Food Habits And Dietary Intake in Italian Children with Spinal Muscular Atrophy Type 1 and 2.

Rationale: Spinal Muscular Atrophy (SMA) is an autosomal recessive neurodegenerative disease characterized by the degeneration of spinal cord motor neurons. SMA patients suffer from swallowing and gastrointestinal problems that could reduce energy and nutrients intake. This study aimed to compare the dietary intake of Italian children affected by SMA type 1 (SMAI) and 2 (SMAII) with national recommendation. Methods: 188 SMAI (94M, 63 ± 40 months) and 177 SMAII patients (88M, 51 ± 37 months) were enrolled. 3-days food dietary records were used to investigate feeding behavior and the type of food used. Dietary records were analyzed using WinFood version 3.0. Italian recommended daily allowances of energy and nutrients (LARN, 2014) were used as reference. Nutrients intakes are expressed as mean and standard deviation (SD) of adequacy indexes calculated. Results: 60.1% of SMAI children was fed per os, 36.2% via gastrostomy and 3.7% used nasogastric tube. 100% of SMAII was fed per os. SMAI consume 85.7 ± 27.5 kcal/kg body weight (BW), SMAII 65.3 ± 24.2 kcal/kg BW. Carbohydrates were 70% of the recommendation. Proteins were 2.7 ± 1.1 g/kg BW in SMAI and 2.6 ± 1.0 g/kg BW in SMAII. Fibers were 68% of recommendation. Fat and sugar intakes agreed with recommendations. Concerning micronutrients, SMAI and SMAII introduced, respectively, 72.8 ± 57.5% and 54.6 ± 44.5% of vitC; 26 ± 18.5% and 25.5 ± 15.1% of vitB9, 39.9 ± 50.9% and 14.4 ± 14.7% of vitD, 46.8 ± 31.4% and 39.4 ± 21% of vitE, 64.6 ± 29.2% and 54.4 ± 23.7% of calcium, 87.5 ± 42.2% and 44.9 ± 21.5% of iron, and 23 ± 16.3% and 30.8 ± 13.4% of magnesium. All micronutrients intake resulted statistically different from recommendation (p < 0.05). Conclusions: Both SMAI and SMAII children have adequate daily but consume more proteins than recommendations, and micronutrients’ intake results 50% lower than recommendations. Therefore, specific dietary reference intakes for SMA children are needed to enhance nutritional management.