BACKGROUND: Although neurocognitive deficits in children with sickle cell disease (SCD) have been well documented, the etiology of these deficits has not been completely clarified. The aim of this study was to investigate the association of laboratory markers of disease severity and radiological parameters with neurocognitive functioning in children with SCD. DESIGN AND METHODS: Participants were 37 children with SCD ((HbSS or HbS-β(0)-thalassemia) aged 6-18 years. All participants underwent extensive neurocognitive assessment. Further data (TCD values, laboratory test results, and MRI data) were obtained from medical charts. Associations were analyzed by hierarchical regression analysis. RESULTS: Hemoglobin was associated with a decrease in verbal short-term memory. There was no association between TCD velocities and neurocognitive functioning, when controlled for age. Children with silent infarcts did not differ from children with normal MRI in neurocognitive functioning. Children with right-left asymmetries in cerebral blood flow as measured by continuous arterial spin labelling (CASL) MRI had better sustained attention than children without asymmetries. CONCLUSIONS: Neurocognitive deficits are associated with the severity of anemia, indicating reduced oxygen delivery to the brain as an etiological mechanism. This implies that children with SCD and normal MRIs may still suffer from neurocognitive impairments, possibly affecting their academic development and full participation in society.
BACKGROUND: Although neurocognitive deficits in children with sickle cell disease (SCD) have been well documented, the etiology of these deficits has not been completely clarified. The aim of this study was to investigate the association of laboratory markers of disease severity and radiological parameters with neurocognitive functioning in children with SCD. DESIGN AND METHODS: Participants were 37 children with SCD ((HbSS or HbS-β(0)-thalassemia) aged 6-18 years. All participants underwent extensive neurocognitive assessment. Further data (TCD values, laboratory test results, and MRI data) were obtained from medical charts. Associations were analyzed by hierarchical regression analysis. RESULTS: Hemoglobin was associated with a decrease in verbal short-term memory. There was no association between TCD velocities and neurocognitive functioning, when controlled for age. Children with silent infarcts did not differ from children with normal MRI in neurocognitive functioning. Children with right-left asymmetries in cerebral blood flow as measured by continuous arterial spin labelling (CASL) MRI had better sustained attention than children without asymmetries. CONCLUSIONS:Neurocognitive deficits are associated with the severity of anemia, indicating reduced oxygen delivery to the brain as an etiological mechanism. This implies that children with SCD and normal MRIs may still suffer from neurocognitive impairments, possibly affecting their academic development and full participation in society.
Authors: Olubusola B Oluwole; Robert B Noll; Daniel G Winger; Olu Akinyanju; Enrico M Novelli Journal: Pediatr Blood Cancer Date: 2016-07-09 Impact factor: 3.167
Authors: Kemar V Prussien; Rachel E Siciliano; Abagail E Ciriegio; Allegra S Anderson; Radha Sathanayagam; Michael R DeBaun; Lori C Jordan; Bruce E Compas Journal: J Pediatr Psychol Date: 2020-03-01
Authors: Allison A King; Mark J Rodeghier; Julie Ann Panepinto; John J Strouse; James F Casella; Charles T Quinn; Michael M Dowling; Sharada A Sarnaik; Alexis A Thompson; Gerald M Woods; Caterina P Minniti; Rupa C Redding-Lallinger; Melanie Kirby-Allen; Fenella J Kirkham; Robert McKinstry; Michael J Noetzel; Desiree A White; Janet K Kwiatkowski; Thomas H Howard; Karen A Kalinyak; Baba Inusa; Melissa M Rhodes; Mark E Heiny; Ben Fuh; Jason M Fixler; Mae O Gordon; Michael R DeBaun Journal: Am J Hematol Date: 2014-08-04 Impact factor: 10.047
Authors: Ann T Farrell; Julie Panepinto; C Patrick Carroll; Deepika S Darbari; Ankit A Desai; Allison A King; Robert J Adams; Tabitha D Barber; Amanda M Brandow; Michael R DeBaun; Manus J Donahue; Kalpna Gupta; Jane S Hankins; Michelle Kameka; Fenella J Kirkham; Harvey Luksenburg; Shirley Miller; Patricia Ann Oneal; David C Rees; Rosanna Setse; Vivien A Sheehan; John Strouse; Cheryl L Stucky; Ellen M Werner; John C Wood; William T Zempsky Journal: Blood Adv Date: 2019-12-10