Unavane Ojas, Tiwari Kritika, Nagral Aabha, Aggarwal Ritika, Garg Nikita, Nagral Nishtha, Verma Bela, Jhaveri Ajay, Setia Maninder S
Seth G.S Medical College and KEM Hospital, Mumbai, India.
Matushree Gomati Hospital, Mumbai, India.
J Clin Exp Hepatol. 2022 Mar-Apr;12(2):461-466. doi: 10.1016/j.jceh.2021.05.013. Epub 2021 Jun 9.
Wilson's disease (WD) is a chronic disease caused by altered copper metabolism requiring lifelong therapy. Its long-term and debilitating nature has the potential to affect the quality of life (Qol) of patients as well as their families. Our study aims to assess this impact of the disease on patients and their families.
We conducted a prospective, observational study over 2 years on 73 patients and 73 age-matched controls with 33 children and 40 adults in each group. The Qol of cases and controls was assessed using the PedsQL Generic Core Scales and World Health Organisation Quality of Life BREF (WHOQOL-BREF) for children and adults, respectively. Families of child and adult patients were interviewed using PedsQL Family Impact Module and Family Attitude Scale (FAS), respectively. The data were statistically analyzed.
Mean age of the cases was 22.04 ± 11.8 years. Qol scores for both adults and children were worse in cases with neuropsychiatric disease than in those with hepatic disease. For children, the mean scores of overall psychological functioning were lower in cases compared with controls ( = 0.0001). Qol of parents of the patients was significantly lower than those of parents of the controls as was the family functioning ( = 0.0001 and = 0.016). Family Attitude Scale scores for adults did not differ significantly between cases and controls.
The Qol of patients with neuro-WD is worse than that of hepatic disease. The disease impacts the psychological functioning of the children and the Qol of their families, which improves with the duration of the disease.
WD is a long-term, debilitating disease. Patients have to take lifelong treatment with frequent medical visits and often multiple hospitalizations.
WD affects the Qol of not only the patients but also their families. Qol of patients with neuro-WD is worse than that of patients with hepatic disease.
威尔逊病（WD）是一种由铜代谢改变引起的慢性疾病，需要终身治疗。其长期且使人衰弱的特性有可能影响患者及其家庭的生活质量（Qol）。我们的研究旨在评估该疾病对患者及其家庭的这种影响。
我们对73例患者和73名年龄匹配的对照者进行了为期2年的前瞻性观察研究，每组包括33名儿童和40名成人。分别使用儿童生活质量量表通用核心量表（PedsQL）和世界卫生组织生活质量简表（WHOQOL-BREF）对儿童和成人的病例组及对照组的生活质量进行评估。分别使用儿童生活质量量表家庭影响模块（PedsQL）和家庭态度量表（FAS）对儿童和成人患者的家庭进行访谈。对数据进行统计学分析。
病例组的平均年龄为22.04±11.8岁。患有神经精神疾病的患者的成人和儿童生活质量得分均低于患有肝脏疾病的患者。对于儿童，病例组的总体心理功能平均得分低于对照组（P = 0.0001）。患者父母的生活质量显著低于对照组父母，家庭功能也是如此（P = 0.0001和P = 0.016）。病例组和对照组成人的家庭态度量表得分无显著差异。
神经型WD患者的生活质量比肝脏疾病患者更差。该疾病影响儿童的心理功能及其家庭的生活质量，随着疾病持续时间的延长，情况会有所改善。
WD是一种长期使人衰弱的疾病。患者必须终身接受治疗，频繁就医且常需多次住院。
WD不仅影响患者的生活质量，还影响其家庭的生活质量。神经型WD患者的生活质量比肝脏疾病患者更差。
