RANK-L 抑制剂治疗难治性广泛颅面骨纤维结构不良：一例报告。
RANK-L inhibitor as a promising agent for refractory extensive craniofacial fibrous dysplasia: A case report.
机构信息
Department of Otolaryngology - Head and Neck Surgery, Tanta University, Tanta, Egypt.
Department of Otolaryngology - Head and Neck Surgery, The Ohio State University Wexner Medical Center, Columbus, Ohio, USA.
出版信息
Head Neck. 2024 Jan;46(1):E1-E5. doi: 10.1002/hed.27546. Epub 2023 Oct 12.
BACKGROUND
McCune-Albright syndrome is a rare disorder characterized by polyostotic fibrous dysplasia (FD), café-au-lait skin pigmentation, and endocrine dysfunction. Extensive FD in the craniofacial region can present significant challenges in terms of disease control and carries a high risk of permanent visual impairment.
METHODS
We present a case of medically and surgically resistant FD that required nine optic nerve decompressions.
RESULTS
The condition was ultimately controlled with the use of the denosumab agent.
CONCLUSION
The case highlights the importance and potential efficacy of denosumab in resistant FD management, particularly in cases involving sensitive organs.
背景
McCune-Albright 综合征是一种罕见疾病，其特征为多骨性纤维结构不良（FD）、咖啡牛奶斑皮肤色素沉着和内分泌功能障碍。颅面区域广泛的 FD 在疾病控制方面会带来重大挑战，并存在永久性视力损害的高风险。
方法
我们报告了一例经药物和手术治疗抵抗的 FD，该患者需要进行九次视神经减压手术。
结果
最终使用地舒单抗药物控制了病情。
结论
该病例突出了地舒单抗在抵抗性 FD 管理中的重要性和潜在疗效，特别是在涉及敏感器官的情况下。
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