Abstract
ABSTRACT Background/Objective: Stroke is a potentially fatal complication of sickle cell disease (SCD). Transcranial Doppler (TCD) is useful at identifying increased risk of stroke in children with SCD and vasospasm after subarachnoid hemorrhage. The main aim of this study was to determine the proportion of patients with SCD in the Gulf region who are at a high risk of stroke, as determined by TCD. Methods: This multicenter (Oman, Qatar, and UAE), descriptive, cross-sectional study in patients (aged 2–16 years) with SCD included a baseline visit, 1 follow-up visit for patients with conditional TCD, and 3-year retrospective data analysis for all patients. Results: Of the 410 eligible patients (Oman, 86.5%; Qatar, 8.2%; UAE, 5.1%), most had a TCD finding (left side, 91.7%; right side, 92.0%) of normal velocity (<155 cm/s) at baseline. For 6 of 7 patients with conditional velocity (155–179 cm/s) and 1 patient with high velocity (≥180 cm/s), baseline TCD results were not confirmed at follow-up. As per bivariate linear regression, age, race, transfusion type, and transfusion frequency were significant predictors of the TCD velocities. Multivariate logistic regressions revealed that TCD velocities were significantly correlated with sex, race, and type of transfusion. No patients reported any adverse events at follow-up. No deaths occurred during the study. Discussion/Conclusions: The study results show that far fewer patients with SCD in the Gulf have abnormal TCD findings than the internationally reported. Larger studies are needed to identify the factors underlying this observation.
Highlights
Sickle cell disease (SCD) is an autosomal recessive, monogenic, a multi-organ disorder associated with high morbidity, mortality, and poor quality of life [1]
The distribution of homozygous SCD in different Middle-East Arab countries of Gulf cooperation council was reported in available literature from Qatar (3.9%), Oman (3.8%), Bahrain (2.1%), United Arab Emirates (UAE; 1.9%) Yemen (0.95%) and SaudiArabia (0.01%–0.10%) [5,6,7,8,9]
The primary objective of the study was to determine the proportion of patients with SCD who were at a high risk of stroke with abnormal Transcranial Doppler (TCD) results, and were eligible for transfusion therapy
Summary
Sickle cell disease (SCD) is an autosomal recessive, monogenic, a multi-organ disorder associated with high morbidity, mortality, and poor quality of life [1]. It is characterized by inefficient sickle-shaped red blood cells, produced due to a mutation in the hemoglobin gene (sickle hemoglobin, HbS) [2,3]. Almost two-thirds of patients with SCD carry a homozygous HbSS genotype (sickle cell anemia) and are severely affected [10]. Patients who carry a heterozygous HbS and normal β-globin (sickle cell trait) are clinically asymptomatic. HbSβ0 is usually as severe as HbSS, while other genotypes vary from mild to moderate in severity [10,11,12]
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