Effects of nutritional interventions on children with Pompe disease at the National Children’s Hospital
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Abstract
In addition to enzyme replacement therapy, nutritional management plays an important role in the care of children with Pompe disease. This study aimed to evaluate the effects of nutritional interventions-including a high-protein, low-carbohydrate diet and a reduced amino acid-free interval-on children with Pompe disease at the National Children’s Hospital. After a 12-week intervention period, the median dietary protein intake increased from 14.37 ± 1.14% to 24.21 ± 1.12% of total energy intakes, with no observed correlation with transaminases levels, creatinine, or body weight. The median of maximal amino acid-free interval decreased from 9.33 to 6.42 hours. The prevalence of malnutrition improved from 25% to 12.5%. Additionally, the ultrasonographic left anterior thigh thickness increased from 22.59 ± 5.59mm to 23.8 ± 3.99mm.
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Keywords
Pompe disease, high protein low carbohydrate diet, nutritional intervention, anterior thigh thickness, children
References
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