Increasing Cases of Chronic Nonbacterial Osteomyelitis in Children: A Series of 215 Cases From a Single Tertiary Referral Center

Sumaya Aden, Stephen Q. Wong, Claire Yang, T. Bui, Travis Higa, Joshua Scheck, R. Iyer, M. Egbert, A. Lindberg, Yongdong Zhao
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引用次数: 8

Abstract

Objective Chronic nonbacterial osteomyelitis (CNO) is a rare autoinflammatory bone disease that is gaining recognition from clinicians and researchers. We aim to publish data from our cohort of patients with CNO living in the northwestern United States to increase the awareness of specific demographics, characteristics, and presentation of this rare disease. Methods A retrospective chart review was performed of our electronic medical records. Patients with complete chart records who met criteria for a diagnosis of CNO from 2005 to 2019 were included. Extracted data including patient demographics, bone biopsy results, and lesion locations on advanced imaging were analyzed. King County census data were used to calculate the annual new case rate within our center. Results A total of 215 CNO cases were diagnosed at our large tertiary pediatric hospital. The majority of cases were of White race residing in Washington’s most populous county, King County. Most cases were diagnosed in 2016 to 2019, showing a significant increase in the annual case rate from 8 to 23 per million children in King County, though there did not appear to be a seasonal predilection. Biopsy rate decreased from 75% to 52%. One hundred fifty-two (71%) children had family history of autoimmunity. With increasing use of whole-body magnetic resonance imaging (WB-MRI), results showed 68% had multiple lesions. Conclusion CNO has been diagnosed at an increased rate in recent years. WB-MRI may assist in identifying other lesions that may be asymptomatic on presentation. Bone biopsy is still required in some children at the time of diagnosis.
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儿童慢性非细菌性骨髓炎病例增加:来自单一三级转诊中心的215例病例
目的慢性非细菌性骨髓炎(CNO)是一种罕见的自身炎症性骨病,越来越受到临床医生和研究者的重视。我们的目标是公布生活在美国西北部的CNO患者队列的数据,以提高对这种罕见疾病的特定人口统计学、特征和表现的认识。方法对我院电子病历资料进行回顾性分析。纳入了2005年至2019年符合CNO诊断标准的完整病历记录的患者。提取的数据包括患者人口统计学、骨活检结果和高级影像学上的病变位置。金县人口普查数据用于计算本中心的年度新病例率。结果我院大型三级儿科医院共诊断CNO 215例。大多数病例是居住在华盛顿人口最多的金县的白人。大多数病例是在2016年至2019年诊断出来的,金县的年病例率从每百万儿童8例显著增加到23例,尽管似乎没有季节性倾向。活检率由75%降至52%。152例(71%)患儿有自身免疫家族史。随着全身磁共振成像(WB-MRI)应用的增加,结果显示68%的患者有多发病变。结论近年来CNO的诊断率呈上升趋势。WB-MRI可以帮助识别其他可能在表现时无症状的病变。一些儿童在确诊时仍需进行骨活检。
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The Journal of rheumatology. Supplement
The Journal of rheumatology. Supplement Medicine-Medicine (all)
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期刊介绍: The Journal of Rheumatology is a monthly international serial edited by Duncan A. Gordon, The Journal features research articles on clinical subjects from scientists working in rheumatology and related fields, as well as proceedings of meetings as supplements to regular issues. Highlights of our 36 years serving Rheumatology include: groundbreaking and provocative editorials such as "Inverting the Pyramid," renowned Pediatric Rheumatology, proceedings of OMERACT and the Canadian Rheumatology Association, Cochrane Musculoskeletal Reviews, and supplements on emerging therapies.
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