scholarly journals Prevalence and Predisposing Factors of Atrial Fibrillation in a Multi-Ethnic Society: The Impact of Racial Differences in Bahrain

2011 ◽  
Vol 4 ◽  
pp. OJCS.S8032 ◽  
Author(s):  
Taysir Garadah ◽  
Saleh Gabani ◽  
Mohamed Al Alawi ◽  
Ahmed Abu-Taleb
Keyword(s):  
Atrial Fibrillation ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Middle Eastern ◽  
Predisposing Factors ◽  
Cell Disease ◽  
Clinical Events ◽  
History Of ◽  
The Mean ◽  
One Year

Background The prevalence and epidemiological data of atrial fibrillation (AF) among multi-ethnic populations is less well studied worldwide. Aim Evaluation of the prevalence and predisposing factors of AF in patients who were admitted to acute medical emergencies (ER) in Bahrain over the period of one year. Methods Two hundred and fifty three patients with onset of AF were studied. The mean difference of biochemical data and clinical characteristics between Middle Eastern (ME) and sub continental (SC) patients was evaluated. The odds ratio of different predisposing factors for the development of clinical events in AF patients was assessed using multiple logistic regression analysis. Results Out of 7,450 patients that were admitted to ER over one year, 253 had AF based on twelve leads Electrocardiogram (ECG), with prevalence of 3.4%. In the whole study, the mean age was 59.45 ± 18.27 years, with 164 (65%) male. There were 150 ME patients (59%), and 107 (41%) SC, 55 (22%) were Indian (IND) and 48 (19%) were South Asian (SA). In the whole study clinical presentation was of 48% for palpitation, pulmonary edema was of 14%, angina pectoris on rest of 12%, 10% had embolic phenomena, 6% had dizziness, and 7% were asymptomatic. The odds ratio of different variables for occurrence of clinical events in the study was positive of 2.2 for history of hypertension, 1.8 for sickle cell disease, 1.2 for high body mass index (BMI) >30, 1.1 for mitral valve disease. The ME patients, compared with SC, were older, had significantly higher body mass index, higher history of rheumatic valve disease, sickle cell disease with high level of uric acid and lower hemoglobin. The history of hypertension, DM and smoking was higher among the SC patients. The rate of thyroid disease was equal in both groups. Conclusion The prevalence of atrial fibrillation was 3.4% with male predominance of 65%. Patients of sub continental origin were younger with a significantly high history of hypertension and ischemic heart disease. The patients of Middle Eastern origin had significantly high rate of rheumatic heart disease, and sickle cell disease. The history of hypertension was the most important independent clinical predictor of adverse events in patients presented with AF.

Chronic tonsillitis, tonsillectomy and sickle cell crises

1987 ◽  
Vol 101 (5) ◽  
pp. 467-470 ◽  
Author(s):  
G. T. A. Ijaduola ◽  
O. O. Akinyanju
Keyword(s):  
Sickle Cell Disease ◽  
General Anaesthesia ◽  
Sickle Cell ◽  
Chronic Tonsillitis ◽  
Cell Disease ◽  
Pain Crisis ◽  
The Mean ◽  
One Year ◽  
The One ◽  
Sickle Cell Crises

AbstractA study of the effect of tonsillectomy on the frequency of sickle cell pain crises was carried out on 15 patients with sickle cell disease (Hb SS) who presented with complaints of frequent pain crises and were found to have chronic tonsillitis. They comprised 9 females and 6 males and ranged in age from 6 to 35 years, with a mean of 15 years. Tonsillectomy was performed under general anaesthesia. The mean number of pain crises in the one-year period after tonsillectomy was 1.5 and was significantly less than the mean number 4.7 in the one year preceding the operation (p<0.001). Four patients failed to show a reduced number of crises and these were those whose tonsils at operation showed no pus in their tonsillar crypts. It is thus observed that chronic tonsillitis, symptoms of which may not be volunteered at examination, is a potent inductor of sickle cell pain crisis and that tonsillectomy is an effective mode of treatment, especially when the tonsillar crypts contain pus.

scholarly journals Sickle Cell Disease Related Outcomes in Patients Evaluated for COVID-19 Infections in South Carolina

Blood ◽  
2020 ◽  
Vol 136 (Supplement 1) ◽  
pp. 38-39
Author(s):  
Laurence Noisette ◽  
Mamatha Mandava ◽  
Mathew Gregoski ◽  
Shayla Bergmann
Keyword(s):  
South Carolina ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Clinical Presentation ◽  
Self Report ◽  
Mortality Data ◽  
Acute Chest Syndrome ◽  
Cell Disease ◽  
History Of ◽  
The Mean

Introduction: The severe acute respiratory syndrome coronavirus 2 (SARS-Cov-2), first identified in Wuhan, China, was declared a pandemic by WHO in March 2020 due to its high transmission rate. Due to the diffuse vasculo-endothelial damage, individuals with Sickle Cell Disease (SCD) are at risk to develop severe clinical complications, if infected with coronavirus 19 (COVID-19).[1] Given this risk, a systematic evaluation of individuals with SCD presenting with COVID19 infection is paramount to identify the variable clinical manifestations and complications encountered in children and adults with SCD. Methods: A retrospective chart review was conducted from January to June 2020 at the Medical University of South Carolina. We included individuals with sickle cell disease of all genotypes, from 0 to 65 years of age found to be positive for COVID19 by polymerase chain reaction (PCR). Patients' past medical history, clinical presentations, admissions, treatment, complications, and mortality data were reviewed. The data was collected with REDCap@ and descriptive data analysis was conducted per SPSS@. Results: Of the identified 23 patients with SCD who tested positive for COVID during the time specified, 19 (82.6%) had Hgb SS genotype, two had Hgb SC (9%) and two Hgb Sβ+ thalassemia (9%) with similar incidence in both genders (47.8% male and 52.2 % female). All patients were African American. The mean age was 26.13+/-11.53 years. In the last three years they had admissions for pain at a mean of 4.29 +/- 5 and admissions for acute chest syndrome 1+/- 2.2. Six participants (26.1%) had history of mild asthma. Two (8.7%) had pulmonary hypertension. No participants had a history of silent stroke. One participant had history of ischemic stroke, three (13%) had history of pulmonary embolism, and six (26.1%) had deep vein thrombosis (DVT). A variable clinical presentation was noted in our population (Table 1). Of the 23, only nine (39%) required admission of which only one met criteria for intensive care (4.3%) requiring respiratory support with high flow nasal canula. All participants recovered well with the mean length of admission 4.36+/- 3.8 days. Treatment included supportive care including transfusion support, two (8.7%) needed simple transfusion, two (8.7%) needed exchange transfusion. Regarding the laboratory values, coagulations studies were noted to be elevated among all those obtained, but overall limited values were obtained. (Table 2) Thus far no complications of stroke, thrombosis, or pulmonary emboli are noted in the patients positive for COVID in sickle cell disease at our institution. No deaths were reported. Conclusion: Our population reflects what has been described thus far in other cohorts regarding patient demographics, clinical presentation and evolution of disease. Missing laboratory results is most likely due to the mild severity which did not require further clinical evaluation. The absence of VTE/PE may be explained by the low rate of ICU admissions. A similar ICU admission rate of 13% in the same age group as our population was described in a study conducted in France with 83 patients. [2] Compared to a study conducted in Detroit, Michigan, our population underwent comparable rates of transfusions with 3 patients compared to 4 in our population, again most likely due to the mild severity. [3] Our results reflect only MUSC's testing sites and we are dependent on patient's self-report which may not represent our entire population. To address this issue, as part of the Sickle Cell South Carolina network, we are partnering with two other institutions to assess SARS-Cov-2 infection in South Carolina. SARS-CoV-2 pandemic has brought to light many disparities encountered in the American health care system. It is premature to evaluate the immediate and long-term ramifications of COVID19 in individuals with sickle cell disease, due to which we plan to continue to monitor for the next 2 years. References 1. Hussain, F.A., et al.,COVID-19 Infection in Patients with Sickle Cell Disease.Br J Haematol, 2020. 2. Arlet, J.B., et al.,Prognosis of patients with sickle cell disease and COVID-19: a French experience.Lancet Haematol, 2020. 3. Balanchivadze, N., et al.,Impact of COVID-19 Infection on 24 Patients with Sickle Cell Disease. One Center Urban Experience, Detroit, MI, USA.Hemoglobin, 2020: p. 1-6. Disclosures Gregoski: National Institutes of Health under Grant Number UL1 TR001450:Current Employment.

scholarly journals Prevalence and Correlates of Growth Failure in African Patients with Sickle Cell Disease: A Multinational Study

Blood ◽  
2017 ◽  
Vol 130 (Suppl_1) ◽  
pp. 971-971
Author(s):  
Laure Alexandre ◽  
Dapa Diallo ◽  
Aissata Tolo ◽  
Saliou Diop ◽  
Ibrahima Sanogo ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Growth Failure ◽  
Case Control ◽  
Sub Saharan Africa ◽  
Cell Disease ◽  
History Of ◽  
The Mean ◽  
Male Sex ◽  
Sub Saharan

Abstract Introduction Growth failure has been a well-known complication of sickle cell disease (SCD) since the 70s. More recent studies show that the proportion of underweight children with SCD has decreased significantly, thanks to modern treatments (in particular hydroxyurea and iterative transfusions), with a tendency towards overweight and even obesity. However, most studies have been carried out in high-income countries, while 80% of the affected children are born in sub-Saharan Africa, where the environment and the medical care are entirely different. We carried out a case-control study nested in a multinational African cohort to study the growth of sickle cell children and the possible factors influencing growth failure. Methods We performed a case-control transversal study nested in the CADRE cohort that includes SCD patients from five African countries: Cameroon, Gabon, Côte d'Ivoire, Mali and Senegal. All children aged 5 to 21 years-old from this cohort were included in our study. Healthy controls were recruited among the patients' siblings or the children of health workers from each center. The main parameters studied were: medical history, height, weight, blood pressure; hemoglobin phenotype (SS, Sβ0, SC or Sβ +); complete blood count; hemolysis markers (LDH and bilirubin); microalbuminuria; echocardiographic parameters. The primary endpoint was growth failure, defined as a weight, height or BMI below the 5th percentile of the WHO growth charts. We described the frequency of growth failure according to hemoglobin phenotype, age and sex. Then we assessed by multivariate logistic regression in two SCD phenotypic groups (SS or Sβ0 and SC or Sβ +) the association between growth failure and the biological characteristics or the history of SCD-related complications. Results 2296 patients (1799 SS, 114 Sβ0, 287 SC, 96 Sβ+ patients and 287 controls were enrolled in Cameroon (n=735), Ivory Coast (n=380), Gabon (n=298), Mali (n=589) and Senegal (n=581). Overall, 48% of the patients were male and their median [interquartile range] age was 12 [8-16] years-old. Growth failure was diagnosed in 51% of SS, 58% of Sβ0, 44% of SC, 38% of Sβ+patients and 32% of controls, with deeper underweight than linear growth retardation. Beyond the age of 18, the mean BMI of SCD patients was again similar to that of controls in girls, but remained lower in boys, whereas the mean height was similar to that of controls regardless of sex (Figures a to d). Growth failure was more frequent in boys than in girls and maximal between 13 and 16 years-old (Figures e and f). In univariate analysis, the prevalence of growth retardation was associated with the country (highest in Senegal, lowest in Cameroon), age, male sex, hemoglobin phenotype, levels of anemia and hemolysis (p &lt;.0001 for all comparisons). After adjusting for age and the country, the prevalence of growth failure was significantly higher in SS-Sβ0 patients compared to controls (OR = 2.55 [1.83-3.56]) but not different between SC-Sβ+ patients and controls (1.35 [0.91-2.01]). In multivariate analysis, growth failure in SCD patients was positively associated with male sex (OR=1.89, 95%IC=1.56-2.28), [12-15] years age class (OR=2.31, 95%IC=1.75-3.07), SS or Sβ0 phenotypes (OR=2.06 and 2.27, 95%IC=1.26-3.36 and 1.22-4.21, respectively), icterus, leukocytes count, and microalbuminuria (OR = 1.4 [1.1-1.8]). It was negatively associated with parents' secondary or upper education, mean blood pressure and hemoglobin level (OR = 1.96 [1.55-2.47] for the first quartile of hemoglobin level vs others). No association was found between growth failure and the history of clinical SCD-related complications (osteonecrosis, leg ulcers, stroke, priapism, pulmonary hypertension defined by echocardiography and retinopathy). Conclusion In sub-Saharan Africa, growth failure occurs in more than half of the SCD children and concerns weight more than height. Its prevalence is particularly high in SS or Sβ0 patients and during adolescence, due to pubertal delay. Growth failure in SCD children is associated with male sex, anemia and high hemolysis markers independently of the hemoglobin phenotype, but is not independently associated with the occurrence of acute or chronic vascular complications of sickle cell disease, apart from microalbuminuria. However, the long-term consequences of growth failure in sickle cell disease should be evaluated in a longitudinal study. Figure 1 Figure 1. Disclosures No relevant conflicts of interest to declare.

Pica In Children with Sickle Cell Disease

Blood ◽  
2010 ◽  
Vol 116 (21) ◽  
pp. 4807-4807
Author(s):  
Michel Aloni ◽  
Pauline Lecerf ◽  
Catherine Heijmans ◽  
Phu-Quoc Le ◽  
Sophie Huybrechts ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Children And Adolescents ◽  
Sickle Cell ◽  
Zinc Deficiency ◽  
Iron Status ◽  
Biological Data ◽  
Cell Phenotype ◽  
Cell Disease ◽  
History Of ◽  
The Mean

Abstract Abstract 4807 Background: Pica defined as a compulsive and persistent intake of inedible substances or atypical food combinations at least one month. Association of pica and sickle cell disease (SCD) are poorly documented. Alerted by parents, we decided to verify systematically a) the occurrence of pica in SCD children b) to determine its duration, c) to identify the substances commonly ingested and d) to determine the characteristics among children and adolescents with SCD who reported practicing pica. Methods: SCD patients were seen in the outpatient clinic at the University Children's Hospital Queen Fabiola at Brussels between May 1, 2010 and July 30, 2010 as part of their regular follow-up. Data on sex, age, weight, height, body mass index, sickle cell phenotype, G6PD deficiency as well as biological data such as hematocrit mean corpuscular volume, fetal hemoglobin, plasma iron, zinc, copper, lead levels were recorded from their medical chart. Parents and patients were interviewed for the presence of eating disorders. Children and care givers completed questionnaires assessing symptoms of pica. Patients with acute illness, pregnancy, developmental delay, cognitive impairment or age younger than 3 years were excluded. Student t-test was used for difference in mean values groups by pica presence or absence. Fisher exact test was used to compare categorical variable. Difference in means values were considered statistically significant at p < 0.05. Results: Fifty-Five patients (24 males and 31 females) with a median age of 8. 9 years (6. 7– 11. 3) were evaluated during the study period. Forty-five patients (81.8%) originate from Central Africa, 10.9% (6/55) from West Africa, 1.8 %(1/55) from respectively East Africa, Italy and 3.6% (2/55) have mixed origin. Fifty –two (94.5%) patients are homozygous for Hb S (Hb SS), 3 are SC and 1 has Sβ° thalassemia. Thirty-one patients (56.4%) reported an history of pica and during the study period 29.1% (16/55) reported practicing pica regularly. The mean age of patient with pica was 7. 4 years significantly lower than non-pica patients (p <0.05). Pica prevalence by substances ingested was 16 for amylophagia, 14 for paper and powder, 13 for geophagia, 7 for pencils and textiles, 5 for foam, 4 for soap, and 3 for hair. In 6 cases, there was a familial history of pica in non SS individuals. Except for the fact that the mean hematocrit was lower in pica patients compared to non-pica ones (p < 0.05), all the other biological variables were similar in both groups (p> 0.05) (Table 1). Iron status was identical. Lead overload was absent in the entire cohort. Hemolytic phenotype assessed by LDH was identical in both group and mild zinc deficiency was present in both groups. We did not observe difference due to hydroxyurée intake (p>0.05) Conclusion: In our study, pica appeared to have a very high prevalence in SCD children and adolescents. However, its etiology is still unknown. Except lower age and significant lower hematocrit value in pica patients when compared to non-pica ones, no differences in iron status, zinc deficiency or hematological parameters was found. The general health status evaluated by BMI was identical in both groups and the trend to more boys in the pica group is not significant. Further studies are necessary to establish the etiology of pica and its possible consequences on SCD. Disclosures: No relevant conflicts of interest to declare.

Increased Pre-Transfusion Reticulocytosis Among Chronically Transfused Sickle Cell Disease Patients Is Associated With More Severe Cerebral Vasculopathy

Blood ◽  
2013 ◽  
Vol 122 (21) ◽  
pp. 1163-1163
Author(s):  
Megha Kaushal ◽  
Ross M. Fasano ◽  
Colleen Byrnes ◽  
Naomi L.C. Luban ◽  
Emily Riehm Meier ◽  
...  
Keyword(s):  
Magnetic Resonance ◽  
Sickle Cell Disease ◽  
Magnetic Resonance Angiography ◽  
Sickle Cell ◽  
Control Group ◽  
Cell Disease ◽  
Transfusion Therapy ◽  
Blood Samples ◽  
History Of ◽  
The Mean

Abstract Chronic red blood cell transfusion therapy is indicated for primary and secondary stroke prevention in children with sickle cell disease (SCD). The main transfusion goal is achievement of pre-transfusion sickle hemoglobin (HbS) levels of 30%. Unfortunately, there continues to be a population of patients with a history of stroke who have progressive vasculopathy and/or secondary stroke, despite chronic transfusion therapy. Predictive markers for vasculopathy and cerebral events are needed to identify patients at risk for disease progression. Increased reticulocytosis was previously associated with other sickle cell disease complications, and adherent reticulocytes may contribute to the vascular pathology. The objective of this study was to explore the hypothesis that pre-transfusion reticulocytosis may serve as a disease severity marker for cerebral vasculopathy among chronically transfused children with sickle cell disease. After obtaining consent and assent, reticulocytosis was studied in a cohort of pediatric sickle cell patients treated with chronic transfusions (n=33, ages 2-17 years). The group was stratified into three groups: group 1 with an abnormal transcranial doppler (TCD) study in the absence of magnetic resonance angiography (MRA) detected vasculopathy [TCD(+), MRA(-), n=14], group 2 with a history of a stroke in the absence of MRA-detected vasculopathy [Stroke(+), MRA(-), n=5], and group 3 with a history of abnormal TCD or stroke and more severe vasculopathy detected by magnetic resonance angiography [MRA(+), n=14]. Pre-transfusion blood samples were analyzed within 72 hours of collection. Steady-state blood samples were also examined from a control group of pediatric SCD patients (>6 years of age) with normal TCD studies who were receiving supportive care in the absence of chronic transfusions or hydroxyurea (n=7). Hematologic data, including automated complete blood counts, absolute reticulocyte counts (ARC) with reticulocyte maturity were obtained. In addition, a flow cytometric approach was developed to further examine and quantitate reticulocyte subsets based upon staining with thiazole orange combined with CD36, CD45, CD49d, CD71, and CD235. The pre-transfusion HbS levels were not statistically different among the three transfused groups ([TCD(+), MRA(-)]: 30.2 ± 11.8%; [Stroke(+), MRA(-)]: 28.4 ± 3.3%; [MRA(+)]: 33.3 ± 9%, p>0.3). The high levels of reticulocytosis in the pre-transfusion samples were similar to those measured in the control group (ARC: 451 ± 126 K/uL in the chronically transfused cohort; ARC: 369 ± 94 K/uL in the control group, p=0.11). Pre-transfusion reticulocytosis was detected in every chronically transfused subject (ARC range 151-701 K/ul). The mean ARC in the [TCD(+), MRA(-)] group was not significantly different from the [Stroke(+), MRA(-)] group (411 ± 135 K/uL and 396 ± 97 K/uL respectively, p=0.82). However, the mean ARC in the [MRA(+)] group (512 ± 107 K/uL) was significantly higher than the control group, the [TCD(+), MRA(-)] group and the [Stroke(+), MRA(-)] group (p<0.05). The increased ARC in the MRA(+) group included higher absolute numbers of circulating reticulocytes at all stages of maturation including the immature reticulocyte subset [CD36(+), CD71(+)] that was detected in every sample. These data suggest that reticulocytosis with the release of immature CD36(+), CD71(+) cells into the peripheral blood remains as a characteristic feature of pediatric SCD even among chronically transfused patients. More severe vasculopathy, detected by MRA, was associated with significantly higher levels of pre-transfusion reticulocytosis. As such, reticulocytosis should be explored further as a marker or a potential contributor to more severe vasculopathy among chronically transfused children with SCD. Disclosures: No relevant conflicts of interest to declare.

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.

scholarly journals History of Pain is Associated with Hospitalization and Severe Course of COVID-19 in Children with Sickle Cell Disease

2021 ◽  
Vol 22 (5) ◽  
pp. 607-608
Author(s):  
Lana Mucalo ◽  
Amanda Brandow ◽  
Mahua Dasgupta ◽  
Sadie Mason ◽  
Pippa Simpson ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Cell Disease ◽  
History Of ◽  
History Of Pain

The natural history of sickle cell disease

1998 ◽  
Vol 10 (1) ◽  
pp. 49-52 ◽  
Author(s):  
Carolyn Hoppe ◽  
Lori Styles ◽  
Elliott Vichinsky
Keyword(s):  
Sickle Cell Disease ◽  
Natural History ◽  
Sickle Cell ◽  
Cell Disease ◽  
History Of

Attention Deficits in Children with Sickle Cell Disease

2002 ◽  
Vol 95 (1) ◽  
pp. 57-67 ◽  
Author(s):  
Nina A. Nabors ◽  
Angela K. Freymuth
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Adaptive Functioning ◽  
Prior History ◽  
Cell Disease ◽  
Attentional Deficits ◽  
Wide Range Achievement Test ◽  
Wide Range ◽  
History Of ◽  
Timed Test

Previous research has suggested that children with sickle cell disease may exhibit cognitive deficits even in the absence of direct cerebrovascular involvement (stroke). This study was designed to assess specific attentional deficits in children with sickle cell disease. 12 children with sickle cell disease (Hb SS) with a prior history of stroke, 14 children with sickle cell disease (Hb SS) without evidence of stroke, and 13 similar aged siblings (Hb AA or Hb AS) were compared on measures of attention, intellectual functioning, achievement, and adaptive Functioning, Significant differences were found between children with sickle cell disease (with or without stroke) and healthy controls on a timed test of visual scanning, the Coding subtest of the Wechsler Intelligence Scale for Children—Revised, and subtests of Reading, Arithmetic, and Spelling from the Wide Range Achievement Test-Revised. The differences between children with sickle cell disease and their healthy siblings appear to be the result of strokes rather than sickle cell disease itself as children with sickle cell disease without strokes did not significantly differ from controls. Implications for the effects of sickle cell disease and stroke on academic performance are discussed.

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