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Abstract：
NF2-related schwannomatosis (NF2-SWN) is a rare genetic disorder and is associated with progressive morbidities. This study aimed to investigate the relationship between NF2-SWN disease severity, health-related Quality of Life (QoL), and mental health aspects of patients. Standardised questionnaires assessing mental health problems (symptoms of depression, anxiety, and somatic burden), psychological factors (resilience, loneliness, and personality functioning), and health-related QoL were administered to 97 patients with NF2-SWN. The results of these questionnaires were compared with physician-rated disease severity. Questionnaires were completed by 77 patients. Physician-rated disease severity scores were available for 55 patients. NF2-SWN patients showed a high prevalence of clinically relevant symptoms of depression (30%), anxiety (16%), and somatic burden (32%). Almost all variables showed moderate to high correlations with NF2-SWN-related QoL. NF2-SWN-related QoL was associated with physician-reported disease severity (r = 0.614). In the stepwise hierarchical linear regression analysis, a significant model with four predictors (disease severity type, depression symptoms, personality functioning, and gender) explained 64% of the variance in NF2-SWN-related QoL. Our results showed a strong association between NF2-SWN-related QoL and depression symptoms. Moreover, personality functioning is an important influencing factor, representing a modifiable construct that can be targeted by prevention programs or psychotherapy.
摘要：
NF2相关神经鞘瘤病（NF2-SWN）是一种罕见的遗传性疾病，与进行性疾病有关。本研究旨在探讨NF2-SWN与疾病严重程度的关系,与健康相关的生活质量（QoL），和患者的心理健康方面。评估心理健康问题的标准化问卷(抑郁症的症状，焦虑,和躯体负担)，心理因素(韧性，孤独,和人格功能)，对97例NF2-SWN患者进行了与健康相关的QoL。将这些问卷的结果与医生评估的疾病严重程度进行比较。77例患者完成问卷调查。医生评定的疾病严重程度评分可用于55名患者。NF2-SWN患者的抑郁症临床相关症状患病率较高（30％），焦虑（16％），和躯体负担（32%）。几乎所有变量都与NF2-SWN相关的QoL表现出中等至高度的相关性。NF2-SWN相关QoL与医生报告的疾病严重程度相关（r=0.614）。在逐步分层线性回归分析中，具有四个预测因子的重要模型(疾病严重程度类型，抑郁症状,人格功能，和性别）解释了NF2-SWN相关QoL中64%的方差。我们的结果显示NF2-SWN相关的QoL与抑郁症状之间有很强的关联。此外，人格功能是一个重要的影响因素，代表一种可修改的结构，可以通过预防计划或心理治疗作为目标。
