scholarly journals Mixed Bullous-Eczematous Contact Dermatitis From a Black Henna Tattoo in an African American Female With Sickle Cell Disease With Post-Dermatitis Pain

Cureus ◽  
2020 ◽  
Author(s):  
Dharam Persaud-Sharma ◽  
Marien Govea ◽  
Robert Hernandez
Keyword(s):  
African American ◽  
Contact Dermatitis ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
African American Female ◽  
Cell Disease

scholarly journals Case Report: Acute Stroke in Young Adult Patient with Moyamoya Syndrome Secondary to Sickle Cell Disease

Blood ◽  
2020 ◽  
Vol 136 (Supplement 1) ◽  
pp. 13-13
Author(s):  
Oladipo Cole ◽  
Asia Filatov ◽  
Javed Khanni ◽  
Patricio Espinosa
Keyword(s):  
Case Report ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Moyamoya Disease ◽  
Conflicts Of Interest ◽  
Acute Chest Syndrome ◽  
African American Female ◽  
Moyamoya Syndrome ◽  
Cell Disease ◽  
Radiographic Findings

Moyamoya disease, well described in literature, is a chronic cerebrovascular occlusive disorder. It is characterized by progressive stenosis/occlusion of the terminal portions of the internal carotid arteries (ICA) and the proximal portions of the middle cerebral arteries (MCA). Less frequently described is Moyamoya syndrome, the name given to radiographic findings consistent with Moyamoya disease, but with an identifiable cause. The diseases associated with Moyamoya Syndrome include Sickle Cell Disease (SCD), Thalassemias, and Down's Syndrome to name a few. Common complications of Moyamoya include both ischemic and hemorrhagic strokes. Upon literature review, Moyamoya syndrome caused by SCD is not well described. When it is, the discussion is centered around the pediatric patient population and surgical management. Our case report describes a 22-year-old African American female with SCD who initially presented with Acute Chest Syndrome. Her hospital course was complicated by development of overt debilitating neurologic deficits. Subsequently, she was found to have Moyamoya Syndrome on neuroimaging. She was successfully treated with medical management without any surgical intervention. This case highlights the necessity of thorough examination, differential diagnosis, imaging findings, and consideration of predisposing syndromes in the work-up for Moyamoya syndrome; especially individuals with Sickle Cell Disease. Disclosures No relevant conflicts of interest to declare.

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.

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

scholarly journals Fetal Hemoglobin Levels in African American and Hispanic Children With Sickle Cell Disease at Baseline and in Response to Hydroxyurea

2011 ◽  
Vol 33 (7) ◽  
pp. 496-499 ◽  
Author(s):  
Katherine L. Ender ◽  
Margaret T. Lee ◽  
Sujit Sheth ◽  
Maureen Licursi ◽  
Jennifer Crotty ◽  
...  
Keyword(s):  
African American ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Fetal Hemoglobin ◽  
Cell Disease ◽  
Hispanic Children

scholarly journals Information-Seeking Coping Behaviors During Painful Procedures in African-American Children with Sickle Cell Disease

2013 ◽  
Vol 14 (3) ◽  
pp. e54-e58 ◽  
Author(s):  
Alyssa M. Schlenz ◽  
Jeffrey Schatz ◽  
Catherine B. McClellan ◽  
Sarah M. Sweitzer ◽  
Carla W. Roberts
Keyword(s):  
African American ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Information Seeking ◽  
African American Children ◽  
Coping Behaviors ◽  
Cell Disease ◽  
American Children

scholarly journals Lupus Nephritis in a Patient with Sickle Cell Disease

2013 ◽  
Vol 2013 ◽  
pp. 1-4
Author(s):  
Vinay Minocha ◽  
Fauzia Rana
Keyword(s):  
Sickle Cell Disease ◽  
Lupus Nephritis ◽  
Renal Biopsy ◽  
Sickle Cell ◽  
Lupus Erythematosus ◽  
Early Recognition ◽  
African American Female ◽  
Cell Disease ◽  
Diagnostic Challenge ◽  
Renal Abnormality

Introduction. The diagnosis of systemic lupus erythematosus (SLE) in patients with sickle cell disease (SCD) can be difficult to establish because the musculoskeletal, central nervous system, and renal manifestations are similar in both diseases. In the presented case, we highlight the diagnostic challenge that can evolve in patients with a concurrence of both diseases and we establish the importance of early recognition and treatment of lupus nephritis in patients with SCD.Case Presentation. We present a case of a 31-year-old African American female with sickle-C disease (hemoglobin SC) who was admitted to our hospital with complaints of periumbilical abdominal pain associated with intractable nausea and vomiting, abdominal distension, and worsening lower extremity edema. Urine studies revealed nephrotic range proteinuria and the immunological investigations were consistent with lupus. A renal biopsy revealed focal proliferative lupus nephritis.Conclusion. It is important to consider the presence of a coexisting autoimmune disease in a patient with sickle hemoglobinopathy who displays an atypical and multisystem presentation that is unresponsive to conventional therapies. When a significant kidney disease is present, a renal biopsy is critical in identifying the etiology of a renal abnormality in the setting of coexisting SLE and SCD.

Sign in / Sign up

Export Citation Format

Share Document