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Last winter we told you about the importance of antioxidants, and how they may be particularly helpful for those with MSUD. In the past year, several additional studies have been published on the subject, so we thought it was worth bringing up again.

As a refresher, or for those of you who missed last year’s article, antioxidants are naturally occurring substances that battle free radicals. Free radicals are molecules that are very unstable and cause damage to cells in our bodies by reacting with oxygen and other substances. They are formed every time we breathe as a result of normal respiration, and can also be generated by external agents such as smog, tobacco, and stress.

Our bodies have a natural system for battling free radicals, called the antioxidant defense system. Sometimes, though, our ability to neutralize free radicals cannot keep up with the generation of new free radicals. This is called oxidative stress. One study published by Quental and colleagues (Molecular Genetics & Metabolism 2008) reported increased markers of oxidative stress in 7 MSUD patients in Brazil. While all of the children were currently being treated for MSUD, it must be noted that they had been diagnosed at variable ages and that all suffered from neurological complications of the disease. In a personal communication with the study’s author, it was learned that compliance with the diet in these patients was poor. Whether the same would be found in children who comply with the diet is unknown.

Clearly more research is needed in this area. Studies of this nature would be strengthened by including a dietary analysis so the antioxidant content of the diets would be known. Meanwhile, we should all be encouraging our children and adults with MSUD to favor fruits and vegetables in their diets. US Dietary Guidelines recommend 2 cups of fruit and 2 cups of vegetables for adults every day.

Interestingly, a paper published in the Journal of the American Dietetic Association (Phillips 2009) reviewed the antioxidant content of various sweeteners. They found that refined sugar, corn sweetener (used in most processed foods) and agave nectar had negligible antioxidant content, while dark and blackstrap molasses had the highest, followed by maple syrup (real maple syrup, not maple flavored syrup such as Aunt Jemima), brown sugar, and honey . As the MSUD diet relies on sugar as a significant source of calories, substituting these less processed sugars for more highly processed forms can improve the antioxidant content of the diet.

Advocacy

As our legislators headed home for their August break, Rare Disease Legislative Advocates got busy.

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From The Editor

Hello to my MSUD family! The power of this family hit home this summer, when Hannah (Classic MSUD age 23 years) and I visited Israel.

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A Child's Life

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