scholarly journals Comprehensive Assessment of Cognitive Function in Patients with Sickle Cell Disease Reveals Deficits in Memory and Processing Speed

Blood ◽  
2021 ◽  
Vol 138 (Supplement 1) ◽  
pp. 975-975
Author(s):  
Gerard Portela ◽  
Meryl Butters ◽  
Maria M Brooks ◽  
Leticia Candra ◽  
Caterina Rosano ◽  
...  
Keyword(s):  
Executive Function ◽  
Cognitive Function ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Processing Speed ◽  
Severe Disease ◽  
Cognitive Domain ◽  
Cell Disease ◽  
History Of ◽  
Domain Scores

Abstract Cognitive impairment (CI) is a serious complication of sickle cell disease (SCD) and can cause significant functional and social limitations. To date, there are only a few studies characterizing CI in adults with SCD. In this study, we comprehensively assessed cognitive function in a cross-sectional sample of patients with SCD compared to healthy controls across ten memory and processing speed domains. Patients with SCD (HbSS, HbSC and HbS/beta thalassemia) ages 18 years and older were recruited from the University of Pittsburgh Medical Center Adult Sickle Cell Program, and sex- and age-matched controls were recruited from the community and by SCD patient referral. Unlike previous studies, we did not restrict participation to patients with HbSS only and we included all SCD comorbidities. All participants underwent neurocognitive assessments using measures that are validated and accurate in discriminating CI from dementia and normal cognition. We conducted linear regression analyses to estimate the difference in each cognitive domain score between patients and controls. We also contrasted pairwise comparisons between controls and patients with and without stroke, with and without chronic blood transfusions, and classified by disease severity based on genotype. Among 86 SCD patients and 66 controls, 43% were male and the average age was 36.2 years. SCD patients had fewer years of education on average (13.3 vs. 14.2) and a higher percent had a history of smoking (46% vs. 29%). Additionally, 45% of patients had milder disease (HbSC or HbSb +-thalassemia) with the rest having severe disease (HbSS or HbSb 0-thalassemia)), 14% had a history of stroke, and 20% were receiving chronic exchange transfusions. Patients with SCD had significantly lower scores on five out of ten cognitive domains: language (b=-5.88, P=0.014), attention/processing (b=-14.47, P<0.001), RBANS total score (b=-7.55, P<0.001), executive function (b=-1.60, P<0.001), and DSST scaled score (b=-2.08, P<0.001). Adjusting for pre-morbid verbal IQ (VIQ), hemoglobin level, and smoking history, attention/processing (b=-10.70, P=0.001), RBANS total (b=-7.27, P=0.003), and DSST scaled scores (b=-1.54, P=0.019) were significantly lower among patients compared to controls. In adjusted models, there were no significant differences in cognitive domain scores between patients with mild disease compared to patients with severe disease; both mild and severe disease subgroups had lower attention/processing scores compared to controls (both P<0.05). Patients with SCD and a history of stroke had significantly lower attention/processing, executive function, RBANS total, and DSST scaled scores (all P<0.01) compared to controls, and SCD patients who did not have a history of stroke had lower attention/processing and RBANS total scores than controls (both P<0.05). Patients with a history of stroke had significantly lower executive function (b=-1.65, P=0.012), RBANS total (b=-8.64, P=0.020), and DSST scaled scores (b=-2.43, P=0.015) than patients without a history of stroke. There were no significant differences in cognitive domain scores between patients receiving chronic transfusions and those not receiving transfusions. To our knowledge, this is the most inclusive real-life study of adult patients with SCD to investigate cognition to date, further characterizing CI among this patient population. Many of the disparities in cognitive function between patients and controls are not explained by pre-morbid VIQ, hemoglobin levels or smoking, indicating further research is needed to understand the pathogenesis of CI in SCD. Figure 1 Figure 1. Disclosures Novelli: Novartis Pharmaceuticals: Consultancy.

scholarly journals Alkaline Phosphatase Is Associated with Red Cell Alloimmunization in the Pulmonary Hypertension and Hypoxic Response (PUSH) Sickle Cell Disease Cohort

Blood ◽  
2020 ◽  
Vol 136 (Supplement 1) ◽  
pp. 20-20
Author(s):  
Victoria Brooks ◽  
Oluwalonimi Adebowale ◽  
Victor R. Gordeuk ◽  
Sergei Nekhai ◽  
James G. Taylor
Keyword(s):  
Risk Factors ◽  
Alkaline Phosphatase ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Research Funding ◽  
Severe Disease ◽  
Case Control ◽  
Red Cell ◽  
Cell Disease ◽  
History Of

Background: Blood transfusion is a common therapy for sickle cell disease (SCD). Although, highly effective, a major limitation is development of alloantibodies to minor blood group antigens on donor red cells. Alloimmunization has a prevalence of 2-5% for transfusions in the general population, but it is significantly higher in SCD. Risk factors for alloimmunization have been poorly characterized, although number of lifetime transfusions is an important risk factor. Alloimmunization has been clinically observed in children with a prevalence of about 7%. With development of each antibody, blood donor matching becomes increasingly difficult and expensive with an increased risk for transfusion reactions and diminished availability of compatible red cell units for treatment of SCD. The ability to identify risk factors for developing alloantibodies would be beneficial for clinicians. To identify markers for alloimmunization in SCD, we have analyzed children and adults who developed this complication. Methods: We analyzed The Pulmonary Hypertension and Hypoxic Response in Sickle Cell Disease (PUSH) study, which enrolled n=468 pediatric and n=59 adult SCD subjects. In both children and adults, alloimmunization cases were defined as a history of at least 1 alloantibody. Controls in both cohorts were defined as subjects with no history of alloantibodies and receipt of more than 10 lifetime red cell transfusions. All others within the study who did not meet these criteria were assigned to a third comparison group. To identify differences between cases, controls and all others, we performed univariate analyses (using ANOVA or Kruskal Wallace where appropriate) for clinical parameters and laboratories. Case control comparisons were also performed for selected variables and plasma levels for 11 cytokines. Results were further analyzed using regression modeling. Results: The overall prevalence of alloimmunization was 7.3% among children (34/468 subjects; median age 12, range 3-20 years) compared to 28.8% in adults (17/59 subjects; median age 37, range 18-73 years). When only considering those with >10 lifetime transfusions, the prevalence was considerably higher at 29.3% and 54.8% in children and adults, respectively. At the same time, 8 pediatric (23.5%) and 5 adult (29.4%) alloimmunization cases had received fewer than 10 transfusions. In a 3-way pediatric cohort comparison (cases, controls and all others), risk factors associated with alloimmunization included SS genotype, older age and markers of more severe disease (higher ferritin, WBCs, platelets and total bilirubin). Comparison of cases to controls showed alkaline phosphatase (P=0.05) was significantly lower in cases, whereas AST (P=0.02) was significantly higher even with adjustment for age. Levels of plasma cytokines MCP-1 (P=0.01) and IFNgamma (P=0.08) were lower in cases from a subset of the pediatric cohort. In adults, only 4/59 (6.8%) subjects had never received a lifetime transfusion (all non-SS). In the adult 3-way comparisons, only SS genotype and higher ferritin were associated with alloimmunization. The adult case control analysis showed higher absolute monocyte count (P=0.02), absolute eosinophil count (P=0.04) and absolute basophil count (P=0.008) in association with alloimmunization cases. In addition, alkaline phosphatase was again significantly lower among cases (P=0.02) as seen in the pediatric cohort. There were no significant differences in cytokine levels among adults. Conclusions: When considering only transfused SCD patients, the prevalence of alloimmunization is higher than 30%. As seen in prior studies, higher lifetime red cell transfusions are an important risk factor especially among adults where most patients have received transfusions. Children who develop alloantibodies appear to have laboratory markers of more severe disease, but this is not observed in adults. A novel association observed across both pediatric and adult subjects is a significantly lower serum alkaline phosphatase in those with alloantibodies. The results of this study suggest a need for improved tracking of red cell transfusion therapy in the US for SCD patients due to a high prevalence of alloimmunization. Further study is also needed to elucidate the significance of the alkaline phosphatase association. Disclosures Gordeuk: CSL Behring: Consultancy, Research Funding; Global Blood Therapeutics: Consultancy, Research Funding; Novartis: Consultancy; Ironwood: Research Funding; Imara: Research Funding.

Cognitive Function Of Nigerian Children With Sickle Cell Disease

Blood ◽  
2013 ◽  
Vol 122 (21) ◽  
pp. 1008-1008
Author(s):  
Olubusola Oluwole ◽  
Robert Noll ◽  
Julie Makani ◽  
Enrico M Novelli
Keyword(s):  
Working Memory ◽  
Cognitive Function ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Teaching Hospital ◽  
Cognitive Deficits ◽  
Processing Speed ◽  
University Teaching ◽  
Cell Disease ◽  
Intelligence Scale

Abstract Background Sickle cell disease (SCD) -related neurological complications include overt stroke, silent infarctions and cognitive impairment (CI). CI significantly impacts developmental growth and quality of life. Despite the high prevalence of SCD in sub-Saharan Africa, there is limited information on the burden of neurological dysfunction. To address this gap, we conducted a study to elucidate the prevalence and correlates of CI in SCD-children in an urban tertiary care setting in Nigeria. Method This case-control, cross-sectional study was approved by the University of Pittsburgh IRB, Lagos University Teaching Hospital (LUTH) and Lagos State University Teaching Hospital (LASUTH) in Nigeria. Participants were recruited from the LUTH sickle cell clinic and the Sickle Cell Foundation-associated clinics, which included a transcranial Doppler (TCD) clinic. Participants were English-speaking children between the ages 6-16 with laboratory-diagnosed homozygous SCD (HbSS, sickle cell anemia). Children who had an on-going sickle cell crisis or who received a blood transfusion within 3 months were excluded from the study. HbAA siblings of the patients and unaffected age-matched children from the LASUTH pediatric primary care clinic were recruited as control subjects. For a comprehensive assessment of cognitive function, subtests of the Wechsler Intelligence Scale for Children (WISC IV) were administered to assess processing speed (Symbol Search, Coding subtests) and Working Memory (Digit Span, Symbol search subtests) indices. Baseline hemoglobin levels were obtained via a Stat-site hemoglobin analyzer, and oxygen saturation levels were obtained using a pulse oximeter. Baseline demographic data was obtained by surveying the parents/guardians. Lastly, children recruited from the Sickle Cell Foundation TCD clinic (n=24) were stratified by stroke risk levels (standard, conditional, high and indeterminate) based on the TCD velocity values. Results A total of 56 children diagnosed with sickle cell disease (M=29, mean age=9.2, SD: 2.76) and 42 unaffected children (M=24, mean age= 9.41, SD: 2.75) participated in this study. We found a higher prevalence of cognitive deficits, especially in areas of processing speed (p=0.013) and short-term auditory memory (p=0.002) in SCD patients as compared to controls. There was also a close association with working memory deficits (p=0.07). Surprisingly, we did not find an association between the cognitive performance of SCD children and their levels of anemia, oxygen levels or body mass index. TCD high risk level was marginally associated with a lower hemoglobin and severe anemia (p=0.05) although there was no significant correlation with working memory and processing speed indices. Conclusion Children with SCD in Nigeria suffer from cognitive deficits in the areas of memory and attention when evaluated with subtests of the Wechsler Intelligence Scale for Children. We found that, unlike other studies of cognitive function in SCD, our study did not find a correlation between cognitive function and hemoglobin level. It is, therefore, possible that other local environmental or disease-specific factors may be associated with CI in our SCD cohort, or that anemia may affect other cognitive domains not explored by our research. Larger, longitudinal studies should be performed to further elucidate the cognitive function of pediatric SCD patients in Nigeria so that appropriate, locally targeted, preventive interventions can be developed. Disclosures: No relevant conflicts of interest to declare.

scholarly journals In Africa, a High Proportion of Adults with HbSC Meet American Society of Hematology's Eligibility Criteria for Severe Sickle Cell Disease and Starting Hydroxyurea Therapy in a Clinical Trial Setting

Blood ◽  
2021 ◽  
Vol 138 (Supplement 1) ◽  
pp. 487-487
Author(s):  
William Kwesi Ghunney ◽  
Eugenia Vicky Asare ◽  
John Ayete-Nyampong ◽  
Yvonne Adomakoh ◽  
Adetola A. Kassim ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Severe Disease ◽  
Incidence Rates ◽  
Cell Disease ◽  
Eligibility Criteria ◽  
Chronic Anemia ◽  
History Of ◽  
Hydroxyurea Therapy ◽  
Trial Setting

Abstract Introduction : Sickle cell disease (SCD), HbSC, is the second most frequent hemoglobinopathy after HbSS. Worldwide, an estimated 54,736 babies are delivered annually with HbSC disease, with the highest HbC gene frequency in West Africa (Piel et al.2013). A retrospective study at the Ghana Institute of Clinical Genetics [(GICG), a sickle cell clinic] reported that 40% of the 3,000 SCD patients attending the clinic yearly are HbSC (Asare et al.2018). HbSC disease usually results in a comparatively milder form of SCD. However, rates of maternal-fetal morbidity, retinopathy, avascular necrosis (AVN) of the hip, priapism, and chronic kidney disease are increased (Powars et al.2002; Oppong et al.2018). While microalbuminuria is lower in HbSC than HbSS, it still occurs in >23% of adults (Drawz et al.2016). In addition, thrombosis, silent cerebral infarction, sensorineural hearing loss, and pulmonary hypertension may be higher than previously suspected (Sathi et al.2019). Unlike HbSS, hydroxyurea is not routinely recommended for HbSC patients because they are all perceived to have a milder phenotype, with improved life expectancy. Thus, HbSC individuals are often excluded from randomized controlled trials in children and adults with SCD (Luchtman-Jones et al.2016). Given the high proportion of adults with HbSC in Ghana, who may benefit from hydroxyurea therapy, we tested the hypothesis that at least 5% of adults with HbSC will meet the ASH criteria for severe disease and treatment. Data indicating that a significant proportion of adults with HbSC are eligible for hydroxyurea could potentially support pilot safety trials to determine the optimal hydroxyurea dose to ameliorate symptoms while limiting toxicity. Data can also facilitate government health policy decisions to subsidize hydroxyurea costs. Methodology : We conducted a medical chart review of all adults with HbSC disease (total: 639; 18-45years) who attended the SCD clinic, GICG (January 1, 2019, to December 31, 2019). We identified a comparison group of 639 adults with HbSS, age, and gender-matched to the HbSC patients from the same clinic. Severe complications were defined as a history of ≥3 sickle cell-associated moderate to severe pain episodes/year, a history of severe acute chest syndrome (ACS), and severe symptomatic chronic anemia that interferes with daily activities or quality of life (Hydroxyurea and Transfusion Therapy for the Treatment of Sickle Cell Disease.2014). We defined acute pain episode as new onset of pain that lasts at least 4 hours, for which there is no explanation other than vaso-occlusion, which requires therapy with parenteral opioids in a medical setting (Ballas et al.2010). Severe symptomatic chronic anemia was defined as a drop in hemoglobin by ≥2g/dL below the steady state. Data were analyzed using SPSS version 23 and summarized as simple descriptive statistics. Study endpoints were the proportion of individuals with SCD who met the definition of severe disease and were eligible for hydroxyurea. We also calculated the pain and ACS incidence rates. Results: The 639 HbSC participants had a mean age of 30.8years during the one-year study period, which was identical to that of the HbSS group. At least 8.5% of HbSC adults had ≥three acute pain episodes/ year, while 1.6% had ACS. No HbSC patient had severe symptomatic chronic anemia . In total, 10.0%(64/639) of patients with HbSC disease met the eligibility criteria for hydroxyurea therapy, compared to 24.1%(154/639) of patients with HbSS, p=<0.001. The pain and ACS incidence rates for the HbSC and HbSS individuals were [74.6; 95% CI(67.9-81.3) and 2.3; 95% CI(1.2-3.5)] events per 100 patient-years vs. [123.0; 95% CI(114.4-131.6) and 5.6; 95% CI(3.8-7.5)] events per 100 patient-years respectively. Also, 1.1%(7/639), 7.7%(49/639), 0.5%(4/639), and 4.4%(11/252) of patients with HbSC had papillary necrosis, AVN, stroke, and priapism, respectively while 8.5%(54/639) of patients with HbSS had proteinuria, Table 1. Only 0.9%(HbSC) and 2.3%(HbSS) took hydroxyurea during the study period. Conclusion: Based on ASH's evidence-based guidelines, in a large SCD clinic at an academic medical center in Ghana, 10.0% of HbSC adults meet the criteria for shared decision-making to consider starting hydroxyurea therapy in a clinical trial setting. The optimal dose of hydroxyurea that maximizes benefit and minimizes toxicity in adults with HbSC is yet to be determined. Figure 1 Figure 1. Disclosures Asare: ASH Global Research Award: Research Funding.

scholarly journals Study of montelukast in children with sickle cell disease (SMILES): a study protocol for a randomised controlled trial

Trials ◽  
2021 ◽  
Vol 22 (1) ◽  
Author(s):  
Anna M. Hood ◽  
Hanne Stotesbury ◽  
Melanie Kölbel ◽  
Michelle DeHaan ◽  
Michelle Downes ◽  
...  
Keyword(s):  
Cognitive Function ◽  
White Matter ◽  
Sickle Cell Disease ◽  
Young Children ◽  
Sickle Cell ◽  
Processing Speed ◽  
Post Treatment ◽  
Cell Disease ◽  
Double Blind ◽  
Randomised Controlled

Abstract Background Young children with sickle cell anaemia (SCA) often have slowed processing speed associated with reduced brain white matter integrity, low oxygen saturation, and sleep-disordered breathing (SDB), related in part to enlarged adenoids and tonsils. Common treatments for SDB include adenotonsillectomy and nocturnal continuous positive airway pressure (CPAP), but adenotonsillectomy is an invasive surgical procedure, and CPAP is rarely well-tolerated. Further, there is no current consensus on the ability of these treatments to improve cognitive function. Several double-blind, randomised controlled trials (RCTs) have demonstrated the efficacy of montelukast, a safe, well-tolerated anti-inflammatory agent, as a treatment for airway obstruction and reducing adenoid size for children who do not have SCA. However, we do not yet know whether montelukast reduces adenoid size and improves cognition function in young children with SCA. Methods The Study of Montelukast In Children with Sickle Cell Disease (SMILES) is a 12-week multicentre, double-blind, RCT. SMILES aims to recruit 200 paediatric patients with SCA and SDB aged 3–7.99 years to assess the extent to which montelukast can improve cognitive function (i.e. processing speed) and sleep and reduce adenoidal size and white matter damage compared to placebo. Patients will be randomised to either montelukast or placebo for 12 weeks. The primary objective of the SMILES trial is to assess the effect of montelukast on processing speed in young children with SCA. At baseline and post-treatment, we will administer a cognitive evaluation; caregivers will complete questionnaires (e.g. sleep, pain) and measures of demographics. Laboratory values will be obtained from medical records collected as part of standard care. If a family agrees, patients will undergo brain MRIs for adenoid size and other structural and haemodynamic quantitative measures at baseline and post-treatment, and we will obtain overnight oximetry. Discussion Findings from this study will increase our understanding of whether montelukast is an effective treatment for young children with SCA. Using cognitive testing and MRI, the SMILES trial hopes to gain critical knowledge to help develop targeted interventions to improve the outcomes of young children with SCA. Trial registration ClinicalTrials.govNCT04351698. Registered on April 17, 2020. European Clinical Trials Database (EudraCT No. 2017-004539-36). Registered on May 19, 2020

scholarly journals Correlates of Cognitive Function in Sickle Cell Disease: A Meta-Analysis

2020 ◽  
Vol 45 (2) ◽  
pp. 145-155
Author(s):  
Kemar V Prussien ◽  
Rachel E Siciliano ◽  
Abagail E Ciriegio ◽  
Allegra S Anderson ◽  
Radha Sathanayagam ◽  
...  
Keyword(s):  
Executive Function ◽  
Cognitive Function ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Doppler Velocity ◽  
Cell Disease ◽  
Verbal Reasoning ◽  
Negatively Associated ◽  
Visual Spatial ◽  
Perceptual Reasoning

Abstract Objective To provide a comprehensive quantitative review of biological, environmental, and behavioral correlates across domains of cognitive function in sickle cell disease (SCD). Methods Forty-seven studies were identified in PubMed, MedLine, and PsycINFO involving 2573 participants with SCD. Results Meta-analytic findings across all identified samples indicate that hemoglobin and hematocrit were positively correlated with Full Scale IQ [FSIQ; r = .15, 95% confidence interval (CI) = .10 to .21], language and verbal reasoning (r = .18, 95% CI = .11 to .24), and executive function (r = .10, 95% CI = .01 to .19) with small effects and significant heterogeneity. Transcranial Doppler velocity was negatively associated with visual spatial and perceptual reasoning (r = −.18, 95% CI = −.31 to −.05). Socioeconomic status was positively associated with FSIQ (r = .23, 95% CI = .17 to .28), language and verbal reasoning (r = .28, 95% CI = .09 to .45), visual spatial and perceptual reasoning (r = .26, 95% CI = .09 to .41), and executive function (r = .18, 95% CI = .07 to .28) with small to medium effects. Finally, total behavioral problems were negatively associated with FSIQ (r = −.12, 95% CI = −.21 to −.02) such that participants with lower FSIQ exhibited greater behavioral and emotional problems. Conclusions Findings provide evidence for biological, environmental, and psychosocial corelates across multiple domains of cognitive function in SCD. More research on more specific cognitive domains and psychosocial correlates is needed in addition to assessments of interactional models among risk factors.

Cerebrovascular Disease and Cognition in Adults with Sickle Cell Disease

Blood ◽  
2019 ◽  
Vol 134 (Supplement_1) ◽  
pp. 789-789
Author(s):  
Princess Ikemenogo ◽  
Taniya Varughese ◽  
Allison L'Hotta ◽  
Kamilya Hunter ◽  
Anna Bauer ◽  
...  
Keyword(s):  
Working Memory ◽  
Executive Function ◽  
Cognitive Function ◽  
Sickle Cell Disease ◽  
Task Performance ◽  
Processing Speed ◽  
Research Funding ◽  
Mean Difference ◽  
Self Awareness ◽  
Cell Disease

Background: Sickle cell disease (SCD) is a genetic hematologic condition affecting more than 300,000 individuals worldwide. Most of the 100,000 individuals with SCD in the United States are African-American. An estimated 50% of those with the most severe genotype of SCD (HbSS) will have an overt or silent cerebral infarct (SCI) by age 30. While a significant number of studies have addressed the cognitive function and brain imaging of children with SCD, very few have included adults. As disease modification has increased over the last decade, a modern assessment of adults with SCD is needed. The aims of this study are to: 1. Determine the prevalence of cognitive impairment in adults with SCD 2. Determine if adults with SCD have deficits in functional task performance skills 3. Assess if and how cognitive function and task performance change over time Methods: This is a cross-sectional analysis of a prospective, observational cohort study. Adults, 18 years of age or older, with any form of SCD were recruited from the SCD clinics at Washington University in St. Louis. During baseline testing, participants completed the Wechsler Abbreviated Scale of Intelligence (WASI-II), National Institutes of Health Toolbox Cognition Battery (NIHTB-CB), and the Medication subtest of the Executive Function Performance Test (EFPT-M). The WASI-II and NIHTB-CB were repeated annually. Mean participant data from the baseline WASI-II and NIHTB-CB were compared with normative individual measure and composite scores using a one-sample t-test. The NIHTB-CB normative mean is a t-score of 50 with a SD of 10. Baseline results on Medication subtest of the EFPT were compared to previously tested control and stroke groups. Higher scores on the EFPT are indicative of greater executive dysfunction. Differences between Time 1 and Time 2 scores were evaluated using a paired-samples t-test. Univariate analyses were used to describe relationships between cognition and patient factors. Annual magnetic resonance imaging (MRI) was also conducted to assess infarct classification, with completion limited to 12-18 months from consent. Radiographical analyses were reviewed and recorded by the study neuroradiologists. Results: Forty participants were assessed at baseline; 9 completed Time 2 testing. Participant demographic information is presented in Table 1. Compared to the normative population, no significant differences were found on the WASI-II. On the NIHTB-CB, participants scored significantly lower on subtests measuring processing speed (Mean difference -13.8, p <.001), executive function (Mean difference -8.93, p = .001), attention (Mean difference -13.15, p < .001), working memory (Mean difference -5.68, p = .001), overall fluid cognition (Mean difference -13.08, p < .001), and total cognition (Mean difference -5.87, p = .002). Participants scored significantly higher than previously tested control (Mean difference 1.68,p <.001) and mild-stroke groups (Mean difference 1.18, p < .001) on the EFPT-M. Deficits in self-awareness were noted on all performance-based measures. No significant differences were found between participant means on Time 1 vs. Time 2 testing (Mean = 1.02 years, Range =.97-1.12). Participants with more severe phenotypes of SCD (HbSS or HbS-beta thal0) performed lower on the NIHTB test of attention when compared to those with HbSC (Mean difference -6.26, p=.039). History of stroke or use of disease modifiers had no relationship with cognitive outcomes. Of the 59 participants consented to yearly MRI evaluation, 45 have completed baseline imaging and 1 completed Time 2 imaging. Radiographical impressions reported: 1) overt strokes in 2 participants on transfusions (TF), 1 on hydroxyurea (HU), and 1 on no disease-modifying regimen (ND); 2) SCIs in 6 participants on TF, 6 on HU, and 4 on ND; 3) no infarct in 1 participant on TF, 18 on HU, and 9 on ND. Conclusion: Adults with SCD in this relatively highly educated cohort have marked deficits in executive function, attention, working memory, processing speed, and self-awareness. Cognitive deficits lead to difficulty completing everyday functional and disease-related activities, ranging from medication adherence to productivity in education and employment. Healthcare providers who treat individuals with SCD should be aware of the cognitive impairments in this population. Routine cognitive screening is needed to initiate referrals for cognitive rehabilitation. Disclosures King: Magenta Therapeutics: Membership on an entity's Board of Directors or advisory committees; Novimmune: Research Funding; RiverVest: Consultancy; Tioma Therapeutics (formerly Vasculox, Inc.):: Consultancy; WUGEN: Equity Ownership; Incyte: Consultancy; Amphivena Therapeutics: Research Funding; Cell Works: Consultancy; Bioline: Consultancy; Celgene: Consultancy.

scholarly journals Slow Wave Sleep and EEG Delta Spectral Power are Associated with Cognitive Function in Parkinson’s Disease

2020 ◽  
pp. 1-12
Author(s):  
Kimberly H. Wood ◽  
Adeel A. Memon ◽  
Raima A. Memon ◽  
Allen Joop ◽  
Jennifer Pilkington ◽  
...  
Keyword(s):  
Executive Function ◽  
Cognitive Function ◽  
Cognitive Performance ◽  
Slow Wave ◽  
Effect Size ◽  
Processing Speed ◽  
Slow Wave Sleep ◽  
Spectral Power ◽  
Z Scores ◽  
Domain Scores

Background: Cognitive and sleep dysfunction are common non-motor symptoms in Parkinson’s disease (PD). Objective: Determine the relationship between slow wave sleep (SWS) and cognitive performance in PD. Methods: Thirty-two PD participants were evaluated with polysomnography and a comprehensive level II neurocognitive battery, as defined by the Movement Disorders Society Task Force for diagnosis of PD-mild cognitive impairment. Raw scores for each test were transformed into z-scores using normative data. Z-scores were averaged to obtain domain scores, and domain scores were averaged to determine the Composite Cognitive Score (CCS), the primary outcome. Participants were grouped by percent of SWS into High SWS and Low SWS groups and compared on CCS and other outcomes using 2-sided t-tests or Mann-Whitney U. Correlations of cognitive outcomes with sleep architecture and EEG spectral power were performed. Results: Participants in the High SWS group demonstrated better global cognitive function (CCS) (p = 0.01, effect size: r = 0.45). In exploratory analyses, the High SWS group showed better performance in domains of executive function (effect size: Cohen’s d = 1.05), language (d = 0.95), and processing speed (d = 1.12). Percentage of SWS was correlated with global cognition and executive function, language, and processing speed. Frontal EEG delta power during N3 was correlated with the CCS and executive function. Cognition was not correlated with subjective sleep quality. Conclusion: Increased SWS and higher delta spectral power are associated with better cognitive performance in PD. This demonstrates the significant relationship between sleep and cognitive function and suggests that interventions to improve sleep might improve cognition in individuals with PD.

scholarly journals To Give or Not to Give: RhD Immunoglobulin for an RHD*39 Pregnant Woman with Sickle Cell Disease

2021 ◽  
pp. 1-5
Author(s):  
Justin E. Juskewitch ◽  
Craig D. Tauscher ◽  
Sheila K. Moldenhauer ◽  
Jennifer E. Schieber ◽  
Eapen K. Jacob ◽  
...  
Keyword(s):  
Pregnant Woman ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Pregnancy Termination ◽  
Acute Chest Syndrome ◽  
Cell Disease ◽  
Childbearing Age ◽  
Procedural Complications ◽  
History Of ◽  
Chronic Hemolysis

Introduction: Patients with sickle cell disease (SCD) have repeated episodes of red blood cell (RBC) sickling and microvascular occlusion that manifest as pain crises, acute chest syndrome, and chronic hemolysis. These clinical sequelae usually increase during pregnancy. Given the racial distribution of SCD, patients with SCD are also more likely to have rarer RBC antigen genotypes than RBC donor populations. We present the management and clinical outcome of a 21-year-old pregnant woman with SCD and an RHD*39 (RhD[S103P], G-negative) variant. Case Presentation: Ms. S is B positive with a reported history of anti-D, anti-C, and anti-E alloantibodies (anti-G testing unknown). Genetic testing revealed both an RHD*39 and homozygous partial RHCE*ceVS.02 genotype. Absorption/elution testing confirmed the presence of anti-G, anti-C, and anti-E alloantibodies but could not definitively determine the presence/absence of an anti-D alloantibody. Ms. S desired to undergo elective pregnancy termination and the need for postprocedural RhD immunoglobulin (RhIG) was posed. Given that only the G antigen site is changed in an RHD*39 genotype and the potential risk of RhIG triggering a hyperhemolytic episode in an SCD patient, RhIG was not administered. There were no procedural complications. Follow-up testing at 10 weeks showed no increase in RBC alloantibody strength. Discussion/Conclusion: Ms. S represents a rare RHD*39 and partial RHCE*ceVS.02 genotype which did not further alloimmunize in the absence of RhIG administration. Her case also highlights the importance of routine anti-G alloantibody testing in women of childbearing age with apparent anti-D and anti-C alloantibodies.

scholarly journals Concurrent Bilateral Central Retinal Artery Occlusion Secondary to Sickle Cell Crisis

2021 ◽  
Vol 9 ◽  
pp. 232470962110283
Author(s):  
Gowri Renganathan ◽  
Piruthiviraj Natarajan ◽  
Lela Ruck ◽  
Roberto Prieto ◽  
Bharat Ved Prakash ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Vision Loss ◽  
Central Retinal Artery Occlusion ◽  
Retinal Artery Occlusion ◽  
Artery Occlusion ◽  
Cell Disease ◽  
Sickle Cell Crisis ◽  
History Of ◽  
Retinal Artery

Vascular occlusive crisis with a concurrent vision loss on both eyes is one of the most devastating disability for sickle cell disease patients. Reportedly occlusive crisis in the eyes is usually temporary whereas if not appropriately managed can result in permanent vision loss. A carefully managed sickle cell crisis could prevent multiple disabilities including blindness and stroke. We report a case of a 24-year-old female with a history of sickle cell disease who had acute bilateral vision loss during a sickle crisis and recovered significantly with a timely emergent erythrocytapheresis.

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