Nutritional Therapy in Children With Spinal Muscular Atrophy in the Era of Nusinersen

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Abstract
Objectives: Spinal muscular atrophy (SMA) is a genetic motor neuron disorder characterized by progressive muscle atrophy. Our aims were to evaluate the impact of nutritional intervention and nusinersen therapy on the nutritional status of SMA patients.Study design: This prospective study included all children and young adults (P = 0.042). There was also a significant increase in total energy and protein consumption in the former group (P = 0.043).Conclusions: Malnutrition is frequent among children with SMA, and the nutritional status of patients that started nusinersen therapy after implementation of a nutritional intervention underwent a more significant improvement. The importance of combining adequate nutritional management with disease‐modifying treatment is highlighted.

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