scholarly journals Discordance between Self-Report and Genetic Confirmation of Sickle Cell Disease Status in African-American Adults

2014 ◽  
Vol 17 (3) ◽  
pp. 169-172 ◽  
Author(s):  
Christopher J. Bean ◽  
W. Craig Hooper ◽  
Dorothy Ellingsen ◽  
Michael R. DeBaun ◽  
Jennifer Sonderman ◽  
...  
Keyword(s):  
African American ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Disease Status ◽  
Self Report ◽  
Cell Disease ◽  
African American Adults

Daily Mood and Stress Predict Pain, Health Care Use, and Work Activity in African American Adults With Sickle-Cell Disease.

2004 ◽  
Vol 23 (3) ◽  
pp. 267-274 ◽  
Author(s):  
Karen M. Gil ◽  
James W. Carson ◽  
Laura S. Porter ◽  
Cindy Scipio ◽  
Shawn M. Bediako ◽  
...  
Keyword(s):  
Health Care ◽  
African American ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Health Care Use ◽  
Cell Disease ◽  
Work Activity ◽  
African American Adults

Self-perceived Loss of Control and Untreated Dental Decay in African American Adults With and Without Sickle Cell Disease

2006 ◽  
Vol 17 (3) ◽  
pp. 641-651 ◽  
Author(s):  
Brian Laurence ◽  
Dexter Woods ◽  
David George ◽  
Onyinye Onyekwere ◽  
Ralph Katz ◽  
...  
Keyword(s):  
African American ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Loss Of Control ◽  
Cell Disease ◽  
Dental Decay ◽  
African American Adults

Hypnotically Induced Pain Control in Sickle Cell Anemia

PEDIATRICS ◽  
1979 ◽  
Vol 64 (4) ◽  
pp. 533-536
Author(s):  
Lonnie Zeltzer ◽  
Jerry Dash ◽  
J. Paul Holland
Keyword(s):  
Sickle Cell Disease ◽  
Children And Adolescents ◽  
Sickle Cell ◽  
Sickle Cell Anemia ◽  
Pain Control ◽  
Gain Control ◽  
Disease Status ◽  
Similar Technique ◽  
Cell Disease

Recurrent painful vaso-occlusive crises often represent sources of frustration and debilitation to those afflicted with sickle cell disease. We present two adolescents with sickle cell disease who have been able to gain control over the frequency and intensity of these crises by utilizing self-hypnosis. We feel that the utilization of similar technique(s) may allow many ill children and adolescents to obtain mastery over abnormal physiologic processes concomitant with their particular disease status.

scholarly journals Height-corrected low bone density associates with severe outcomes in sickle cell disease: SCCRIP cohort study results

2019 ◽  
Vol 3 (9) ◽  
pp. 1476-1488 ◽  
Author(s):  
Oyebimpe O. Adesina ◽  
James G. Gurney ◽  
Guolian Kang ◽  
Martha Villavicencio ◽  
Jason R. Hodges ◽  
...  
Keyword(s):  
Bone Mineral Density ◽  
African American ◽  
Cohort Study ◽  
Sickle Cell Disease ◽  
Children And Adolescents ◽  
Sickle Cell ◽  
Chronological Age ◽  
Cell Disease ◽  
Mineral Density ◽  
Z Scores

AbstractLow bone mineral density (BMD) disproportionately affects people with sickle cell disease (SCD). Growth faltering is common in SCD, but most BMD studies in pediatric SCD cohorts fail to adjust for short stature. We examined low BMD prevalence in 6- to 18-year-olds enrolled in the Sickle Cell Clinical Research and Intervention Program (SCCRIP), an ongoing multicenter life span SCD cohort study initiated in 2014. We calculated areal BMD for chronological age and height-adjusted areal BMD (Ht-aBMD) z scores for the SCCRIP cohort, using reference data from healthy African American children and adolescents enrolled in the Bone Mineral Density in Childhood Study. We defined low BMD as Ht-aBMD z scores less than or equal to –2 and evaluated its associations with demographic and clinical characteristics by using logistic regression analyses. Of the 306 children and adolescents in our study cohort (mean age, 12.5 years; 50% female; 64% HbSS/Sβ0-thalassemia genotype; 99% African American), 31% had low areal BMD for chronological age z scores and 18% had low Ht-aBMD z scores. In multivariate analyses, low Ht-aBMD z scores associated with adolescence (odds ratio [OR], 7.7; 95% confidence interval [CI], 1.94-30.20), hip osteonecrosis (OR, 4.0; 95% CI, 1.02-15.63), chronic pain (OR, 10.4; 95% CI, 1.51-71.24), and hemoglobin (OR, 0.74; 95% CI, 0.57-0.96). Despite adjusting for height, nearly 20% of this pediatric SCD cohort still had very low BMD. As the SCCRIP cohort matures, we plan to prospectively evaluate the longitudinal relationship between Ht-aBMD z scores and markers of SCD severity and morbidity.

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

Blood ◽  
2010 ◽  
Vol 116 (21) ◽  
pp. 259-259
Author(s):  
Lisa M. Williams ◽  
Zongming Fu ◽  
Pratima Dulloor ◽  
Kun Yan ◽  
John J. Strouse ◽  
...  
Keyword(s):  
Blood Pressure ◽  
African American ◽  
Sickle Cell Disease ◽  
Systolic Blood Pressure ◽  
Sickle Cell ◽  
Vascular Endothelium ◽  
Validation Cohort ◽  
Plasma Samples ◽  
Cell Disease ◽  
Control Subjects

Abstract Abstract 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. Disclosures: No relevant conflicts of interest to declare.

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