Pediatric Rheumatology and Osteology, University Children's Hospital Wuerzburg, Josef-Schneider-Str. 2, 97080, Wuerzburg, Germany.
Pediatric Pulmonology and Sports Medicine, University Children's Hospital Wuerzburg, Wuerzburg, Germany.
Pediatr Rheumatol Online J. 2019 Jul 18;17(1):45. doi: 10.1186/s12969-019-0351-4.
Chronic non-bacterial osteomyelitis (CNO) is an autoinflammatory disorder of the skeletal system of yet unknown etiology. Patients present with local bone pain and inflammation and - to our experience - often suffer from functional impairment with significant disabilities of daily life. The objective of this study was to assess physical activity, fitness and health-related quality of life (HRQOL) in adolescents with established diagnosis of CNO versus healthy controls (HC).
15 patients with CNO and 15 age and gender matched HC aged 13-18 years, completed questionnaires, performed an incremental exercise test with gas exchange measures up to voluntary fatigue and wore an accelerometer over 7 days at home to assess physical activity behavior.
At the time of assessment, 5 CNO patients were in clinical, one in radiological and 5 in clinical and radiological remission. 7 did not receive any therapy at the time of assessment. The results of the exercise test and of the accelerometry did not show any significant difference between CNO and HC. However, reported sports participation was lower in patients with CNO and PedsQL3.0 and 4.0 showed significant lower values in most of the scores indicating reduced HRQOL.
Although most CNO patients showed a favorable course of disease without any relevant differences in objective measurements of physical activity and fitness versus HC at the time of assessment, questionnaires revealed perceived limitations. Further studies are needed to measure HRQOL and to validate questionnaires in patients with CNO against objective measures including more participants with a higher level of disease activity.
慢性非细菌性骨髓炎（CNO）是一种病因不明的骨骼系统自身炎症性疾病。患者表现为局部骨痛和炎症，根据我们的经验，他们经常因功能障碍而遭受日常生活的严重残疾。本研究的目的是评估已确诊 CNO 的青少年与健康对照组（HC）的身体活动、健康相关体能和生活质量（HRQOL）。
15 名 CNO 患者和 15 名年龄和性别匹配的 13-18 岁 HC 完成了问卷调查，进行了递增运动试验，测量了气体交换直至自愿疲劳，并在家中佩戴加速度计 7 天以评估身体活动行为。
在评估时，5 名 CNO 患者处于临床缓解期，1 名处于放射学缓解期，5 名处于临床和放射学缓解期。7 名患者在评估时未接受任何治疗。运动试验和加速度计的结果在 CNO 和 HC 之间没有显示出任何显著差异。然而，CNO 患者报告的运动参与度较低，PedsQL3.0 和 4.0 的大多数评分显示 HRQOL 降低。
尽管大多数 CNO 患者的疾病表现出良好的缓解趋势，与评估时的 HC 相比，在身体活动和体能的客观测量方面没有任何显著差异，但问卷调查显示出了感知到的局限性。需要进一步的研究来测量 CNO 患者的 HRQOL，并使用客观测量（包括更多疾病活动水平较高的参与者）来验证针对 CNO 患者的问卷。
