Abbreviated Scale of Intelligence. Accuracy was 75%, with 75% sensitivity and 76% specificity. Administration of a brief cognitive battery may be the most feasible approach to screen for silent infarctions in children with sickle cell disease.

MR imaging and 1H-MR spectroscopy in a case of cerebral infarction with transient cerebral arteriopathy

A case of cerebral infarction with transient cerebral arteriopathy in a 12-year-old female is described. The child showed with an acute onset of left hemiplegia, central facial palsy and aphasia. She was diagnosed with right internal carotid artery obstruction with no predisposing factors for cerebral infarction. A serial MR image and MR spectroscopy of the brain was performed. MR angiography revealed reversible vascular changes in the right middle cerebral artery after several months. The ratio of choline/creatine was elevated and that of N-acetylaspartate (NAA)/creatine was depressed in the cerebral lesion, however, they were normalized during the remission phase with long echo time-MR spectroscopy. The elevation of the lactate peak was clarified with short echo time-MR spectroscopy. The longitudinal follow up using MR angiography, and MR spectroscopy with long and short echo time was useful for the evaluation of reversible vascular change with cerebral infarction in children.