杜氏肌营养不良（DMD）患儿营养状况与膳食营养摄入情况的横断面研究

目的 调查杜氏肌营养不良症（Duchenne muscular dystrophy, DMD）患儿营养状况及膳食摄入情况，初步探索两者的相关关系，为制定DMD患儿合理营养治疗提供理论依据。方法 选择2017年7月–2021年4月于四川大学华西第二医院就诊并经基因检测法诊断为DMD的共223名2～14岁患儿作为研究对象，采用3天24小时膳食回顾法进行膳食调查，采用化学发光法测定血清维生素D水平。结果 营养状况正常DMD患儿仅占33.2%，生长迟缓、低体质量、超重、肥胖发生率分别为13.5%、14.4%、14.3%、8.1%，血清维生素D缺乏和不足患儿分别占9.0%和89.7%；膳食调查DMD患儿每日碳水化合物、蛋白质和脂肪供能比分别为（47.40±6.64）%、（14.46±2.22）%和（38.17±5.30）%；每日膳食钙和维生素D摄入量分别为（433.32±164.39） mg/d、（155.73±89.30） IU/d；生长迟缓DMD患儿蛋白质摄入量/蛋白质平均需要量的比值（P=0.003）和能量摄入量/能量需要量的比值（P=0.007）低于营养状况正常DMD患儿。结论 DMD患...

Cross-Sectional Study of Nutritional Status and Dietary Nutrient Intake in Children with Duchenne Muscular Dystrophy (DMD) in China

  Objective  To investigate the dietary nutrient intake and the nutritional status of children with Duchenne muscular dystrophy (DMD), and to explore the correlation between them, so as to provide theoretical basis for the formulation of proper nutritional treatment for children with DMD.  Methods  A total of 223 children aged 2 to 14 years who came to West China Second University Hospital, Sichuan University from July 2017 to April 2021, and who were diagnosed with DMD by genetic testing were enrolled as the subjects of the study. Dietary assessment was conducted with a 3-day 24-hour dietary recall, and serum vitamin D level was measured by chemiluminescence method.  Results  Only 33.2% of the children with DMD were found to be of normal nutritional status. The incidences of stunted growth, underweight, overweight and obesity were 13.5%, 14.4%, 14.3% and 8.1%, respectively. Among the children with DMD, those with serum vitamin D deficiency and insufficiency accounted for 9.0% and 89.7%, respectively. According to the dietary recall of the children with MDM, the daily energy ratio of carbohydrate, protein and fat were (47.40±6.64)%, (14.46±2.22)%, and (38.17±5.30)%, respectively. The daily intake of dietary calcium and vitamin D were (433.32±164.39) mg per day and (155.73±89.30) IU per day, respectively. The ratio of daily protein intake to the estimated average requirement for protein (P=0.003) and ratio of daily energy intake to the estimated energy requirement (P=0.007) were lower in children with stunted growth than those of DMD children of normal nutritional status.  Conclusion  The dietary structure of children with DMD is obviously not suited to their condition and nutritional deficiency coexists with overnutrition among them. Further research needs to be done for developing appropriate nutritional guidance programs and standardized nutritional management measures for children with DMD.