Purpose

Patients with primary immunodeficiency disease (PID) have an increased risk of experiencing physical activity limitations, social difficulties, and psychological problems due to their chronic condition. Evaluation of their health-related quality of life (HRQOL) and fatigue is crucial in these patients to help understand their complex disease and provide adequate medical care.

Methods

In this study, we evaluated HRQOL and fatigue in pediatric and young adult patients with PID attending our center. Participants completed the Pediatric Quality of Life Inventory (PedsQL), version 4.0, and the PedsQL multidimensional fatigue module, standard version.

Results

Fifty-three PID patients were recruited (age range: 2–23 years). The mean HRQOL score obtained was 66.61 (SD: 18.73) out of 100, and the emotional and work/school dimensions were the ones most highly affected. There were no significant differences in reported quality of life between patients and their caregivers. The mean patient-reported fatigue value was 68.81 (SD: 17.80) out of 100, and the rest-related dimension was the one most highly affected. In the caregivers’ assessment, general fatigue was the most highly affected dimension.

Conclusions

The results of this study show that quality of life is poor and fatigue measures are considerably increased in our young adult and pediatric patients with PIDs. These findings can indicate areas requiring more intensive interventions, and they will serve as a basis for comparison of future results.

中文翻译：

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原发性免疫缺陷儿童的健康相关生活质量和多维疲劳量表。

目的

原发性免疫缺陷病 (PID) 患者由于其慢性病而经历身体活动受限、社交困难和心理问题的风险增加。评估他们的健康相关生活质量 (HRQOL) 和疲劳对于这些患者来说至关重要，以帮助了解他们的复杂疾病并提供足够的医疗护理。

方法

在这项研究中，我们评估了在我们中心就诊的儿童和年轻成人 PID 患者的 HRQOL 和疲劳。参与者完成了儿科生活质量量表 (PedsQL) 4.0 版和 PedsQL 多维疲劳模块标准版。

结果

招募了 53 名 PID 患者（年龄范围：2-23 岁）。获得的平均 HRQOL 得分为 66.61（SD：18.73）（满分 100 分），情绪和工作/学校维度受到的影响最大。报告的患者与其照料者之间的生活质量没有显着差异。患者报告的平均疲劳值为 68.81（SD：17.80），共 100 分，与休息相关的维度是受影响最大的维度。在护理人员的评估中，全身疲劳是受影响最大的维度。

结论

这项研究的结果表明，在我们患有 PID 的年轻成人和儿童患者中，生活质量很差，疲劳程度大大增加。这些发现可以表明需要更深入干预的领域，它们将作为未来结果比较的基础。