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). RESULTS: 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.
Evaluation of body composition as a potential biomarker in spinal muscular atrophy / G. Baranello, R. De Amicis, M.T. Arnoldi, R. Zanin, C. Mastella, R. Masson, A. Leone, K. Alberti, A. Foppiani, A. Battezzati, S. Bertoli. - In: MUSCLE & NERVE. - ISSN 0148-639X. - 61:4(2020), pp. 530-534. [10.1002/mus.26823]
Evaluation of body composition as a potential biomarker in spinal muscular atrophy
R. De AmicisSecondo
;A. Leone;A. Foppiani;A. BattezzatiPenultimo
;S. BertoliUltimo
2020
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). RESULTS: 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.File | Dimensione | Formato | |
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2020. Baranello et al. - Body composition as biomarker in SMA.pdf
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