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尿毒症性狮子病,继发于甲状旁腺功能亢进的严重肾性骨营养不良的罕见表现。

Uremic leontiasis ossea, a rare presentation of severe renal osteodystrophy secondary to hyperparathyroidism.

机构信息

Faculty of Dentistry, University of Chile, Chile; Maxillofacial Surgery Service, San Juan de Dios Hospital, Chile.

Faculty of Dentistry, University of Chile, Chile.

出版信息

J Stomatol Oral Maxillofac Surg. 2018 Feb;119(1):56-60. doi: 10.1016/j.jormas.2017.10.006. Epub 2017 Oct 14.

Abstract

Renal osteodystrophy is a common complication of end-stage renal failure patients. It's most severe osseous complication is characterized by massive thickening of the cranial vault and facial bones, called uremic leontiasis ossea (ULO), with only few cases reported in the literature. A case of a 47-year-old female patient with ULO is presented. Physical examination showed enlargement of the jaws, which hinders proper ventilation and feeding. The computed tomography examination showed marked osseous proliferation in the jaws causing severe bony expansion and loss of normal bony architecture in the skull and the skull base. The most relevant clinical, histopathological and laboratory findings are discussed. The uremic leontiasis ossea causes significant aesthetic and functional changes. Correct diagnosis and management of the factors responsible for the development of bone lesions due to altered bone metabolism are key factors. The maxillofacial surgeon must have the proper knowledge of patient's medical condition and bone maturation status to address an adequate surgical strategy.

摘要

肾性骨营养不良是终末期肾衰竭患者的常见并发症。其最严重的骨骼并发症表现为颅穹窿和面部骨的广泛增厚,称为尿毒症性狮子头骨病(ULO),文献中仅报道了少数病例。本文报告了一例 47 岁女性 ULO 患者。体格检查显示颌骨增大,妨碍了正常的通气和进食。计算机断层扫描检查显示颌骨有明显的骨质增生,导致颅骨和颅底严重骨扩张和正常骨结构丧失。讨论了最相关的临床、组织病理学和实验室发现。尿毒症性狮子头骨病会引起明显的美观和功能改变。正确诊断和处理由于骨代谢改变而导致骨病变的因素是关键。颌面外科医生必须充分了解患者的身体状况和骨成熟状态,以制定出合适的手术策略。

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