School of Psychology, University of Queensland, Brisbane, Australia; Surgical Treatment and Rehabilitation Service, Brisbane, Australia; Radiological Sciences, Mental Health and Clinical Neuroscience, School of Medicine, University of Nottingham, Nottingham, United Kingdom.
Radiological Sciences, Mental Health and Clinical Neuroscience, School of Medicine, University of Nottingham, Nottingham, United Kingdom; Sir Peter Mansfield Imaging Centre, University of Nottingham, Nottingham, United Kingdom.
Eur J Paediatr Neurol. 2022 Sep;40:34-39. doi: 10.1016/j.ejpn.2022.07.004. Epub 2022 Jul 21.
To explore neurological factors affecting quality of life (QoL) in children and young people with ataxia-telangiectasia (A-T), from both child and parent perspective.
24 children/young people with A-T (mean age 11.2 ± 3.5 years; 13 males) and 20 parents were recruited, and 58% were reassessed after an average interval of 3.4 years. Participants completed the PedsQL QoL assessment. Participants with A-T underwent structured neurological examination. QoL data from 20 healthy controls and their parents was used for comparison.
Children/young people with A-T rated their QoL higher than parental ratings across time points, with no longitudinal change. Higher age of the child participant correlated with lower parental (r = -0.43, p = .008) but not child ratings of QoL (r = -0.16, p = .380). Child and parent QoL ratings from the A-T group were lower than respective ratings from controls (η = 0.44 and η = 0.75 respectively, both p < .0005, controlled for socioeconomic status). Parental, but not child, ratings of QoL was predicted by a regression model based on neurological scores (R = 0.44, p=<.001).
Neurological disability does not determine child/young person QoL ratings in A-T. While certain aspects of neurological disability predict parent-proxy ratings, there is no decline in QoL over time. These results may reflect resilience in the face of a complex life-limiting disorder.
从儿童和家长的角度探讨影响共济失调毛细血管扩张症（A-T）患儿生活质量（QoL）的神经学因素。
共招募了 24 名 A-T 患儿/青少年（平均年龄 11.2±3.5 岁，男性占 58%）及其 20 名家长，其中 58%的患儿在平均 3.4 年后接受了再次评估。参与者完成了 PedsQL QoL 评估，A-T 患者接受了结构化神经学检查。同时使用了 20 名健康对照及其家长的 QoL 数据进行比较。
A-T 患儿/青少年的自评 QoL 评分高于家长报告的评分，且无纵向变化。患儿年龄越大，其家长报告的 QoL 评分越低（r=-0.43，p=0.008），但患儿的自评 QoL 评分则无变化（r=-0.16，p=0.380）。A-T 组的患儿和家长的 QoL 评分均低于相应的对照组（η=0.44 和 η=0.75，均 p<0.0005，调整社会经济地位后）。基于神经学评分的回归模型可以预测家长的 QoL 评分（R=0.44，p<.001），但不能预测患儿的 QoL 评分。
神经学障碍并不能决定 A-T 患儿/青少年的 QoL 评分。尽管某些神经学障碍方面可以预测家长的代理评分，但 QoL 并没有随时间下降。这些结果可能反映了在面对复杂的生命限制疾病时的适应能力。
