Hereditary fructose intolerance occurs when there is a deficiency of the enzyme fructose-1,6-biphosphate aldodase.
In case of deficiency of fructose-1,6-diphosphate aldolase (aldolase B) 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-bisphosphate to form triose phosphate and glyceraldehyde phosphate. The same enzyme also hydrolyzes fructose-1-phosphate. Aldolase B deficiency leads to rapid accumulation of fructose-1-phosphate, and severe symptoms of poisoning develop when fructose is consumed.
The true incidence 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 mutations have been identified that lead 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).
As long as fructose or sucrose is not ingested into the body (usually from fruits, fruit juices or sugary cereals), patients do not experience any health problems. Symptoms of fructose intolerance may occur soon after birth if complementary foods contain these sugars. Early manifestations resemble galactosemia and include jaundice, hepatomegaly, vomiting, somnolence, irritability, and seizures. Laboratory tests reveal prolongation of blood clotting time, hypoalbuminuria, increased activity of bilirubin and transaminases in the blood serum, and dysfunction of the proximal renal tubules. A single intake of fructose provokes hypoglycemia with accompanying symptoms, and chronic consumption of this sugar leads to developmental delays and liver damage. Continued intake of this carbohydrate is accompanied by repeated attacks of hypoglycemia, progression of liver and kidney failure, and ultimately leads to death.
Suspicion of aldolase deficiency arises from the occasional appearance of a reducing substance in the urine. Confirmation of the diagnosis requires an intravenous fructose tolerance test. When the enzyme is deficient, the level of phosphate in the blood first rapidly decreases, and then glucose. After this, an increase in concentration is observed uric acid and serum magnesium. Fructose should not be prescribed orally, as the patient may develop acute attack. Final diagnosis established by determining the activity of aldolase B in liver tissue.
It is necessary to completely exclude from the diet foods containing sucrose, fructose and sorbitol, which is not always easy, since these sugars are widely used as fillers in most medicines. Following the diet usually improves liver and kidney function and normalizes growth rate. Mental development, as a rule, does not suffer. With age, symptoms become less severe, even with fructose consumption. The long-term prognosis is good. Due to the refusal of sucrose, patients rarely develop caries.
Hereditary fructose intolerance results from the congenital absence of fructose-1-phosphate aldolase and, additionally, fructose-1,6-biphosphate aldolase. Occurring as a result of this metabolic disorders are expressed in the presence of excess 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 incidence of this disease in France is probably due to the fact that many newborns receive milk with added sucrose, which contributes to early manifestation violations. It is possible that in other countries this metabolic disease is detected later and its consequences are immediately minimized due to the appearance of an aversion to sweet foods.
Broadcast. Transmission is carried out according to an autosomal recessive type. Some heterozygous children, as well as homozygous ones, 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. It should be taken into account that in heterozygotes, fructose load tests or determination of aldolase activity in the liver will be negative.
Symptoms appear as soon as sucrose and, therefore, 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 beginning birth received a diet without fructose (group III).
In group I, children constantly vomited; anorexia was less common; None of the newborns developed an aversion to sweet foods. After eating, the appearance of pallor and shock was observed only in 2 children. Liver enlargement was found constantly, while splenomegaly was found only in 3 cases. There was often hemorrhagic syndrome, spontaneous or at the injection site. 3 children with jaundice had temporary and incomplete discoloration of stool. 8 newborns with edema and (or) ascites had a tendency to bleeding. Finally, 22 children were urgently hospitalized due to acute liver failure, infection, hemorrhagic syndrome, state of shock or dehydration.
Diagnostic studies are primarily aimed at detecting signs of a metabolic disease, since in hereditary fructose intolerance there are general symptoms with galactosemia and hereditary tyrosinemia. From this point of view, the possible consanguinity of the parents, cases of death with a similar clinical picture of members of this family in the past, the contrast between hepatomegaly and the severity of hepatic cell failure, and combination with signs of renal tubular damage become important. For less severe and shorter forms that occur under a mask chronic disease liver, histological examination of the liver should look for a combination of steatosis and non-inflammatory fibrosis.
Among metabolic diseases, vomiting is an important argument indicating hereditary fructose intolerance; its absence in a child receiving food containing sucrose represents important element against the assumption of this diagnosis. Appearance clinical symptoms in the days after weaning the baby is also 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 to confirm the diagnosis.
1. Elimination 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. A fructose load test causes a hypoglycemic reaction, which is 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 determined. Intravenous administration fructose at a dose of 0.25-0.30 g/kg for 2-3 minutes causes rapid decline blood glucose and phosphorus with a simultaneous increase in the blood levels of fructose, magnesium and lactic acid. A similar hypoglucosemic effect after intravenous administration of fructose can only be observed with hereditary fructose-1,6-biphosphatase deficiency.
3. Determination of fructose-1-phosphate aldolase activity in the liver, intestinal mucosa and renal cortex. In 52 patients, aldolase activity towards fructose-1-phosphate was much lower than that towards fructose-1,6-bisphosphate, 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 aldolase B activity in the liver, apparently due to a mutation in a structural gene.
The mechanism of liver damage seems to be quite similar to that of galactosemia and hereditary tyrosinemia: intrahepatocyte accumulation of non-metabolized 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, apparently, can explain the lack of response to hypoglycemia caused by fructose injection, which is also confirmed by the absence of a rise in glucose in response to glucagon administration after fructose administration. However, this does not explain the severity of hypoglucosemia, not accompanied by an increase in insulin secretion, after the administration of fructose.
Treatment of fructose intolerance. The main treatment method is to eliminate fructose and sucrose from the diet. The presence of pronounced signs of hepatic cellular failure may serve as an indication for emergency measures. should continue indefinitely, which is not difficult for older children, given their relative or absolute aversion to sweets. Re-introduction against this background, large amounts of fructose can cause the same disorders as in small amounts infants. Intravenous administration of solutions containing fructose or sorbitol should be strictly excluded, given the known cases deaths during such perfusion. Constant supplementation of vitamin C is also necessary, since it is found mainly in foods to be avoided, especially fruits.
The immediate prognosis after treatment is favorable. The long-term prognosis is very good, as children quickly recover their developmental delays in height and body weight. Only the enlarged liver remains for many years. Repeated histological studies of the liver showed rapid disappearance of intralobular fibrosis observed in small infants and stabilization or regression of portal fibrosis. On the contrary, steatosis remains, especially in the hepatocytes of the periportal zone, but becomes less diffuse. This does not appear to be related to poor diet; steatosis does not disappear with the addition of dextrin, maltose or glucose. Preliminary observations suggest the possibility of disappearance of steatosis in children on a diet with a loose exclusion of fructose. It is interesting to note that children on a fructose-free diet do not suffer from dental caries, so monitoring dental health may be an excellent means of monitoring treatment.
Newborns belonging to a family with a history of fructose intolerance should be placed on a fructose-free diet immediately after birth. Their tolerance to fructose should be tested subsequently, at the age of three months, by conducting a stress test, which makes it possible to decide on the indications for continuing the diet or its cancellation. Five newborns in this group, who did not receive any sweet foods, still had hepatic steatosis a few weeks after birth; This fact raises the question of whether fructose in small quantities is an essential food product.
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Fructose intolerance in adults and children is a genetically determined disease in which there is a deficiency of fructose-1-phosphate aldolase in the body. This causes excess accumulation fructose-1-phosphate in organs such as liver, kidneys, and intestines.
This type pathological conditions the body manifests itself in childhood while eating foods rich in fruit sugar. Characteristic symptoms include vomiting, nausea, hepatomegaly, and hypoglycemia. If treatment is not started promptly, it can cause death.
The body exhibits hypersensitivity to fructose and its rejection in almost identical ways. The symptomatic complex is similar in many ways. This is an itchy syndrome on the skin and mucous membranes, a characteristic rash, pain syndrome in the epigastric region, stool disturbance.
An allergenic symptom complex occurs against the background of the body’s immune response to a specific irritant. In our case, this is fructose that a person consumed. The body perceives such a substance as foreign, producing a large volume of protective antibodies - immunoglobulins, which cause allergy symptoms.
With food intolerance, this reaction immune system doesn't happen. Experts believe that increased sensitivity to carbohydrate is associated with the production of immunoglobulins type G (IgG), which are not characteristic of an allergic reaction.
For food allergies A small amount of the allergen consumed is sufficient for the corresponding symptoms to occur, whereas with food intolerance there is a delayed reaction (usually after a few days).
The appearance of signs of food intolerance is diagnosed many times more often than an allergic reaction to a particular product (in particular, to fructose).
Characteristic symptoms occur more often in persons suffering from metabolic disorders, gastrointestinal pathologies, smoking abuse or leading a sedentary lifestyle.
If fructose allergy is present, symptoms in adults may vary in severity and intensity. It depends on what the level is hereditary predisposition, the amount of carbohydrate consumed, as well as what age the person is and what his condition is digestive system.
After consuming an allergen, a person may experience the following symptoms:
An allergy sufferer can noticeably lose weight with frequent contact with the allergen, develop jaundice, and delay physical development(especially in children).
If there is carbohydrate intolerance, symptoms in adults occur even when consuming a small amount of such a substance (this includes sucrose - regular sugar). In the body, sugar is reformed into fructose, which reduces the amount of sugar in the blood.
Fructose intolerance in children and adults occurs with the following symptoms:
If a person continues to eat improperly for a long time and include fructose products in the diet, the activity of the liver and kidneys begins to be disrupted, which in the future will require other specific therapy.
For diabetics, foods containing carbohydrates do not pose a health hazard in the following cases:
If a person has type 1 diabetes or another insulin-dependent type, monitor the rate of increase in blood sugar after consuming a sweetener. For such people, insulin therapy varies depending on the characteristics of the growth rate.
If type 2 diabetes develops, it is important to follow daily norm fructose, because this makes the diet much easier. So, a sweetener is added to jam and those foods that require great content natural sugar.
The main enemy for people with diabetes is not the carbohydrate fructose, but foods rich in glucose. These are cakes and other confectionery products. In the process of preparing such dishes, glucose is replaced with fruit sugar.
Is it possible to replace sugar with fructose during a weight loss diet? This is possible and, moreover, such a replacement does not in any way affect the balance of nutrients in the body. If you decide to lose weight, it will be useful to eat the following foods:
Newborn baby having genetic type fructose intolerance, after birth should be switched to special feeding that does not contain fructose.
Diagnosis of tolerability of such a substance is carried out when the baby reaches 3-4 months. For such infants, special stress tests have been developed, the results of which determine whether it is necessary to continue following the diet or whether it needs to be discontinued.
After contacting medical center the doctor prescribes a series diagnostic measures which help identify intolerance.
First of all, information is collected about the nature of the symptoms that arise, how long ago they appeared, and the characteristics of clinical signs intolerance, previous factors. They find out whether a person has a hereditary predisposition to the pathology.
Using a biopsy, you can determine the condition of the liver and the activity of aldolase it produces, an enzyme that promotes the breakdown of fructose.
They also conduct special provocative test using fructose. To a person intravenously a certain volume of the substance is administered (calculating body weight), after which a blood sample is examined for any changes that occur. Such diagnostics are carried out in the laboratory. You can't do this at home.
If the cause of unpleasant symptoms is carbohydrate intolerance, it is necessary to change the diet, eliminating from it foods that contain such a substance. Proper nutrition helps improve the functioning of the liver and kidneys and normalize the general condition.
In case of pathology developing in children, compliance proper nutrition has no effect on mental development and growth of the organism as a whole. Over time, the severity of symptoms decreases, even if the child consumes fructose in small quantities.
A useful drug in this case is Bifiform, which will help normalize general condition and bowel activity after stool disorder.
In case of hypersensitivity to carbohydrates, allergenic products containing this substance are excluded from the diet. Even small size eating food causes characteristic symptoms. Sorbent preparations help remove allergens from the body.
You can eliminate the unpleasant manifestations of allergies with the help of antihistamines, which block the production of histamine, the substance responsible for allergic symptoms.
Fructose is almost 1.7 times sweeter than regular sugar. A combination of fructose and ascorbic acid: 4 tsp. mixtures replace the daily amount of vitamin.
The norm of fructose consumption per day is 30-40 grams. If this ratio is not observed, the possibility of developing heart and vascular diseases increases.
Prohibited foods for carbohydrate intolerance include:
It is allowed to eat foods that contain more glucose than fructose: strawberries, raspberries, cranberries, blueberries, oranges, tangerines, bananas, kiwi.
Allergy to fructose in children and adults is less common than negative reactions to oranges or full-fat milk, but once the diagnosis is confirmed, the patient has to significantly change his diet. Many fruits and vegetables contain fruit sugar in large quantities, and if you refuse them, the body loses a lot nutrients.
What to do if a child has fructose intolerance? Which products contain the highest percentage of valuable substances? How much fructose can you consume per day without harming your health? The answers are in the article.
Unlike other types of allergies, a negative reaction when consuming foods rich in fructose develops due to a lack of a special enzyme that breaks down fruit sugar. Weakness of the immune system is only an additional factor that increases the sensitization of the body.
Deficiency of fructose-1-phosphathaldolase interferes with normal digestion of food, rotting residues accumulate, and fermentation processes are activated. As a result, not only the absorption of nutrients is impaired, but fructose also accumulates in the intestines and liver, which can lead to cirrhosis and problems with activity digestive tract.
Excess of toxins provokes the activation of protective forces. Attempts to remove excess allergens by turning on the immune system lead to negative reactions on the skin, stomach, and intestines. Other signs of allergic inflammation often develop.
Risk negative reaction on fruit sugar is enhanced by the following factors:
Allergy to fructose ICD code - 10 - T78.1 (“Other manifestations of a pathological reaction to certain types of food”).
Intolerance to fruit sugar, immune reactions due to a lack of a special enzyme that breaks down fructose are complex processes, the elimination of which will require maximum attention to the preparation of the diet. It is important to start treating the disease at an early age: If the nutritional rules are followed, many children outgrow the “allergies”; with congenital fructose intolerance, they will have to adjust their diet throughout their lives.
Why does fructose cause allergies? How does the disease manifest itself and how to treat it? Find out the answers after watching the following video:
Fruits – useful and valuable elements of the diet, providing a person with almost everything essential vitamins and minerals. At the same time, they can pose a serious danger to human health. Many people suffer from fruit sugar allergies.
Allergy to fructose
Fruit sugar (fructose) is present in all fruits, berries and some vegetables. Despite the fact that it is considered healthier than regular sugar, it can provoke an allergic reaction. Therefore, if negative reactions to this element occur, the patient has to give up all fruits and most berries.
In children, allergies develop mainly as a result of improper complementary feeding. Parents must understand that every fruit or berry has its time. It is worth listening to the pediatrician’s recommendations and not deviating from the established standards.
In adults, the disease can be caused by many reasons. It is worth noting that adults experience more severe reactions than children. Perhaps this is due to greater weakness immunity and other “adult” factors. However, there are a number of specific reasons that lead to a negative reaction to fructose.
Causes of allergies
The main reason for the problem is not even a weakness of the immune system, but a lack of an enzyme. The element is designed to break down and absorb fructose. With its deficiency, undigested residues succumb to the fermentation process. As a result, the body forms increased amount toxic substances that protective forces and strive to remove it from the body as quickly as possible.
In addition, the disease can be provoked by:
In a baby, an allergy can be caused by excessive consumption of various fruits by the mother. In this case, the mother needs to reconsider her attitude to nutrition and seek help from an allergist. This will make it possible to prevent the occurrence serious problems with the health of the baby in the future.
Regardless of what caused the illness, there is a clinic that can suggest the presence of an allergy.
Symptoms of the disease
It should be noted that clinical picture at different patients may vary significantly. It all depends on many factors, including the amount of dangerous substances entering the body. Great value have a number of other factors, including age, genetics and so on.
The main signs of the pathological condition:
Important: manifestations of the disease can be life-threatening for the patient, especially if he already has any allergic disease. These signs include anaphylactic reactions, accompanied by a sharp decrease in blood pressure.
Therefore, without help medical personnel can't get by. In addition, only a doctor can determine accurate diagnosis and recommend an individual wellness program.
Treatment of fructose allergy
The patient is recommended to undergo tests that will make it possible to determine the true allergen and the main cause of the disease. After this, the allergist creates a diet. If the diagnosis is confirmed, the person should give up all fruits containing large quantities of fructose.
In addition, admission is required pharmaceuticals, helping to eliminate skin problems and reduce the level of histamine in the blood. The following groups of drugs are most often prescribed:
For both adults and children, medications are selected exclusively by a doctor, taking into account certain factors. Changing the drug or its dosage on your own is strictly prohibited, as this can lead to irreversible consequences. You can try to eliminate skin rash using alternative medicine methods.
Folk remedies
For children the best medicine For allergies, chamomile decoction is considered (provided there is no negative reaction to the plant). To obtain a decoction, take 20 grams. dry raw materials, pour 0.5 liters hot water, bring to a boil, insist. After the strained broth, give 1 teaspoon to drink 3 times a day (for very small ones). How older patient, the higher the dosage.
The same decoction is used to wipe the skin affected by rashes or redness. For healing baths, a decoction of the string is used. It is worth noting that baths with medicinal decoctions cannot be taken daily or for a long time. For example, baths with a decoction of the string are taken every other day and the duration of bathing should not be more than 15–20 minutes.
Vegetables and fruits have always been classified as healthy products and an integral part of a balanced diet. For example, the German Nutrition Society (DGE) recommends eating fruits and vegetables at least five times a day. But these recommendations are not true for everyone. The one who gets these after every apple or banana he eats. unpleasant symptoms, such as abdominal pain or bouts of diarrhea, most likely suffers from fructose intolerance.
This problem is caused by a defect in one of the proteins responsible for ensuring that fructose from food is absorbed into the body. small intestine and entered the blood. Due to impaired metabolism, there are typical symptoms: belching, diarrhea, nausea and vomiting, heaviness in the stomach. But do not despair - it is enough to reduce the amount of fructose consumed per day to the amount that the patient’s body can still absorb, and this will bring quick relief. At the same time, you should also pay attention to the sorbitol content in the food you consume, since this sugar alcohol additionally interferes with the absorption of fructose. Very often it is used as a sugar substitute in food products for patients. diabetes mellitus, dietary and “light” products, in various drinks and chewing gum.
After a doctor has diagnosed fructose intolerance, you must immediately change your diet. This is done in two stages. The first involves the complete exclusion from the menu of products containing fructose and sorbitol. The second stage comes down to the gradual inclusion of foods containing fructose in the daily diet in order to determine the individual norm of its daily amount that the patient’s body is able to absorb. Then the norm of products containing sorbitol is determined in the same way.
Different vegetables and fresh fruits contain different amounts of fructose. Vegetables containing low amounts of fructose include: eggplant, carrots, red beets, cauliflower, broccoli, chicory, peas, lettuce, asparagus, tomatoes, zucchini, radishes, celery, spinach. Fruits such as pineapple, melon, tangerines, grapefruits, papaya, lemons and bananas can also be included in the diet. It is better to avoid vegetables and fruits such as artichokes, white and red cabbage, apricots, apples, pears, kiwis, mangoes, peaches, plums, watermelons and grapes.
You need to be very careful when choosing the food products you purchase in the store. There are many designations indicating the fructose content in a particular product: fruit sugar, fruit syrup, invert sugar, corn syrup. You should carefully review the ingredients of a food product before adding it to your shopping cart. This also applies to dairy and sausage products, various drinks and confectionery - first make sure that they do not contain fructose additives, and then you can buy.
Hello! Three years ago, I suddenly stopped digesting almost all the fruits and half of the vegetables, including white cabbage, apples, bananas, beets, melon and many others. At the same time, there are no problems with products such as potatoes, carrots, tomatoes, cucumbers, arugula, strawberries, raspberries, dill, onions, etc. Among my symptoms: instant bloating even after a small bite, rumbling, flatulence, spasmodic or aching pain in the intestines, constipation (constant). Symptoms such as nausea, vomiting, and diarrhea are absent. I was examined by a gastroenterologist. General analysis blood - normal, ESR - 8, Immunoglobulin IgE - 99.14 (1.31-165.3), liver tests - normal, on occult blood- negative, coprogram - normal for all points, except: starch - large quantity, iodophilic form - moderate quantity, abundant bacterial flora, oxalate crystals +++. Ultrasound of the liver, gallbladder, and pancreas did not reveal any significant abnormalities. Hormones thyroid gland- normal. (in 2000 she underwent surgery to partial removal thyroid gland). With a height of 176 cm, I weigh 60 kg. Gastroscopy revealed erosive duodenitis, H.pylory. I completed a full course of antibiotics with antacids prescribed by my doctor. And yet...
Two years have passed - intolerance to vegetables and fruits has not gone away, and the only way to avoid bloating is to avoid these foods. In addition, frequent rashes appeared on the face and problems with hair. I don’t drink alcohol, don’t smoke, and eat exclusively “right” foods (even though I have a very limited choice of vegetables and fruits). My diet consists mainly of oatmeal, buckwheat, rice porridge, pasta, chicken, turkey, lean fish, chicken and quail eggs, potatoes, cucumber and tomato salads, some fruits, almonds, dried apricots, homemade yeast bread made from white flour. Rarely - low-fat cottage cheese, a little butter, sour cream. Dairy should be mentioned separately. Although dairy products do not cause bloating, itchy rashes on the hands are often observed after them (especially after kefir and yogurt, as well as after the drug Hilak-Forte). I don’t eat smoked meats, mayonnaise, canned food, canned vegetables, or ice cream. Very rarely - low-fat confectionery, pancakes, hard and soft cheese (although I digest them well).
I would like to know which diagnostic direction I should go in and what tests I need to take in order to understand the causes of food intolerance and the appearance of rashes on the face (about hormonal background no talk yet). If for enzymes, then which ones? Is there any point in doing a test for dysbacteriosis? I took a monthly course of lactovit on my own, but it didn’t bring any significant changes. Is it possible to cleanse the intestines using oat bran? After all, this is coarse fiber that can only do harm.
Thanks in advance for your answer. Sincerely.
Normally, disaccharides are broken down into monosaccharides by disaccharidases (in particular, lactase, maltase, isomaltase, sucrase) located on the brush border of enterocytes. Unsplit disaccharides have high osmolarity, which attracts water and electrolytes into the intestinal lumen, which is manifested by watery diarrhea. Due to the processing of carbohydrates by microflora, gases (H2, CO2, methane) are formed in the colon, which is accompanied by excessive discharge of gases, bloating and distension of the abdomen, and the appearance of abdominal pain.
Causes of carbohydrate intolerance
Disaccharidase deficiency in origin can be congenital, acquired (primary) and secondary. Congenital deficiency(in particular, sucrose-isomaltase deficiency) is rare.
Primary lactase deficiency (primary adult hypolactasia) is the most common form of carbohydrate intolerance. Lactase activity is high in newborns, which promotes milk absorption; in most ethnic groups (80% of blacks and Hispanics, > 90% of Asians) its content decreases after weaning, so adolescents and adults sometimes do not tolerate large amounts of lactose well. However, 80% of white Nordic people produce lactase throughout their lives, so they can tolerate dairy products well. It remains unclear why lactase deficiency can be considered a normal condition in >75% of the world's population.
Secondary lactase deficiency develops in conditions of mucosal damage small intestine. In young children, transient secondary disaccharidase deficiency complicates intestinal infections or surgical interventions. Resolution of the underlying disease is accompanied by an increase in enzyme activity.
Symptoms and signs of carbohydrate intolerance
The clinical manifestations of all types of disaccharidase deficiency are similar. A child suffering from lactase intolerance develops diarrhea after consuming a significant amount of milk and may experience a lack of weight gain. For adults, after consuming lactose, it is common to develop watery diarrhea, bloating, excess gas, nausea, rumbling in the abdomen, and cramping pain. Patients usually recognize the connection between the onset of symptoms and the consumption of dairy products in childhood and try to avoid them. Dairy products containing 250-375 ml (8-12 oz) of milk are usually required for symptoms to appear. Diarrhea can be quite severe and be accompanied by the removal of other nutrients that have not had time to be absorbed.
Lactose intolerance can usually be recognized through a careful analysis of the medical history and the results of a trial elimination diet. Patients typically report developing diarrhea and/or bloating after consuming dairy products; other symptoms such as rash, wheezing and other signs of anaphylaxis (especially in infants and older children) indicate an allergy to cow's milk. Milk allergy is rare in adults and may be accompanied by vomiting and signs of gastroesophageal reflux, which do not fit into the picture of disaccharidase deficiency. An acid reaction also indicates disaccharidase deficiency. feces(pH 20 ppm compared to baseline. Sensitivity and specificity of the test >95%.
The lactose tolerance test is less sensitive (about 75%), but its specificity is > 95%. The patient takes lactose. The glucose content in the serum is determined before taking lactose and after 60 and 120 minutes. With lactose intolerance, diarrhea, bloating, discomfort develops within 20-30 minutes and there is no increase in blood glucose > 20 mg/dL compared to the initial level.
Treatment of carbohydrate intolerance
Manifestations of carbohydrate malabsorption respond well to treatment by avoiding the consumption of carbohydrates whose absorption is impaired (for example, following a lactose-free diet in cases of lactase deficiency). But due to the wide variability in the degree of lactose malabsorption, many patients can tolerate up to 375 ml of milk (18 lactose) or dairy products. Usually yogurt is better tolerated because... contains a sufficient amount of lactase produced by the Lactobacilli culture. The lactose content of cheese is lower than that of milk, and cheese is often well tolerated depending on the amount consumed.
If a patient suffering from manifestations of lactase deficiency wants to consume milk, it is advisable to add lactase preparations to it for preliminary processing of lactose; Milk already treated with lactase is available on sale. Enzyme preparations act as an addition to dietary restrictions, but not as replacement therapy. Patients with lactase deficiency should take calcium supplements (1,200-1,500 mg/day).
Among them:
The body's intolerance to any substances and their failure to assimilate indicates specific deviations in its functioning. This problem limits the supply of useful and nutritious substances and leads to the appearance of various dysfunctions and diseases. Fructose intolerance is the most common.
Lack of fructose absorption makes normal balanced diet. Healthy and vitamin-rich vegetables and fruits become poison for the body, which in small quantities causes damage to the digestive system.
Fortunately, this problem is quite easy to overcome. It is necessary to organize your diet in such a way that the amount of fructose consumed does not exceed the permissible dose. However, how do you know if you have fructose intolerance and how can this affect your future health?
Fructose intolerance is a special type congenital disease. The disease is associated with a defect in one of the active enzymes, responsible for converting fructose into other sugars.
Complete or partial inability to absorb fructose, also known as fructosemia or fructose malabsorption, manifests itself in childhood. Unfortunately, this disease It is not yet treatable, but only partially corrected.
When intolerance occurs, the body begins to experience a shortage of fructose-1-phosphaldolase. Thus, due to deficiency, fructose begins to be deposited in tissues internal organs- liver, kidneys, intestines.
Any consumption of foods that contain fructose or sucrose provokes attacks, as the body becomes oversaturated. A person begins to suffer from serious attacks, which manifest themselves in nausea, vomiting, and muscle cramps.
Fructose intolerance is hereditary disease, which is transmitted from parents to children.
If it is not absorbed, there is a defect in the gene responsible for the production of enzymes - fructose phosphate aldolase and fructose diphosphatase. These organic compounds are responsible for processing fructose and converting it into glucose.
In 25% of cases, children inherit this gene defect. In this case, the liver completely loses its ability to produce these enzymes. Thus, the ability to convert fructose into glycogen is also lost.
The body's metabolism begins to disturb, which leads to excessive fat formation. In the future, it is possible that other disorders will appear in the body, which will affect a person’s mental activity, as well as his well-being.
Although fructose intolerance begins to manifest itself quite actively at an early age, it may take time to identify it.
Very often, parents confuse the symptoms of intolerance with a number of diseases, due to which they miss valuable time. Thus, when the first warning symptoms appear and do not go away within a couple of days, an examination and consultation with a doctor is necessary.
The use is not even large quantity fructose leads to the following symptoms:
With further consumption of foods containing fructose, a person may fall into a coma. In the future, it is also possible to develop pathologies of the kidneys and liver.
There are two main categories of forms for fructosemia:
Ignoring severe symptoms and lack of treatment threatens a person with serious complications. In some cases, not only human health, but also human life is at risk.
Determining whether a person has fructose intolerance involves several diagnostic methods, including:
It is also possible to assign the patient consultations with other specialized specialists, including a nutritionist or medical geneticist.
Separately, physical diagnostics are distinguished. This method is based on the search for signs of illness that affect appearance patient.
So, in the case of children who are suspected of fructose intolerance, the following external signs are noted:
However, children with this disease show other, positive signs, such as excellent skin condition and perfect teeth.
Treatment of the condition involves excluding from the patient’s diet those foods that contain even minimal doses of fructose. Among them:
When fructose stops entering the patient's body, the patient begins to feel significant relief. Vomiting stops and appetite is restored, the patient gains significant weight.
Diet therapy is prerequisite speedy recovery. Thus, for newborn babies, a number of special milk formulas are prescribed, which contain only lactose and dextrin-maltose.
Fruits are replaced with glucose in the amount of 30-60 g. In complementary foods, foods such as eggs, cheese, meat and fish are prescribed. This dietary food must be strictly observed until 5-6 years.
Only from 6-7 years old can a child be gradually introduced to prohibited foods. The only rule is to do this with the consent of the doctor and under his periodic supervision.
Adults can eat the following products:
Patients also need to use medications with extreme caution. Some of them may contain sugar.
The consequences of fructose intolerance can be very dangerous. Frequent complications are the following states:
Some of these complications can lead to disability and sometimes death of the patient. It is especially worth noting that when consuming fructose, a patient with fructose intolerance almost always develops cirrhosis of the liver.
Prevention involves studying family history for timely detection illness in one of the relatives. This is the only thing that can be done for the patient. Others preventive measures does not exist, since the development of the disease is determined by genetics.
An allergy to fructose is associated with many problems for a person. This diagnosis forces the patient to almost completely reconsider his diet - he has to give up most fruits and vegetables, which contain fruit sugar. And, of course, healthy vitamins and minerals have to be replenished through other foods.
Such a diagnosis will be especially upsetting for patients with diabetes mellitus - their menu already has a lot of restrictions, and fructose is very important for them. important product, replacing regular sugar.
Although allergies are a common disease in modern world, she is still picky in her choice of “victims.”
Her favorite “objects”:
If a product enters the human body that the immune system considers dangerous, it will immediately begin to build a defense against it. A substance called histamine, present in all cells and not active in normal conditions, will be put on alert and will begin to enter the blood in large quantities.
Peculiarity! Weak immunity is an additional factor that provokes an allergic reaction.
As a result of “internal discord,” a person will develop symptoms characteristic of allergic reactions: the skin, respiratory organs, mucous membranes of the nose and eyes may be damaged.
Since intolerance to this product is rarely diagnosed, many people do not know whether it exists at all. Doctors say: yes, it is possible, only the mechanism of its development is somewhat different from the traditional scheme.
To start it, the body must be deficient in a special enzyme (fructose-1-phosphate aldolase), responsible for the breakdown fruit sugar.
Enzyme deficiency leads to the fact that food is not digested normally, rotting residues accumulate, and fermentation processes begin. To work on removal from the body toxic substances the immune system gets involved, and that’s when a person gets the full set of symptoms that accompany an allergy.
The disease occurs in both children and adults. In infants, it can manifest itself after the first feeding. Doctors explain it this way: if the baby has not been given regular sugar before (for example, in a drink), an allergic reaction to fructose can occur, like a rejection of any sweet product containing sugar or its analogues.
Factors that provoke a reaction in children may be:
Manifestations of an allergic reaction in children:
Pay attention! Young patients with this diagnosis may experience rapid weight loss. They may experience jaundice and developmental delays.
Symptoms of fructose allergy in adults:
If the patient has all listed symptoms immediately, there is practically no doubt about the accuracy of the diagnosis.
Any type of food allergy (including fructose) should be treated by specialists. If the patient's condition is serious, it is necessary to call ambulance. After the acute stage has been overcome, regular long-term treatment will be required to help alleviate the patient’s condition and prevent another outbreak of the disease.
Diagnostics must be carried out to be sure which product has become an allergen for the patient.
The following are used in treatment:
A course of treatment with calcium gluconate helps improve metabolism, strengthen the immune system and, thereby, protect the patient from possible relapses of the disease.
The patient will need to change his diet by eliminating foods containing fructose from the menu. Glucose or lactose can be used as a sweetener.
If the patient is a child, parents need to be especially careful, monitoring the composition of milk formulas and various vitamins (fructose may be in their shell).
If allergy treatment is carried out promptly and comprehensively, the body will be able to further protect itself on its own - it will neutralize fructose that accidentally gets into it, preventing an allergic reaction.
Important! Usage folk remedies, especially for children, you need to check with your doctor.
Here are a few folk ways allergy treatment:
In terms of prevention, for those who are already sick or at risk, doctors recommend:
Fructose is found in many foods, the names of which are unlikely to be constantly remembered in your head. Allergists recommend having a table at home with a complete list of them.
If there is no absolute ban on fructose, but there is only a predisposition to an allergy to this substance, a table indicating the amount of fructose in a particular product will help to make it easier to choose the safest one.
We must not forget that fructose is found not only in fresh fruits. Juice, sauce, and filling of pies may contain fruit sugar that is dangerous for allergy sufferers. It is especially difficult to begin to suspect products that, as experts say, contain “hidden fructose.” These include, for example, hard cheese.
It is important for an allergy sufferer to take care of the digestive organs, since they are the first to take the hit if you are allergic to fructose. Periodic visits to the doctor, laboratory tests will help avoid exacerbation of symptoms.
Allergy to fructose, which not so long ago many did not even suspect, today affects even infants - there are cases where children are born with this disease. The disease requires adherence to a diet that excludes foods with fructose from the menu, as well as regular medical monitoring for preventive purposes.
Fructose intolerance is uncommon, but if diagnosed, significant dietary changes must be made. There is a lot of this substance in the most necessary products, the refusal of which leads to a deficiency of components important for the body. Therefore, you need to contact a specialist to create the right diet.
Intolerance and allergies
If the body reacts negatively to any foods or drinks as a result of digestive problems, then a food intolerance is diagnosed. Many people consider this condition an allergy, but these are two different concepts.
An allergic reaction causes rashes, swelling, and disturbances in the digestive system and other organs. Food intolerance accompanied by fullness and weakness, urolithiasis, fatigue. Sometimes it has no symptoms and only causes you to feel unwell. Therefore, it is difficult to determine the problem.
Causes of dangerous conditions
Fructose intolerance in adults and children has its own reasons. Typically, pathology develops when there is not enough enzyme that breaks down fruit sugar.
The situation is aggravated by a weak or immature immune system. If there is not enough fructose-1-phosphate aldolase, then food is not completely digested, its remains accumulate and begin to rot. At the same time useful substances are not absorbed, fructose accumulates in the intestines and liver.
This can cause cirrhosis and other dangerous diseases.
Due to the excessive content of toxic substances, protective forces are activated. The immune system tries to neutralize allergens, which is accompanied by various reactions from organs and systems. The likelihood of an allergic reaction under the influence of fruit sugar increases:
Signs
Fructose allergy has different symptoms in adults. The severity of reactions depends on age, the amount of fruit sugar in the body, and the state of the digestive system.
If high level fructose is associated with overeating, then:
An allergy to fructose in an infant occurs if the child was born with intolerance to this substance. If the baby does not have enough enzyme to break down fructose, then he cannot eat fruits and the body suffers from a lack of vitamins. This has a bad effect on the entire body.
In this situation, it is difficult for a child to gain weight, he becomes capricious due to intestinal colic, and sleeps poorly. Allergic reactions can also occur in children:
Many babies experience fructose intolerance when introducing complementary foods. Most parents think that this substance is found only in fruits, sweets, and honey.
This is a mistake, since there are many useful components in berries, vegetables and legumes. It is even found in potatoes and cucumbers.
How to replace fructose
An allergy to fructose causes difficulties in creating a diet, since many products are prohibited. To replace fructose, doctors prescribe medications and foods that contain glucose and lactose.
If an allergy develops, you need to give different foods in small quantities, observing the condition of the skin and digestive system.
How to eat when sick
If fruit sugar continues to enter the body despite an allergic reaction, it will lead to various problems in the body. Therefore on initial stages For the development of the disease, doctors recommend completely eliminating all items that contain fructose.
After the manifestations of hypersensitivity are stopped, you need to gradually introduce foods into the diet, controlling the body’s reaction. The patient should eat this way for several weeks. After this, the doctor determines which products are most suitable for the patient’s body and composes safe diet. It usually includes foods with minimal glucose content. The patient is allowed to eat some zucchini, eggplant, spinach, potatoes, and cauliflower. Apples, pears, honey, and chocolate bars are prohibited.
It is also very important to know that this component is included in some medications. It is added to the shell of tablets and medicinal syrups.
When purchasing products, the patient should study the composition of the products and check whether they contain fruit sugar.
Treatment options available
Symptoms of fructose intolerance in adults require a thorough examination. After confirming the diagnosis, treatment is prescribed.
To avoid disorders in the digestive system, if there is not enough enzyme that breaks down fructose, diet alone will not work. Eliminating the irritant from your diet is very important, but it is not the only thing that needs to be done.
In this situation, antihistamines are needed. Thanks to their influence, the production of serotonin, histamine and other components that provoke inflammatory processes allergic type.
To get rid of the symptoms of intoxication of the body, it is necessary to use sorbents. Also prescribed local remedies to eliminate allergic rashes.
Usually, the disease is treated with syrups, tablets, drops. If the symptoms of the disease are pronounced, the face, eyelids, and ulcers swell, the use of Suprastin, Diazolin, and Diphenhydramine is required.
Moderate and mild allergic reactions are treated with antihistamines new generation. They are allowed to be taken for a long time.
Expert opinion
Allergist Dmitriev Mikhail Lvovich
Education: First Moscow State University medical university named after I.M. Sechenov, 1997
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Reducing the likelihood of relapses is achieved with the help of calcium gluconate. Thanks to this, it is possible to normalize the condition of blood vessels, strengthen the functioning of the immune system, and improve metabolic processes in the body. Therefore, the drug is always prescribed
Preventive measures
Allergy to fructose in children and adults can subside and recur. To avoid repeated exacerbations, you must follow all the recommendations of specialists:
Parents who are suffering allergic reactions, wondering whether the baby may be allergic to fructose or not. The likelihood of a problem with a hereditary predisposition is 25% higher than for all others.
If a child has an increased sensitivity to fructose, he is recommended to follow the diet until the age of six.
A poor reaction of the body to fruit sugar, the development of immune reactions when there is insufficient amount of enzyme in the body to break down fructose are complex processes. Therefore, the patient should pay a lot of attention to his own diet.
Treatment of the disease must continue with early age. If you follow all the rules, then most patients manage to outgrow the disease, but this only works in the case of acquired intolerance.
If a child was born with the disease, he will have to monitor the amount of fructose in foods throughout his life. But the right approach will help avoid feeling unwell and all allergy symptoms.
