Disease severity and slower psychomotor speed in adults with sickle cell disease

Blood Advances
Dana R JorgensenEnrico M Novelli

Abstract

Psychomotor slowing is common in children with sickle cell disease (SCD), but little is known about its severity in adults. We conducted a cross-sectional study to quantify psychomotor speed, measured with the digit symbol substitution test (DSST), in relationship with disease severity in adults with SCD attending an outpatient clinic (n = 88, age 36.3 years). Genotype was used to group patients in "severe" (homozygous for hemoglobin S or compound heterozygous with β0thalassemia) or "moderate" groups (compound heterozygous for HbS, with either HbC or β+thalassemia). Analyses were repeated after exclusion of patients with a history of stroke (n = 11). Mild impairment in processing speed was detectable in both the "severe" and the "moderate" group (30% and 9%, respectively; age-adjustedP= .14). Compared with the "moderate" group, those in the "severe" group had significantly lower standardized DSST scores (P= .004), independent of adjustment for factors that differed between the groups: hemoglobin, ferritin, hydroxyurea use, blood pressure parameters, and stroke history. Results were similar after excluding patients with stroke. Psychomotor slowing in SCD differs in relationship to genotype; this difference appears unrelated to h...Continue Reading

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Citations

May 23, 2020·JMIR Research Protocols·Jane S HankinsUNKNOWN Sickle Cell Disease Implementation Consortium
Jul 4, 2021·NeuroImage. Clinical·Tales SantiniTamer S Ibrahim
Jan 1, 2022·Pediatric Blood & Cancer·Andrew M HeitzerJane S Hankins

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