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家族性地中海热患者的生活质量及其父母的认知

The Quality of Life in Patients with Familial Mediterranean Fever and Their Parents Perception.

作者信息

Ensari Esra, Akyol Onder Esra Nagehan, Bilac Oznur, Ertan Pelin

机构信息

Department of Pediatric Nephrology, Celal Bayar University School of Medicine, Manisa, Turkey.

Pediatric Nephrology, Aksaray Training and Research Hospital, Department of Paediatric Nephrology, Aksaray, Turkey.

出版信息

Klin Padiatr. 2024 Aug 6. doi: 10.1055/a-2339-3742.

Abstract

BACKGROUND

Familial Mediterranean fever (FMF) is a chronic disease characterized by recurrent episodes of fever and polyserositis. This study aimed to assess children's quality of life (QoL), as reported by children and their parents, and to compare the results according to clinical variables.

MATERIAL AND METHODS

The study examined 107 children with FMF, evaluating their demographic and genetic data, utilizing the Pediatric Quality of Life Inventory (PedsQL) to assess QoL, and comparing scores based on disease severity.

RESULTS

The severity of FMF is inversely correlated with QoL scores, with mild cases having the highest scores (97±4), followed by moderate (76±11) and severe cases (52±10.3) (p<0,001). Disease severity, treatment adherence, healthcare utilization, genetic mutations, family income, and maternal age at birth all significantly impact perceived quality of life in FMF patients (p<0,001). Additionally, parents reported lower QoL for children with FMF who experienced various adverse factors such as low family income, household smoking, frequent attacks, hospitalizations, irregular medication use, and low maternal education levels (p<0,001).

CONCLUSION

Children's daily activities, academic performance, and family functioning are all significantly impacted by FMF. Physicians caring for patients with FMF should be aware of the QoL changes in the management of these patients. As a result, medical therapy, patient education, and indicators of psychological and social support can all be offered more effectively.

摘要

背景

家族性地中海热(FMF)是一种以发热和多浆膜炎反复发作为特征的慢性疾病。本研究旨在评估儿童及其父母报告的儿童生活质量(QoL),并根据临床变量比较结果。

材料与方法

该研究对107名FMF患儿进行了检查,评估了他们的人口统计学和遗传数据,使用儿童生活质量量表(PedsQL)评估生活质量,并根据疾病严重程度比较得分。

结果

FMF的严重程度与生活质量得分呈负相关,轻度病例得分最高(97±4),其次是中度(76±11)和重度病例(52±10.3)(p<0.001)。疾病严重程度、治疗依从性、医疗利用、基因突变、家庭收入和母亲生育年龄均对FMF患者的感知生活质量有显著影响(p<0.001)。此外,父母报告称,经历各种不利因素(如家庭收入低、家庭吸烟、频繁发作、住院、用药不规律和母亲教育水平低)的FMF患儿生活质量较低(p<0.001)。

结论

FMF对儿童的日常活动、学业成绩和家庭功能均有显著影响。治疗FMF患者的医生应意识到这些患者管理中的生活质量变化。因此,可以更有效地提供药物治疗、患者教育以及心理和社会支持指标。

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