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Individuals with tyrosinenmia have food patterns very similar to the one for PKU discussed above. PKU is more common than tyrosinemia. Foods that are high in tyrosine and phenylalanine are high in protein. Thus, children with tyrosinemia should eat foods that are low in protein. Each child can "tolerate" a different amount of tyrosine/phenylalanine, so each child will have a sliglltly differenl food pattern. The most important food for a child with tyrosinemia, however, is his/her formula. Formula provides energy (calories), protein, and the vitamins and minerals (including those that are found in high protein foods).
Foods that all children with tyrosinemia should avoid are foods rich in protein i.e. meat, chicken, fish, milk, cheese, beans and legumes, peanut butter, and eggs.
5. Maple syrup urine disease is a rare inborn error of metabolism. It derives its name from the odor of the urine of affected individuals. If untreated, affected children die soon
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