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Management of age-associated medical complications in patients with β-thalassemia.β地中海贫血患者年龄相关医学并发症的管理
Expert Rev Hematol. 2020 Jan;13(1):85-94. doi: 10.1080/17474086.2020.1686354. Epub 2019 Nov 12.
4
Brain functional impairment in beta-thalassaemia: the cognitive profile in Italian neurologically asymptomatic adult patients in comparison to the reported literature.β-地中海贫血所致脑功能障碍:意大利神经学无症状成年患者的认知特征与文献报道的比较。
Br J Haematol. 2019 Aug;186(4):592-607. doi: 10.1111/bjh.15959. Epub 2019 May 20.
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Prevalence of Mild Cognitive Impairment and Dementia in Saudi Arabia: A Community-Based Study.沙特阿拉伯轻度认知障碍和痴呆症的患病率:一项基于社区的研究。
Dement Geriatr Cogn Dis Extra. 2018 Mar 20;8(1):98-103. doi: 10.1159/000487231. eCollection 2018 Jan-Apr.
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Neurocognitive dysfunction in children with β thalassemia major: psychometric, neurophysiologic and radiologic evaluation.重型β地中海贫血患儿的神经认知功能障碍:心理测量、神经生理学及放射学评估
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Magnitude and concurrence of anxiety and depression among attendees with multiple sclerosis at a tertiary care Hospital in Oman.阿曼一家三级护理医院中多发性硬化症患者焦虑和抑郁的程度及并发情况。
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阿曼重型β地中海贫血成年患者的认知功能:一项初步研究。

Cognitive Function in Adults with Beta-Thalassemia Major in Oman: A Pilot Study.

作者信息

Daar Shahina, Al Saadoon Muna, Wali Yasser, Al Mujaini Rawan, Al Rahbi Sarah, Chan Moon Fai, Al-Siyabi Alya, Alansary Niveen, Mahadevan Sangeetha, Al-Adawi Samir

机构信息

Department of Hematology, College of Medicine and Health Sciences, Sultan Qaboos University, Muscat, Oman.

Stellenbosch Institute for Advanced Study, Wallenberg Research Centre at Stellenbosch University, Stellenbosch 7600, South Africa.

出版信息

Oman Med J. 2021 Nov 30;36(6):e322. doi: 10.5001/omj.2021.101. eCollection 2021 Nov.

DOI:10.5001/omj.2021.101
PMID:34868668
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC8630422/
Abstract

OBJECTIVES

Neurocognitive dysfunction has been established in several studies in children with beta-thalassemia major (TM). However, despite its wide occurrence in populations across the Arabian Peninsula, scant attention has been paid to shedding light on neuropsychological functioning among adults with TM. This study aimed to examine the level of neuropsychological functioning among Omani adults with TM regularly followed-up at a tertiary care hospital in Oman. A related aim was to examine the factors associated with neuropsychological performance.

METHODS

Standard neuropsychological tests were used to measure attention and concentration, learning and remembering, verbal fluency, and executive functioning. Participants were also gauged on indices of intellectual ability and affective range. As normative data for neuropsychological functioning in Oman is scarce, healthy age- and sex-matched controls underwent the same testing procedure. The log-linear model was used to identify factors associated with TM patients on demographic and neuropsychological performance.

RESULTS

This study recruited 28 adult patients with TM (age 30.0±6.5) and 39 healthy controls (age 29.2±6.1). Findings suggested that having a diagnosis of TM was significantly associated with symptoms of depression ( < 0.001) and anxiety ( < 0.001), indices of executive functioning (verbal fluency) (0.003), working memory (digit span) ( < 0.001), and verbal and auditory attention scores (California Verbal Learning, 0.002).

CONCLUSIONS

The data appears to suggest that short-term memory capacity, verbal fluency, and verbal and auditory attention may be impaired in TM compared to controls. Studies on a larger cohort are therefore warranted.

摘要

目的

多项研究已证实重型β地中海贫血(TM)患儿存在神经认知功能障碍。然而,尽管TM在阿拉伯半岛各地人群中广泛存在,但对于成年TM患者的神经心理功能却鲜有研究。本研究旨在调查阿曼一家三级护理医院定期随访的成年阿曼TM患者的神经心理功能水平。另一个相关目的是研究与神经心理表现相关的因素。

方法

使用标准神经心理测试来测量注意力、专注力、学习与记忆、语言流畅性和执行功能。还对参与者的智力能力和情感范围指标进行了评估。由于阿曼缺乏神经心理功能的规范数据,因此选取了年龄和性别匹配的健康对照者进行相同的测试程序。采用对数线性模型来确定与TM患者人口统计学和神经心理表现相关的因素。

结果

本研究招募了28名成年TM患者(年龄30.0±6.5岁)和39名健康对照者(年龄29.2±6.1岁)。研究结果表明,TM诊断与抑郁症状(<0.001)、焦虑症状(<0.001)、执行功能指标(语言流畅性)(0.003)、工作记忆(数字广度)(<0.001)以及语言和听觉注意力得分(加利福尼亚语言学习测试,<0.002)显著相关。

结论

数据似乎表明,与对照组相比,TM患者的短期记忆能力、语言流畅性以及语言和听觉注意力可能受损。因此,有必要对更大的队列进行研究。