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Evaluation of body composition as a potential biomarker in spinal muscular atrophy
Author(s) -
Baranello Giovanni,
De Amicis Ramona,
Arnoldi Maria Teresa,
Zanin Riccardo,
Mastella Chiara,
Masson Riccardo,
Leone Alessandro,
Alberti Katia,
Foppiani Andrea,
Battezzati Alberto,
Bertoli Simona
Publication year - 2020
Publication title -
muscle and nerve
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.025
H-Index - 145
eISSN - 1097-4598
pISSN - 0148-639X
DOI - 10.1002/mus.26823
Subject(s) - sma* , spinal muscular atrophy , medicine , anthropometry , biomarker , dual energy x ray absorptiometry , physical medicine and rehabilitation , motor function , lean body mass , body mass index , physical therapy , body weight , biology , bone mineral , biochemistry , mathematics , disease , osteoporosis , combinatorics
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.