波兰肝豆状核变性队列的社会人口学特征及治疗持续性的影响。
Social and demographic characteristics of a Polish cohort with Wilson disease and the impact of treatment persistence.
机构信息
Department of Experimental and Clinical Pharmacology, Medical University of Warsaw, Warsaw, Poland.
2nd Department of Neurology, Institute of Psychiatry and Neurology, Warsaw, Poland.
出版信息
Orphanet J Rare Dis. 2019 Jul 5;14(1):167. doi: 10.1186/s13023-019-1133-2.
BACKGROUND
Wilson disease (WD) is a genetic disorder involving impaired copper metabolism, which presents with hepatic, neurological, and/or psychiatric manifestations. WD requires lifelong pharmacotherapy and treatment persistence may be problematic. We studied social characteristics, education, and work-related activities and how they are affected by WD symptoms and treatment persistence.
METHODS
In a cross-sectional study, data on demographic characteristics, achieved education level, household and marital status, plus a primary source of income were collected from 202 Polish subjects (mean ± standard deviation age of 36.4 ± 9.9 years at assessment) with WD.
RESULTS
Overall, WD appeared to have a negative impact on achieved level of education and influenced the ability to work as compared with the general Polish population. Patients with neurological manifestations less often achieved upper-secondary/post-secondary or higher education compared with those with hepatic manifestations (65.5% vs. 83.6%; p = 0.003). They also significantly less frequently stated salary (19.6% vs. 56.2%; p < 0.0001) as the primary income and more often were on disability pension (53.3% vs. 26.0%; p = 0.0003). The percentage of married patients with WD appeared lower than in the general population (47.0% vs. 54.6%), although the difference was not significant (p = 0.2). The 27.6% of patients who were non-persistent with WD treatment less frequently achieved upper/post-secondary or higher education compared with persistent patients (66.0% vs. 76.3%; NS) and their primary source of outcome was significantly less often a salary (18.9% vs. 40.3%; p = 0.001).
CONCLUSIONS
Neurological manifestations had an adverse effect on education level and work ability. Treatment non-persistence had a further negative impact regardless of the disease form. Patients with WD should receive appropriate treatment, with the need for persistence emphasized and monitored to avoid a detrimental effect on their lives.
背景
威尔逊病（WD）是一种遗传性铜代谢紊乱疾病，可导致肝脏、神经和/或精神症状。WD 需要终身药物治疗，治疗的持续性可能是个问题。我们研究了社会特征、教育和与工作相关的活动，以及它们如何受到 WD 症状和治疗持续性的影响。
方法
在一项横断面研究中，我们从 202 名波兰 WD 患者（评估时的平均年龄为 36.4±9.9 岁）收集了人口统计学特征、已完成的教育水平、家庭和婚姻状况以及主要收入来源的数据。
结果
总体而言，与一般波兰人群相比，WD 似乎对已完成的教育水平产生负面影响，并影响工作能力。与肝表现相比，神经表现的患者获得高中后/高等教育的比例较低（65.5%比 83.6%；p=0.003）。他们作为主要收入来源的工资（19.6%比 56.2%；p<0.0001）也明显较少，而更多的人领取残疾抚恤金（53.3%比 26.0%；p=0.0003）。WD 患者的已婚比例似乎低于一般人群（47.0%比 54.6%），尽管差异无统计学意义（p=0.2）。27.6%的 WD 治疗非持续性患者与持续性患者相比，获得高中后/高等教育的比例较低（66.0%比 76.3%；NS），其主要收入来源明显较少是工资（18.9%比 40.3%；p=0.001）。
结论
神经表现对教育水平和工作能力有不良影响。无论疾病形式如何，治疗非持续性都有进一步的负面影响。WD 患者应接受适当的治疗，强调并监测治疗的持续性，以避免对其生活造成不利影响。
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