intolerance of monosaccharides

August 12, 2017 17:51 | Genetic Diseases

intolerance monosaccharides are generally characterized by the presence of defects in the active transport of glucose intestinal mucosa.

Now allocate both primary and acquired disorders of the absorption of glucose and galactose.

Under primary forms understand genetically determined pathology with an autosomal recessive mode of inheritance, which is characterized by impaired glucose active transport in the renal tubules, and later accompanied by a temporary increase in the excretion of glucose in the urine (glycosuria transient).

Acquired forms of malabsorption (malabsorption) monosaccharides are found in the syndrome of celiac disease, enteritis, cystic fibrosis, as well as combined with fructose intolerance.

Thus, violation of intestinal absorption of carbohydrates associated with various biochemical defects of enzymatic systems.The clinical picture is very similar and is characterized by the following common syndromes: diarrhea, sometimes with the release of excess fat feces, vomiting, increase in

the abdomen, weakness, weight loss, decreased appetite, increased irritability, delayed physical development.Defects

enzyme synthesis can lead to the fact that not absorbed disaccharides uncleaved and entering the more distal portions of the intestine are subjected to bacterial decomposition.Because fermentation processes gut pH shifts to the acidic side due to the development of organic acids (lactic and acetic), which in turn leads to intestinal dysfunction and contribute to the spread of microorganisms in the upper sections.Despite the fact that some of the water and electrolyte is absorbed from the intestinal lumen, yet it remains an excessive amount of water, whereby the chair becomes liquid.The resulting bacterial waste products, sucking can cause, in addition to diarrhea, toxic effects, shock and in some cases are the cause of deaths.Severe disease, even fatal, have been reported in adults even after a few sips of milk.

molochnogosahara intolerance, lactose - evident from the first days of life and is characterized by severe as a result of dehydration and toxicity.There have been prolonged vomiting after each feeding with breast milk, as well as diarrhea, which can not be treated by conventional methods.

Lactose malabsorption Holzel type is characterized by a favorable course, usually occurs in older children.In this case interesting is the fact that in children at an early age, when milk is the only food product, milk sugar tolerance is quite satisfactory.Children with this disease often refuse to write breast soon after receiving them appear abdominal pain, bloating (flatulence), general weakness and lethargy, vomiting and diarrhea.The presence of lactose in the urine, usually in patients not detected feces contain lactose, a large amount of organic acids.In rare cases, there is the appearance in the feces of large amounts of fat.

Primary lactose intolerance is characterized by the late appearance of clinical symptoms.This type of deficiency of the enzyme that breaks down milk sugar, is called the adult.In this disease, the clinical picture is expressed in a mild, usually appears after 5 years of the disease, is characterized by abdominal distention, nausea, rumbling in the intestine, colic, increased discharge of gases and mushy or watery stools with a sour smell.

Clinical symptoms of sucrase insufficiency usually appear after the transition to a mixed or artificial feeding, as well as the appointment of foods, namely at a time when the baby begins to flow sucrose, maltose and foods containing starch.The disease is manifested incessant diarrhea, rarely vomiting, dehydration episodes (symptoms of loss of a large amount of body fluids), with an increase in body temperature to febrile digits (above 38 ° C).

Glyukozogalaktoznaya malabsorption diarrhea manifested in the first 2-4 days after birth.Child about 10 to 20 times per day allocates watery feces with urine resembling sour odor, containing large amounts of glucose and galactose, sometimes with mucus.Rapidly developing dehydration, there is thirst, stomach swells.Vomiting is usually absent, often the body temperature rises.Abrupt disappearance of diarrhea at the termination of milk feeding, intravenous fluids and assigning fructose indicate the presence of the disease.

Identifying hereditary intolerance of mono- and di-saccharides is based on a study of tolerance (tolerance) loads the corresponding mono- and disaccharides.Thus necessarily determine the concentration of sugar in the solution to be investigated drink, as well as determine its concentration in the blood, urine and feces.X-ray revealed increasing levels of the liquid in the intestines, increased motor activity, accelerating the passage of the contrast agent through the small intestine.The phenomenon of "psevdoastsita" also revealed by ultrasound of the abdominal cavity.

Use hydrogen breath test is based on detecting the hydrogen content after lactose load.Streets without lactose malabsorption hydrogen concentration in the breath is small, with a deficiency of this enzyme is increased.

the treatment of disaccharide intolerance essential component of a comprehensive treatment is diet, the basic principle of which consists in the elimination or restriction of products, leading to the development of pathological symptoms.Recommended early use of vegetable puree.When saharaznoy failure appoint human milk and predominantly dairy mixture without sucrose.Use apple and carrot puree, pectin, while limiting intake of starch with food.

When glucose-galactose malabsorption recommended the appointment of fructose.Along with diet prescribed vitamins, minerals, enzyme preparations in physiological doses and other therapy aimed at relief of symptoms of the disease.Require correction of intestinal dysbiosis, detected in all children with intolerance di-sugars.To this end, the children are assigned bifidobacteria.

With early diagnosis of hereditary defects in the intestinal system disaccharidases and timely appointment of adequate therapy reasonable prognosis in most cases, is generally favorable.