Reem A Tarazi1,2, Kristina E Patrick3, Mary Iampietro4, Nataly Apollonsky2,5. 1. Department of Psychiatry, Drexel University College of Medicine, Philadelphia, PA. 2. Division of Hematology, St. Christopher's Hospital for Children, Philadelphia, PA. 3. Department of Neurology, Division of Pediatric Neurology, University of Washington School of Medicine, Seattle Children's Hospital, Seattle, WA. 4. Department of Child and Adolescent Psychiatry and Behavioral Sciences, The Children's Hospital of Philadelphia, Philadelphia, PA. 5. Department of Pediatrics, Drexel University College of Medicine, Philadelphia, PA.
Abstract
OBJECTIVE: Hydroxyurea (HU) is used in children with sickle cell disease (SCD) to increase fetal hemoglobin (HF), contributing to a decrease in physical symptoms and potential protection against cerebral microvasculopathy. There has been minimal investigation into the association between HU use and cognition in this population. This study examined the relationship between HU status and cognition in children with SCD. METHODS: Thirty-seven children with SCD HbSS or HbS/β0 thalassaemia (sickle cell anemia; SCA) ages 4:0-11 years with no history of overt stroke or chronic transfusion completed a neuropsychological test battery. Other medical, laboratory, and demographic data were obtained. Neuropsychological function across 3 domains (verbal, nonverbal, and attention/executive) was compared for children on HU (n = 9) to those not taking HU (n = 28). RESULTS: Children on HU performed significantly better than children not taking HU on standardized measures of attention/executive functioning and nonverbal skills. Performance on verbal measures was similar between groups. CONCLUSIONS: These results suggest that treatment with HU may not only reduce physical symptoms, but may also provide potential benefit to cognition in children with SCA, particularly in regard to attention/executive functioning and nonverbal skills. Replication with larger samples and longitudinal studies are warranted.
OBJECTIVE:Hydroxyurea (HU) is used in children with sickle cell disease (SCD) to increase fetal hemoglobin (HF), contributing to a decrease in physical symptoms and potential protection against cerebral microvasculopathy. There has been minimal investigation into the association between HU use and cognition in this population. This study examined the relationship between HU status and cognition in children with SCD. METHODS: Thirty-seven children with SCD HbSS or HbS/β0 thalassaemia (sickle cell anemia; SCA) ages 4:0-11 years with no history of overt stroke or chronic transfusion completed a neuropsychological test battery. Other medical, laboratory, and demographic data were obtained. Neuropsychological function across 3 domains (verbal, nonverbal, and attention/executive) was compared for children on HU (n = 9) to those not taking HU (n = 28). RESULTS:Children on HU performed significantly better than children not taking HU on standardized measures of attention/executive functioning and nonverbal skills. Performance on verbal measures was similar between groups. CONCLUSIONS: These results suggest that treatment with HU may not only reduce physical symptoms, but may also provide potential benefit to cognition in children with SCA, particularly in regard to attention/executive functioning and nonverbal skills. Replication with larger samples and longitudinal studies are warranted.
Authors: Andrew M Heitzer; Diana L Cohen; Victoria I Okhomina; Ana Trpchevska; Brian Potter; Jennifer Longoria; Jerlym S Porter; Jeremie H Estepp; Allison King; Misham Henley; Guolian Kang; Jane S Hankins Journal: Pediatr Blood Cancer Date: 2021-12-31 Impact factor: 3.838
Authors: Andrew M Heitzer; Jennifer Longoria; Victoria Okhomina; Winfred C Wang; Darcy Raches; Brian Potter; Lisa M Jacola; Jerlym Porter; Jane E Schreiber; Allison A King; Guolian Kang; Jane S Hankins Journal: Br J Haematol Date: 2021-07-16 Impact factor: 8.615