Abstract
INTRODUCTION: Sickle Cell Disease (SCD) ranks among the most prevalent genetic disorders globally. The incidence in sub-Saharan African countries has been estimated to be 230.000/y with a high prevalence (1%) in the Democratic Republic of Congo (DRC). Stroke is a significant complication of Sickle Cell Disease (SCD), and carries a high risk of disability and mortality. Transcranial Doppler (TCD) is currently the non-invasive exploration recommended for the prevention of stroke in young SCD patients. OBJECTIVE: To determine the prevalence of pathological TCD in a population of young Congolese SCA patients and to assess its association with hematological parameters. POPULATION AND METHODS: This cross sectional study was carried out on 150 Congolese SS homozygous children between the ages 2-16 years old (mean age: 8.5 ± 4.0 years) in stable condition, and followed from January 1 to December 31, 2013. TCD was performed using the STOP I method in the main cerebral arteries. The risk of stroke was absent when the average maximum speed during a cycle (TAMMV) in middle cerebral artery (MCA) was < 170 cm/s, but present when TAMMV was borderline or conditional for values between 170 and 199 cm/s and pathological for values ≥ 200 cm/s. RESULTS: The prevalence of pathological TCD was 4% while the conditional TCD prevalence was 10%. The Mean blood velocity in MCA was 114.0 cm/s. There was a significant difference in the means of WBC (p = 0.003), Hb (p < 0.001), Hct (p < 0.001), MCV (p = 0.005) parameters when comparing normal and at risk TCD (conditional and abnormal). However, no significant association was found for the categorical corresponding parameters. CONCLUSION: Globally, 14% of patients were at risk of stroke, hence the interest in integrating TCD in the routine monitoring of children with SCD in order to prevent overt stroke by implementing a chronic blood transfusion program or the use of hydroxycarbamide.