scholarly journals The Relationship of Transcranial Doppler Ultrasonography with Attention, Motor, and Social-Emotional Functioning in Pediatric Sickle Cell Disease

2019 ◽  
Vol 34 (6) ◽  
pp. 842-842
Author(s):  
M Balderrama ◽  
C Merrill ◽  
A Whitaker ◽  
K Kayser

Abstract Objective Children with sickle cell disease (SCD) are at greater risk for certain neuropsychological deficits due to their medical condition and associated complications. Previous studies have explored the relationship between Transcranial Doppler (TCD) ultrasonography and various domains of neuropsychological functioning in pediatric SCD populations; however, these studies are dated, present variable and inconsistent findings, and are somewhat limited in scope. The goal of this study was to update and expand upon previous research by examining the predictive relationship of TCD results with measures of attention, motor, and social-emotional functioning. Methods Thirty-six patients ages 3-19 with SCD with no known history of stroke, with TCD completed within the past 12 months, underwent a brief neuropsychological exam. Attention, motor, and social-emotional functioning were assessed as appropriate for age using the Conners Continuous Performance Test (K-CPT2/CPT3), Purdue Pegboard, and PROMIS, respectively. TCD values were gathered via medical record review, using the highest value of most recent TCD. Results TCD significantly predicted certain aspects of attention and motor ability, but not social-emotional functioning. Specifically, TCD significantly predicted Detectability (p = .005), Omissions (p = .001), Commissions (p = .012), Perseverations (p = .035), and HRT SD (p = .046) on K-CPT2/CPT-3 and the non-dominant (p = .009) and bilateral (p = .024) trial scores on the Purdue Pegboard. Conclusion This study provides new evidence that TCD may be predictive of motor functioning in pediatric SCD. Results confirm that TCD is predictive of attentional function, though subdomains impacted varied from previous research. While no significant relationship between psychosocial symptoms and TCD were identified, further studies utilizing more comprehensive measurement within this domain is warranted.

Blood ◽  
2013 ◽  
Vol 122 (21) ◽  
pp. 1740-1740
Author(s):  
Rodney S Theodore ◽  
Maa-Ohui Quarmyne ◽  
James Bost ◽  
Carlton Dampier ◽  
Peter A. Lane

Abstract Background Sickle cell disease (SCD) is characterized by marked heterogeneity in clinical manifestations, severity and utilization of health care services. This heterogeneity is particularly evident with regards to utilization of inpatient services, which account for a large percentage of total health care costs and significant absence from school and work. Previous studies of utilization, based on analysis of administrative datasets, have been limited by inaccuracies in ICD-coding, inability to differentiate among SCD genotypes, and/or inability to capture individuals never hospitalized. Single center studies have been limited by potential disease severity referral bias, inability to capture service utilization at outside facilities, and relatively small numbers. Objectives We sought to identify children and adolescents with SCD with high hospital utilization (HHU) and to determine the relationship of HHU with age, SCD genotype, and insurance coverage. We also sought to examine trends over a three year period (2010-2012) and explore the relationship of any trends in hospital utilization over time. Methods The clinical database of the SCD Program at Children's Healthcare of Atlanta (CHOA) was used to identify all patients with SCD, ages ≥ 5 years, who received comprehensive outpatient, acute care, and inpatient services at three CHOA facilities in metro Atlanta during 2010-2012. SCD genotype was confirmed for each patient by review of hematologic and clinical data, including results of diagnostic hemoglobin electrophoresis. All outpatient clinic, emergency department, and inpatient utilization was captured as well as insurance (payer) status at each encounter. HHU was defined as ≥3 admissions during a calendar year. Data were analyzed for significance by Chi-square. The extent to which the data were population-based was determined from the Georgia Hospital Association database with includes DRG-level data for all inpatient hospitalizations in Georgia. Results Hospitalizations at CHOA accounted for 96.1% of all SCD admissions for children within the 28-county greater metro Atlanta area. During 2010-2012, 1348 individual children and adolescents ≥ 5yr of age with SCD received comprehensive care at CHOA (1076 in 2010, 1112 in 2011, 1159 in 2012): 928 (68.8%) had SS/S β°thal, 334 (24.8%) SC, 86 (6.4%) S β+thal. Of 1159 active patients in 2012, 441 (38.1%) were 5-9 yr of age, 419 (36.2%) 10-14 yr, 299 (25.8%) ≥ 15 yr; 474 (40.9%) had traditional medicaid (MC), 205 (17.7%) managed-care medicaid (MMC), 374 (32.3%) private payer (PP), and 105 (9.1%) multiple payers (MP). HHU (≥ 3 admissions/yr) decreased from 119 of 1076 (11.1%) in 2010 to 96 of 1112 (8.6%) in 2011 and 95 of 1159 (8.2%) in 2012 (p <.01). The Table shows percentage HHU for each year by age, genotype, and payer. Conclusions For children and adolescents with SCD, HHU decreased during the three year period. HHU was significantly associated with older age and with MC and MP insurance coverage during all three years. HHU was significantly associated with the SS/S β°thal genotype in 2010, but not in 2011-12. The extent to which the decline of HHU among patients with SS/S β°thal genotype was related to increasing use of hydroxyurea is being investigated. Disclosures: Off Label Use: Hydroxyurea is not FDA approved for use in children.


2021 ◽  
Vol 27 ◽  
pp. 107602962110029
Author(s):  
Mira Merashli ◽  
Alessia Arcaro ◽  
Maria Graf ◽  
Matilde Caruso ◽  
Paul R. J. Ames ◽  
...  

The relationship between antiphospholipid antibodies (aPL) and sickle cell disease (SCD) has never been systematically addressed. Our aim was to evaluate potential links between SCD and aPL in all age groups. EMBASE/PubMed was screened from inception to May 2020 and Peto odds ratios for rare events were calculated. The pooled prevalence (PP) of IgG anticardiolipin antibodies (aCL) was higher in individuals with SCD than in controls (27.9% vs 8.7%, P < 0.0001), that of IgM aCL was similar in the two groups (2.9% vs 2.7%); only individuals with SCD were positive for lupus anticoagulant (LA) (7.7% vs 0%, P < 0.0001). The PP of leg ulcers was similar between aPL positive and negative individuals (44% vs 53%) and between patients in acute crisis and stable patients (5.6% vs 7.3%). Reporting of aPL as a binary outcome and not as a titer precluded further interpretation. The results indicate that a prospective case-control study with serial measurements of a panel of aPL in SCD patients might be warranted, in order to understand further the possible pathogenic role of aPL in SCD.


2014 ◽  
Vol 8 (4) ◽  
pp. 31-35
Author(s):  
Sheyda Javadipour ◽  
Shiva Javadipour ◽  
Bijan Keikhaeidehdezi ◽  
Meymanat Akbari

Correction: On 20/08/2014 the spelling of the third author was changed from Bijan Keykhah DehdeziTO Bijan Keikhaeidehdezi.Correction: On 20/08/2014 the affiliation of the first author was changed fromMusculoskeletal Rehabilitation Research Center, Jundishapur University of Medical Sciences of Ahvaz, IranTO Musculoskeletal Rehabilitation Research Center, Ahvaz Jundishapur University of Medical Sciences, Ahvaz, Iran Abstract:Background: psychometric properties of a 9-item self-appraisal scale for studying self-efficacy in sickle cell disease was evaluated in order to survey people’s comprehension of their capabilities to be engaged in functional activities of daily living who were suffering from sickle cell disease and in order to manage syndromes.Methods: the participants of this study were aged between 11-19 years with sickle cell disease under treatment for at least one year.Result: reliability analysis showed the internal consistency and correlation coefficient of the scale to be acceptable values. Construct validity analysis showed moderate positive correlation between the scores obtained using Sickle Cell Self-Efficacy Scale and those obtained using Mental Components Scale of SF36. There is a weak correlation between the score obtained from SCSES and that of Physical Components Scale of SF36.Conclusion: this scale can be used in the future for future studies on self-efficacy and also to find the relationship between this component and other components.DOI: http://dx.doi.org/10.3126/ijls.v8i4.10931


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