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神经纤维瘤病 1 型成人的社会功能。

Social functioning in adults with neurofibromatosis type 1.

机构信息

The Institute for Neuroscience and Muscle Research, The Children's Hospital at Westmead, Locked Bag 4001, Westmead, NSW 2145, Australia; Discipline of Paediatrics and Child Health, Faculty of Medicine, The University of Sydney, NSW 2006, Australia.

出版信息

Res Dev Disabil. 2013 Oct;34(10):3393-9. doi: 10.1016/j.ridd.2013.07.011. Epub 2013 Aug 2.

Abstract

Neurofibromatosis type 1 (NF1) is a common single-gene disorder characterised by a diverse range of cutaneous, neurological and neoplastic manifestations. It is well recognised that children with NF1 have poor peer interactions and are at risk for deficits in social skills. Few studies, however, have examined social functioning in adults with NF1. We aimed to determine whether adults with NF1 are at greater risk for impairment in social skills and to identify potential risk factors for social skills deficits. We evaluated social skills in 62 adults with NF1 and 39 controls using self-report and observer-report measures of social behaviour. We demonstrate that adults with NF1 exhibit significantly less prosocial behaviour than controls. This deficit was associated with social processing abilities and was more evident in males. The frequency of antisocial behaviour was comparable between the two groups, however was significantly associated with behavioural regulation in the NF1 group. These findings suggest that poor social skills in individuals with NF1 are due to deficits in prosocial behaviour, rather than an increase in antisocial behaviour. This will aid the design of interventions aimed at improving social skills in individuals with NF1.

摘要

神经纤维瘤病 1 型(NF1)是一种常见的单基因疾病,其特征是具有多种皮肤、神经和肿瘤表现。众所周知,NF1 患儿的同伴互动能力较差,存在社交技能缺陷的风险。然而,很少有研究检查 NF1 成年患者的社交功能。我们旨在确定 NF1 成年患者是否更容易出现社交技能障碍,并确定社交技能缺陷的潜在危险因素。我们使用社交行为的自我报告和观察报告测量方法,评估了 62 名 NF1 成年患者和 39 名对照者的社交技能。我们证明,NF1 成年患者的亲社会行为明显少于对照组。这种缺陷与社交加工能力有关,在男性中更为明显。两组的反社会行为频率相当,但在 NF1 组中与行为调节显著相关。这些发现表明,NF1 个体的社交技能差是由于亲社会行为缺陷所致,而不是反社会行为增加所致。这将有助于设计旨在提高 NF1 个体社交技能的干预措施。

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