Neurocognitive functioning in symptomatic adults with sickle cell disease: A description and comparison with unaffected siblings.
Neurocognitive functioning in symptomatic adults with sickle cell disease: A description and comparison with unaffected siblings.
复制标题
DOI：
10.1080/09602011.2019.1598876
复制
发表时间：
2020-10
影响因子：
2.7
通讯作者：
Tisdale J
中科院分区：
文献类型：
--
作者：
Martin S;Roderick MC;Abel C;Wolters P;Toledo-Tamula MA;Fitzhugh C;Hsieh M;Tisdale J
患有镰状细胞病(SCD)的儿童和成人存在神经心理缺陷的风险；然而，SCD及其相关合并症成人的神经认知功能在文献中尚未广泛报道。我们检查了有症状的SCD成人的特定认知领域，并将他们与他们未受影响的兄弟姐妹进行了比较。我们还检查了认知评分、患者报告的结果(PRO)和医学/实验室价值之间的关系。30对患者-兄弟姐妹(M患者年龄=32.5岁，M兄弟姐妹年龄=32.1岁)作为医学临床试验(NCT00061568)的一部分完成了评估。所有患者和兄弟姐妹的神经认知测试分数都在正常范围内。患者在韦氏加工速度指数上的得分(M=91.0±11.3)显著低于他们的同胞(M=100.6±12.3；t=−3.5，p<；0.01)。在执行功能问卷上，他们也显示出比兄弟姐妹更多的问题，尽管在考虑抑郁症状后，这些差异并不显著。较高的胎儿血红蛋白和较低的肌酐与特定认知和预防措施的较好分数相关。综上所述，我们的成人症状SCD样本显示出比他们的兄弟姐妹更慢的处理速度和更多的执行挑战，尽管使用了羟基脲治疗。这些相对的虚弱可能与疾病过程有关，但具体的生理机制尚不清楚。
Children and adults with sickle cell disease (SCD) are at risk for neuropsychological deficits; however, the neurocognitive functioning of adults with SCD and related comorbidities has not been widely reported in the literature. We examined specific cognitive domains in symptomatic adults with SCD and compared them with their unaffected siblings. We also examined relationships between cognitive scores, patient-reported outcomes (PROs), and medical/laboratory values. Thirty patient-sibling pairs (M patient age = 32.5 years, M sibling age = 32.1 years) completed evaluations as part of a medical clinical trial (NCT00061568). All patient and sibling neurocognitive test scores were within normal limits. Patients scored significantly lower (M=91.0±11.3) than their siblings (M=100.6±12.3; t=−3.5, p<.01) on the Wechsler Processing Speed Index. They also indicated more problems than siblings on an executive functioning questionnaire, although these differences were nonsignificant after accounting for depressive symptoms. Higher fetal hemoglobin and lower creatinine correlated with better scores on particular cognitive and PRO measures. In summary, our sample of adults with symptomatic SCD demonstrated worse processing speed and experience more executive challenges than their siblings, despite treatment with hydroxyurea. These relative weakness likely relate to disease processes but the specific physiological mechanism is unclear.
登录
查看更多内容
影响因子：
3.8
作者：
Crawford, Regina D.;Jonassaint, Charles R.
通讯作者：
Jonassaint, Charles R.
影响因子：
2.4
作者：
Gelb, Shannon R.;Shapiro, R. J.;Thornton, W. J. L.
通讯作者：
Thornton, W. J. L.
影响因子：
5.8
作者：
Kral, MC;Brown, RT;Hynd, GW
通讯作者：
Hynd, GW
影响因子：
2.2
作者：
Puffer, Eve;Schatz, Jeffrey;Roberts, Carla W.
通讯作者：
Roberts, Carla W.
影响因子：
4.2
作者：
Rajagopalan, Priya;Refsum, Helga;Thompson, Paul M.
通讯作者：
Thompson, Paul M.