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.

Melvin Carruth

This letter is a tribute to my brother Melvin Carruth! We believe he is one of the oldest living African Americans with Maple Syrup Urine Disease.

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NBS-MSUD Connect: Your One-Stop Shop For MSUD Resources

NBS-MSUD Connect was launched as part of the Newborn Screening Connect patient registry (NBS Connect) in 2013 through a partnership between the Department of Human Genetics at Emory University, the Maple Syrup Urine Disease (MSUD) Family Support Group and other key stakeholders.

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Cambrooke Therapeutics

Cambrooke Therapeutics continues to expand its line of delicious and nutritious low protein foods to help improve the lives of individuals with Inborn Errors of Metabolism such as MSUD.

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Updates To Nutrition Management Guidelines

The Nutrition Management Guideline for MSUD was first published in 2014. Since that time, there have been reports of new research and experiences that have prompted updates of the guideline.

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Emory Metabolic Camp 2018 Announcement

Join us June 18-23, 2018 for the 24th Annual Metabolic Camp at Emory University in Atlanta, GA!

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From The Chairman’s Desk

As I sit here reflecting on how much the care and treatment of MSUD has changed over the years, my mind goes back to 1978 when our son Keith was born.

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MSUD Advocacy Report

Medical Nutrition Equity Act

The Medical Nutrition Equity Act (MNEA) would require all private insurance plans (state regulated or self-insured/self-funded) and federal health programs, including Children’s Health Insurance Program, Tricare, Medicaid, Medicare, and Federal Employee Health Benefit Plans, to provide coverage for formula and low-protein foods for all children and adults with MSUD.

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The MSUD Family Support Group Is Excited To Announce Their Participation In The Million Dollar Bike Ride

The MSUD Family Support Group is excited to announce their participation in The Million Dollar Bike Ride

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19th Biennial MSUD Symposium

I can’t believe that almost two years have passed and it is time for another MSUD Symposium! I am especially excited about this conference because I’ve built in extra time for social interaction.

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A Child's Life

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