Turner syndrome (TS) is a genetic disorder, affecting 1/2500 to 1/3000 live female births, induced by partial or total deletion of one X chromosome. The neurocognitive profile of girls with TS is characterized by a normal Verbal IQ and weaknesses in visual-spatial, mathematics, and social cognitive domains. Executive functions (EFs) impairments have also been reported in these young patients. However, methodological differences across studies do not allow determination of which EFs are impaired and what is the magnitude of these impairments. The aim of this review was to clarify the EF profile of children and adolescents with TS. Sixteen samples, from thirteen studies, were included in the current meta-analysis. EFs measures used in these studies were classified into working memory, inhibitory control, cognitive flexibility, or higher-order EFs tasks in accordance with Diamond’s model, Annual Review of Psychology, 64, 135–168 (2013). Results confirmed that girls with TS had significant executive impairments with effect sizes varying from small (inhibitory control) to medium (cognitive flexibility) and large (working memory, higher-order EFs). Analyses by task revealed that cognitive inhibition may be more impaired than the other inhibitory control abilities. Heterogeneity across cognitive flexibility measures was also highlighted. Between-sample heterogeneity was observed for three tasks and the impact of participants’ characteristics on EFs was discussed. This meta-analysis confirms the necessity to assess, in patients living with TS, each EF by combining both visual and verbal tasks. Results also underline that, when studying girls with TS’ executive profile, it is important to explore the impact of moderator variables, such as IQ, parental socio-economic status, TS karyotype, psychiatric comorbidities, and hormonal treatment status.

中文翻译：

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特纳综合征儿童和青少年的执行功能：系统评价和荟萃分析

特纳综合症（TS）是一种遗传疾病，会影响一部分X染色体的部分或全部缺失，从而导致1/2500至1/3000活着的女性出生。患有TS的女孩的神经认知特征是正常的言语智商以及视觉空间，数学和社会认知领域的弱点。在这些年轻患者中也有执行功能（EFs）受损的报道。但是，研究之间的方法学差异无法确定哪些EF受损以及这些损害的严重程度。这篇综述的目的是阐明患有TS的儿童和青少年的EF概况。目前的荟萃分析包括来自十三项研究的十六个样本。这些研究中使用的EFs措施分为工作记忆，抑制性控制，认知灵活性，心理学年度评论，64，第135-168页（2013年）。结果证实，患有TS的女孩有严重的执行力障碍，其影响大小从小（抑制性控制）到中等（认知灵活性）和大（工作记忆，高阶EF）不等。通过任务分析表明，认知抑制可能比其他抑制控制能力受到的损害更大。还强调了认知灵活性指标之间的异质性。观察到三个任务的样本间异质性，并讨论了参与者特征对EF的影响。这项荟萃分析证实有必要通过合并视力和言语任务来评估患有TS的患者中的每个EF。结果还强调，在研究具有TS执行力特征的女孩时，重要的是要探索主持人变量（例如智商，