Transcranial doppler and brain MRI in children with sickle cell disease and high hemoglobin F levels

Abstract

While overt stroke and silent brain infarcts (SBI) are uncommon among Kuwaiti patients with sickle cell disease (SCD), there have been no previous transcranial Doppler (TCD) studies in this population. The main objective of this study is to determine TCD velocities in a group of Kuwaiti children with SCD and correlate same with brain magnetic resonance imaging (MRI) and angiography (MRA) findings. Forty-three steady-state, pediatric patients with SCD aged 10.1 ± 3.9 years (21 SS, 19 Sβ(0)Thal, and 3 SD) were studied. Twenty-six age-matched, normal siblings of the patients served as controls. TCD was performed using a General Electric (GE), Vivid 3 equipment and MRI/MRA with a GE Signa Excite HD 1.5 Tesla magnet. Complete blood count was with an electronic counter and Hb quantitation with cation-exchange high performance liquid chromatography (HPLC). The mean time-averaged mean of the maximum velocity (TAMV) was significantly higher in the SCD group than the controls, but was normal (<170 cm/second) in all. The mean values were comparable among the SS and Sβ(0)thal groups. Five (11.1%) patients had SBI and all were between 12 and 16 years of age. There was no significant difference of TAMV in this group compared to those without infarcts. No patient showed evidence of stenosis or any other abnormalities in the circle of Willis vessels. The mild phenotype among Kuwaiti patients with SCD is reflected in normal TCD velocities and a low prevalence of SBI in children with the disease.