COGNITIVE DEFICITS IN CHILDREN AND ADOLESCENTS WITH SICKLE CELL DISEASE (GENOTYPE SS) IN BRAZZAVILLE

Introduction: Sickle cell disease has an negative impact on neurodevelopment in child. Objectives: To determine the frequency of cognitive deficits in children and adolescents with sickle cell disease (genotype SS) in Brazzaville; to identify the associated factors. Methods: An analytical prospective study was conducted at the National Center of Reference of Sickle Cell Disease in Brazzaville, from March to September 2019. It included 6-16-year-old children with homozygous sickle cell disease (genotype SS). Neurocognitive domains were evaluated by means of the Wechsler Intelligence Scale for Children, Fifth Version. Analyzed parameters were sociodemographic and clinical. Results: Among the 130 children with homozygous sickle cell disease presenting for a routine clinic visit for follow-up, 83 (63.84%) had cognitive deficits. Of these, there are 43 (51.80 %) boys and 40 (48.20%) girls. Mean age was 10.93 +/- 2.83 years. Deficits of language, visuo-spatial functions, attention and executive functions were detected in patients. Strokes, severe anemia and age had an impact on these cognitive deficits. Working memory was not impaired. General intelligence was preserved. Conclusion: In our setting, cognitive deficits are very frequent in children and adolescents with homozygous sickle cell disease. The factors associated with neurocognitive impairment in our patients get closer to those reported in literature. It is important to include a systematic neurocognitive assessment in the follow-up of these children and to support them with adapted teaching methods or cognitive rehabilitation programs.