Nutritional, Lipid Metabolic and Body Composition Features of Children with Spinal Muscular Atrophy

Abstract

Background Spinal Muscular Atrophy (SMA) is a progressive neuromuscular disease affecting multiple organs and systems, including nutritional and metabolic status. There is still a lack of relevant large sample size research. We aimed to investigate the nutritional status, serum lipid profile, and body composition characteristics in children with SMA. Methods A hospital-based, case-control study was conducted on 91 children with SMA (17.6% of type I, 48.4% of type II, 34.1% of type III) and 91 age- and gender-matched healthy children in a single hospitalized center in China. Anthropometric information, serum lipids parameters and body composition (in subgroup of patients older than 3 years) were collected. According to the anthropometric Z scores, nutritional status was categorized into normal and malnutrition (including under- and over-nutrition) groups. Results The prevalence of malnutrition and dyslipidemia in children with SMA was higher than that of controls (49.45% vs. 23.08% and 56.04% vs. 34.07%, respectively and p<0.01). Particularly, the prevalence of lower-than-normal levels of high density lipoprotein (HDL) and apolipoprotein A1 (Apo A1) were significantly higher than that in healthy controls in children with SMA (26.37% vs. 7.69% and 41.76% vs. 6.59%, respectively and p<0.01). In terms of the body composition characteristics in children with SMA II & III, on average, the total body fat mass percentage (FM%) was 43.38% (36.99%, 48.20%), the fat mass index (FMI) was 7.30 (5.07, 8.72), the lean body mass index (LBMI) was 7.70 (6.96, 9.02) and the bone mineral density-z score (BMD-z) was -1.40 (-2.80, -0.60), generally different from the reference profiles in published literature. Conclusion Children with SMA in China have obvious nutritional imbalances, abnormal levels of serum lipid and unbalanced body composition. Further research is warranted to explore the potential mechanisms and health management strategies on nutritional and metabolic disorders of SMA.