Presentation Details
| Brain Volumes and Neurocognitive Outcomes in Children with Hemophilia A Silvia Verhofste1, Ahmad Al-Huniti1, 2, Marci Novak1, Amy L.Conrad1, 5, 6, Ellen van der Plas3, 4, Lyndsay Harshman1, 5, 6, Janice M.Staber1, 5, 6. 1Stead Family Department of Pediatrics, University of Iowa, Iowa City, IA, USA.2*Current Location - Mayo Clinic, Rochester, MN, USA.3Department of Psychiatry, University of Iowa, Iowa City, IA, USA.4*Current Location -University of Arkansas for Medical Sciences, Pediatrics Arkansas Children's Hospital, Hematology/Oncology, Little Rock, AR, USA.5The University of Iowa Roy J and Lucille A Carver College of Medicine, Iowa City, IA, USA.6Iowa Neuroscience Institute, Iowa City, IA, USA |
Abstract
Background: People with hemophilia are at an increased risk of intracranial hemorrhage. In addition, hemophilia-related sequelae, such as an increased risk of neuropsychiatric disorders and impairments in executive function, may exert a direct, negative impact on the developing brain. The effect of factor VIII deficiency, a protein essential for blood coagulation, on neuroimaging and neurocognitive outcomes in children with hemophilia A (HA) is not well characterized.
Objectives: To compare neuroanatomical features between children with severe HA and healthy controls using quantitative structural magnetic resonance imaging (MRI). To examine differences in neurocognition between children with severe HA and healthy controls.
Methods: This single-center study included 32 males aged 6-16 years: nine with severe FVIII deficiency and 23 healthy controls. Volumetric/structural data from MRI and neurocognitive testing including Delis-Kaplan Executive Function System (DKEFS) and Behavioral Regulation Index of Executive Function (BRIEF) were compared using linear models including age to evaluate the association between brain volume and function.
Results: Children with HA had decreased mean cerebellar and hippocampal volumes (Figure 1A and 1B). Cerebellar gray matter volume was significantly smaller in the HA cohort compared to healthy controls, (Estimate=-0.375, 95% CI=-0.732:-0.019), t(26)=−2.07, p = 0.049) (Figure 1A). A reduction in cerebellar gray matter was associated with neurocognitive executive dysfunction as noted by abnormal scores on two executive function assessments: the DKEFS total switching accuracy (Estimate=0.549, 95% CI= -0.876:0.221), t(25) = -3.28, p=0.003) (Figure 2A) and total correct category switching (Estimate=0.538, 95% CI=-0.868:0.207), t(25)=−3.19, p=0.004) (Figure 2A), as well as the BRIEF behavioral regulation index score (Estimate=0.531, 95% CI=0.228:0.835), t(25)=3.44, p=0.002) (Figure 2B).
Conclusion: Our study provides key insight into the lower brain volumes found in persons with HA and corresponding executive dysfunction. Structural brain volume assessment in persons with HA may provide an integrated measure and with further research could be a useful clinical tool when assessing risk for neurocognitive dysfunction.
No part of this publication may be reproduced, distributed, or transmitted in any form or by any means, including photocopying, recording, or other electronic or mechanical methods, without the prior written permission of the author.
Objectives: To compare neuroanatomical features between children with severe HA and healthy controls using quantitative structural magnetic resonance imaging (MRI). To examine differences in neurocognition between children with severe HA and healthy controls.
Methods: This single-center study included 32 males aged 6-16 years: nine with severe FVIII deficiency and 23 healthy controls. Volumetric/structural data from MRI and neurocognitive testing including Delis-Kaplan Executive Function System (DKEFS) and Behavioral Regulation Index of Executive Function (BRIEF) were compared using linear models including age to evaluate the association between brain volume and function.
Results: Children with HA had decreased mean cerebellar and hippocampal volumes (Figure 1A and 1B). Cerebellar gray matter volume was significantly smaller in the HA cohort compared to healthy controls, (Estimate=-0.375, 95% CI=-0.732:-0.019), t(26)=−2.07, p = 0.049) (Figure 1A). A reduction in cerebellar gray matter was associated with neurocognitive executive dysfunction as noted by abnormal scores on two executive function assessments: the DKEFS total switching accuracy (Estimate=0.549, 95% CI= -0.876:0.221), t(25) = -3.28, p=0.003) (Figure 2A) and total correct category switching (Estimate=0.538, 95% CI=-0.868:0.207), t(25)=−3.19, p=0.004) (Figure 2A), as well as the BRIEF behavioral regulation index score (Estimate=0.531, 95% CI=0.228:0.835), t(25)=3.44, p=0.002) (Figure 2B).
Conclusion: Our study provides key insight into the lower brain volumes found in persons with HA and corresponding executive dysfunction. Structural brain volume assessment in persons with HA may provide an integrated measure and with further research could be a useful clinical tool when assessing risk for neurocognitive dysfunction.
No part of this publication may be reproduced, distributed, or transmitted in any form or by any means, including photocopying, recording, or other electronic or mechanical methods, without the prior written permission of the author.
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