VP.21 Baseline nutrition investigation in a Chinese cohort of pediatric patients with spinal muscular atrophy

Abstract

Nutritional problem is frequently observed in pediatric patients with spinal muscular atrophy (SMA). In this study, we aimed to investigate the nutritional status in Chinese patients with SMA. Clinical data including the body weight, length, body mass index (BMI) and the levels of serum 25-hydroxy Vitamin D (25-OHD) of SMA patients from January 2022 to March 2022 were collected and analyzed. We used the Z-score (standard deviation) to analyze the BMI. The levels of serum 25-OHD were graded as follows: 75-250nmol/L for normal, 50-75nmol/L for insufficiency, less than 50nmol/L for deficiency. A total of 41 pediatric patients with SMA (8 case of SMA type 1, 20 case of SMA type 2, and 13 case of SMA type 3) with ages ranging from 12 months old to 15 years old were included. Nearly 25% of SMA patients showed nutritional problems. Malnutrition was observed in 50% (4/8) of SMA 1 patients, 10% (2/20) of SMA 2 patients and 15.3% (2/13) of SMA 3 patients; overweight was found in 5% (1/20) of SMA 2 patients and 15.3% (2/13) of SMA 3 patients. The 25-OHD deficiency was observed in 25% (2/8) of SMA 1 patients, 30% (6/20) of SMA 2 patients, and 38.5% (5/13) of SMA 3 patients. The 25-OHD insufficiency was found in 25% (5/20) of SMA 2 patients and 23.1% (3/13) of SMA 3 patients. This study showed that malnutrition was common in SMA 1 patients, 25-OHD deficiency or insufficiency was frequent among the three types of SMA. Disease management with adjusting the diet structure and more outdoor activities would improve the nutritional problems. In this study, we demonstrated the baseline nutrition situations in a Chinese cohort of pediatric patients with SMA.