Abstract
Body composition is sparsely described in spinal muscular atrophy (SMA). Body (BMI, mass/height in m 2), fat-free (FFMI, lean mass/height in m 2) and fat (FMI, fat mass/height in m 2) mass indexes were estimated in 25 children (aged 5–18) with SMA (2 type I, 13 type II, 10 type III) using dual-energy radiograph absorptiometry and anthropometric data referenced to gender and age-matched healthy children (NHANES III, New York Pediatric Rosetta Body Project). BMI was ⩾50th percentile in 11 (44%) and ⩾85th in 5 (20%). FFMI was reduced ( p < 0.005) and FMI was increased ( p < 0.005) in the overall study cohort. FMI was ⩾50th, ⩾85th and 95th percentiles in 19 (76%), 10 (40%) and 5 (20%) subjects, respectively. Using a receiver operator characteristic curve, BMI above 75th, 50th and 3rd percentiles maximized sensitivity and specificity for FMI ⩾95th, ⩾85th and ⩾50th percentiles, respectively. Children with SMA have reduced lean and increased fat mass compared to healthy children. Obesity is a potentially important modifiable source of morbidity in SMA.
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