inborn errors of metabolism…

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galactosemia, galactosemia, nutritional considerations, fructose intolerance, nutritional considerations, maple sugar urine disease, msud, nutritional considerations, phenylketonuria, pku, nutritional considerations, branched chain ketoaciduria, nutritional considerations, inborn errors of metabolism, rare genetic disorders, the body cannot turn food into energy, metabolize food, disorders, defects, in the enzymes involved, in the biochemical pathways that break down food components, a food product that is not metabolized, broken down into energy, build up, a wide array of symptoms, several inborn errors of metabolism cause mental retardation, controlled, please see each specific disease, exact symptoms, inborn errors of metabolism often demand diet changes, type, extent, the changes depends on the specific metabolic error, registered dietitians, physicians, the diet modifications needed, each disease, examples of inborn errors of metabolism, fructose intolerance, fructose intolerance, genetic disorder involving the breakdown, the carbohydrate fructose, potentially life-threatening but treated by diet changes, food sources of fructose include fruits, fruit juices, sucrose, all sugars, cane, beet, white, brown, corn syrups, honey, sorbitol, levulose, invert sugar, vegetable, starches, all fructose, avoided, in the diet, severity, the restriction depends on individual tolerance, sugar, fructose, found in many foods, making the diet difficult to follow, galactosemia galactosemia, genetic disorder involving the breakdown, the carbohydrate galactose to glucose, result in cataracts, enlarged liver, enlarged spleen, mental retardation, disease is found in milk-fed infants shortly after birth, milk contains large amounts of galactose, food sources of galactose include mammalian milks, dairy products, foods containing them, recommended that milk, milk products, avoided, yogurt, cheese, ice cream, galactose, lactose-free milk substitutes, foods, sources of galactose, sugar beets, gums, seaweed, flaxseed, mucilage, whey, vegetables, women who carry the genetic trait should also follow the diet, galactose, mental retardation, fetus, registered dietitian, complete information on a galactose free diet, maple sugar urine disease, msud, maple sugar urine disease, rare genetic disorder involving the breakdown, the branch chain amino acids valine, leucine, isoleucine, the disease is found shortly after birth, characterized by maple syrup odor, the urine, vomiting, refusal to eat, increased reflex actions, left untreated, life-threatening neurological damage, treatment includes a special diet, strict compliance is necessary, prevent neurological damage, close supervision by a registered dietitian, physician, cooperation by parents, phenylketonuria, pku, phenylketonuria, rare genetic disorder, result in severe progressive mental retardation, untreated by diet, most states, require blood, urine testing, pku is all newborns, a low phenylalanine diet, strict compliance, diet is necessary to reduce, prevent mental retardation, close supervision by a registered dietitian, physician, cooperation, the parent, child.