Nutrition status survey of type 2 and 3 spinal muscular atrophy in Chinese population.

OBJECTIVE: To evaluate the nutritional status of children with SMA types II and III in a Chinese population. METHODS: We performed a retrospective medical record review of prospectively collected data from children with SMA types II and III in a single centre. We analysed data including clinical parameters, anthropometrics, and 24-hour dietary intake records in our clinic. RESULTS: We analysed the anthropometric data from 86 children with 69 (80%) SMAII and 17 (20%) SMAIII; 47 (55%) were female, mean age was 5.22 ± 3.73 years. The WAZ of the SMAII (n = 69) and SMAIII (n = 17) were -0.48 (IQR -1.69, 0.57) vs -0.53 (IQR -1.60, 0.55), P = 0.926; the HAZ were -0.62 (IQR -1.4, 0.3) vs -0.6 (IQR -1.61, 0.4), P=0.72; the BMIZ were -0.51 (IQR -1.53, 0.99) vs -0.08 (IQR -1.625, 1.125), P = 0.537.The dietary intake of 51 children was compared to the Chinese Dietary Reference Intakes (DRIs). The actual energy intake in SMAII was similar to the DRIs, but which in SMAIII was less than the DRIs (1312.4 ± 329.5 kcal vs. 1655 ± 640.1 kcal, P = 0.028). The protein intake in SMAII and SMAIII was higher than the DRIs (55 ± 16.3 g/d vs 30.2 ± 4.6 g/d, P < 0.05; 56.8 ± 18.1 g/d vs 41.5 ± 17.5 g/d, p = 0.22), and calcium intake was lower than the recommendation (507.7 ± 177.8 mg/d vs 731.7 ± 123.4 mg/d, P < 0.05; 478.4 ± 207.4 mg/d vs 478.4 ± 207.4 mg/d, P = 0.01). Swallowing on the Neuromuscular Disease Status Scale was 7.41 ± 0.5. CONCLUSIONS: Children with SMAII and SMAIII were at risk for malnutrition and low calcium intake.