[纤维发育不良]。

Orthopadie (Heidelberg, Germany) Pub Date : 2024-10-01 Epub Date: 2024-09-05 DOI:10.1007/s00132-024-04548-w
A Waltermann, B Westhoff
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引用次数: 0

摘要

纤维发育不良是一种零星发生的良性骨骼疾病,其特点是正常骨组织被过度增殖的细胞纤维组织取代。它可以单骨质或多骨质形式出现。根据病变的位置、数量和大小,临床表现可从无症状到严重残疾不等。典型的问题是骨痛、骨畸形和病理性骨折。如果合并有内分泌疾病和/或皮肤表现(咖啡斑),则被称为麦库恩-阿尔布莱特综合征。主要通过放射学进行诊断,骨骼病变的特征是浑浊、磨砂玻璃样。无法进行病因治疗。骨科治疗包括缓解疼痛、稳定骨骼、矫正畸形,必要时还包括病灶清理以及通过抗骨质吸收药物防止病情恶化。病理性骨折最好采用髓内骨合成术进行稳定。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
[Fibrous dysplasia].

Fibrous dysplasia is a sporadically occurring benign skeletal disease characterized by the replacement of normal bone tissue with excessively proliferating cellular fibrous tissue. It can occur in a monostotic or polyostotic form. Depending on the location, number and size of the lesions, the clinical picture can vary from an asymptomatic disease to a severe disability. Typical problems are bone pain, bone deformities and pathological fractures. In combination with endocrinopathies and/or skin manifestations (café au lait spots), it is referred to as the McCune-Albright syndrome. The diagnosis is mainly carried out radiologically and the bony lesions are characterized by a cloudy, frosted glass-like aspect. Causal treatment is not possible. Orthopedic treatment includes pain relief, bone stabilization, deformity correction and, if necessary, lesion cleansing as well as the prevention of progression by means of antiresorptive medication. Pathological fractures are preferably stabilized with intramedullary osteosynthesis procedures.

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