Prevalence and risk factors of cognitive impairment in children with sickle cell disease in Egypt.

ABSTRACT

Little is known about cognitive impairment in patients with sickle cell disease in Africa. This study aimed to assess cognitive impairment and identify possible risk factors in patients with sickle cell disease in Egypt. This study was conducted at Cairo University Children Hospital. Patients with sickle cell disease, between ages of 6-20 years were enrolled. Cognitive ability was tested using the Stanford Binet intelligence quotient (IQ) test, fourth edition. Transcranial Doppler, magnetic resonance imaging and magnetic resonance angiography of the brain were performed within a week of the IQ test. Among the 40 enrolled patients, 55% had a Full Scale IQ at least 1 standard deviation below the mean, and 27.5% had an IQ 2 standard deviations below the mean. High lactate dehydrogenase was significantly associated with low IQ (p = 0.004). In univariate analyses, IQ was significantly correlated with older age (p = 0.025), high lactate dehydrogenase (p = 0.008) and older age at the start of hydroxyurea (p = 0.025). Impaired cognition is prevalent among sickle cell disease patients. Early initiation of hydroxyurea therapy, which should also reduce hemolysis and lactate dehydrogenase, may be a simple measure to preserve mental abilities in these patients.