Burosumab and Dental Abscesses in Children With X-Linked Hypophosphatemia

Margaux Gadion; Agathe Hervé; Julia Herrou; Anya Rothenbuhler; Violaine Smail-Faugeron; Frédéric Courson; Agnès Linglart; Catherine Chaussain; Martin Biosse Duplan

doi:10.1002/jbm4.10672

Burosumab and Dental Abscesses in Children With X-Linked Hypophosphatemia

JBMR Plus. 2022 Sep 20;6(11):e10672. doi: 10.1002/jbm4.10672. eCollection 2022 Nov.

Authors

Margaux Gadion^{1

2}, Agathe Hervé^{1

2}, Julia Herrou^{1

3}, Anya Rothenbuhler⁴, Violaine Smail-Faugeron^{1

2}, Frédéric Courson^{1

2}, Agnès Linglart^{4

5}, Catherine Chaussain^{1

2

6}, Martin Biosse Duplan^{1

2

7}

Affiliations

¹ Université Paris Cité Faculté de Santé (Unité de formation et de recherche Odontologie et Unité de formation et de recherche de médecine) Paris France.
² Assistance publique des hopitaux de Paris, Reference Center for Rare Diseases of the Calcium and Phosphate Metabolism, OSCAR Network, Fédération hospitalo-universitaire DDS-net, European reference network BOND, Department of Dental Medicine Bretonneau Hospital Paris France.
³ Assistance publique des hopitaux de Paris, Reference Center for Rare Diseases of the Calcium and Phosphate Metabolism, OSCAR Network, European reference network BOND, Department of Rheumatology Cochin Hospital Paris France.
⁴ Assistance publique des hopitaux de Paris, Reference Center for Rare Diseases of the Calcium and Phosphate Metabolism, OSCAR Network, Endo-European reference network and European reference network BOND, Department of Endocrinology and Diabetology for Children Bicêtre Paris Saclay Hospital Le Kremlin Bicêtre France.
⁵ Université Paris Saclay Institut national de la santé et de la recherche médicale unité mixte de recherche 1185 Physiologie et physiopathologie endocrinienne Le Kremlin Bicêtre France.
⁶ Université Paris Cité Laboratory Unité de recherche2496 Orofacial Pathologies, Imaging and Biotherapies Paris France.
⁷ Institut Imagine, INSERM U1163 Paris France.

Abstract

X-linked hypophosphatemia (XLH) is a rare genetic disorder that disrupts skeletal and dental mineralization. In addition to rickets in children, XLH patients also have frequent spontaneous dental abscesses that increase the risk of tooth loss and may lead to facial cellulitis. Hypomineralized and hypoplastic dentin is the main driver of these infections. Conventional treatment (CT) of XLH improves this tissue defect and reduces the occurrence of dental abscesses. Burosumab is a recent treatment for XLH that targets excess circulating fibroblast growth factor 23 (FGF23), and its benefits on rickets have been demonstrated. It is not yet known whether burosumab improves dental manifestations of XLH. The main objective of our study was to compare the incidence of dental abscesses with XLH treated with either CT or burosumab. In this monocentric retrospective study, we measured and compared the incidence of dental abscess in children with XLH treated with either CT or burosumab, followed at our dental center for at least 1 year. The primary endpoint was the number of dental abscesses per month of dental follow-up. A total of 71 children were included in the study, with a mean ± standard deviation (SD) age at the start of dental follow-up of 7.86 ± 3.76. Thirty-eight children were treated with CT (53.5%) and 33 with burosumab (46.5%). All children treated with burosumab had previously been treated with CT. The mean number of dental abscesses per month of dental follow-up was significantly reduced in the burosumab group compared with the CT group (0.01 versus 0.04; p = 0.04). Burosumab treatment appears to be associated with a reduction in the number of dental abscesses in XLH children, compared with CT. © 2022 The Authors. JBMR Plus published by Wiley Periodicals LLC on behalf of American Society for Bone and Mineral Research.

Keywords: BONE MATRIX; CELL/TISSUE SIGNALING – ENDOCRINE PATHWAYS; DENTAL BIOLOGY; DISORDERS OF CALCIUM/PHOSPHATE METABOLISM; PTH/VIT D/FGF23; |MATRIX MINERALIZATION.