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Interdisciplinary management of FGF23-related phosphate wasting syndromes : A Consensus Statement on the evaluation, diagnosis and care of patients with -linked hypophosphataemia
Trombetti, Andrea Al-Daghri, Nasser Brandi, Maria Luisa Cannata-Andia, Jorge B. Cavalier, Etienne Chandran, Manju Chaussain, Catherine Cipullo, Lucia Cooper, Cyrus Haffner, Dieter
Trombetti, Andrea
Al-Daghri, Nasser
Brandi, Maria Luisa
Cannata-Andia, Jorge B.
Cavalier, Etienne
Chandran, Manju
Chaussain, Catherine
Cipullo, Lucia
Cooper, Cyrus
Haffner, Dieter
Author
Trombetti, Andrea
Al-Daghri, Nasser
Brandi, Maria Luisa
Cannata-Andia, 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.
Minisola, Salvatore
Yerro, Maria Concepción Prieto
Rosa, Mario Miguel
Seefried, Lothar
Vlaskovska, Mila
Zanchetta, María Belén
Rizzoli, René
Al-Daghri, Nasser
Brandi, Maria Luisa
Cannata-Andia, 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.
Minisola, Salvatore
Yerro, Maria Concepción Prieto
Rosa, Mario Miguel
Seefried, Lothar
Vlaskovska, Mila
Zanchetta, María Belén
Rizzoli, René
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.
Keywords
Date
2022
Type
Journal article
Journal
Nature Reviews Endocrinology
Book
Volume
18
Issue
6
Page Range
366-384
Article Number
ACU Department
Mary MacKillop Institute for Health Research
Faculty of Health Sciences
Faculty of Health Sciences
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Relation URI
Source URL
Event URL
Open Access Status
License
All rights reserved
File Access
Controlled