Department of clinical nutrition, Children's Hospital of Fudan University, Shanghai, People's Republic of China.
Department of neurology, Children's Hospital of Fudan University, Shanghai, People's Republic of China.
Nutr Neurosci. 2022 Jul;25(7):1488-1494. doi: 10.1080/1028415X.2020.1871212. Epub 2021 Jan 23.
To evaluate the nutritional status of children with SMA types II and III in a Chinese population.
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.
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 (= 69) and SMAIII (= 17) were -0.48 (IQR -1.69, 0.57) vs -0.53 (IQR -1.60, 0.55), = 0.926; the HAZ were -0.62 (IQR -1.4, 0.3) vs -0.6 (IQR -1.61, 0.4), =0.72; the BMIZ were -0.51 (IQR -1.53, 0.99) vs -0.08 (IQR -1.625, 1.125), = 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, = 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, < 0.05; 56.8 ± 18.1 g/d vs 41.5 ± 17.5 g/d, = 0.22), and calcium intake was lower than the recommendation (507.7 ± 177.8 mg/d vs 731.7 ± 123.4 mg/d, < 0.05; 478.4 ± 207.4 mg/d vs 478.4 ± 207.4 mg/d, = 0.01). Swallowing on the Neuromuscular Disease Status Scale was 7.41 ± 0.5.
Children with SMAII and SMAIII were at risk for malnutrition and low calcium intake.
评估中国人群中 2 型和 3 型脊肌萎缩症（SMA）患儿的营养状况。
我们对单中心的 2 型和 3 型 SMA 患儿前瞻性收集的数据进行了回顾性病历审查。我们分析了包括临床参数、人体测量学和我们诊所 24 小时饮食摄入记录在内的数据。
我们分析了 86 名儿童的人体测量数据，其中 69 名（80%）为 2 型 SMA，17 名（20%）为 3 型 SMA；47 名（55%）为女性，平均年龄为 5.22±3.73 岁。2 型 SMA（=69）和 3 型 SMA（=17）的 WAZ 分别为-0.48（IQR-1.69，0.57）和-0.53（IQR-1.60，0.55），=0.926；HAZ 分别为-0.62（IQR-1.40，0.30）和-0.60（IQR-1.61，0.40），=0.72；BMIZ 分别为-0.51（IQR-1.53，0.99）和-0.08（IQR-1.625，1.125），=0.537。将 51 名儿童的饮食摄入量与中国膳食参考摄入量（DRIs）进行了比较。2 型 SMA 的实际能量摄入量与 DRIs 相似，但 3 型 SMA 的能量摄入量低于 DRIs（1312.4±329.5kcal 与 1655±640.1kcal，=0.028）。2 型 SMA 和 3 型 SMA 的蛋白质摄入量均高于 DRIs（55±16.3g/d 与 30.2±4.6g/d，<0.05；56.8±18.1g/d 与 41.5±17.5g/d，=0.22），钙摄入量低于推荐值（507.7±177.8mg/d 与 731.7±123.4mg/d，<0.05；478.4±207.4mg/d 与 478.4±207.4mg/d，=0.01）。神经肌肉疾病状态量表的吞咽评分为 7.41±0.5。
2 型和 3 型 SMA 患儿存在营养不良和低钙摄入的风险。
