CONTENTS INTRODUCTION TO MAPLE SYRUP 03 Introduction to Maple Syrup Urine Disease 10 Your Child’s Growth and Development URINE DISEASE 03 What is MSUD? 11 Teaching Your Child Diet Management Your child has a condition called Maple Syrup Urine Disease, or MSUD for short. Children who have inherited this 04 MSUD: An Inherited Disorder 12 Feeding Problems condition cannot use the essential amino acids isoleucine 05 Types of MSUD 13 The Role of Family and Other Care Providers in (i-so-lu-seen) (ILE), leucine (lu-seen) (LEU), and valine Managing MSUD (vay-leen) (VAL) in a normal way. 05 Diagnosis of MSUD These amino acids are found in all foods that contain protein. 05 Symptoms of MSUD 13 Your Child’s Diet During Illness You will need to feed your child all the foods necessary for 06 Nutrition Support of MSUD 14 A Look to the Future normal growth and development, but only the amount of Figure 1. Amino acids are joined together like beads on a necklace to ILE, LEU, and VAL he can safely use. form protein. Enzymes act like scissors to remove amino acids from 07 International System of Measurement 15 Recipes Learning some medical terms in nutrition and genetics will protein. (Metric System) 17 Resources help you understand and manage your child’s diet better. 09 Checking Your Child’s Progress If you have any questions, write them down and ask the nutritionist (dietitian), nurse, or doctor at the metabolic clinic. WHAT IS MSUD? MSUD is an inherited disorder of amino acid metabolism. Proteins, which are made up of amino acids, are found in many parts of the human body, including hair, blood, skin, and muscles. Most foods contain protein. When we eat foods containing protein, this protein is split into amino acids during digestion. The amino acids are later put back together like beads on a necklace to form new protein. These new proteins are used to build and repair the body’s tissues. Twenty amino acids occur commonly in the human body and in the foods we eat. Three of these essential amino acids are ILE, LEU, and VAL. Because of their chemical structure, these amino acids are described as branched chain. They are frequently grouped together and called branched-chain amino acids (BCAAs). All foods with protein contain BCAAs. High-protein foods are dairy products, eggs, meat, poultry, nuts, soy products, seafood, seeds, nut butters, beans and peas. Fruits, grains, and vegetables have less protein, and so have less ILE, LEU, and VAL. These are allowed in the diet in measured Abbott provides this booklet to health care quantities. professionals to help them counsel families, and Splitting protein into amino acids requires a special to families to help them learn about MSUD substance that does the actual work. Think of the splitting substance as a pair of scissors snipping beads off a necklace © 2023 Abbott (Figure 1). The “scissors” are called enzymes (n-simes). 2 3