Denosumab Therapy in Cherubism.

Autor: Liles SI; School of Medicine, University of Mississippi Medical Center, Jackson, MS, USA., C Hoppe I; Division of Plastic Surgery, Department of Surgery, University of Mississippi Medical Center, Jackson, MS, USA., Arnold L; Division of Hematology/Oncology, Department of Pediatrics, University of Mississippi Medical Center, Jackson, MS, USA.
Jazyk: angličtina
Zdroj: The Cleft palate-craniofacial journal : official publication of the American Cleft Palate-Craniofacial Association [Cleft Palate Craniofac J] 2023 Dec; Vol. 60 (12), pp. 1665-1673. Date of Electronic Publication: 2022 Jul 12.
DOI: 10.1177/10556656221113891
Abstrakt: 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.
Competing Interests: Declaration of Conflicting InterestsThe author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.
Databáze: MEDLINE