By Elaina Jurecki, M.S., R.D., Kaiser Permanente Medical Center, Oakland, CA

Adapted with permission from the Organic Acidemia Association Newsletter (Vol. VI, No. 2., Aug. 1996).

So you are walking down the isle in the grocery store and wondering whether or not a new food you see on the shelf is okay to include in your child's diet. Now-a-days it seems like there is a new food item every time you go into the store. This is especially true with cereals and snack foods that can frequently contribute to a large portion of your child's intake. The best place to look to determine if this food is okay is by checking out the label and evaluating its nutrient content.

The food label was revised a couple of years ago and now includes a lot more useful information. The new label is illustrated here. This information is provided by the manufacturers and regulated by the U.S. Food and Drug Administration (FDA). The nutrient content is listed per serving size, and the number of servings per container is also indicated. Serving sizes have been standardized by the FDA to allow for comparing nutrient content of similar products made by different manufacturers.

You can now find the amount of calories per serving size and the number of calories that come from fat. The total amount of fat is listed in grams and includes the amount that comes from saturated sources. The cholesterol and sodium contents are listed in milligram amounts. The carbohydrate content provides the gram total amount and that amount from dietary fiber and sugar. The total grams of protein content is also listed.

The percent of Daily Value listed next to several of the nutrients is a new reference value. This is used to determine what the recommended intake per day should be based on a daily intake of 2,000 calories. The chart at the bottom of the label shows Daily Values for fat, saturated fat, carbohydrate, and fiber for the 2,000 calorie diet and for a 2,500 calorie diet. Consumers can use the Daily Value percentages to see how a food fits into an overall diet. It should be kept in mind, however, that Daily Values may vary, depending on an individual's calorie needs. Keep in mind that these calorie needs are more typical for an adult's intake, and for an individual not following a special diet.

The grams of protein, fat, and carbohydrate have been rounded to the nearest whole number. In other words, a food that contains from 0.0 to 0.4 grams will be listed to contain 0 grams, and a food that has 0.5 to 1.4 grams will be listed to contain 1.0 grams. Therefore, you may select a food that shows zero grams of protein, but it actually could contain up to 0.4 grams of protein per serving. This difference in protein content could cause an individual with a disorder in protein metabolism to get out of metabolic control and even result in hospitalization. So what should you do to verify the actual protein content? Contact the manufacturer for that food to find out the exact protein content. You can also verify the amounts of other nutrients listed. The name, address, and telephone number (and in many instances a toll free number is provided) for the manufacturer will be listed on the food product.

The ingredients of that food will also be listed on the label. The ingredients are listed by descending order by weight. For example, when the amount of each ingredient is weighed, the ingredient that weighs most is listed first. The ingredient that weighs the least is listed last. Ingredients that make up 2% or less of a food are listed at the end of the ingredients listing in no particular order. Because of the fancy terminology for ingredients food companies use, you may need help with determining what they actually are. For example, cellulose gum is a fiber, corn syrup solids are simple sugar, and mono and diglycerides are fats. Check out these ingredients with your nutritionist to make sure that the food is acceptable on your child's diet.

The only vitamin and mineral values listed on food labels include vitamin A, vitamin C, calcium and iron and are indicated as a percent of Daily Value based on a 2,000 calorie diet. Additional information regarding the vitamin and mineral content of foods can be found in food composition tables. You may need to know the amounts of other nutrients that are not included on the label. For example, an individual with MSUD needs to know the amino acids: leucine, valine and isoleucine content for that food. A person with a disorder in a long chain fatty acid oxidation may need to know what the essential fat content is for that food. This information can be found in published food composition tables. There are also many computer nutrition programs available that include this information. The USDA Nutrient Database includes the largest amount of food composition data for over 5,000 different food items. This governmental agency has previously published their food composition tables, but now makes it more readily available via the Internet.

So don't be afraid to check out new foods. They may help to provide more variety in your child's diet. This is especially important when the diet is restricted in some way. But if you are unsure whether the food is truly acceptable in your child's diet, or if you are unsure of how much to give your child, then the safest thing to do is contact your nutritionist and/or Metabolic Health Care team for further assistance. Bring the food labels with you to your next appointment with your Metabolic Health Care Team, and they can help you determine if this is a good food and/or how much your child can eat.


The MSUD Family Support Group is currently funding several research projects and we are proactively looking for researchers interested in developing new treatments or finding a cure for MSUD. Significant funding is necessary if we are to accomplish this goal.
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