Intolerance to monosaccharides
Intolerance of monosaccharides, as a rule, is characterized by the presence of defects in the active transport of glucose in the intestinal mucosa.
Currently, both primary and acquired disorders of glucose and galactose uptake are isolated.
By primary forms we mean a genetically determined pathology with an autosomal recessive type of inheritance, which is characterized by a violation of the active transport of glucose in the renal tubules, and is subsequently accompanied by a temporary increase in the excretion of glucose in the urine( transient glucosuria).
Acquired forms of malabsorption( impaired absorption) of monosaccharides occur in celiac disease, enteritis, cystic fibrosis, and also are combined with intolerance to fructose.
Thus, the violation of intestinal absorption of carbohydrates is associated with various biochemical defects of enzymatic systems. The clinical picture has much in common and is characterized by the following common syndromes: diarrhea, sometimes with excess fecal fat, vomiting, abdominal enlargement, weakness, weight loss, decreased appetite, increased irritability, delayed physical development.
Defects in the synthesis of enzymes can lead to the fact that unsplit disaccharides are not absorbed and, when entering more distant parts of the intestine, are exposed to bacterial decomposition. Due to fermentation processes, the pH of the intestinal contents shifts to the acidic side due to the development of organic acids( lactic and acetic), which in turn leads to bowel dysfunction and promotes the spread of microorganisms to the upper sections. Despite the fact that some of the water and electrolytes are absorbed from the intestinal lumen, there is still an excessive amount of water, as a result of which the stool becomes liquid. The resulting bacterial products of vital activity, absorbed, can cause, in addition to diarrhea, toxic effects, shock and in some situations are the cause of death. Severe diseases, even with a fatal outcome, were noted in adults even after several sips of milk.
Intolerance of lactic sugars-lactose - manifested from the first days of life and is characterized by severe course as a result of dehydration and toxicosis. Long-term vomiting after each breast-feeding is noted, as well as diarrhea, which are not amenable to treatment by conventional methods.
Lactose malabsorption type Holzel is characterized by a more favorable course, usually found in older children. At the same time, it is interesting that in children at an early age, when milk is the only food, the tolerance of milk sugar is quite satisfactory. Children with this disease often refuse to write milk, soon after receiving it, they have abdominal pain, bloating( flatulence), general weakness and lethargy, vomiting and diarrhea. The presence of lactose in the urine, as a rule, is not found in patients, the excrements contain lactose, a large amount of organic acids. In rare cases, a large amount of fat appears in the stool.
Primary lactose intolerance is characterized by late appearance of clinical symptoms. This type of deficiency of the enzyme that breaks down milk sugar is called an adult. With this disease, the clinical picture is more mild, usually after 5 years of the disease, characterized by bloating, nausea, rumbling in the intestines, colic, increased gas leakage and a mushy or watery stool with an acidic odor.
Clinical symptoms of insufficiency of sucrose appear usually after the transition to mixed or artificial feeding, as well as the appointment of complementary foods, namely at the time when the child's body begins to receive sucrose, maltose and products containing starch. The disease is manifested by persistent diarrhea, less often by vomiting, episodes of dehydration( symptoms of loss of a large amount of fluid by the body) with fever to febrile figures( above 38 ° C).
Glucosogalactose malabsorption is manifested by diarrhea already in the first 2-4 days after birth. A child about 10-20 times a day excretes watery urine-like excrement with a sour smell containing large amounts of glucose and galactose, sometimes with mucus. Dehydration is rapidly developing, strong thirst is observed, the stomach is swollen. Vomiting is usually absent, often the body temperature rises. The sudden disappearance of diarrhea with the termination of milk supply, intravenous fluids and the appointment of fructose indicate the presence of this disease.
Detection of hereditary intolerance of mono- and disaccharides is based on the study of tolerance( tolerance) of loads to the corresponding mono- and disaccharides. At the same time, the concentration of sugar in the solution that must be drunk is determined without fail, and also its content in blood, urine and feces is determined. X-ray reveals an increase in fluid levels in the intestine, increased motor activity, acceleration of passage of contrast medium through the small intestine. The phenomenon of "pseudoacsita" is also revealed in the ultrasound examination of the abdominal cavity.
A hydrogen breathing test is used, based on the detection of the hydrogen content after loading with lactose. A street without lactose malabsorption, the hydrogen concentration in the exhaled air is negligible, with a deficiency of this enzyme it increases.
In the treatment of intolerance to disaccharides, the most important component of complex therapy is dietary nutrition, the main principle of which is to exclude or limit products leading to the development of pathological symptoms. An early appointment of vegetable purees is recommended. With saccharine deficiency, women's milk and mostly fermented milk mixtures without sucrose are prescribed. Use apple and carrot purees, pectins, while limiting the intake of starch from food.
In glucose-galactose malabsorption, fructose is recommended. Along with dietary therapy, vitamins, microelements, enzyme preparations in physiological doses and other therapy aimed at stopping the symptoms of the disease are prescribed. They require correction of the intestinal dysbiosis, which are detected in all children with intolerance to di-sugars. To this end, children are prescribed bifidobacteria.
With the early diagnosis of hereditary defects in the intestinal disaccharidase system and the timely administration of reasonable adequate therapy, the prognosis is generally favorable in most cases.