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Bone Quality in Patients with a Congenital Myopathy: A Scoping Review.


ABSTRACT:

Background

Congenital myopathies are rare neuromuscular disorders presenting with a wide spectrum of clinical features, including long bone fractures (LBFs) that negatively influence functional prognosis, quality of life and survival. Systematic research on bone quality in these patients is lacking.

Objective

This scoping review aims to summarize all evidence on bone quality and to deduce recommendations for bone quality management in congenital myopathies.

Methods

Five electronic databases (Pubmed, Embase, Cochrane, Web of Science, CINAHL) were searched. All studies on bone quality in congenital myopathies were included. Decreased bone quality was defined as low bone mineral density and/or (fragility) LBFs. Study selection and data extraction were performed by three

SUBMITTER: Bouman K 

PROVIDER: S-EPMC9881028 | biostudies-literature | 2023

REPOSITORIES: biostudies-literature