Nutrition management may include an LEU-free medical movement (psychomotor abnormalities), seizures, and food paired with a low-protein diet (restricting LEU). Your nervous system impairment. Reye-like symptoms, including Table 1. General Guide to Foods on LEU-Restricted Diets metabolic doctor may also recommend carnitine and/or low blood sugar (hypoglycemia), acidosis, and high ammonia Foods That Are Not Allowed Foods That Are Limited Foods That May Be Eaten Freely glycine supplementation. Carnitine and glycine help the body levels (hyperammonemia), have also been reported. get rid of toxic metabolites that build up in the body as well Additional Therapies. The goal of treatment during Dairy products (cheese, milk, ice Breast milk, infant formula, bread, Gumdrop candy, hard candy, as help to prevent possible deficiencies. acute episodes is prevention of body protein breakdown cream, yogurt), soy milk and soy crackers, fruit, fruit juices, low jelly, Kool-Aid®, lemonade, protein cereals, popcorn, potato ® 3-Hydroxy-3-Methylglutaryl-CoA Lyase Deficiency (catabolism) by carbohydrate administration and correction products, beans and peas, eggs, fish chips, special low-protein foods, lollipops, Popsicles , pure sugar (3-hi-drox-ee 3-meth-ill-glue-tar-ill-Co-A lie-ase), also of the acidosis. The effect of long-term nutrition management and seafood, meat, nuts, nut butters, vegetables, vegetable juices and fat, soda known as HMG-CoA lyase deficiency. This deficiency of this rare disorder has not been established. Nutrition poultry, seeds, tofu not only blocks the body’s ability to break down LEU, but management may include an LEU-free medical food paired also prevents the body from making ketones (key-tones), with a low-protein diet (restricting LEU). Your metabolic Requirements for LEU, Protein, and Energy. A child vitamins. Supplemental minerals and vitamins are not usually which are used for energy during periods without food doctor may also recommend carnitine supplementation. with a DLC who eats enough protein to grow properly gets needed when the diet is followed as directed. (fasting). HMG-CoA lyase deficiency symptoms may include Carnitine helps the body get rid of toxic metabolites that build too much LEU. Foods high in protein are cheese, milk, soy vomiting, excessive tiredness (lethargy), metabolic acidosis, up in the body as well as help to prevent possible deficiencies. milk, eggs, meat, poultry, fish and seafood, nuts, beans and I-Valex®-2 Amino Acid-Modified Medical Food With Iron low blood sugar (hypoglycemia), high ammonia levels DIAGNOSIS OF A DLC peas, seeds, and nut butters. Foods low in protein include is a medical food used for the dietary management of children (hyperammonemia), and an enlarged liver (hepatomegaly), some cereals, grains, fruits, fats, vegetables, and sweets. and adults with a DLC. I-Valex-2 contains no LEU, so LEU frequently triggered by an illness or fasting. Infection, high Most states require all babies to be screened for disorders Eating these foods in the amounts needed to provide just must be met by using other food sources. Your nutritionist protein intake, and prolonged fasting can lead to metabolic of LEU catabolism and other conditions before they are enough LEU does not provide enough protein to meet your will tell you which medical food is right for your child, as decompensation, resulting in low blood sugar with a small discharged from the hospital. In cases where DLCs are not child’s needs for growth. Depending on your child’s protein well as which foods and how much of each your child can eat. amount or no ketones produced (hypoketotic hypoglycemia), tested on newborn screening, an individual may be diagnosed tolerance, a special medical food that is high in protein and I-Valex-1 and I-Valex-2 must be used under the supervision and an increase in several organic acids. The inability to after showing symptoms of the condition. free of LEU may be required. of a physician. produce ketones in response to fasting can cause severe low If the initial screening tests show that a baby may have a To be sure your child is getting enough energy, protein, and I- Valex-1 and I-Valex-2 taste different from milk. Most blood sugar. DLC, additional blood and urine are collected for more adequate LEU for growth and development, a nutritionist children and adults get accustomed to the flavors of the foods Additional Therapies. Paired with a low-protein diet precise measurements that will confirm the diagnosis. Some carefully calculates the amount of each nutrient needed. Too that they eat. They may seem distasteful to you, but it is very (restricting LEU intake), additional therapies include doctors may hospitalize the infant to confirm diagnosis so that little LEU, protein, or energy can result in growth failure. important not to show this to your child, either by word or prompt attention to illness, decreased fat intake, carnitine treatment can be started sooner if the baby has a DLC. Frequent diet adjustments are necessary, especially during action. Your child may refuse the medical food just because supplementation, and avoidance of fasting. The goal of long- the first 6 months of life when babies grow rapidly. The you appear not to like it. term nutrition management is to prevent low blood sugar, NUTRITION SUPPORT OF A DLC nutritionist or metabolic doctor will make these diet changes One mother disliked the odor of the medical food so much limit long periods of fasting, and minimize elevations of LEU A diet that reduces LEU intake is used to help prevent most based on your baby’s health, growth, LEU intakes, blood that she made a face every time she gave it to her son. metabolites. A high-carbohydrate, low-fat diet with LEU of the symptoms of a DLC. This diet, which is different for levels of LEU, and urinary excretion of organic acids. Because of this, he refused the medical food for several days restriction results in normal growth and development. A each person with a DLC, can lower the blood and urinary I-Valex®-1 Amino Acid-Modified Infant Formula with until she and her family realized what was wrong. As she said high carbohydrate intake with reduced fat intake is required organic acid levels to a range that may allow normal mental Iron is an infant formula used to provide protein for infants later, “We changed our attitude to thinking this wonderful to prevent the build-up of metabolites arising from the development and growth. and toddlers. I-Valex-1 does not contain any LEU. Similac diet will make it possible for our child to have a happy life.” breakdown of stored body fat (fatty acid oxidation). Carnitine Advance Infant Formula with Iron, breast milk, or other helps the body get rid of toxic metabolites that build up in the The special diet for your child is designed for his individual intact protein must also be fed to provide the specific amount Other children in the family should be told that I-Valex is body as well as help to prevent possible deficiencies. needs, and following it is very important. With proper of LEU your baby needs for growth and development. Breast very important, and they should not emphasize the difference nutritional management, your child will grow and develop milk is lower in LEU than infant formula or cow’s milk and in taste or odor between milk and medical food to the child 3-Methylglutaconic Aciduria Type I (meth-ill-glue-ta-con- normally. with a DLC. ic a-cid-ur-ee-a) is a deficiency of methylglutaconyl-CoA can be used to supply the required LEU. The decision to hydratase (meth-ill-glue-ta-con-ill Co-A hi-dra-tase) that Many foods contain protein. Those foods also contain LEU. breastfeed should be discussed with your nutritionist and Most children taking medical foods for a DLC like them IF results from an increased amount of 3-methylglutaconic acid Your child with a DLC must limit the amount of foods that metabolic doctor. The nutritionist or metabolic doctor will the medical foods are started early and IF their family has a released and blocks the body’s ability to break down LEU contain protein. Table 1 is a general guide of foods that are not tell you the exact amount of breast milk needed in addition positive attitude. Older children who start on the diet after (Figure 2). Symptoms range from isolated speech retardation allowed, foods that are limited, and foods that may be eaten to your child’s infant formula. I-Valex-1 contains carnitine drinking cow’s milk may not like I-Valex at first, but in time to slowing down of thought and a reduction in physical freely if obesity is not a problem. and is well supplied with fat, carbohydrate, minerals, and accept it. 6 7