Regional brain volumes and their relationship to neurocognitive outcomes in children with severe hemophilia A

Blood Vessels, Thrombosis & Hemostasis Pub Date : 2024-07-26 DOI:10.1016/j.bvth.2024.100021

Silvia Verhofste , Ahmad Al-Huniti , Marci Novak , Amy L. Conrad , Ellen van der Plas , Lyndsay Harshman , Janice M. Staber

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Abstract

The effect of factor VIII (FVIII) deficiency on neurocognitive outcomes in children with hemophilia A (HA) is not well characterized. This study aimed to examine differences in brain volume and neurocognition between children with severe HA and healthy controls.This single-center study included 32 males aged 6 to 16 years, 9 with severe FVIII deficiency and 23 healthy controls. Volumetric data from magnetic resonance imaging and neurocognitive testing were compared using linear models including age to evaluate the association between regional brain volume and function. Cerebellar gray matter volume was significantly smaller in the HA cohort than in healthy controls (estimate, –0.375; 95% confidence interval [CI], –0.732 to –0.019; t₍₂₆₎ = 2.07; P = .049). A reduction in cerebellar gray matter was associated with neurocognitive executive dysfunction as noted by abnormal scores on 2 executive function assessments: the Delis-Kaplan Executive Function System, total switching accuracy (estimate, 0.549; 95% CI, –0.876 to 0.221; t₍₂₅₎ = –3.28; P = .003) and total correct category switching (estimate, 0.538; 95% CI, –0.868 to 0.207; t₍₂₅₎ = –3.19; P = .004), and the Behavior Rating Inventory of Executive Function, behavioral regulation index score (estimate, 0.531; 95% CI, 0.228-0.835; t₍₂₅₎ = 3.44; P = .002). Our study provides key insights into the lower brain volumes found in patients with HA and the corresponding executive dysfunction. Quantitative brain volume assessment in patients with HA may provide an integrated measure and with further research could be a useful clinical tool when assessing risk for neurocognitive dysfunction.

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严重血友病 A 患儿的区域脑容量及其与神经认知结果的关系

摘要因子 VIII（FVIII）缺乏症对 A 型血友病（HA）儿童神经认知能力的影响尚不十分明确。这项单中心研究纳入了 32 名 6-16 岁的男性，其中 9 名患有严重的 FVIII 缺乏症，23 名为健康对照组。研究使用包括年龄在内的线性模型比较了磁共振成像和神经认知测试的体积数据，以评估区域脑体积与功能之间的关联。HA 组群的小脑灰质体积明显小于健康对照组（估计值，-0.375；95% 置信区间 [CI]，-0.732 至 -0.019；t(26) = 2.07；P = .049）。小脑灰质的减少与神经认知执行功能障碍有关，表现为2项执行功能评估的异常评分：Delis-Kaplan执行功能系统，总转换准确性（估计值，0.549；95% CI，-0.876 至 0.221；t（25）= -3.28；P = .003）和类别转换总正确率（估计值，0.538；95% CI，-0.868 至 0.207；t(25) = -3.19；P = .004），以及执行功能行为评级量表（Behavior Rating Inventory of Executive Function）行为调节指数得分（估计值，0.531；95% CI，0.228-0.835；t(25) = 3.44；P = .002）。我们的研究为了解 HA 患者较低的脑容量和相应的执行功能障碍提供了重要依据。对HA患者进行脑容量定量评估可提供一种综合测量方法，随着研究的深入，它将成为评估神经认知功能障碍风险的一种有用的临床工具。

本文章由计算机程序翻译，如有差异，请以英文原文为准。

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Blood Vessels, Thrombosis & Hemostasis

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