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Evaluation of body composition as a potential biomarker in spinal muscular atrophy

Baranello, G; De Amicis, R; Arnoldi, MT; Zanin, R; Mastella, C; Masson, R; Leone, A; ... Bertoli, S; + view all (2020) Evaluation of body composition as a potential biomarker in spinal muscular atrophy. Muscle & Nerve 10.1002/mus.26823. (In press). Green open access

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Abstract

INTRODUCTION We aimed to investigate the correlation between body composition (BC) and spinal muscular atrophy (SMA)‐specific motor function assessments. METHODS Patients with SMA types I or II, aged 1 to 10 years, were recruited in this cross‐sectional study. The protocol included anthropometric measurements, and dual‐energy X‐ray absoprtiometry to assess fat mass (FM), lean mass (LM), fat‐free mass (FFM), FM and FFM indexes (FMI, FFMI), and motor function assessments (Children's Hospital of Philadelphia Infant Test of Neuromuscular Disorders scale for SMAI, and Hammersmith Functional Motor Scale―Expanded for SMAII). DISCUSSION Eighty‐eight children were included. All had a higher FM percentage than reference values. Motor function was moderately correlated with body mass index (BMI), FFMI, and LMI in SMAI, and weakly correlated with FFMI, LMI, and LM:FM ratio in SMAII. DISCUSSION BC shows promise as a potential biomarker for SMA, but further studies are needed.

Type: Article
Title: Evaluation of body composition as a potential biomarker in spinal muscular atrophy
Location: United States
Open access status: An open access version is available from UCL Discovery
DOI: 10.1002/mus.26823
Publisher version: https://doi.org/10.1002/mus.26823
Language: English
Additional information: This is an open access article under the terms of the Creative Commons Attribution‐NonCommercial‐NoDerivs License, which permits use and distribution in any medium, provided the original work is properly cited, the use is non‐commercial and no modifications or adaptations are made. http://creativecommons.org/licenses/by-nc-nd/4.0/
Keywords: body composition, fat-free mass, Hammersmith Functional Motor Scale―Expanded, spinal muscular atrophy, The Children's Hospital of Philadelphia Infant Test of Neuromuscular Disorders
UCL classification: UCL
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Population Health Sciences
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Population Health Sciences > UCL GOS Institute of Child Health
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Population Health Sciences > UCL GOS Institute of Child Health > Developmental Neurosciences Dept
URI: https://discovery.ucl.ac.uk/id/eprint/10091228
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