Seizures risk factors in sickle cell disease. The cooperative study of sickle cell disease

Nawaiseh, Mohammed; Shaban, Ala; Abualia, Mohammad; Haddadin, Rund; Nawaiseh, Yara; AlRyalat, Saif Aldeen; Yassin, Ahmed; Sultan, Iyad

doi:10.1016/j.seizure.2021.05.009

Cited by 6 publications

(2 citation statements)

References 36 publications

(52 reference statements)

Supporting

Mentioning

Contrasting

Order By: Relevance

“…Further research is warranted to investigate the connection between the kidney and the retina in SCD. Although, a previous report from the CSSCD had found an association between eye diseases and neurological manifestation of SCD, such as seizures and strokes, we did not find an association between the neurological manifestations of SCD and the development of SCR [36].…”

Section: Discussioncontrasting

confidence: 99%

Risk factors associated with sickle cell retinopathy: findings from the Cooperative Study of Sickle Cell Disease

et al. 2022

Self Cite

View full text Add to dashboard Cite

Background Sickle cell retinopathy (SCR) is one of the most important ocular manifestations of sickle cell disease (SCD). This study aims to assess the prevalence of SCR in SCD, identify risk factors for its development and progression to proliferative sickle cell retinopathy (PSCR), and evaluate the potential implications of these results on clinical practice. Methods This research is a secondary analysis of patients diagnosed with SCD from the epidemiological, multicenter Cooperative Study of Sickle Cell Disease (CSSCD). We included all patients who completed a full ophthalmic evaluation. We identified clinical and laboratory SCD characteristics associated with SCR using multivariate logistic regression models. Proliferative sickle cell retinopathy (PSCR) was diagnosed according to the Goldberg classification system. Results Of the 1904 study participants with SCD who met the inclusion criteria, 953 (50.1%) had retinopathy; of which 642 (67.3%) had bilateral disease. SCR was associated with older age (p < 0.001), history of smoking (p = 0.001), hematuria (p = 0.050), and a lower hemoglobin F (HbF) level (p < 0.001). PSCR risk increased with smoking (p = 0.005), older age (p < 0.001) higher hemoglobin level (p < 0.001) and higher white blood cell count (p = 0.011). Previous blood transfusion (p = 0.050), higher reticulocyte count (p = 0.019) and higher HbF level (p < 0.001) were protective factors against the development of PSCR. Ocular symptoms were associated with progression to PSCR in patients with SCR (p = 0.021). Conclusion In this cohort of individuals with SCD, half of the participants had signs of SCR. Smoking and blood hemoglobin level were the two modifiable risk factors associated with increased retinopathy progression. Screening to identify the different stages of retinopathy, actively promoting smoking cessation, and optimizing the hematological profile of patients with SCD should guide treatment protocols designed to prevent the vision-threatening complications of the disease.

show abstract

Section: Discussioncontrasting

confidence: 99%

Risk factors associated with sickle cell retinopathy: findings from the Cooperative Study of Sickle Cell Disease

et al. 2022

Self Cite

View full text Add to dashboard Cite

show abstract

“…Sickle cell disease (SCD) is the most common inherited blood disorder, with about 275,000 babies born annually worldwide ( 1 ). In the homozygous form [sickle cell anaemia (SCA); HbSS], a combination of low haemoglobin and low oxygen saturation means that children are at risk of central nervous system complications, including stroke and seizures ( 2 , 3 ). Accumulation of silent cerebral infarcts (SCI) on Magnetic Resonance Imaging (MRI) from an early age may result in cognitive difficulties that impact not only on academic attainment and life achievements, but also quality of life ( 4 ).…”

Section: Introductionmentioning

confidence: 99%

Mind the gap: trajectory of cognitive development in young individuals with sickle cell disease: a cross-sectional study

et al. 2023

View full text Add to dashboard Cite

Study objectivesCompared to typically developing children and young adults (CYA-TD), those living with Sickle Cell Disease (CYA-SCD) experience more cognitive difficulties, particularly with executive function. Few studies have examined the relative importance of silent cerebral infarction (SCI), haemoglobin and arterial oxygen content on age-related cognitive changes using cross-sectional or longitudinal (developmental trajectory) data. This study presents cohort data from a single timepoint to inform studies with multiple timepoints.MethodsWe compared cross-sectional raw and scaled scores as age-related changes in cognition (trajectories) in CYA-SCD and age-and ethnicity-matched CYA-TD. We also compared cross-sectional age-related changes in cognition (trajectories) in CYA-SCD with and without SCI to CYA-TD. General cognitive abilities were assessed using Wechsler Intelligence Scales, including the Verbal Comprehension Index (VCI) and Perceptual Reasoning Index (PRI) underpinning IQ. Executive function was evaluated using the Delis-Kaplan Executive Function System (D-KEFS) Tower subtest and the Behaviour Rating Inventory of Executive Function (BRIEF) questionnaire. SCI were identified from contemporaneous 3 T MRI; participants with overt stroke were excluded. Recent haemoglobin was available and oxygen saturation (SpO2) was measured on the day of the MRI.ResultsData were available for 120 CYA-SCD [62 male; age = 16.78 ± 4.79 years; 42 (35%) with SCI] and 53 CYA-TD (23 male; age = 17.36 ± 5.16). Compared with CYA-TD, CYA-SCD experienced a delayed onset in VCI and slower rate of development for BRIEF Global Executive Composite, Metacognition Index (MI), and Behaviour Regulation Index. The rate of executive function development for the BRIEF MI differed significantly between CYA-TD and CYA-SCD, with those with SCI showing a 26% delay compared with CYA-TD. For CYA-SCD with SCI, arterial oxygen content explained 22% of the variance in VCI and 37% in PRI, while haemoglobin explained 29% of the variance in PRI.ConclusionAge-related cognitive trajectories of CYA-SCD may not be impaired but may progress more slowly. Longitudinal studies are required, using tests unaffected by practice. In addition to initiation of medical treatment, including measures to improve arterial oxygen content, early cognitive intervention, educational support, and delivery of extracurricular activities could support cognitive development for CYA-SCD.

show abstract

Terminology for Retinal Findings in Sickle Cell Disease Research

Reilly,

Xie,

Scherer

et al. 2024

Ophthalmology Retina

View full text Add to dashboard Cite

Seizures risk factors in sickle cell disease. The cooperative study of sickle cell disease

Cited by 6 publications

References 36 publications

Risk factors associated with sickle cell retinopathy: findings from the Cooperative Study of Sickle Cell Disease

Risk factors associated with sickle cell retinopathy: findings from the Cooperative Study of Sickle Cell Disease

Mind the gap: trajectory of cognitive development in young individuals with sickle cell disease: a cross-sectional study

Terminology for Retinal Findings in Sickle Cell Disease Research

Contact Info

Product

Resources

About