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原发性慢性骨髓炎是一种单一的疾病吗?基于对过去30年治疗患者的回顾性分析提出一种分类方法。

Is primary chronic osteomyelitis a uniform disease? Proposal of a classification based on a retrospective analysis of patients treated in the past 30 years.

作者信息

Baltensperger Marc, Grätz Klaus, Bruder Elisabeth, Lebeda Richard, Makek Miro, Eyrich Gerold

机构信息

Department of Cranio-Maxillofacial Surgery, University Hospital Zürich, Switzerland.

出版信息

J Craniomaxillofac Surg. 2004 Feb;32(1):43-50. doi: 10.1016/j.jcms.2003.07.008.

Abstract

INTRODUCTION

Primary chronic osteomyelitis of the jaw is a rare, non-suppurative, chronic inflammatory disease of unknown aetiology. To date, classification is confusing due to a non-uniform terminology. The aim of this study was to establish a simple (clinical) classification based on patient data from our clinic.

METHODS

Retrospective analysis revealed 30 cases of which clinical course, radiology, pathology, therapy and outcome were analysed.

RESULTS

Both sexes were equally represented. The mean age at onset of disease was 35 years (range 5-76 years). Onset of disease revealed two peaks of incidence, one in adolescence and one after age 50 years. While clinical symptoms were similar in all cases, an increased intensity of these symptoms was noted in younger individuals as well as in the early stages of the disease. Five adults and one adolescent presented with additional non facial bone, joint and skin manifestations consistent with the diagnosis of SAPHO (synovitis, acne, pustulosis, hyperostosis, osteitis) syndrome, or chronic recurrent multifocal osteomyelitis. Radiology demonstrated sclerosis, osteolysis and periosteal reaction in variable stages in all cases. However, findings were more extensive in younger patients. Histology revealed different stages of chronic inflammation in all cases. Microabscess formation was noted in 11 cases, six of which were children/adolescents. Therapy consisted mainly of surgery, antibiotics and hyperbaric oxygen therapy. At the end of the follow up period, 11 patients demonstrated complete remission, while in 14 cases amelioration and in 5 no significant improvement was noted.

CONCLUSION

Based on differences in age at presentation, clinical appearance and course, radiology and histology, a subclassification into early and adult onset primary chronic osteomyelitis has been established. Cases with purely mandibular involvement should further be distinguished from cases associated with other syndromes.

摘要

引言

颌骨原发性慢性骨髓炎是一种罕见的、非化脓性的、病因不明的慢性炎症性疾病。迄今为止,由于术语不统一,分类较为混乱。本研究的目的是根据我们诊所的患者数据建立一种简单的(临床)分类方法。

方法

回顾性分析显示有30例病例,对其临床病程、放射学、病理学、治疗方法及结果进行了分析。

结果

男女比例相同。疾病发病的平均年龄为35岁(范围5 - 76岁)。疾病发病呈现两个发病高峰,一个在青春期,另一个在50岁以后。虽然所有病例的临床症状相似,但在较年轻个体以及疾病早期,这些症状的强度有所增加。5名成年人和1名青少年出现了与滑膜炎、痤疮、脓疱病、骨肥厚、骨炎(SAPHO)综合征或慢性复发性多灶性骨髓炎诊断相符的额外非面部骨骼、关节和皮肤表现。放射学检查在所有病例中均显示出不同阶段的骨质硬化、骨质溶解和骨膜反应。然而,年轻患者的表现更为广泛。组织学检查在所有病例中均显示出慢性炎症的不同阶段。11例出现微脓肿形成,其中6例为儿童/青少年。治疗主要包括手术、抗生素和高压氧治疗。在随访期结束时,11例患者完全缓解,14例有所改善,5例无明显改善。

结论

基于发病年龄、临床表现及病程、放射学和组织学的差异,已建立了早发型和成人型原发性慢性骨髓炎的亚分类。单纯下颌骨受累的病例应进一步与其他综合征相关的病例相区分。

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