Distribution of sickle cell disease and assessment of risk factors based on transcranial Doppler values in the Gulf region

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.

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“…Acute anemia might present as a single presentation or in accordance with splenomegaly or hepatomegaly 10 . However, when aplastic crisis diagnosis is made it is essential to rule out any possibility of delayed hemolytic transfusion reaction as more cautions are required if RBCs transfusion is the plan 1 …”

“…Sickle Cell disease (SCD) is one of the most common hemoglobinopathies, and it is due to mutation on the beta chain hemoglobin that causes deformity in red blood cells (RBCs) shape. The sickle shape of RBCs affects their function leading to hypoxia and sickle cell crisis including hemolysis when triggers like infections happen 1 …”

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