Increased fat mass and high incidence of overweight despite low body mass index in patients with spinal muscular atrophy.
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.
Division of Pediatric Neurosciences, Department of Neurology, SMA Clinical Research Center, Columbia University Medical Center, Harkness Pavilion, HP-514, 180 Fort Washington Avenue, New York, NY 10032-3791, USA. dsproule@neuro.columbia.edu
Douglas M
DM
Sproule
Jacqueline
J
Montes
Megan
M
Montgomery
Vanessa
V
Battista
Dorcas
D
Koenigsberger
Wei
W
Shen
Mark
M
Punyanitya
Darryl C
DC
De Vivo
Petra
P
Kaufmann
D015502:Absorptiometry, Photon; D000273:Adipose Tissue / Q000000981:diagnostic imaging; D000293:Adolescent; D000886:Anthropometry; D001823:Body Composition; D015992:Body Mass Index; D002648:Child; D002675:Child, Preschool; D015331:Cohort Studies; D005260:Female; D006801:Humans; D015994:Incidence; D008297:Male; D009134:Muscular Atrophy, Spinal / Q000453:epidemiology*; D050177:Overweight / Q000453:epidemiology*; D012372:ROC Curve