Transcranial doppler as screening method for sickling crises in children with sickle cell anemia: a latin America cohort study

Background Sickle cell anemia (SCA) is the leading cause of childhood stroke. We aimed to evaluate whether altered cerebral flow velocities, as measured by transcranial Doppler (TCD), are associated with vaso-occlusive complications in addition to stroke in pediatric SCA patients. Methods We evaluated 37 children aged between 2 and 16 years with SCA who underwent screening for TCD between January 2012 and October 2018. Genotypic profiles and demographic data were collected, TCD examinations were performed during follow-up, and the presence of sickling crises was compared. Survival analyses were performed using simple frailty models, in which each predictor variable was analyzed separately in relation to the occurrence of a sickling crisis. Results The variables related to sickle cell crises in the univariate analysis were peak systolic velocity (PSV) in the middle cerebral artery (MCA), hazard ratio (HR) 1.01 (1.00—1.02) p = 0.04; end-diastolic velocity (EDV) in the MCA, HR 1.02 (1.01—1.04) p = 0.01; time average mean maximum velocity (TAMMV) in the basilar artery (BA), HR 1.02 (1.00—1.04) p = 0.04; hemoglobin, HR 0.49 (0.38—0.65) p < 0.001; hematocrit, HR 0.78 (0.71—0.85) p < 0.001; leukocyte counts, HR 1.1 (1.05—1.15) p < 0.001; platelets counts, HR 0.997 (0.994—0.999) p = 0.02; and reticulocyte numbers, HR 1.14 (1.06—1.23) p < 0.001. Conclusions Our results indicate PSV and EDV in the MCA and TAMMV in the BA as markers of risk for the occurrence of sickling crises in SCA.