Paediatric Neurology, University Hospitals Bristol, University College London, UK.
Paediatric Neurology, University Hospitals Bristol, UK.
Eur J Paediatr Neurol. 2019 Nov;23(6):801-807. doi: 10.1016/j.ejpn.2019.09.006. Epub 2019 Sep 13.
The quality of life (QoL) of patients with Tuberous Sclerosis Complex (TSC) has not been studied before. We aimed to investigate the impact of the disease on QoL. We studied the QoL of 91 TSC patients who have attended the Bath TSC clinic, UK over 6 months. QoL was evaluated using the PedsQL for children, and SF-36 for adults.
Impaired QoL is found in all patients with TSC regardless of the presence of epilepsy and learning disabilities (LD). Total mean self-reported score for children was 71 out of 100, compared to a UK norm of 84, p < 0.000. The proxy mean score was 48, (UK norm 85, p < 0.000). Physical Functioning score for adults with TSC was 70, compared to a UK norm of 94, p < 0.000. The Social Functioning score for adults with TSC was 71, (UK norm 88, p < 0.000).
Impaired QoL is found in all patients with TSC regardless of the presence of epilepsy and learning disabilities. The psychosocial domain is most affected. The quality of life of children with TSC is lower than children who suffer from asthma, diabetes, cancer and inflammatory bowel disease. To improve health related quality of life in TSC, a focus on patient's physical health, educational performances, and overall quality of life is crucial. In order to achieve this, coordinated medical care across disciplines, and psychosocial and social support is necessary.
之前从未研究过结节性硬化症 (TSC) 患者的生活质量 (QoL)。我们旨在研究该疾病对 QoL 的影响。我们研究了在英国巴斯 TSC 诊所就诊的 91 名 TSC 患者在过去 6 个月的 QoL。使用 PedsQL 对儿童进行 QoL 评估，使用 SF-36 对成人进行 QoL 评估。
所有 TSC 患者（无论是否患有癫痫和学习障碍 (LD)）的 QoL 均受损。儿童自我报告的总平均得分为 100 分中的 71 分，而英国的标准为 84 分，p<0.000。代理平均得分为 48 分，（英国标准为 85 分，p<0.000）。TSC 成年患者的身体机能评分为 70 分，而英国标准为 94 分，p<0.000。TSC 成年患者的社会功能评分为 71 分，（英国标准为 88 分，p<0.000）。
无论是否存在癫痫和学习障碍，所有 TSC 患者的 QoL 均受损。受影响最严重的是心理社会领域。TSC 儿童的生活质量低于患有哮喘、糖尿病、癌症和炎症性肠病的儿童。为了提高 TSC 的健康相关生活质量，必须关注患者的身体健康、教育表现和整体生活质量。为了实现这一目标，需要跨学科协调医疗保健以及心理社会和社会支持。
