严重疲劳在原发性免疫缺陷的儿科患者中很常见，与疾病活动无关。
Severe Fatigue Is Common Among Pediatric Patients with Primary Immunodeficiency and Is Not Related to Disease Activity.
机构信息
Department of Pediatrics, Wilhelmina Children's Hospital, University Medical Center Utrecht, Surface mail: HP KE.04.133.1, Post box 85090, 3508 AB, Utrecht, The Netherlands.
Department of medical physiology, Child Development and Exercise Center, Wilhelmina Children's Hospital, University Medical Center Utrecht, Utrecht, The Netherlands.
出版信息
J Clin Immunol. 2021 Aug;41(6):1198-1207. doi: 10.1007/s10875-021-01013-7. Epub 2021 Mar 17.
PURPOSE
Fatigue is a distressing symptom commonly reported among pediatric patients with primary immunodeficiency (PID). However, the relationship between fatigue and disease activity is currently unknown.
METHODS
In this cross-sectional study, we examined the prevalence of severe fatigue, the effect of fatigue on health-related quality of life (HRQoL), and the effects of disease activity and comorbidity on fatigue severity among pediatric patients 2-18 years of age with PID. Fatigue and HRQoL were assessed using the pediatric quality of life inventory multidimensional fatigue scale (PedsQL MFS) and generic core scales (PedsQL GCS), respectively. Linear regression analyses and an analysis of covariance were used to compare the fatigue scores with the scores obtained from a healthy control group. Data were adjusted for age and sex.
RESULTS
Of the 91 eligible patients, 79 were assessed (87% participation rate), with a mean age of 10.4 ± 4.4 years. Pediatric patients with PID reported significantly higher fatigue levels compared to healthy peers, with an 18.9% prevalence of severe fatigue. Moreover, higher fatigue levels were inversely associated with HRQoL in all domains and directly associated with school absences. We found that severe fatigue was comparable between common variable immunodeficiency (CVID), combined immunodeficiency (CID), and selective immunoglobulin A deficiency (SIgAD) patients, but was not reported in the X-linked agammaglobulinemia (XLA) patients studied. Finally, fatigue severity was not significantly associated with disease activity or comorbidity.
CONCLUSIONS
Nearly 20% of pediatric patients with PID reported experiencing severe fatigue, and fatigue was reported among a wide range of PID subcategories. In addition, severe fatigue negatively affected the patient's quality of life and daily functioning, but was not associated with disease activity or comorbidity. Thus, targeting severe fatigue might be a promising strategy for improving the overall well-being and quality of life of pediatric patients with PID.
目的
疲劳是原发性免疫缺陷（PID）儿科患者常报告的一种令人痛苦的症状。然而，目前尚不清楚疲劳与疾病活动之间的关系。
方法
在这项横断面研究中，我们检查了严重疲劳的患病率、疲劳对健康相关生活质量（HRQoL）的影响，以及疾病活动和合并症对 PID 儿科患者 2-18 岁患者疲劳严重程度的影响。疲劳和 HRQoL 分别使用儿科生活质量清单多维疲劳量表（PedsQL MFS）和通用核心量表（PedsQL GCS）进行评估。线性回归分析和协方差分析用于比较疲劳评分与健康对照组的评分。数据根据年龄和性别进行调整。
结果
在 91 名符合条件的患者中，有 79 名接受了评估（87%的参与率），平均年龄为 10.4±4.4 岁。PID 儿科患者报告的疲劳水平明显高于健康同龄人，严重疲劳的患病率为 18.9%。此外，更高的疲劳水平与所有领域的 HRQoL 呈负相关，与缺课直接相关。我们发现，常见变异性免疫缺陷（CVID）、联合免疫缺陷（CID）和选择性免疫球蛋白 A 缺乏症（SIgAD）患者的严重疲劳程度相当，但在所研究的 X 连锁无丙种球蛋白血症（XLA）患者中并未报告。最后，疲劳严重程度与疾病活动或合并症无显著相关性。
结论
近 20%的 PID 儿科患者报告出现严重疲劳，且疲劳报告存在于广泛的 PID 亚类中。此外，严重疲劳严重影响患者的生活质量和日常功能，但与疾病活动或合并症无关。因此，针对严重疲劳可能是改善 PID 儿科患者整体健康和生活质量的有前途的策略。
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