Salera Simona, Menni Francesca, Moggio Maurizio, Guez Sophie, Sciacco Monica, Esposito Susanna
Pediatric Highly Intensive Care Unit, Department of Pathophysiology and Transplantation, Università degli Studi di Milano, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, 20122 Milan, Italy.
Neuromuscular and Rare Disease Unit, Department of Neuroscience, Foundation IRCCS Ca' Granda Ospedale Maggiore Policlinico, University of Milan, 20122 Milan, Italy.
Nutrients. 2017 Jun 10;9(6):594. doi: 10.3390/nu9060594.
Neuromuscular diseases (NMDs) represent a heterogeneous group of acquired or inherited conditions. Nutritional complications are frequent in NMDs, but they are sometimes underestimated. With the prolongation of survival in patients with NMDs, there are several nutritional aspects that are important to consider, including the deleterious effects of overnutrition on glucose metabolism, mobility, and respiratory and cardiologic functions; the impact of hyponutrition on muscle and ventilatory function; constipation and other gastrointestinal complications; chewing/swallowing difficulties with an increased risk of aspiration that predisposes to infectious diseases and respiratory complications; as well as osteoporosis with an associated increased risk of fractures. The aim of this review is to provide a comprehensive analysis of the nutritional aspects and complications that can start in children with Duchenne muscular dystrophy (DMD) and increase with ageing. These aspects should be considered in the transition from paediatric clinics to adult services. It is shown that appropriate nutritional care can help to improve the quality of life of DMD patients, and a multidisciplinary team is needed to support nutrition challenges in DMD patients. However, studies on the prevalence of overnutrition and undernutrition, gastrointestinal complications, infectious diseases, dysphagia, and reduced bone mass in the different types of NMDs are needed, and appropriate percentiles of weight, height, body mass index, and body composition appear to be extremely important to improve the management of patients with NMD.
神经肌肉疾病(NMDs)是一组由后天获得或遗传因素导致的异质性疾病。营养并发症在NMDs中很常见,但有时会被低估。随着NMDs患者生存期的延长,有几个营养方面的问题需要重点关注,包括营养过剩对糖代谢、活动能力、呼吸及心脏功能的有害影响;营养不足对肌肉和通气功能的影响;便秘及其他胃肠道并发症;咀嚼/吞咽困难以及误吸风险增加,这会导致感染性疾病和呼吸并发症;还有骨质疏松及骨折风险增加。本综述的目的是全面分析杜氏肌营养不良症(DMD)患儿可能出现并随年龄增长而增多的营养问题及并发症。在从儿科诊所向成人医疗服务过渡的过程中,应考虑这些方面。研究表明,适当的营养护理有助于改善DMD患者的生活质量,并且需要一个多学科团队来应对DMD患者的营养挑战。然而,仍需要对不同类型NMDs中营养过剩和不足、胃肠道并发症、感染性疾病、吞咽困难及骨量减少的患病率进行研究,合适的体重、身高、体重指数和身体成分百分位数对于改善NMD患者的管理似乎极为重要。