Strategy of nutrition intervention on Duchenne muscular dystrophy / 中华临床营养杂志

ABSTRACT

Duchenne muscular dystrophy (DMD) is a fatal X-linked genetic disease caused by complete absence of Dystrophin due to mutation of DMD gene. Typical symptoms are progressive muscle loss which cause slow walking, gait abnormalities, falls, and difficult squatting. The adult patients often died from the respiratory and cardiovasucula failure in their early life stage.The early introduction of steroids has altered the natural history of the disease, but can exacerbate weight gain in a population already susceptible to obesity. Prior to commencing steroids, anticipatory guidance for weight management should be provided. Malnutrition is a feature of end stage disease requiring a multidisciplinary approach, such as texture modification and supplemental feeding. Micronutri- ent requirements are yet to be determined but, as a result of corticosteroid treatment, vitamin D and calcium should be supplemented. The multidisciplinary management of boys with DMD including nutrition treatment has progressed significantly and improved the life qulity of both patients and their parents.