Journal
NEUROMUSCULAR DISORDERS
Volume 22, Issue 11, Pages 966-973Publisher
PERGAMON-ELSEVIER SCIENCE LTD
DOI: 10.1016/j.nmd.2012.04.008
Keywords
Spinal muscular atrophy type I; Body composition; Bone density; Nutrient and caloric intake; Dual-energy X-ray absorptiometry; Growth status; Nutrient deficiencies
Categories
Funding
- SMA Angels Charity, Inc.
- NIH, National Institute of Child Health and Human Development [R01-HD054599]
- National Center for Research Resources [UL1RR025764]
- SMA
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Clinical experience supports a critical role for nutrition in patients with spinal muscular atrophy (SMA). Three-day dietary intake records were analyzed for 156 visits in 47 SMA type I patients, 25 males and 22 females, ages 1 month to 13 years (median 9.8 months) and compared to dietary reference intakes for gender and age along with anthropometric measures and dual-energy X-ray absorptiometry (DEXA) data. Using standardized growth curves, twelve patients met criteria for failure to thrive (FTT) with weight for age <3rd percentile; eight met criteria based on weight for height. Percentage of body fat mass was not correlated with weight for height and weight for age across percentile categories. DEXA analysis further demonstrated that SMA type I children have higher fat mass and lower fat free mass than healthy peers (p < 0.001). DEXA and dietary analysis indicates a strong correlation with magnesium intake and bone mineral density (r = 0.65, p < 0.001). Average caloric intake for 1-3 years old was 68.8 +/- 15.8 kcal/kg - 67% of peers' recommended intake. Children with SMA type I may have lower caloric requirements than healthy age-matched peers, increasing risk for over and undernourished states and deficiencies of critical nutrients. Standardized growth charts may overestimate FTT status in SMA type I. (C) 2012 Elsevier B.V. All rights reserved.
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