Protein-free diet for children with phenylketonuria. What is phenylketonuria? Therapeutic nutrition for phenylketonuria in children

There are several variations in the preparation of this dish. Let's look in detail at how to prepare a delicious pie with cabbage and mushrooms. All that remains is to choose the most suitable recipe.

Lenten pie with cabbage and mushrooms

Compound

wheat flour – 400 g;
champignons – 300 g;
salt and black pepper - to taste;
white cabbage – 400 g;
carrots – 1 pc.;
dry yeast (fast-acting) – 1.5 tsp;
vegetable oil– 100–150 ml;
sugar – 4 tsp;
purified water – 220 ml.

Preparation

Jellied pie with cabbage and mushrooms

Compound

chicken eggs - 3 pieces;
cabbage – 200 g;
mayonnaise – 150 ml;
champignons – 250 g;
sour cream – 150 ml;
onion – 1 head;
wheat flour – 3 tsp;
salt and spices “for casseroles” - to taste;
baking powder (can be replaced with soda) – 1 teaspoon.

Preparation

Pie with cabbage and mushrooms made from puff pastry

Compound

puff pastry – 1 kg;
cabbage – 1.5 kg;
a mixture of peppers and salt - to taste;
onion – 1 head;
egg - 1 piece;
champignons or porcini mushrooms – 600 g.

Preparation

Pie with mushrooms and cabbage on kefir

Compound

eggs – 3 pcs.;
carrots – 1 pc.;
wheat flour – 1 cup;
kefir – 200 ml;
soda – 0.5 tsp;
cabbage – 200 g;
salt and spices - to taste;
fresh mushrooms – 300 g;
mayonnaise – 200 g;
onion – 1 head;
sugar – 1 tsp.

Preparation

Grate the cabbage.
Chop the onion into cubes.
Cut the mushrooms into slices.
Grate the carrots on a medium grater.
Heat a frying pan and fry the onion over medium heat until soft.
Add mushrooms, salt and carrots.
When the champignons release their juice, add cabbage to the pan.
Stir and pepper the vegetables. Leave them to simmer over low heat with the lid on.
Let the finished filling cool.
Break 3 eggs into a large bowl. Add a spoonful of sugar and 2 pinches of salt. Whisk the mixture.
Pour kefir and mayonnaise into the dough. Mix everything.
Add baking soda and sifted flour. Beat the mixture with a mixer until smooth. If it is too runny, add more flour.
Grease the pan and pour in half the batter.
Spread the mushroom filling on it.
Pour in the remaining dough.
Bake the cabbage and mushroom pie for about half an hour in an oven preheated to 200°.
Cool the finished baked goods and serve.

Pie with mushrooms, cabbage and minced meat

This recipe is different from the others because it is cooked not in the oven, but in a frying pan.

Compound:

fresh cabbage – 350 g;
eggs – 2 pcs.;
saffron milk caps or porcini mushrooms – 100 g;
dill (or parsley) - several sprigs;
minced chicken – 150 g;
flour – 2.5 tbsp. l.;
onion – 1 head;
salt and pepper mixture - to taste.

Preparation

Chop the onion into cubes.
Cut the saffron milk caps into several pieces.
In a preheated frying pan, fry the onion until soft.
Add minced chicken and saffron milk caps. Sprinkle the filling with salt and pepper mixture. Fry it over medium heat until golden brown.
Beat the eggs with a whisk. Mix them with sifted flour. Add finely chopped dill and salt.
Shred the cabbage by hand or grate it. Mix it into the dough.
Grease a frying pan with vegetable oil and add half of the cabbage mixture.
Place the meat filling on top.
Use the last ball to spread the cabbage dough.
Set the heat to low and fry the pie until golden brown.
Then turn the product over.
When the pie turns golden brown on all sides, it is ready. Serve it with your favorite sauce.

Filling options

Fried fish;
hard or processed cheese;
boiled eggs;
spinach;
sauerkraut;
green onions;
boiled potatoes.

These are just a few interesting recipes cabbage and mushroom pies. We hope everyone enjoys these hearty baked goods.

Cook with love!

How to calculate your diet correctly

To start calculating the diet, you need to know several parameters of the child - exact weight, age and the amount of food eaten (exactly the amount that you counted on the days of the phenylalanine-free diet). We make the initial calculation, which will be adjusted only by your child’s tests, using tabular data, and subsequently you will determine the data specifically for your baby, taking into account his individual tolerance and health status.

Let's proceed with the calculation according to the following scheme:
Child's weight multiplied by required amount phenylalanine per day (see Table 1);
Divide the result by 50 to obtain protein from natural products;
Multiply the child's weight by total protein;
From the result of multiplying the weight and total protein take away the protein from natural products. The amount of therapeutic protein is obtained;
Convert the amount of medicinal protein into the amount of dry medicinal mixture.

The table shows protein consumption standards from Polish nutritionists. Protein consumption standards in the CIS countries are higher - from 2.9 g per kg of weight of a child aged 0 to 3 months to 3.5 g/kg (Republic of Belarus). When choosing standards for calculation, you should be guided by tests and the child’s appetite.

Protein, phenylalanine and calorie intakes
Table 1
Age Protein, g Phenylalanine, mg Energy, kcal
0-6 months (per 1 kg of weight) 2.4 g per kg 30-60 108
6-12 months (per 1 kg of weight) 2.2 g per kg 30-60 96
1-3 years (per 1 kg of weight) 2.1-1.95 g per kg 20-30 100-86
4-6 years 30-35 15-20 1400-1600
7-9 years 35-40 10-15 2000-2200*
10-12 years 45-50 10-15 2200-2500*
13-"teenagers" + adults 0.9-1g per kg 5-15 2200-2500*
*Here - bottom line for girls, top for boys

For example:
The child’s weight is 4.2 kg, age is 1 month, the amount of phenylalanine per kg of weight is 60, let’s take the norm of total protein for age – 2.4 g/kg (if the child cannot handle larger amounts of total protein). I want to emphasize that each child is an individual and one child at that age requires a total protein norm of 2.0 g/kg, while another 2.8 g/kg will not be enough.
4.2 x 60 = 252 (FA per day)
252: 50 = 5.04 (g natural protein per day)
4.2 x 2.4 = 10.08 (g total protein per day)
10.08 – 5.04 = 5.04 (g medicinal protein per day)
Let's convert 5.04 g of therapeutic protein into the amount of the amino acid mixture Afenilak 15 (protein equivalent of 15 g of protein in 100 g of dry mixture). Let's make a proportion:
If 100 g of the mixture contains 15 g of medicinal protein, then
in X g of mixture – 5.04 g of medicinal protein.
From here we find
X = 5.04*100/15 = 33.6 g of dry mixture Aphenilac 15.
Where is the volume that we so insistently asked you to count? And now the volume comes into play!
According to the tabular data, the volume baby nutrition from birth to 2 months should not exceed 1/5 of body weight. For a child weighing 4.2 kg, the volume of food should not be more than 4.2/5 = 0.84 dl - should not exceed 840 ml.
Let us clarify right away – these are average statistical data. You can calculate the volume needed specifically for your child yourself.
The volume that your child ate when he was fed unlimitedly only with a medicinal formula will show whether your baby can handle the amount of nutrition that you calculated theoretically. In this case, the volume of the therapeutic mixture will be 45 x 8 = 360 ml (at a dilution of 1 to 8) and the volume breast milk is:
5.04 x 100 / 1.2 = 420 ml
360 + 420 = 780 ml
If you see that the child is not eating enough, because... In terms of volume, you could still eat 60 ml, then dilute the mixture with a little more water to increase the volume of the medicinal mixture. But usually the volume of the mixture is enough. Often the problem is the opposite - to feed the child all the “norm” he is entitled to. Here, a reduction in the calculation of the norm of total protein per kg of the child’s weight (from 2.4 to 2.2), diluting the mixture in a smaller proportion, but not less than 1 to 7, can be used.
And one more nuance of feeding. If your child is breastfeeding, then first you need to feed the amino acid mixture (with eight feedings a day - 1/8 of the calculated daily norm medicinal mixture), and then the baby is offered the breast until full. If the child is bottle-fed, then it’s the other way around: first, the child is fed with an adapted milk formula (also 1/8 of the norm of the total volume of natural protein), and then the child is fed until satiety with a medicinal formula.
Now your task is to monitor the analysis. It has increased above normal (above 4), it immediately increases medicinal mixture and this amount of protein reduces natural protein. If, on the contrary, the analysis is low (less than 0.7), then the therapeutic protein decreases and, accordingly, the amount of natural protein increases.

When making calculations, a balance must be maintained:

Total Protein = Medicinal Protein + Natural Protein

Phenylketonuria (PKU)- a hereditary disease based on a violation of amino acid metabolism. PKU combines several genetically heterogeneous forms of phenylalanine metabolism disorder, similar in clinical signs. This is like classic PKU, caused by phenylalanine 4-hydroxylase deficiency (PKU). I type), and atypical (malignant) forms associated with a defect in the pterin cofactor (PKU II And III type).

The average incidence of PKU among newborns in Russia, according to mass survey data, is 1:8000. The most common form of PKU is the classical one, in which diet therapy is the only effective treatment method.

Clinical manifestations of the disease

At birth, a child with classic phenylketonuria looks well outwardly, but he already has pronounced disturbances in the amino acid composition of the blood, with the level of phenylalanine in the blood serum exceeding 20 mg% (1200 µmol/l). Phenotypic features include hypopigmentation of the skin, hair, iris, and sometimes there is a peculiar “mouse” smell of urine.

If there is no treatment, Clinical signs diseases. PKU manifestation occurs between 2 and 6 months of age. The first symptoms of the disease are nonspecific and are a consequence of the manifestation of vegetative-visceral lability and increased neuro-reflex excitability. Subsequently, mood instability progresses (lethargy, increased irritability, anxiety), lack of interest in the child’s surroundings, regurgitation, disturbances muscle tone(more often muscle hypotonia), signs of atopic dermatitis, there may be seizures. Subsequently, a delay in motor and psycho-speech development is formed, and microcephaly is often noted.

From the second half of the year, untreated patients may develop epileptic seizures, severe mental retardation (even idiocy), and social maladjustment.

Medical nutrition

Diet therapy is the only effective method treatment of classic PKU. Its main goal is to prevent the development of damage to the central nervous system, mental defect, and impaired physical development.

To organize therapeutic nutrition for a child with PKU, it is necessary to have specialized products based on mixtures of amino acids or protein hydrolysates with low content phenylalanine, which are the main sources of protein in the diet.

When organizing diet therapy for patients with PKU, it is necessary to consider:

clinical form of the disease;

child's age;

phenylalanine tolerance;

blood phenylalanine level;

the amount of natural protein obtained from food;

realize differentiated approach to the use of natural products according to the child’s age.

Dietary therapy must begin within the first three weeks of the child's life.

In the first year of life, medicinal products are used that are balanced in all respects. nutrients, but lacking or low in phenylalanine. These include “Aphenilak 13”, “Aphenilak 15”, “XP Analogue LCP”, “MEmil PKU 0”, “Phenyl Free 1” (Table 57).

The needs of PKU patients for basic food ingredients are close to physiological norms; the amount of protein per day is calculated based on 2.2-2.9 g/kg body weight. Since phenylalanine is an essential amino acid, the minimum requirement must be met to ensure normal height and development of a child with PKU. How smaller child, those in more he needs phenylalanine, since (unlike adults) 40% of dietary phenyl-

In children, lanine is used for the synthesis of the body’s own proteins. Equivalent replacement for protein and phenylalanine is carried out using the “portion” method of calculation: 50 mg of phenylalanine is approximately equivalent to 1 g of protein, which allows for adequate replacement of products for protein and phenylalanine.

During the first year of life for children with PKU, permissible quantity phenylalanine per day ranges from 90 to 35 mg/kg body weight (Table 58).

Treatment begins at a serum phenylalanine level of 15 mg% (900 µmol/l) or higher. The main criterion for diagnosing PKU and assessing the effectiveness of treatment is the level of phenylalanine in the blood.

Specialized products based on a mixture of amino acids are introduced into the diet gradually, over 7-10 days, the initial doses are 1/5-1/10 of their required daily amount. At the same time, the proportion of protein contained in natural products in the diet is reduced, and a specialized product is added to each meal. In the first months of life, the only source of protein from natural products is expressed human milk or infant formula with a minimal protein content (1.2-1.4 g per 100 ml of ready-to-use formula). Expressed human milk or formula is combined with the required amount of a specialized product, diluted boiled water or special water for baby food, while the total amount of nutrition corresponds to the patient’s age. It is recommended to prepare food immediately before each feeding.

Other approaches to prescribing a diet for an infant are also possible. If the level of phenylalanine in the blood is very high (900-1200 µmol/l), then when switching to therapeutic diet It is recommended to feed the patient for 2-3 days only a specialized product based on a mixture of amino acids without phenylalanine or a protein hydrolyzate with a low content. This allows you to more intensively reduce the level of phenylalanine in the blood; only after it has normalized, expressed human milk or infant formula is gradually included in the diet.

Purpose of complementary feeding

From 4 months of age, the diet of a PKU patient is expanded to include fruit and berry juices (apple, pear, plum, etc.), and from 4.5 months - fruit puree. Complementary foods in the form of vegetable puree or canned fruits and vegetables for baby food without the addition of milk are introduced into the diet from 5 months. At 5.5 months, 10% porridge made from ground sago, low-protein cereal or dairy-free porridge industrial production based on corn and rice flour, containing no more than 1.0 g of protein per 100 ml of ready-to-eat food. From 6-7 months, mousses and jelly are introduced into the diet, which are prepared using amylopectin swelling starch and fruit juice. Features of the composition of complementary feeding products, as well as the timing of their introduction, are presented in Table. 59.

Table 59. Timing of introduction of complementary foods to children of the first year of life with phenylketonuria

Products and dishes	Age, months
Fruit juice
Fruit puree
Vegetable puree
Low protein porridge
Kissel, low protein mousse
Vermicelli low protein
Low protein bread
Milk porridge




Rusks, cookies
Vegetable oil
Butter

Dietary treatment of patients with PKU must be carried out under strict control of phenylalanine levels in the blood serum. For patients with PKU, it should be in the average range (3-4 mg%, or 180-240 µmol/l). If the level of phenylalanine decreases to 2 mg% and below (120 µmol/l and below) or exceeds 8 mg% (480 µmol/l), protein correction in the child’s diet is necessary.

Used in Russia next diagram control of phenylalanine content in the blood for children of the first year of life:

up to 3 months - 1 time per week (until receiving stable results) and then at least 2 times a month,

from 3 months to 1 year - once a month, if necessary - 2 times a month

In children with PKU, the prognosis depends on the time of initiation and adequacy of diet therapy. With timely treatment of the classic form of PKU, the prognosis is relatively favorable. With late and inadequate dietary therapy, the prognosis worsens significantly.

Materials for this chapter were also provided by: Ph.D. Rybakova E.P. (Moscow), Ph.D. Bushueva T.V. (Moscow), Ph.D. Zvonkova N.G. (Moscow).

Diagnosis of phenylketonuria long years associated with the concept of mental retardation. And it is not just words. Quite recently, literally 25-30 years ago, serious clinical researches diseases that gave following results(hereinafter quoted from the academic publication Lebedev B.V., Blyumina M.G. Phenylketonuria in children. - M.: Medicine, 1972. -152 p.).
Psychopathological disorders in phenylketonuria are complex and polymorphic. The vast majority of patients (about 92-96%) have severe degrees mental retardation- idiocy and imbecility. In 3-4% are detected mild deficiency intelligence and 0.2-0.3% have almost normal mental abilities.
The structure of an intellectual defect has a number of features. Children are emotionally monotonous, unexpressive, and do not strive for emotional communication with parents and peers. There are often periods psychomotor agitation, reaching psychotic expression with impulsiveness, stereotypical pretentious movements, mannerisms, grimaces, echopraxia and echolalia. Sometimes states of excitement alternate with substuporous and stuporous phenomena, sometimes occurring with signs of waxy flexibility.
Most children, often with profound mental underdevelopment, experience epileptiform seizures. Attacks can be both episodic and systematic, tend to be polymorphic, and are resistant (insensitive) to anticonvulsant treatment without the use of specific pathogenetic therapy.

You read it and are horrified. That's how it was. Through the efforts of many scientists and practitioners, specific dietary therapy was created, tested, and introduced, which helps prevent the formation clinical picture diseases. Many children (and adults!) retain normal intelligence, study, work, give birth to healthy children, live full life. To do this, you need only one thing - strict discipline in following a diet. That's all! Such a little separates " severe degree mental retardation» from happiness healthy life. It would seem that everything is so clear. In practice, everything happens completely differently.
Experience working with parents and children with PKU makes it possible to assert that a very small percentage of parents have a real understanding of the risk of diet violations. In 78% of cases, it was relatives who provoked children to eat foods that were harmful to them. How can I do that? There are a few bad advice on this score.

Take pity on the poor child, deprived of life, let him try just a little tasty food. He’s so worried about the fact that everyone around him eats meat (fish, cheese, buns, etc.), but he doesn’t. This microscopic dose will not cause a rise in phenylalanine. She'll just move taste preferences child. And the baby will understand that if it’s not possible, but you really want to, then it’s possible. And then, on his own, with a 100% probability he will definitely break the diet.

Have pity on the poor child, do everything for him. He's sick, it's so difficult for him. There is no need to put away toys, wash dishes, or wipe off dust. All this can be done easily and quickly by a mother, grandmother, aunt, nanny, etc. You will gradually teach your child to be irresponsible and passive towards life. He won't be able to school age just like that, “suddenly” you start to control yourself, resist temptations, it will be easier for him to go with the flow, because you taught him that way!

Have pity on the poor child, don’t traumatize his psyche with stories about illness. This is so scary! And the child will know little about the consequences of breaking the diet. And he won’t understand what he’s risking. And he will start eating anything because he wants to.

It seems that all these words are a mockery of the feelings of parents. This is wrong. It is infinitely difficult, painful, and terrible to watch when a child’s personality disappears before your eyes, his intellect disintegrates, his bright future, to which he had the right, melts away!!! And the main responsibility lies with the parents. Only they can raise a child in such a way as to preserve his path forward, in order to realize his capabilities and abilities to the fullest.
Everyone knows that smoking, alcoholism, drug addiction are definitely bad, they are harmful to the body, they addictive behavior which needs to be fought, treated, overcome with the help of psychologists, social support etc. For a child with PKU, “human” food is just as unequivocally evil. This is the first and most important thing that parents should always remember. No sane person would ever pour vodka instead of water into a baby's glass. Why do some adults think it’s okay to give a child with PKU a “taste” of a piece of cookie or candy?
The PKU diet is simply a way of life. You can learn it only if you have long-term daily experience of doing what you can labor activity. When a child is deprived of this burden, it will be incredibly difficult for him to cope with any regime, any violence against desires.
And one last thing. Without awareness, motivation for any activity is never formed. A child at any age is able to understand what will happen to him if he breaks his diet. PKU is not atopic dermatitis, not gastritis or colitis. There, the consequences of breaking the diet are immediately clear and there is no need to explain anything. I ate candy and became covered with a crust that itches and does not allow me to live in peace; or you ate candy - and you die from terrible stomach pain. With phenylketonuria, the consequences are not felt by the person himself. People around them see them. And these consequences are much worse than abdominal pain or redness of the skin.
Parents! Think about the future of children!!! Really feel sorry for them!
Sincerely,
Nadezhda Mazurova,

Metabolic disorders that occur due to gene mutations, are called hereditary metabolic diseases.

Disorders in genes can also occur due to insufficient or excess nutrition, poisoning (toxic) effects heavy metals, some medicines, products that are formed in the body during its own metabolism (free radicals).

As a result, metabolic processes inside the cell (intracellular metabolism) are disrupted, causing diseases such as phenylketonuria, histidinemia, galactosemia, etc.

If absorption processes are disrupted nutrients in the intestines, they arise the following diseases: celiac disease, cystic fibrosis, sucrose intolerance, etc.

All these and similar diseases currently have the main treatment method diet therapy. Nutrition in general can act as a regulator of metabolism in the body, and individual foods introduced (or removed) into the daily diet can cure metabolic diseases.

In almost all diseases of this group, there is one substance or another that accumulates in the body and poisons it due to the fact that there are no necessary enzymes that would break it down. It is enough to exclude from the diet those substances that cannot be absorbed by the body, and the disease will not develop.

Congenital disorders amino acid metabolism
Amino acids- These are components of protein molecules. If at birth there is no special enzyme in the body that breaks down a particular amino acid, it accumulates in the blood, urine, and cerebrospinal fluid.

Such disorders appear in the first months or even weeks of a child’s life in the form of severe changes in the central nervous system, liver, kidneys. To the means special treatment Most congenital enzymatic pathologies include a diet based on the principle of excluding from the diet that nutritional factor that cannot be normally absorbed by the body. In some cases, diet is combined with drug treatment.

Dietary nutrition for disorders of amino acid metabolism is carried out using specially created dietary preparations, the addition of vitamins and mineral components, as well as natural products, i.e. the principle is combined use drugs and a special set of products. At the same time, it is very important to know amino acid composition some food products.

Phenylketonuria
Refers to the number hereditary diseases, in which therapeutic nutrition is the only method of treatment. The disease was first described in 1934 and occurs in peoples of all races, but most often in the north. The disease manifests itself in different age categories- from 6 weeks to 70 years. It occurs in men and women, although women get sick more often, it is inherited, and the parents of patients, as a rule, do not suffer from this disease.

Causes
In the liver, as a result of gene changes, the gene is absent or is in insufficient quantities a special enzyme (phenylalanine oxidase) that normally converts the amino acid phenylalanine into tyrosine. Instead, phenylalanine becomes a source of toxic products - phenylpyruvic, phenyllactic and phenylacetic acids. In addition, the content of unconverted phenylalanine in the blood and tissues increases significantly (up to 0.2 g/l instead of 0.02 g/l), and there is a lack of tyrosine, which is very important for the development of the nervous system. If we add to the above that phenylalanine is the main part of almost all proteins, the scale of disturbances in the growth and development of the body becomes clear.

This disease is known as phenylpyruvic oligophrenia. And this is no coincidence, since greatest violations develop in the central nervous system and ultimately lead to dementia. And all this happens due to insufficient supply of the amino acid tyrosine to growing brain tissue.

Symptoms
Signs of phenylketonuria are detected already from the first weeks of a child’s life: lethargy, excessive childishness are noted, sometimes, on the contrary, increased excitability, the need for prolonged rocking, and sleep may be short.

Characteristic features are and. As the child grows, his lag behind his peers becomes obvious: the child later begins to hold his head up, sit, some do not walk or speak. Convulsions in the form of nodding, bowing, shuddering, and short blackouts may be observed.

Seizures are observed in early age, gradually they become less common, and by the age of 10 they completely disappear. Because of increased tone individual muscle groups, patients have characteristic pose: they stand with their legs spread wide apart, knees bent and hip joints, the shoulders are lowered. They walk in small steps, while swaying. In severe forms of dementia, patients sit with their legs crossed (tailor's pose). Children are blond, with fair skin and blue eyes, the facial expression is often normal and pretty. They may be excessively sweaty with a characteristic mouse (or wolf) odor.

The skin of such children is especially sensitive to sun rays and the most common injuries, they have eczema and dermatitis.

According to foreign authors, 60% of patients are idiots, 30% are weak-minded, and only 10% of patients have weak degree oligophrenia (dementia). Spontaneous improvement mental functions has never been observed with phenylketonuria, which is why it is so important to identify the disease as early as possible and prescribe appropriate nutrition.

Already during the first examination of the child in the clinic (most often when he is one month old, but not later than the second month of life), the local pediatrician conducts a test for phenylketonuria according to special program screening (prescribes a Fehling test for phenylpyruvic acid in the urine). A few drops of a special reagent (5% solution of ferric chloride and acetic acid) is applied to a diaper soaked in the baby’s urine. The appearance of a green color within 30 minutes is considered positive result, pointing to increased risk phenylketonuria and requiring further examination.

Treatment
Transferring a child to special nutrition when early detection disease guarantees normal neuropsychological development. Diet therapy is based on the principle of sharply limiting phenylalanine, which comes from food. Any protein contains up to 8% phenylalanine, and therefore high-protein foods (meat, fish, liver, sausages, eggs, cottage cheese, baked goods, cereals, beans, peas, nuts, chocolate) are completely excluded from the diet. Milk, vegetables, and fruits are introduced into the diet with careful calculation of the phenylalanine they contain.

Acceptable natural foods include low-protein cereals, cornflakes, mushrooms, sunflower oil, vegetables, fruits, juices, honey, jams.

The daily amount of phenylalanine is 15-50 mg per 1 kg of body weight, and the need for this amino acid decreases with age. On average, children receive between 3.5 and 8 g of protein per day. natural products, which is completely insufficient for normal growth, and therefore a special protein component in the form of protein hydrolysate. In fact, milk is eliminated and replaced with protein hydrolyzate, it becomes the main food product, introduced with fruit and vegetable juices, purees and soups in the first or second year of life - 2 times a day in the morning and in the afternoon with juices or sweet tea. The drug is introduced into the diet gradually, starting with 1/3 daily dose and within 1-2 weeks, bring it to the required amount, while simultaneously reducing the dose of protein from natural products in the diet.

With exclusion from the diet high protein foods A fat deficiency is inevitable, which is replenished with butter and vegetable oils, and in children of the first year of life - with fish oil. The fat content in the diet is up to 35%.

The need for carbohydrates is met by vegetables, fruits, sugar, and starchy foods. The carbohydrate content of the diet is 60% of the total calorie content.

Patients receive calcium, iron and vitamins in the form of appropriate medications. However, avoiding high-protein foods (in particular, pasta, cereals and bakery products) does not provide the required amount of food, and therefore protein-free bread, pasta, noodles, and cereals have been developed. For the preparation of first and second courses, artificial sago made from corn starch is used as a cereal substitute. Corn, wheat, potato starch used for preparing jelly, protein-free bread, and second vegetable dishes.

Method for preparing a mixture with protein hydrolyzate for children of the first year of life
Corn starch, expressed breast milk, sugar are added to the vegetable oil, everything is thoroughly mixed. Add water (1/2 of the total amount), boil for 2 minutes (the amount of added water is calculated from the total daily requirement child in liquid, taking into account the amount of juices, soups).

The required amount of protein hydrolyzate is diluted in the remaining water, boiled, both liquids are mixed, juice is added and bottled. The mixture is stored in the refrigerator.

If the child is transferred to artificial feeding, a special mixture is prepared on the basis of dry formulas close to human milk. Patients with phenylketonuria receive earlier complementary foods in the form of canned vegetables and fruits for baby food. Porridges are prepared on the basis of protein-free cereals.

Exemplary daily ration(in grams) for children of the first 6 months, patients with phenylketonuria (body weight - 5-6 kg):

breast milk - 350,
sugar - 20,
glucose - 20,
butter - 8,
vegetable oil - 10,
corn starch - 20,
apple juice - 50,
applesauce — 30,
fish fat- 1 tsp,
protein hydrolyzate - 23.

Approximate daily diet (in grams) for children in the second half of life with phenylketonuria (body weight - 10 kg):

formula replacing human milk - 250,
sago porridge (20%) - 100,
vegetable puree - 100,
applesauce – 50,
Apple juice — 50,
sugar - 20, glucose - 20,
butter - 8,
vegetable oil - 10,
corn starch - 15,
fish oil - 2 tsp,
protein hydrolyzate - 32.

For children over 1 year of age, the daily diet is expanded to include various vegetables, fruits, protein-free bread and pasta.

Average daily set of products (in grams) for patients with phenylketonuria from 1 year to 1.5 years:

protein-free vermicelli - 6,
protein-free cereal - 14,
dry formula replacing human milk - 13,
artificial sago – 37,
butter - 26,
sugar - 68,
apples - 246,
pickled cucumbers - 3,
onion - 12,
carrots - 122,
tomato juice - 32,
apricot juice – 42,
cherry juice – 28,
apple juice – 28,
plum juice – 42,
grape juice - 21,
vegetable oil - 25,
cabbage - 97,
potatoes - 85,
beets - 54,
jam - 10,
prunes - 2,
tomato paste - 3,
corn starch - 3,
zucchini - 64,
sour cream - 1,
spinach - 34,
apricot puree - 28.

Approximate one day menu for children with phenylketonuria aged 1 to 1.5 years (in grams):

Breakfast: sago porridge (mashed) - 150 (yield of ready-made dishes), applesauce - 50, sweet tea - 150.
Dinner: pickle with protein-free cereals (mashed) - 150, vegetable puree - 150, apricot juice- 150, corn starch bread - 20, butter — 10.

Afternoon snack: fruit puree with sugar - 150.
Dinner: beet caviar with apples - 150, cherry mousse - 50, sweet tea - 150.

Average daily set of products (in grams) for patients with phenylketonuria aged 1.5 to 3 years:

corn flour - 3,
artificial sago – 37,
potatoes - 80,
cabbage - 135,
carrots - 141,
beets - 68,
zucchini - 107,
pumpkin - 35,
tomatoes - 13,
onion - 12,
cucumbers - 3.6,
spinach - 14,
fruits – 261,
prunes - 2,
raisins - 1.4,
cranberries - 10,
sugar - 81,
tea - 0.3,
butter - 26,
vegetable oil - 31,
sour cream - 0.7,
tomato juice - 35,
apricot juice – 43,
cherry juice – 21,
grape juice - 21,
fruit puree – 79,
tomato paste - 1.3,
jam - 2.8,
protein-free vermicelli - 10,
protein-free cereal - 10,
Protein-free bread - 25.

Sample menu for children with phenylketonuria aged 1.5 to 3 years (in grams):

6.00 h: juice - 100.
Breakfast: Stewed carrots - 150, plum or apple juice - 50, sweet tea - 150.
Dinner: Vegetarian borscht (mashed) - 150, sago porridge - 150, cranberry jelly - 150, protein-free bread - 50.
Afternoon snack: fruits - 200, sugar - 15.
Dinner: stewed cabbage - 150, cherry juice - 150.

Average daily set of products (in grams) for patients with phenylketonuria aged 3 to 5 years:

corn flour - 3,
artificial sago – 45,
potatoes - 95,
cabbage - 150,
carrots - 150,
beets - 126,
zucchini - 58,
pumpkin - 110,
tomatoes - 35,
onion - 13,
cucumbers - 12,
spinach - 10,
fruits - 250,
prunes - 2,
raisins - 1,
cranberries - 10,
sugar - 85,
tea - 0.3,
butter - 26,
vegetable oil - 40,
sour cream - 1,
tomato juice - 35,
apricot juice – 43,
cherry juice – 21,
grape juice - 21,
fruit puree - 80,
tomato paste - 2,
jam - 3,
protein-free vermicelli - 20,
protein-free cereal - 20,
Protein-free bread (corn) - 30.

Sample menu for patients with phenylketonuria aged 3 to 5 years (in grams):

Breakfast: mashed potatoes with cucumber - 170, corn starch bread - 20, sweet tea - 180.
Dinner: Vegetarian cabbage soup - 150, sago porridge - 150, cranberry jelly - 150, corn bread - 20.
Afternoon snack: fruits - 200, sugar - 20.
Dinner: vinaigrette - 200, sweet tea - 150, corn bread - 20.

Average daily set of products (in grams) for children with phenylketonuria, aged 5 to 7 years:

corn flour - 3,
sago - 45,
potatoes - 101,
cabbage - 150,
carrots - 126,
beets - 58,
zucchini - 78,
pumpkin - 36,
tomatoes - 11,
onion - 14,
cucumbers - 10,
spinach - 14,
fresh fruits – 257,
prunes - 2,
raisins - 50,
sour cream - 2,
tomato juice - 35,
apricot juice – 48,
cherry juice – 21,
apple juice - 14,
grape juice - 14,
fruit puree – 114,
tomato paste - 3,
protein-free vermicelli - 20,
protein-free cereal - 20,
Protein-free bread (corn) - 50.

Sample menu for children with phenylketonuria aged 5 to 7 years (in grams):

Breakfast: vegetable salad - 200, plum juice - 100, sweet tea - 150.
Dinner: Beetroot soup - 200, sago pudding - 200, grape juice- 150, corn bread - 50.
Afternoon snack: fruits - 200, sugar - 20.
Dinner: stewed vegetables - 200, cranberry jelly - 150.

Recipes for dishes (in grams) from protein-free products

Vegetarian soup with noodles – 150.
Protein-free vermicelli - 15, carrots - 10, onions - 5, butter (peasant) - 5.
Vegetarian rassolnik with sago – 150.
Potatoes - 70, cucumbers - 30, sago - 7, carrots - 10, onions - 5, butter - 5.
Vegetarian soup with protein-free cereals - 150.
Protein-free cereals - 15, carrots - 10, potatoes - 30, onions - 5, tomato - 2, butter - 5.
Fruit soup with sago – 150.
Sago - 7, dried fruits - 15, sugar - 5.
Puree vegetable soup with sago – 150.
Zucchini - 20, carrots - 5, turnips - 20, tomatoes - 10, potatoes - 20, sago - 7, onions - 5, vegetable oil - 5.
Boiled protein-free vermicelli - 150.
Protein-free vermicelli - 50, butter (peasant) - 10, sugar - 5.
Sago porridge with pumpkin - 150.
Artificial sago - 40, pumpkin - 50, sugar - 10, butter - 10.
Protein-free cereal porridge with raisins – 150.
Protein-free cereals - 30, sugar - 10, butter - 10, raisins - 10.
Cabbage cutlets - 130.
Cabbage - 165, vegetable oil - 12, aminopectin swelling starch - 12.
Sago casserole with apples – 60.
Sago - 50, apples - 20, vegetable oil - 12, sugar - 10, aminopectin swelling starch - 12.
Protein-free cereal cutlets - 130.
Protein-free cereal - 50, aminopectin swelling starch - 10, vegetable oil - 10, sugar - 10.
Cornstarch pancakes - 150.
Corn starch - 45, aminopectin swelling starch - 10, vegetable oil - 10, baking soda - 1.2, sugar - 5, salt - 1.5, water - 120, lemon acid — 0,1.
Protein-free bread made from corn starch - 300.
Corn starch - 176 g, aminopectin swelling starch - 24, sugar - 7, vegetable oil - 10, salt - 6, soda - 2.4, water - 230, citric acid - 0.5.
Protein-free mousse - 100.
Aminopectin swelling starch - 12, sugar - 12, apple juice - 80.
Cranberry jelly - 150.
Cranberries - 15, corn starch - 5, sugar - 15.

Treatment results directly depend on the age at which treatment was started special food: if therapeutic nutrition is introduced from the first two months of a child’s life, this guarantees his full neuropsychic development; with more late start Medical nutrition can only improve the condition.

The criterion for the effectiveness of therapeutic nutrition and its withdrawal is the level of phenylalanine in the blood serum (from 4 to 8 mg%), as well as the age of the child: the withdrawal of therapeutic nutrition with normal stable levels of phenylalanine in the blood serum is carried out before the child enters school (at 7 or 8 years ).

Big preventive value has observation of families that already have children with phenylketonuria. Newly born babies from such families must undergo special biochemical research blood, and according to indications - early dietary nutrition.

Examination of all children in one month old according to mass screening programs, which is strictly mandatory, carried out by a local pediatrician in the presence of the baby’s mother, it also helps prevent the development of severe mental disability.

Women's milk - 0.056
Cow's milk - 0.136.
Kefir - 0.138.
Cottage cheese - 0.491.
Chicken egg - 0.732.
Beef meat - 0.789.
Chicken meat - 0.932.
Beef liver - 0.845.
Cod - 0.651.
Rice groats - 0.313.
Semolina - 0.399.
Buckwheat - 0.395.
Oatmeal - 0.363.
Millet groats - 0.48.
Pearl barley - 0.331.
Peas - 0.763.
Wheat flour - 0.322.
Pasta - 0.488.
Rye bread - 0.278.
Wheat bread - 0.330.
Cookies - 0.334.
Potatoes - 0.083.
Carrots - 0.059.
White cabbage - 0.073.
Tomatoes - 0.023.
Oranges - 0.04.
Lemons - 0.032.
Apple juice - 0.021.
Orange juice - 0.042.
Lemon juice - 0.036.