Fructose intolerance: Lifelong diet or temporary inconvenience. Allergy to sweets

Fructose allergy in children and adults is less common than negative reactions to oranges or full-fat milk, but when the diagnosis is confirmed, the patient has to significantly change the diet. Many fruits and vegetables contain fruit sugar in large quantities, and if you refuse them, the body receives less nutrients.

What to do with fructose intolerance in a child? What foods contain the highest percentage of a valuable substance? How much fructose can be consumed per day without compromising health? Answers in the article.

Reasons for the development of pathology

Unlike other types of allergies, a negative reaction when eating foods rich in fructose develops when there is a lack of a special enzyme that breaks down fruit sugar. Weakness of immunity is only an additional factor that increases the sensitization of the body.

Deficiency of fructose-1-phosphataldolase interferes with the normal digestion of food, rotting residues accumulate, and fermentation processes are activated. As a result, not only the absorption of nutrients is disturbed, but also fructose accumulates in the intestines and liver, which can lead to cirrhosis, problems with the activity of the digestive tract.

An excess of toxins provokes the activation of protective forces. Attempts to remove excess allergens with the inclusion of the immune system lead to negative reactions on the skin, in the stomach, and intestines. Other signs of allergic inflammation often develop.

The risk of a negative reaction to fruit sugar is increased by the following factors:

• frequent overeating, addiction to sweets, fruits, honey, fruit drinks;
• improper, early introduction of complementary foods;
• hereditary predisposition;
• malnutrition of the mother during breastfeeding (excessive consumption of fruits, syrups rich in fructose, sugar, honey);
• pathology of the pancreas;
• hypersensitivity of the body.

Fructose allergy ICD code - 10 - T78.1 ("Other manifestations of a pathological reaction to certain types of food").

• with a tendency to be allergic to fructose, print and store at home a table indicating the types of food, the content of fruit sugar in each item;
• when compiling a diet, select foods in which the amount of fructose is at a minimum level;
• do not overeat, eat berries, fruits, juices, vegetables, honey in moderation;
• take in recommended doses;
• refuse syrups with synthetic fillers;
• monitor the condition of the digestive tract, treat diseases of the intestines, liver, stomach;
• always study the composition of foods, medicines, drinks before buying: "hidden fructose" is often present. A good example is hard cheese.

Intolerance to fruit sugar, immune reactions with a lack of a special enzyme that breaks down fructose are complex processes that will require maximum attention to diet planning to eliminate. It is important to start treatment of the disease at an early age: if the rules of nutrition are observed, many children outgrow the “allergy”, with congenital fructose intolerance, you will have to adjust the diet all your life.

Why does fructose cause allergies? How does the disease manifest itself and how is it treated? Find out the answers after watching the following video:

In some cases, the body stops perceiving some kind of food. It can be caused by congenital features or acquired problems. In addition, such a violation can be a symptom of some serious diseases that require close attention and adequate correction. So a fairly common problem is lactose intolerance - a milk protein, it is faced by both newborn children and adult patients. But this violation is quite amenable to correction - you just have to choose the right diet. Possible problems of this type include fructose intolerance in a child, the symptoms of which in adults will also be considered in detail.

Fructose intolerance is also known as fructosemia or fructose malabsorption. This is a hereditary disease that is caused by a lack of fructose-1-phosphaldolase. Due to this deficiency, the fructose that enters the body is deposited as fructose-1-phosphate in the tissues of the liver, intestines, and kidneys. The disease makes itself felt at an early age, and is not yet curable.

Fructose intolerance - symptoms in adults and children

Manifestations of fructosemia can appear soon after the baby is born, if he is given complementary foods containing fructose or sucrose. Most often, the first symptoms of this disorder become noticeable after the introduction of fruit puree, juices, etc. into the baby’s diet. Now such an expansion of the diet is practiced mainly after the baby reaches six months of age, so fructose intolerance is rarely diagnosed until this time.

At an early age, the intake of sucrose or fructose in such children can cause vomiting, drowsiness and / or excitability. The baby's skin may become pale, and the baby himself looks lethargic. Excessive sweating is often recorded. Fructose intolerance is usually manifested by diarrhea, while loose stools have a rather significant volume (more than an ordinary serving).

If doctors fail to immediately determine the causes of such symptoms, and fructose and / or sucrose continue to enter the body, the patient loses body weight (which leads to the development of malnutrition), his liver may increase in size. Ascites is also quite often recorded - the accumulation of fluid inside the abdominal cavity.

Sometimes patients with fructosemia develop jaundice - the skin and mucous membranes turn yellowish, and the sclera may also turn yellow.

If a significant amount of fructose enters the body at a time, then a patient with intolerance to this substance may develop acute hypoglycemia. In this condition, there is a significant decrease in the amount of glucose (sugar) in the blood. Such a violation makes itself felt by a noticeable trembling of the limbs, a general violation of well-being. The patient may experience convulsions - paroxysmal involuntary muscle contractions, which are accompanied by an extreme degree of muscle tension. In some cases, loss of consciousness, and even the development of coma, is possible. In this case, the patient ceases to respond to any stimuli, which poses a serious threat to his life.
Hypoglycemia is usually fixed not in infants, but in older children.

Cataracts do not develop with such an ailment, and the mental development of babies is also not disturbed.

As practice shows, in most cases, fructose intolerance occurs in a mild form. Such a condition is far from always diagnosed on time, because the intake of a small amount of this substance does not worsen the general well-being of a person. In both children and adults, this does not lead to alarming symptoms. In this case, adult patients are sure that they simply do not like sweets in any of its manifestations.

If the disease proceeds in a severe form, then it is impossible not to notice it. Indeed, in this case, even a slight intake of fructose with baby food is fraught with the development of life-critical conditions.

Most often, with age, the condition of patients with even a severe form of fructose intolerance improves slightly, they can eat a small amount of products containing it without worsening their well-being.

Is it possible to cope with fructosemia?

Unfortunately, it is impossible to eliminate fructose intolerance. To correct this disease, the patient needs to forever abandon the consumption of foods containing fructose. And, as you know, such fruit sugar is found in all sweet fruits, as well as berries. In addition, it is present in honey and even in some vegetables. Also, patients have to exclude products with sucrose (cane or beet sugar) from the diet. Sorbitol (glucite, a sugar substitute) is also banned. It should be noted that the listed components are often included in products with a complex composition, drugs, etc.

Additional Information

Since patients with fructose intolerance cannot eat fruits and berries, they often lack some of the nutrients found in these foods. They are often prescribed additional multivitamin supplements, and herbs can also benefit.

So the next collection will be an excellent source of many vital vitamins. For its preparation, linden inflorescences and St. Grind and mix all these ingredients well. Brew a teaspoon of the finished collection with a glass of boiled water only. Infuse the remedy for half an hour under the lid, then strain. Take hot medicine twice or thrice a day about half an hour before meals.

The feasibility of using traditional medicine should be discussed with your doctor without fail.

Hereditary fructose intolerance is a consequence of congenital absence of fructose-1-phosphate aldolase and, additionally, fructose-1,6-diphosphate aldolase. The resulting metabolic disorders are expressed in the presence of an excess of fructose-1-phosphate in various tissues.

Frequency. The frequency of this pathology is estimated differently and is about 1: 30,000. In our group, 52 children with hereditary fructose intolerance were observed. The relatively high frequency of this disease in France is probably due to the fact that many newborns receive milk with the addition of sucrose, which contributes to the early manifestation of disorders. It is possible that in other countries this metabolic disease is detected later and its consequences are immediately reduced to a minimum due to the appearance of an aversion to sweet foods.

Broadcast. Transmission is carried out in an autosomal recessive manner. Some heterozygous children, as well as homozygous children, show a pronounced aversion to sweet foods. However, the disease in heterozygous children can only be detected by deduction, based on the presence of homozygous individuals in the family. In this case, it should be taken into account that in heterozygotes, tests with a fructose load or determination of aldolase activity in the liver will be negative.

Symptoms appear as soon as sucrose and, consequently, fructose are included in the diet. Of the 52 children with hereditary fructose intolerance, 22 were hospitalized in the first two months (group I), 25 - 3 months or more after the introduction of fructose (group II), while the last 5, born after the discovery of one disease in the family, from the very births received a fructose-free diet (group III).

In group I, children consistently vomited; anorexia was less common; none of the newborns developed an aversion to sugary foods. After eating, the appearance of pallor and a state of shock was observed only in 2 children. Liver enlargement was found constantly, while splenomegaly - only in 3 cases. Often there was a hemorrhagic syndrome, spontaneous or at the injection site. 3 children with jaundice had temporary and incomplete discoloration of stools. 8 newborns with edema and (or) ascites had a bleeding tendency. Finally, 22 children were urgently hospitalized due to acute liver failure, infection, hemorrhagic syndrome, shock or dehydration.

Diagnostic studies are primarily aimed at detecting signs of a metabolic disease, since with hereditary fructose intolerance there are common symptoms with galactosemia and hereditary tyrosinemia. From this point of view, the possible consanguinity of the parents, deaths with a similar clinical picture of members of this family in the past, the contrast between hepatomegaly and the severity of hepatocellular insufficiency, and the combination with signs of damage to the renal tubules become important. In less severe and shorter-lived forms masquerading as chronic liver disease, liver histology should look for a combination of steatosis and non-inflammatory fibrosis.

Among metabolic diseases, vomiting is an important argument pointing to hereditary fructose intolerance; its absence in a child fed sucrose is an important element against the assumption of this diagnosis. The appearance of clinical symptoms in the days after weaning is also an important diagnostic sign. If there are disturbances after eating, fructosuria, these should also be taken into account. Finally, in a certain number of observations, the fact of a family aversion to sweets is revealed.

The following three circumstances are important for confirming the diagnosis.

1. The exclusion of fructose consumption entails the disappearance of vomiting and hemorrhagic manifestations within a few hours and, after a few days, the normalization of hemostasis and the disappearance of signs of damage to the kidney tubules.

2. The fructose loading test causes a hypoglycemic reaction very characteristic of hereditary fructose intolerance. For evidence, it should not be carried out in a child receiving fructose, but 2-3 weeks after its exclusion from the diet. Taking into account the risk of hypoglycemia, the conditions for its implementation must be carefully defined. Intravenous administration of fructose at a dose of 0.25-0.30 g/kg for 2-3 minutes causes a rapid decrease in blood glucose and phosphorus with a simultaneous increase in blood levels of fructose, magnesium and lactic acid. A similar hypoglucosemic effect after intravenous administration of fructose can be observed only with hereditary deficiency of fructose-1,6-diphosphatase.

3. Determination of the activity of fructose-1-phosphate aldolase in the liver, intestinal mucosa and the cortical layer of the kidneys. In 52 patients, the activity of aldolase in relation to fructose-1-phosphate was much lower than in relation to fructose-1,6-diphosphate, while the ratio between fructose-1,6-diphosphate aldolase and fructose-1-phosphate aldolase was abnormal. high.

Pathophysiology. Enzyme deficiency is associated with a significant decrease in the activity of aldolase B in the liver, apparently due to a structural gene mutation.

The mechanism of liver damage seems to be quite similar to that in galactosemia and hereditary tyrosinemia: intrahepatocyte accumulation of non-metabolizable fructose and fructose-1-phosphate, accompanied by a decrease in the content of ATP and inorganic phosphorus. Glycogenolysis is inhibited at the level of the phosphorylase system and, temporarily, neoglucogenesis. These two mechanisms seem to explain the lack of response to fructose injection-induced hypoglycemia, which is also confirmed by the lack of an increase in glucose in response to glucagon administration after fructose administration. However, this does not explain the severity of hypoglycemia, not accompanied by an increase in insulin secretion, after fructose administration.

Treatment of fructose intolerance. The main method of treatment is the elimination of fructose and sucrose from the diet. The presence of pronounced signs of hepatocellular insufficiency may serve as an indication for urgent measures. should continue indefinitely, which is not difficult for older children, given their relative or absolute aversion to sweets. Repeated administration against this background of large amounts of fructose can cause the same disorders as in small infants. Intravenous administration of solutions containing fructose or sorbitol should be strictly excluded, given the known cases of death during such perfusion. It is also necessary to constantly prescribe vitamin C, since it is found mainly in products to be excluded, especially in fruits.

The immediate prognosis against the background of treatment is favorable. The long-term prognosis is very good, as children quickly restore their developmental delay in height and body weight. Only enlargement of the liver remains for many years. Repeated histological examinations of the liver showed rapid resolution of intralobular fibrosis seen in small infants and stabilization or regression of portal fibrosis. Conversely, steatosis remains, especially in the hepatocytes of the periportal zone, but becomes less diffuse. This does not appear to be related to the diet; steatosis does not disappear with the addition of dextrin, maltose, or glucose. Preliminary observations suggest the possibility of the disappearance of steatosis in children who are on a diet with a non-strict exclusion of fructose. It is interesting to note that children on a fructose-free diet do not develop dental caries, so dental monitoring can be an excellent means of monitoring treatment.

Newborns belonging to a family with hereditary fructose intolerance should be switched to a fructose-free diet immediately after birth. Their fructose tolerance should be tested later, at three months of age, by a stress test to decide whether the diet should be continued or discontinued. Five newborns in this group, who did not receive any sugary foods, still had hepatic steatosis a few weeks after birth; this fact raises the question of whether fructose in small quantities is not an essential food product.
Women's magazine www.

E. N. Preobrazhenskaya, Ph.D. nutritionist of the highest qualification category (North-Western State Medical University named after I. I. Mechnikov, St. Petersburg).

Diagnosis of lactose intolerance

Lactose Tolerance Test

Before the analysis, you can not eat anything. On the day of the test, the patient drinks a liquid containing lactose, which can cause gas and abdominal pain. After that, blood is checked every 30 minutes for 2 hours. If blood sugar levels do not rise, this may indicate lactose intolerance. This test does not apply to diabetic patients and infants.

Lactose intolerance in a child is established by exclusion. For 2 weeks, milk and dairy products are excluded from the baby's diet. After the disappearance of the symptoms of dyspepsia, they begin to gradually reintroduce milk into the child's diet in small portions. If symptoms of lactose intolerance appear 4 hours after the child has drunk milk or milk formula, this means that the child does not have enough lactase enzyme.

Exhaled hydrogen test

This is the most accurate analysis of lactose intolerance. Determination of the hydrogen content in the exhaled air makes it possible to identify the activity of microflora in the digestion of lactose. The concentration of gases is determined after a metered load of regular or labeled lactose. The method is used to diagnose lactose intolerance in adults and older children.

Analysis of stool acidity

Determination of the total carbohydrate content in feces, which reflects the body's ability to absorb carbohydrates.

Treatment of lactase deficiency

Only those cases of lactose intolerance that are clinically manifest need treatment. The main principle of treatment is a differential approach to therapy depending on:

• on the age of the patient (term or premature newborn, infant, early, older, adult patient);
• degree of enzymatic deficiency (alactosia, hypolactosia);
• genesis of fermentopathy (primary or secondary).

The main treatment for patients with absolute lactase deficiency (alactasia) is the complete rejection of the use of milk and dairy products. In parallel, therapy is carried out aimed at correcting intestinal dysbacteriosis, replacement therapy (the use of lactase preparations: lactase, tilactase, lacteide) and symptomatic treatment.

With secondary lactase deficiency, the main attention is paid to the treatment of the underlying disease. The decrease in the amount of lactose in the diet is temporary, until the mucosa of the small intestine is restored.

In patients with primary or secondary lactase deficiency, the degree of restriction on the consumption of milk and dairy products is strictly individual, since some patients cannot tolerate only milk, but are able to eat fermented milk products with a low lactose content. And patients with a slight degree of hypolactasia without harm to health can consume even small amounts of fresh milk (up to 100-150 ml per day). In such cases, it is allowed to take milk not on an empty stomach, slowly, in small portions no more than 1-2 times a week. Restrictions are removed with an increase in the patient's endurance to dairy products.

Keep a food diary!

The patient must keep a food diary. Recordings provide answers to two important questions:

• Which product caused flatulence and diarrhea?
• How much lactase should be added to food containing lactose?

In order to identify the patient's reaction to milk, it is recommended to carry out test loads with milk or lactose. Also shown is the training of the corresponding enzyme systems by prescribing small amounts of fermented milk products in a satisfactory condition of a sick child or adult patient. An indicator of an increase in the tolerance threshold for milk is the absence of intestinal disorders.

Carefully!

In the food industry, lactose is used in the production of many food products. In addition to milk and dairy products, lactose is present in packaged sausages, bagged soups, ready-made sauces, chocolate, and cocoa powder. It is used to increase the viscous properties of the product and the convenience of its use. Lactose in sweetness is 30-35% inferior to sucrose, it is added in large volumes. When baked, milk sugar takes on a brown tint, so it is an invariable ingredient in french fries, croquettes, confectionery and bakery products.

Lactose is also widely used in the manufacture of pharmaceuticals; it is one of the main components of flavors, flavor enhancers, sweeteners, etc.

Read labels on foods and medicines.

The amount of lactose in products

According to a special list of products, you can determine the amount of lactose present in the diet. Below are data from different authors.

• Dessert cream - 2.8-6.3.
• Coffee whitener - 10.0.
• Powdered milk yogurt - 4.7.
• Whole milk yogurt (3.5%) - 4.0.
• Milk yogurt (1.5%) - 4.1.
• Milk yogurt (3.5%) - 4.0.
• Natural yogurt - 3.2.
• Creamy yogurt - 3.7.
• Low-fat fruit yogurt - 3.1.
• Fat-free fruit yogurt - 3.0.
• Yoghurt ice cream - 6.9.
• Creamy fruit yogurt - 3.2.
• Cocoa - 4.6.
• Mashed potatoes - 4.0.
• Semolina porridge - 6.3.
• Rice porridge with milk - 18.0.
• Kefir - 6.0.
• Low-fat kefir - 4.1.
• Sausages - 1.0-4.0.
• Margarine - 0.1.
• Butter - 0.6.
• Sour milk - 5.3.
• Non-fat milk - 4.9.
• Pasteurized milk (3.5%) - 4.8.
• Condensed milk (7.5%) - 9.2.
• Condensed milk (10%) - 12.5.
• Condensed milk with sugar - 10.2.
• Powdered milk - 51.5.
• Skimmed milk powder - 52.0.
• Whole milk (3.5%) - 4.8.
• Whole milk powder - 38.0.
• Milkshakes - 5.4.
• Milk chocolate - 9.5.
• Ice cream - 6.7.
• Milk ice cream - 1.9-7.0.
• Ice cream ice cream - 1.9.
• Creamy ice cream - 5.1-6.9.
• Fruit ice cream - 5.1-6.9.
• Nougat - 25.0.
• Buttermilk - 3.5.
• Dry buttermilk - 3.5.
• Donuts - 4.5.
• Pudding - 2.8-6.3.
• Whipped cream (10%) - 4.8.
• Whipped cream (30%) - 3.3.
• Cream for coffee (10%) - 3.8.
• Pasteurized cream - 3.3.
• Pasteurized whole cream - 3.1.
• Sour cream (10%) - 2.5.
• Whey dry - 70.0.
• Gouda cheese (45%) - 2.0.
• Camembert cheese (45%) - 0.1-3.1.
• Mozzarella cheese - 0.1-3.1.
• Parmesan cheese - 0.05-3.2.
• Roquefort cheese - 2.0.
• Steppe cheese - 0.1.
• Cottage cheese (20%) - 2.7.
• Cottage cheese (40%) - 2.6.
• Fat-free cottage cheese - 3.2.

• Kernel buckwheat - 0.03.
• Oatmeal - 0.05.
• Oatmeal - 0.02.
• Winter wheat - 0.05.
• Soft spring wheat - 0.02.
• Women's milk - 6.6-7.0 (cow - 4.8, sterilized cow - 4.7, mare - 5.8, sheep - 4.8, goat - 4.5, camel - 4.9, whole dry - 37.5, dry fat-free - 49.3).
• Fatty kefir - 3.6.
• Yogurt - 4.1.
• Acidophilus - 3.8.
• Yogurt - 3.5.
• Koumiss - 5.0.
• Pasteurized buttermilk - 4.7.
• Dried cream - 26.3.
• Sterilized cream (25%) - 3.3.
• Condensed milk with sugar - 12.5.
• Condensed milk without sugar - 9.5.
• Low-fat cottage cheese - 1.8.
• Fat cottage cheese - 2.8.
• Cream (10%) - 4.0.
• Cream (20%) - 3.7.
• Sour cream (30%) - 3.1.
• Cocoa with condensed milk and sugar - 11.4.
• Coffee with condensed milk and sugar - 9.0.
• Coffee with condensed cream and sugar - 9.0.
• Hard cheeses (the indicator depends on the fat content of the product) - 2.0-2.8.
• Cheese - 2.9.
• Oil of traditional composition - 0.81 ("Peasant" - 1.35, "Sandwich" - 1.89).
• Creamy ice cream - 5.8.
• Dry milk mixture, low lactose, with rice flour - 0.36, with buckwheat flour - 0.36, with oatmeal - 0.36.

Products that almost always contain milk sugar:

• Milk and dairy products.
• Packaged sausage products, including boiled ham.
• Soups in packages.
• Ready sauces.
• Bakery products.
• Nut butter.
• Ice cream.
• Breadcrumbs.
• Cakes and pies.
• Dumplings.
• Croquettes with cheese.
• Hamburgers.
• Cheeseburgers.
• Ham.
• Ketchup.
• Mustard.
• Mayonnaise.
• Flavor enhancers.
• Astringent component for preparation of sauces.
• Sweeteners in prepared packaged foods.
• Condensed milk.
• Loose spices.
• Chocolate bars, sweets such as lollipops, chocolate (with the exception of some types of dark chocolate).
• Cocoa powder.
• Nutritional supplements.
• Light sauces.
• Puddings, soups.
• Donuts and omelets.
• Mashed potatoes.
• Saccharin tablets.

Products approved for use in lactase deficiency:

• Soy milk and soy drinks.
• Low lactose milk formulas.
• Raw meat, poultry, fish.
• Eggs.
• Lard.
• Vegetable oil.
• All fruits and vegetables.
• Potato.
• Cereals (rice, etc.).
• Legumes.
• Nuts.
• Jam, honey, syrups.
• Any type of sugar, except milk (sorbitol, fructose).
• Liquid saccharin.
• Fruit and vegetable juices.
• Tea coffee.
• Pasta made from wheat flour without additives.
• Flour is natural.
• Rye and wheat bread without whey and food additives.

Nutrition for lactose intolerance

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In primary (constitutional) lactase deficiency, the amount of lactose in the diet is reduced, up to its complete exclusion for life. You can reduce the amount of lactose in the diet by reducing or completely eliminating the consumption of lactose-containing foods, primarily whole milk (see Table 1). This method is acceptable for adults and older children with adult-type lactase deficiency.

It is important to note that neither adults nor children show a correlation between the level of lactase activity and the severity of clinical symptoms. With the same degree of enzyme deficiency, there is a large variability in symptoms (including diarrhea, flatulence, and abdominal pain). However, in each individual patient, clinical manifestations depend on the amount of lactose in the diet (dose-dependent effect).

With secondary lactase deficiency, the use of fermented milk products, butter, hard cheeses is allowed. Pasteurized sour-milk yoghurts are absorbed worse by patients, since the microbiological effect of lactase is destroyed during heat treatment. You should buy foods with live lactic acid bacteria because the bacteria in yogurt have already digested some of the lactose. There is yogurt, or acidophilus, or yogurt preferably every day.

Cheeses are divided according to the degree of ripening: the longer the cheese ripens, the less milk sugar remains in it. Consequently, hard and semi-hard cheeses (Swiss, cheddar) lose most of their lactose.

Fats and semi-fat cream contain less lactose than milk, so their use in small quantities in exceptional cases is allowed. The higher the fat content in the product, the less lactose it contains. A glass of milk contains 12 g of lactose. If necessary, milk and cheese from milk can be replaced with soy milk and soy cheese.

It is advisable to combine dairy products with, for example, grain foods, breads, pies, writes Edward Claflin in his book The Home Doctor for Children (1997).

A dairy-free diet may reduce calcium intake.

Infancy and lactase deficiency

In infancy, the problem of correcting the diet for lactase deficiency becomes more complex. If the child is breastfed, then reducing the amount of breast milk in the diet is undesirable. In this case, the best option is to use lactase preparations, which are mixed with expressed breast milk and break down lactose without affecting the rest of the properties of breast milk. If it is impossible to use lactase preparations, the issue of using low-lactose mixtures is being decided.

For artificially fed children, the mixture is selected with the maximum amount of lactose that does not cause the appearance of clinical symptoms and an increase in carbohydrates in the feces. If the child's condition is not disturbed, then you can start with a diet containing up to ⅔ carbohydrates in the form of lactose. This ratio can be achieved by combining the usual adapted formula with a low-lactose or lactose-free formula, or by prescribing a fermented milk formula. In the case of using two mixtures, it is necessary to distribute them evenly throughout the day. For example: in each feeding - 40 ml of a low-lactose formula and 80 ml of a standard one. After changing the diet, the content of carbohydrates in the stool should be checked. After 1 week, decide on the need to further reduce the amount of lactose.

With a pronounced deficiency of lactase, no effect in case of a decrease in the amount of lactose by half, it is recommended to use low-lactose mixtures as the main food product.

There are several types of low lactose products:

• milk formula with malt extract for feeding children during the first 2 months of life;
• milk formula with flour (rice, buckwheat, oatmeal) or oatmeal for feeding children from 2 to 6 months;
• low-lactose milk for feeding children over 6 months and for cooking instead of natural milk;
• you can make your own low-lactose mixture based on eggs with sugar, margarine and rice flour.

Ready-made low-lactose formula is a powder that resembles powdered milk in appearance and taste. The composition of such mixtures includes corn oil and milk fats in a ratio of 25:75, sucrose, malt extract or dextrin-maltose, starch, flour for baby and diet food, vitamins A, D, E, PP, C, group B, macro- and trace elements (iron, sodium, potassium, calcium, phosphorus, magnesium).

Lactose-free formulas are labeled "SL" (sine lactose) or "LF" (lactose free). Soy-based formulas that do not contain lactose can be used. Low-lactose formulas can be developed based on casein, soy and milk protein hydrolysates.

Table 1. A set of products that are allowed and excluded from the diet for lactase deficiency in children (A.I. Kliorin et al., 1980)

The first food for a baby with lactase deficiency may be mashed vegetables. It contains pectin, vitamins, mineral components. It is desirable to give preference to zucchini, potatoes, cauliflower, carrots, pumpkin. Only one type of vegetable should be introduced and not mixed until all vegetables have been tried one by one. Monitor the child's well-being.

It is allowed to use baby kefir. It should be given to a child with lactase deficiency on the third day after preparation. You can use cottage cheese, well cleaned of whey.

Complementary foods (cereals, vegetable purees) for children of the first year of life with lactase deficiency are prepared not with milk, but with a low- or lactose-free product. You can use baby food. Fruit juices are introduced into the diet of children later, usually in the second half of life. Fruit purees of industrial production are prescribed from 3-4 months of age. Sick children older than 1 year and adults usually tolerate fermented milk products (kefir, yogurt) well. Condensed and concentrated milk is excluded from the patient's diet.

The duration of therapy is determined by the genesis of the disease.

In primary congenital lactase deficiency, a low-lactose diet is prescribed for life. With transient lactase deficiency in premature babies, usually by 3-4 months of life (by the postconceptual age of 36-40 weeks or more), children restore the ability to tolerate lactose. Full-term infants immature for gestation need treatment for 1-2 months.

An indication for the gradual withdrawal of therapy is the reduction of stool and its compaction in consistency.

With secondary hypolactasia, the symptoms of lactase deficiency are transient. Therefore, when resolving (achieving remission) of the underlying disease after 1-3 months, the diet should be gradually expanded by introducing lactose-containing dairy products, under the control of clinical symptoms (diarrhea, flatulence) and excretion of carbohydrates with feces. If the symptoms of lactose intolerance persist, one should think about the presence of a primary (constitutional) lactase deficiency in the patient.

Sample lactose free menu

Breakfast

• Porridge with water or soy milk, with the addition of fruits or dried fruits.
• Egg or scrambled eggs (no milk) in vegetable oil.
• Whole wheat bread.
• Tea with sugar or honey.

Snack

• Coffee without milk.
• Sandwich with boiled meat and herbs.
• Apple.

Dinner

• Vegetable soup.
• Fish (boiled or fried) or poultry (boiled or baked).
• Steamed vegetables with vegetable oil.
• Juice or compote from fruits (dried fruits).

Snack

• Yogurt.

Dinner

• Rice with vegetables or vermicelli with vegetables, vegetable oil.
• Canned tuna or salmon.
• Jam tea.
• Fruit.

Metabolic disorders with a lactose-free or low-lactose diet

Exclusion from the diet of milk and dairy products leads to a decrease in calcium intake, which is so necessary during the period of growth of the child. Adults, especially women, need calcium to build strong bones and reduce the risk of osteoporosis.

Non-dairy foods that contain calcium include:

• Broccoli (100g - 90mg), okra, brauncol, spinach, collard greens, turnip greens, lettuce (50g - 10mg).
• Canned sardines (100g - 250mg), tuna (100g - 10mg), salmon (100g - 205mg).
• Oranges (1 piece - 50 mg).
• Calcium-fortified juices - orange juice (100 g - 308-344 mg).
• Calcium-fortified soy products, cereals.
• Beans (50 g - 40 mg).
• Almond.

To absorb calcium, the body needs vitamin D, which is found in soy milk, butter, margarine, egg yolks, and liver.

The main criteria for the effectiveness of treatment

1. Clinical signs: normalization of stool, reduction and disappearance of flatulence and abdominal pain.
2. In children: age-appropriate rate of weight gain, normal physical and motor development.
3. Reducing and normalizing the excretion of carbohydrates (lactose) with feces.

Appeal to patients

If you feel constant discomfort after a glass of milk, ice cream, or another dairy product, this may indicate lactose intolerance. Sometimes symptoms appear or worsen with age. If you suspect that you have lactose intolerance, consult your doctor.

Hereditary fructose intolerance occurs when there is a deficiency of the enzyme fructose-1,6-diphosphate aldodase.

With fructose-1,6-diphosphate aldolase (aldolase B) deficiency, severe symptoms in infants appear when juices containing fructose are added to the diet. Enzyme activity is reduced in the liver, kidneys and intestines. Aldolase B catalyzes the hydrolysis of fructose-1,6-diphosphate to form triose phosphate and glyceraldehyde phosphate. The same enzyme also hydrolyzes fructose-1-phosphate. Deficiency of aldolase B leads to a rapid accumulation of fructose-1-phosphate, and severe symptoms of poisoning develop when fructose is consumed.

Epidemiology and genetics

The true frequency of hereditary fructose intolerance is unknown, but may be as high as 1:23,000. The aldolase B gene is located on chromosome 9. Several of its mutations have been identified, leading to fructose intolerance. An accurate diagnosis is made by direct DNA analysis. Prenatal diagnosis is possible by obtaining amniocytes and chorionic villi (mutation analysis or linkage analysis).

Clinical manifestations

As long as fructose or sucrose does not enter the body (usually with fruits, fruit juices or sugary cereals), patients do not experience any health problems. Symptoms of fructose intolerance can occur soon after birth if complementary foods contain these sugars. Early manifestations resemble galactosemia and include jaundice, hepatomegaly, vomiting, drowsiness, irritability, and convulsions. In laboratory studies, an increase in blood clotting time, hypoalbuminuria, an increase in the activity of bilirubin and transaminases in the blood serum, and impaired function of the proximal renal tubules are found. A single intake of fructose provokes hypoglycemia with accompanying symptoms, and chronic consumption of this sugar leads to developmental delay and liver damage. Continued intake of this carbohydrate is accompanied by repeated bouts of hypoglycemia, progression of liver and kidney failure, and eventually leads to death.

Diagnosis of fructose intolerance

Suspicion of aldolase deficiency arises from the episodic appearance of a reducing substance in the urine. Confirmation of the diagnosis requires an intravenous fructose tolerance test. With an enzyme deficiency, the level of phosphate in the blood quickly decreases first, and then glucose. After that, an increase in the concentration of uric acid and magnesium in serum is observed. Fructose cannot be administered orally, as the patient may develop an acute attack. The final diagnosis is established by determining the activity of aldolase B in the liver tissue.

Treatment of fructose intolerance

It is necessary to completely eliminate foods containing sucrose, fructose and sorbitol from the diet, which is not always easy, since these sugars are widely used as excipients in most drugs. Dieting usually improves liver and kidney function and normalizes growth rate. Mental development, as a rule, does not suffer. With age, the symptoms become less pronounced, even with the consumption of fructose. The long-term prognosis is good. Due to the refusal of sucrose, caries rarely occurs in patients.