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Interdisciplinary management of FGF23-related phosphate wasting syndromes: a Consensus Statement on the evaluation, diagnosis and care of patients with X-linked hypophosphataemia.

Authors :
Trombetti, Andrea
Al-Daghri, Nasser
Brandi, Maria Luisa
Cannata-Andía, Jorge B.
Cavalier, Etienne
Chandran, Manju
Chaussain, Catherine
Cipullo, Lucia
Cooper, Cyrus
Haffner, Dieter
Harvengt, Pol
Harvey, Nicholas C.
Javaid, Muhammad Kassim
Jiwa, Famida
Kanis, John A.
Laslop, Andrea
Laurent, Michaël R.
Linglart, Agnès
Marques, Andréa
Mindler, Gabriel T.
Source :
Nature Reviews Endocrinology. Jun2022, Vol. 18 Issue 6, p366-384. 19p.
Publication Year :
2022

Abstract

X-linked hypophosphataemia (XLH) is the most frequent cause of hypophosphataemia-associated rickets of genetic origin and is associated with high levels of the phosphaturic hormone fibroblast growth factor 23 (FGF23). In addition to rickets and osteomalacia, patients with XLH have a heavy disease burden with enthesopathies, osteoarthritis, pseudofractures and dental complications, all of which contribute to reduced quality of life. This Consensus Statement presents the outcomes of a working group of the European Society for Clinical and Economic Aspects of Osteoporosis, Osteoarthritis and Musculoskeletal Diseases, and provides robust clinical evidence on management in XLH, with an emphasis on patients' experiences and needs. During growth, conventional treatment with phosphate supplements and active vitamin D metabolites (such as calcitriol) improves growth, ameliorates leg deformities and dental manifestations, and reduces pain. The continuation of conventional treatment in symptom-free adults is still debated. A novel therapeutic approach is the monoclonal anti-FGF23 antibody burosumab. Although promising, further studies are required to clarify its long-term efficacy, particularly in adults. Given the diversity of symptoms and complications, an interdisciplinary approach to management is of paramount importance. The focus of treatment should be not only on the physical manifestations and challenges associated with XLH and other FGF23-mediated hypophosphataemia syndromes, but also on the major psychological and social impact of the disease. [ABSTRACT FROM AUTHOR]

Details

Language :
English
ISSN :
17595029
Volume :
18
Issue :
6
Database :
Academic Search Index
Journal :
Nature Reviews Endocrinology
Publication Type :
Academic Journal
Accession number :
156891005
Full Text :
https://doi.org/10.1038/s41574-022-00662-x