Bisphosphonate Use and Fractures in Adults with Hypophosphatasia

Bibliographic Details
Title: Bisphosphonate Use and Fractures in Adults with Hypophosphatasia
Authors: Kate Rassie, Michael Dray, Toshimi Michigami, Tim Cundy
Source: JBMR Plus, Vol 3, Iss 10, Pp n/a-n/a (2019)
Publisher Information: Oxford University Press, 2019.
Publication Year: 2019
Collection: LCC:Orthopedic surgery
LCC:Diseases of the musculoskeletal system
Subject Terms: ALPL, ATYPICAL FEMORAL FRACTURES, GENE MUTATIONS, HYPOPHOSPHATASIA, OSTEOMALACIA, OSTEOPOROSIS, Orthopedic surgery, RD701-811, Diseases of the musculoskeletal system, RC925-935
More Details: ABSTRACT Adults with hypophosphatasia (HPP) may suffer femoral fractures resembling the atypical femoral fractures that can occur with long‐term bisphosphonate treatment, and there is an emerging consensus that bisphosphonates should not be used in adults with HPP and low bone mass. However, the spectrum of HPP in adults is wide: ranging from the severely affected—who commonly have osteomalacia—through to the minimally affected. The former typically have biallelic and the latter, heterozygous ALPL mutations. We have reviewed reports of fractures in adults with genetically proven HPP which suggest that the risk of fracture is at least 200‐fold greater in those with biallelic mutations. We also discuss two cases of postmenopausal women with heterozygous ALPL mutations. One had fractures and severe osteoporosis, but histology revealed no evidence of osteomalacia. The second had taken alendronate for 8 years, but despite profound suppression of bone turnover, histology again revealed no evidence of osteomalacia. The management of adults with HPP who have coexisting osteoporosis is challenging. More data are clearly needed, but we suggest that the risks of bisphosphonate therapy may be relatively low in patients who have heterozygous mutations and no histological evidence of osteomalacia. © 2019 The Authors. JBMR Plus published by Wiley Periodicals, Inc. on behalf of American Society for Bone and Mineral Research © 2019 The Authors. JBMR Plus published by Wiley Periodicals, Inc. on behalf of American Society for Bone and Mineral Research.
Document Type: article
File Description: electronic resource
Language: English
ISSN: 2473-4039
Relation: https://doaj.org/toc/2473-4039
DOI: 10.1002/jbm4.10223
Access URL: https://doaj.org/article/15ba7041856a4875a37d6d33958927bc
Accession Number: edsdoj.15ba7041856a4875a37d6d33958927bc
Database: Directory of Open Access Journals
More Details
ISSN:24734039
DOI:10.1002/jbm4.10223
Published in:JBMR Plus
Language:English