Clinical and translational pharmacological aspects of the management of fibrous dysplasia of bone.
McCune-Albright syndrome
RANK-L
bisphosphonates
bone tumours
fibrous dysplasia
Journal
British journal of clinical pharmacology
ISSN: 1365-2125
Titre abrégé: Br J Clin Pharmacol
Pays: England
ID NLM: 7503323
Informations de publication
Date de publication:
06 2019
06 2019
Historique:
received:
01
07
2018
revised:
08
11
2018
accepted:
10
11
2018
pubmed:
25
11
2018
medline:
14
4
2020
entrez:
25
11
2018
Statut:
ppublish
Résumé
Fibrous dysplasia (FD) is a genetic, noninheritable rare bone disease caused by a postzygotic activating mutation of the α subunit of the stimulatory G-protein causing increased abnormal bone formation leading to pain, deformity and fractures. To date, no cure has been identified for FD/McCune-Albright syndrome (MAS) and treatment is symptomatic and aimed at decreasing pain and/or local bone turnover. Various drugs have been used to achieve clinical improvement in FD/MAS patients including bisphosphonates and denosumab, however further translational studies are also warranted to address unresolved pathophysiological issues and explore novel pharmacological targets for the management of FD/MAS. In this article, we review literature on the medical treatment of FD/MAS, discuss the unresolved pathophysiological issues and explore novel pharmacological targets for the management of FD/MAS.
Identifiants
pubmed: 30471134
doi: 10.1111/bcp.13820
pmc: PMC6533424
doi:
Substances chimiques
Bone Density Conservation Agents
0
Types de publication
Journal Article
Research Support, Non-U.S. Gov't
Review
Langues
eng
Sous-ensembles de citation
IM
Pagination
1169-1179Informations de copyright
© 2018 The Authors. British Journal of Clinical Pharmacology published by John Wiley & Sons Ltd on behalf of British Pharmacological Society.
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