Nutritional Status of Children with Pompe disease at the Vietnam National Children’s Hospital

Abstract

Pompe disease is a serious neuromuscular disorder caused by a genetic defect of lysosomal acid alpha glucosidase (GAA). With dysphagia, skeletal muscle weakness and smooth muscle dysfunction, children with Pompe disease are more susceptible to malnutrition. This cross-sectional study therefore aimed to assess the prevalence of malnutrition and micronutrient deficiencies among 34 pediatric Pompe patients at the Vietnam National Children’s Hospital. The results showed that 100% had at least medium risk of developing malnutrition according to the STRONGkids screening tool, of which 26.47% had high risk. The prevalence of underweight, stunting, wasting, overweight and obese were 11.76%, 11.76%, 14.71%, 5.88% and 2.94%, respectively. The prevalence of zinc deficiency and vitamin D insufficiency were 67.65% and 8.82%, respectively. The nutritional and zinc status were correlated with the duration of disease and the gross motor function.