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Diet in phenylketonuria


02.06.2015
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Diet in phenylketonuria


Diet in phenylketonuria guidelines:

   Ensuring an adequate dietary therapy allows the normal development of the child. Phenylketonuria is a metabolic disease, which is the lack of an enzyme catalyzing the conversion of one of the amino acids (supplied with food) - phenylalanine into another amino acid - tyrosine. Phenylalanine is a component of most plant and animal proteins, is involved in the biosynthesis of epinephrine, melanin, thyroxine. It has a genetic basis. This disease, unfortunately, can not be prevented. But thanks to early detection can protect ourselves from its consequences. The diagnosis of PKU is based on the demonstration of elevated levels of phenylalanine in the blood. Introduction of appropriate medical nutrition therapy, even before the symptoms allows your child to develop normally, both mentally and physically.





No treatment results

   Consequences of non-detection of the disease and failure to implement the treatment can be very dangerous. An excess of phenylalanine and its abnormal metabolites leads to damage of the nervous system and, consequently, to mental retardation, neurological disorders and worse physical development. These changes are irreversible. Untreated children are often irritable and restless, may have dry skin, rashes, seizures.



Effect of diet

   The most vulnerable to the harmful effects of excess phenylalanine are infants, in whom the nervous system is only emerging. It is therefore important to introduce adequate nutrition in patients that are in the first month of life. Treatment started only after the third month of life no longer offers such good results. Diet must be checked on the content of phenylalanine for many years until the child reaches the 8-10 years of age. Myelination of central nervous system is totally completed and the excess phenylalanine is no longer harmful. Women who have phenylketonuria, must necessarily follow a diet during pregnancy and its planning. Breaking the diet leads to damage of the fetus.



The main principles of the diet

• The demand for phenylalanine is determined individually for each child, in addition to age and body weight because the level of amino acid in the blood has to be taken into account.
• The PKU diet formulations are based on protein hydrolysates poor in phenylalanine. And is able to cover the demands of the body for protein. In the first months of life diet is simple. It includes ready-made preparations, and only small amounts of breast milk (it contains about 3 times more phenylalanine than cow's milk).
• Older infants and children in addition to formulated low phenylalanine preparations, dishes from the natural foods with a low content of phenylalanine should be introduced. This helps cover the demand for nutrients (often in the diet of children with phenylketonuria meet deficiencies of zinc, selenium, iron and calcium), and improves mood by introducing new flavors and avoid monotony.
• Diet prepared for the child must be strictly calculated and arranged with the participation of a physician and dietitian. Dietary preparations are adjusted to a certain age and should be changed as your child grows. They differ among themselves in composition. Some of them may be the only source of food, while others serve only to supplement the diet. Preparations are available in all pharmacies. A bit difficult is to buy products with a reduced protein content, such as low protein bread, low-protein flour, wheat starch,  corn starch, and waffles. These products enrich the child's diet.
• Arranging menus, you control the content of phenylalanine derived from both the preparations and products, so that the amount does not exceed the acceptable daily supply of fixed individually to the child. At the same time care must be taken to cover the demand for energy and nutrients.
• If the diet is strictly followed, the blood phenylalanine levels should not exceed 6 mg/100 ml of blood.
• Food can be divided into three groups:
1. Products with high levels of phenylalanine, which should not be administered to children at all. These are mainly products of animal origin with high protein content, such as meat and meat products, fish, eggs, poultry, milk and milk products. Legumes, nuts, chocolate and its participation, cereal products that have not been made from low protein flour should also not be given.
2. Products with a low content of phenylalanine allowed in limited quantities: vegetables, potatoes, fruit, fruit, rice, cereal flour low in protein, rice, margarine, butter.
3. Products allowed, not containing in its composition phenylalanine oil, sugar, honey, mineral water.



Example - diet in phenylketonuria:

Breakfast: Preparation, low protein bread with jam
Lunch: Banana
Dinner: Vegetable soup, low protein flour pancakes with vegetables, apple juice
Afternoon snack: Preparation, starch wafers
Supper: Preparation, low protein flour noodles with cabbage, apple




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