Yildirim Deniz Gezgin, Bakkaloglu Sevcan Azime, Acar A Sebnem Soysal, Celik Bulent, Buyan Necla
Department of Pediatric Rheumatology, Diyarbakir Child Disease Hospital, Diyarbakir, Turkey.
Department of Pediatric Rheumatology, Gazi University Faculty of Medicine, Ankara, Turkey.
North Clin Istanb. 2021 Apr 26;8(3):255-260. doi: 10.14744/nci.2020.90093. eCollection 2021.
A favorable quality of life (QoL) is important in children with chronic disease, and it reflects successful disease management. The aim of our study was to evaluate QoL and its association with clinical parameters in pediatric patients with familial Mediterranean fever (FMF).
The Kinder Lebensqualität Fragebogen (KINDL) questionnaires (kiddy: 4-7 years; kid: 8-16 years) for children and the proxy version for parents were implemented as a QoL measure. A total of 171 FMF patients, 69 healthy peers and their parents were enrolled in the study.
The KINDL QoL scores of the FMF patients were significantly lower than their healthy peers. The physical and emotional well-being KINDL QoL scores of the FMF children were significantly lower than their healthy peers (p=0.017 and p=0.020, respectively). In the evaluation of the KINDL QoL scores between the kiddy and kid groups, only the self-esteem score was higher in the kiddy group (p=0.004), and the school functioning scores were higher in the kid group (p=0.002). The scores in the physical well-being and disease module had significant differences between patients who were adherent and those who were non-adherent to colchicine therapy (p=0.042 and p=0.047, respectively). The scores in the physical well-being and disease module were significantly higher in patients with fewer attacks than those who had many attacks per year (p=0.004 and p=0.014, respectively).
This study suggests that FMF patients have significantly impaired QoL. The irregular use of colchicine and more frequent attacks affect QoL even more. A QoL assessment with multidisciplinary follow-up and control of the disease activity are essential, and if necessary, individualized support should be given to patients.
良好的生活质量（QoL）对慢性病患儿很重要，它反映了疾病管理的成功与否。我们研究的目的是评估家族性地中海热（FMF）儿科患者的生活质量及其与临床参数的关联。
采用儿童生活质量问卷（Kinder Lebensqualität Fragebogen，KINDL）（儿童版：4 - 7岁；青少年版：8 - 16岁）以及家长代理版作为生活质量测量工具。本研究共纳入171例FMF患者、69名健康对照儿童及其家长。
FMF患者的KINDL生活质量评分显著低于其健康对照儿童。FMF患儿的身体和情感幸福感KINDL生活质量评分显著低于其健康对照儿童（分别为p = 0.017和p = 0.020）。在评估儿童版和青少年版KINDL生活质量评分时，仅自尊评分在儿童版组中较高（p = 0.004），而学校功能评分在青少年版组中较高（p = 0.002）。坚持使用秋水仙碱治疗和未坚持治疗的患者在身体幸福感和疾病模块的评分有显著差异（分别为p = 0.042和p = 0.047）。每年发作次数较少的患者在身体幸福感和疾病模块的评分显著高于发作次数多的患者（分别为p = 0.004和p = 0.014）。
本研究表明FMF患者的生活质量显著受损。秋水仙碱的不规律使用和更频繁的发作对生活质量的影响更大。进行多学科随访的生活质量评估以及控制疾病活动至关重要，如有必要，应给予患者个体化支持。
