Institute of Neurology CCS, School of Medicine, University of Belgrade, Belgrade, Serbia.
Mov Disord. 2011 Jul;26(8):1503-8. doi: 10.1002/mds.23608. Epub 2011 May 18.
Health-related quality of life (HRQoL) in Wilson's disease (WD) has not been extensively studied. Therefore, the purpose of this cross-sectional study was to identify clinical and demographic factors influencing HRQoL in 60 treated, clinically stable patients with WD using a generic questionnaire, the Medical Outcomes Study Short-Form 36-Item Health Survey (SF-36). The level of disability and grading of WD multisystemic manifestations were assessed by the Global Assessment Scale for WD (GAS for WD). The Mini Mental State Examination (MMSE) and the 21-item Hamilton Depression Rating Scale (HDRS) scoring were also applied by the same trained interviewers. Lower scores on the SF-36 domains were found in patients with neurological compared with those with a predominantly hepatic form of WD. The HRQoL of patients with WD and psychiatric symptoms was also lower than that of those without them. Finally, significant inverse correlations were obtained between the various SF-36 domains and all the following: period of latency from the first symptoms/signs appearance and treatment initiation, MMSE and HDRS scores, and different domains of the GAS for WD.
Wilson 病（WD）患者的健康相关生活质量（HRQoL）尚未得到广泛研究。因此，本横断面研究的目的是使用通用问卷，即医疗结局研究 36 项简明健康调查问卷（SF-36），确定 60 例经治疗且临床稳定的 WD 患者的临床和人口统计学因素对 HRQoL 的影响。WD 多系统表现的残疾程度和分级通过 WD 全球评估量表（GAS for WD）进行评估。同样由同一位受过培训的访谈者进行简易精神状态检查（MMSE）和 21 项汉密尔顿抑郁量表（HDRS）评分。与主要表现为肝型 WD 的患者相比，神经型 WD 患者的 SF-36 各领域得分较低。伴有精神症状的 WD 患者的 HRQoL 也低于无精神症状的患者。最后，SF-36 的各个领域与以下各项均呈显著负相关：首次出现症状/体征与开始治疗之间的潜伏期、MMSE 和 HDRS 评分以及 GAS for WD 的不同领域。
