Liles Steele I, C Hoppe Ian, Arnold Laura
School of Medicine, University of Mississippi Medical Center, Jackson, MS, USA.
Division of Plastic Surgery, Department of Surgery, University of Mississippi Medical Center, Jackson, MS, USA.
Cleft Palate Craniofac J. 2023 Dec;60(12):1665-1673. doi: 10.1177/10556656221113891. Epub 2022 Jul 12.
Cherubism is a rare disorder characterized by proliferative fibro-osseous lesions that result in bilateral bony hyperplasia of the face. Management varies based on symptom severity and includes longitudinal follow-up, pharmacotherapy, and/or surgical debulking. Off-label treatment with denosumab, a human monoclonal antibody that binds RANKL and inhibits osteoclast function to reduce bone resorption, can be beneficial in suppressing the proliferation of bone to minimize the need for surgery and to control postoperative reproliferation. Close follow-up is needed to maintain appropriate electrolyte levels. The present case demonstrates the achievement of symptomatic control with denosumab in a child with severe refractory cherubism.
cherubism是一种罕见的疾病，其特征为增殖性纤维骨性病变，导致面部双侧骨质增生。治疗方法因症状严重程度而异，包括长期随访、药物治疗和/或手术减容。地诺单抗是一种结合RANKL并抑制破骨细胞功能以减少骨吸收的人单克隆抗体，其标签外治疗有助于抑制骨增殖，从而减少手术需求并控制术后再增殖。需要密切随访以维持适当的电解质水平。本病例展示了地诺单抗在一名患有严重难治性 cherubism的儿童中实现症状控制的情况。
