274th ENMC international workshop: recommendations for optimizing bone strength in neuromuscular disorders. Hoofddorp, The Netherlands, 19–21 January 2024

•Bone strength is impaired in most NMDs.•Bone strength needs to be addressed and treated in NMDs.•Time to initiate bone health monitoring, indication and type of therapy, and duration of clinical follow-up are not defined for most NMDs.•Longitudinal skeletal phenotyping is key to understand the indi...

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Veröffentlicht in:Neuromuscular disorders : NMD 2024-10, Vol.43, p.1-13
Hauptverfasser: Voermans, Nicol C., Dittrich, Anne T.M., Liguori, Sara, Panicucci, Chiara, Moretti, Antimo, Weber, David R., Ward, Leanne M., de Groot, Ingrid, Guglieri, Michela, Wood, Claire, Iolascon, Giovanni, Kostera-Pruszczyk, Anna, Erasmus, Corrie E., de Visser, Marianne, Ward, Leanne, Weber, David, Schlüter, Silke, Crabtree, Nicola, Fattore, Andrea Del, Claeys, Kristl G., Kroneman, Madelon, Wong, Sze Choong, Voermans, Nicol
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Sprache:eng
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Zusammenfassung:•Bone strength is impaired in most NMDs.•Bone strength needs to be addressed and treated in NMDs.•Time to initiate bone health monitoring, indication and type of therapy, and duration of clinical follow-up are not defined for most NMDs.•Longitudinal skeletal phenotyping is key to understand the individual's bone health trajectory.•Bisphosphonates seem efficacious as first-line therapy in bone fragility treatment in NMDs. The 274th ENMC workshop for optimizing bone strength in neuromuscular disorders (NMDs) was held on January 19–21, 2024. The group of participants included experts in the fields of bone health and neuromuscular medicine along with the patient voice. Bone strength represents a crucial aspect of the management of pediatric and adult patients with NMDs. Bone strength may be compromised due to different pathophysiologic mechanisms, including disrupted bone–muscle “cross-talk”, loss of biomechanical loading, nutritional insufficiency, inadequate weight-bearing physical activity, muscle weakness and/or immobility, and drug treatment. While for Duchenne muscular dystrophy recommendations for evaluation and treatment of bone strength have been published, evidence on bone strength in other hereditary and acquired NMDs is scarce. Enhanced knowledge is needed to understand the development and maintenance of bone strength in patients with NMDs. This workshop aimed to develop a strategy to improve bone strength and thus prevent fractures in patients with NMDs.
ISSN:0960-8966
1873-2364
1873-2364
DOI:10.1016/j.nmd.2024.07.009