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Absorption disturbance syndromes - Diseases of digestive organs at children

Table of contents
Diseases of digestive organs at children
Oral cavity
Diseases of teeth
Malformations of the sky and soft tissues of an oral cavity
Diseases of a mucous membrane of an oral cavity and gums
Diseases of lips and language
Sialadens
Digestive tract
Basic reasons of gastrointestinal frustration
Gullet
Atresia and esophageal and tracheal fistula
Guttural and tracheal and esophageal crevice, inborn stenosis of a gullet
Other diseases of a gullet
Stomach and intestines
Peptic ulcer
Inborn hypertrophic pyloric stenosis
Inborn impassability of intestines
Inborn impassability of a duodenum
Disturbances of turn of intestines
Inborn impassability of a small bowel
Inborn megacolon
Diverticulums and duplikatura
The acquired impassability of intestines
Intestines invagination
Foreign bodys of a stomach and intestines
Motive frustration. stomach and intestines
Anomalies of a structure of anorectal area
Infectious diseases of intestines
Nonspecific ulcer colitis
Illness Krone
Necrotic coloenteritis of newborns
The coloenteritis connected with treatment by antibiotics
Gastrointestinal symptoms of anaphylactoid purpura, gemolitiko-uraemic syndrome
Intolerance of food proteins
Eosinophilic gastroenteritis
Absorption disturbance syndromes
Malabsorption
Immunodeficiency and intestines
Syndrome of "a congestive loop"
Syndrome of a short small bowel
Gee's disease
Sprue after acute enteritis
Tropical to a spr
Whipple's illness, intestines lymphangiectasia, Uolmap's illness, idiopathic diffusion defeat of mucous
Enzymopathies and disturbances of mechanisms of transport of nutrients
Irritable colon
Acute appendicitis
Diseases of an anus, direct and large intestine
Tumors of a digestive tract at children
Hernias of a digestive tract at children
Exocrine part of a pancreas
Pancreatitis
Embryonic development of structure and function of a liver
Diagnosis of diseases of a liver
Cholestatic states at babies
Parenchymatous jaundices at children of chest age
Disturbances of a metabolism of a liver and zhelchevydelitelny system
Anomalies of a structure of bilious ways
Cysts of bilious channels
Cholestasia and diseases of a liver connected with completely parenteral food
Medicinal damage of a liver
Ray's syndrome
Chronic hepatitis
Wilson's illness — Konovalova
Indian juvenile cirrhosis
Damages of a liver at chronic colitis
Cirrhosis and chronic liver failure
Portal hypertensia and varicosity of a gullet
Fatty infiltration of a liver
Cholecystitis
Peritoneum diseases
Peritonitis
Peritoneum hernias

Активная глютеновая болезнь
Fig. 12-18. An active gluten illness at the boy of 18-month age. The hanging-down skin folds, an atrophy of proximal muscles, the increased stomach are visible.

ABSORPTION DISTURBANCE SYNDROMES

The terms "syndrome of the broken absorption", or "sprue", cover a wide range of states at which digestion of various nutrients is broken. Disturbance of digestion and absorption of many components of food is shown at once by the increase in a stomach decoloured by the smelling stake, a muscular atrophy liquid badly, especially proximal groups of muscles, a growth inhibition and increases of the body weight (fig. 12-18). Usually these states describe under the name "Gee's disease syndrome" or "sprue". Many chronic diseases, in addition to gastrointestinal at which there are an insufficiency of food and a growth inhibition are known. For example, at children with chronic diseases of kidneys or intracranial defeats the clinical symptomatology can be similar to that which is observed at absorption disturbances. Many years ago some specific frustration of digestive organs were included in a Gee's disease syndrome. One of them, a gluten enteropathy, call a Gee's disease, or with p ru. Basic reasons of generalized disturbances of transformation and absorption of food are summed up in tab. 12-10 where syndromes are separately provided, often and seldom meeting in North America and Europe.

Table 12-10. The reasons of disturbances of nutritive absorption at children


The struck body

Frequent reasons

Rare reasons

Exocrine part under * gastric gland

Mucoviscidosis
Chronic belkovoenergetichesky insufficiency

Shvakhman's syndrome — Daymonda Chronic pancreatitis

Liver, biliary path

Atresia of bilious ways

Other cholestatic states

Intestines Anatomic defects
Persistent infection (immunodeficiency)

Extensive resection Syndrome of a congestive loop
Lambliasis
Coccidiosis

Inborn short gut

Diseases of the mixed etiology

Gee's disease
Malabsorption after enteritis

Intolerance of food proteins (milk, soy)
Tropical to a spr Whipple's Illness Idiopathic diffusion damage of mucous membranes

Such inborn diseases at which only some one specific mechanism of digestion of nutrients is broken are known. In these cases the clinical picture often differs from manifestations of generalized disturbances of absorption. At one diseases intestinal symptoms, in particular diarrhea whereas others are shown only by insufficiency of food are observed and are not followed by gastrointestinal symptoms. Diseases at which specific mechanisms of absorption are broken are listed in tab. 12-11. All of them meet seldom, except for insufficiency of disaccharidases, and only some of them are shown by abdominal syndromes.
Table 12-11. Specific disturbances of digestive and vsasyvatelny function at children


Illness

Intestines \Fats \Proteins \

Abetalipoproteinemiya Defitsit of enterokinase
Disturbances of transport of amino acids (cystinuria, Hartnup's illness, methionine malabsorption, syndrome of "blue diapers")

Carbohydrates

Insufficiency of disaccharidases (inborn: deficit of invertase, isomaltase, lactase; age tranzitorny: deficit of lactase; acquired: generalized malabsorption of glucose and galactose)

Vitamins

B12 vitamin malabsorption (juvenile pernicious anemia, deficit of transcobalamine II, Immerslund's syndrome)
Malabsorption of folic acid

Ions, microelements

Diarrhea with chlorine loss the Enteropatichesky acrodermatitis the Syndrome Menkesa Vitamin - In - dependent rickets Primary hypomagnesiemia

Drugs

Salazosulfapyridinum (malabsorption of folic acid)
Holestiramin (malabsorption of fats and Sa)
Dilantinum (Sa)

Pancreas
gland

Selective enzymatic insufficiency (lipase, trypsinogen)

Inspection of patients at suspicion on malabsorption

Accuracy of the differential diagnosis between a true syndrome of the broken absorption, chronic nonspecific diarrhea and the abenteric reasons of a growth inhibition and development depends first of all on the correct interpretation of clinical data. Descriptions of the diseases proceeding with malabsorption are provided in appropriate sections; in this section special attention is paid for a role of the anamnesis and physical inspection in diagnosis of the specified pathology.
Clinical manifestations. As such symptoms as a growth inhibition or stretching of a stomach, are not specific, extremely important carefully and fully to collect the anamnesis. The special attention is deserved by instructions on an illness of the central nervous system and uric ways. Many diseases of a digestive tract are genetically determined therefore the family anamnesis can be essential help directed by the diagnosis. An important role is played by time of emergence of the first symptoms as their early emergence testifies in favor of an inborn disease. It is very important to establish connection between emergence of a symptom and consumption of food for the reason that selective disturbances of transport of nutrients through an intestinal wall or development of malabsorption under the influence of the irritants which are in food are possible; for example, the products containing gluten provoke a Gee's disease.     
Some parties of the anamnesis which are usually stated excessively in details have no essential value. Description calla, as a rule, very subjectively; the volume of fecal masses is of a certain interest, but data on a smell and consistence often do not give useful information. In the same way data on communication between food and diarrhea have to be logical, otherwise they do not make sense. If Lactose is the reason of ponos, then the food containing one lactose has to provoke ponosa as well as the food containing except lactose, and other carbohydrates.
It is the best of all to estimate impact of an illness on the general condition of the child on dynamics of growth and body weight, considering at the same time if perhaps, constitutional features. Such signs of disturbance of absorption as a hypomyotonia, hypostases, the milkwoman, the "varnished" language and excessive fragility of vessels, it is necessary to interpret taking into account the nutrients given about consumption. At diarrhea parents and the doctor often apply strictly limited diet for a long time, aggravating with that insufficiency of food which can be mistakenly regarded as result of malabsorption.
At suspicion on malabsorption an important diagnostic method is rectal research. In addition to an assessment of a condition of an anus and a rectum, it allows to take a piece a calla for the macroscopic, microscopic and chemical analysis at once. If the patient receives full-fledged food, then at insufficiency of a pancreas in Calais, triglycerides in a large number, and also undigested muscle fibers are found; at a sprue accumulations of crystals of a monoglyceride and fatty acids are visible.
Datas of laboratory. Absorption of fat is determined by a ratio between amount of the fat which arrived with food, and its losses with a stake. If the child receives no more than 20 g of fat a day, then in Calais, completely collected during 4 days of loss should not exceed 15% of the consumed quantity at children of early age and 10% — at children of advanced age. Instead of a labor-consuming, long and unpleasant method of the analysis a calla the screening methods allowing to estimate absorption and to find a steatorrhea are offered. The simplest of them — determination of concentration of carotene in the serum taken on an empty stomach. At normal intake of carotene with food contents it in blood of healthy faces makes 1 g/l, and at disturbances of absorption of fats — less than 0,5 g/l. However this screening method yields a lot of false positive and false-negative results. Experienced laboratory workers prefer to reveal a steatorrhea by means of direct mikrokopichesky definition of particles of fat in fecal masses.
Absorption of carbohydrates cannot be estimated on their consumption and loss as sugar collapse colibacilli. usually Calais is found only by sugar traces; the exception makes kcal of the children who are on breastfeeding. Excess of sugar in fresh Calais demonstrates intolerance of carbohydrates, but lack of sugar does not exclude it. The reducing carbohydrates in Calais can be defined by the special tableted reactants (klinptest, the USA); this test is used at mass inspections. On disturbance of absorption specifies detection in Calais more than 0,5% of the eaten sugar (at enough the reducing sugars in food). The majority of food sugars, except for sucrose, belongs to reducing therefore if necessary to define sucrose it it is necessary to subject previously to hydrolysis, heating kcal together with hydrochloric acid. Usually at intolerance of pH carbohydrates the calla is displaced in the acid party (less than 6,0) that is caused by education by colibacilli of organic acids from sugars. An indirect method of an assessment of absorption of carbohydrates — determination of t of an olerantnost to loading sugar. On an empty stomach the patient is given inside sugar which absorption needs to be estimated at the rate of 0,5 g/kg of body weight, and then define concentration of glucose in plasma in 15, 30, 60 and 120 min. Glucose level normal has to increase at least on 0,2 g/l at loading lactose or sucrose and on 0,5 g/l — at loading glucose. It should be noted that the result is influenced by many accessory factors, except digestion and absorption, in particular evacuation speed from a stomach and intensity of utilization of glucose. One more indicator — the content of hydrogen in expired air — is also determined after loading by sugar (2 g/kg of body weight, at most 50 g). If the studied sugar is not absorbed in proximal part of a small bowel, then in its distal part and in a large intestine it decays colibacilli with formation of hydrogen. The last is quickly soaked up and allocated with lungs. Increase of content of hydrogen in expired air more than on 10 h/one million within the first two hours indicates sugar absorption disturbance. At the patients receiving antibiotics and at 2% of healthy faces the microflora producing hydrogen is absent.
Absorption of proteins cannot be quantitatively estimated in customary clinical practice. Definition of absorption on a difference between consumption and loss does not reflect process of their assimilation as there are endogenous sources of fecal protein; however indicative data can be obtained by means of definition of nitrogen in Calais. Losses of protein through intestines can be calculated, entering intravenously 51sgs1 and determining its content in Calais during 4 days. If more than 0,8% of the entered dose come to light, then it indicates excessive loss of protein. Protein loss is also estimated on fecal clearance of antitrypsin; though experience of use of this method is limited, it was succeeded to establish that the clearance exceeding 15 ml/days (during 48 h), demonstrates the increased protein loss.

Content of other nutrients in blood can be determined against their adequate receipt with food therefore decrease in their concentration reflects absorption disturbance in plasma. Iron which amount in blood depends both on absorption, and on transferrin level in blood belongs to such substances. Content of folic acid in erythrocytes more precisely (than the content in serum) characterizes a condition of food. It is also necessary to call serumal calcium and magnesium vitamin D and its metabolites, vitamins A and B12. At the broken absorption sometimes there pass several years before B12 vitamin reserves are spent.
Some tests allow to define in what department of intestines absorption is broken. Iron and d-xylose (the pentose which almost is not metabolized in a human body) are soaked up in proximal department of a small bowel. If in 1 h after administration of this carbohydrate orally in a dose of 14,5 g/m2 of a body surface (to 25 g) concentration in blood is lower than 0,25 g/l of it, then it is possible to assume damage of a mucous membrane of proximal department of a small bowel; it should be noted that this method gives both false positive, and false-negative results. In distal departments of a gut B12 vitamin is absorbed and bile acids reabsorbirutsya. Absorption of B12 vitamin is estimated by a direct method of Shilling. After saturation of reserves of vitamin B an organism give a dose of radioactive vitamin (separately or in combination with an internal factor) and determine vitamin C excretion by urine during the subsequent 24 h. Excretion less than 5% of the accepted dose speaks about absorption disturbance; it occurs at damage of a mucous membrane of distal department of an ileal gut on a big extent or after its considerable resection.
Malabsorption diagnostic methods. Microbiological. The only eurysynusic infectious disease causing chronic malabsorption — a lambliasis. Trofozoita and their cyst it is possible to find in a mucous membrane of a duodenum or its contents. If protective mechanisms of a small bowel are weakened (owing to stagnation of contents or disturbances of immunity), then the intestinal juice taken from proximal department of a small bowel contains a huge number of bacteria.
Hematologic. At research of usual blood smears it is possible to find an iron deficiency anemia. Existence of megaloblasts in a smear indicates deficit and, therefore, malabsorption of folic acid and B12 vitamin. At an abetalipoproteinemiya it is possible to observe transformation of erythrocytes in acanthocytes. Decrease in maintenance of lymphocytes or a neutropenia are characteristic of Shvakhman's syndrome.

Met of the ode of obtaining image. These methods use first of all for detection of local defeats of digestive organs. At children with a sprue survey and contrast X-ray inspection allows to define the place and the reason of an intestinal staz. For example, the wrong turn of an intestinal tube — the most frequent anomaly of intestines causing incomplete impassability — comes to light only by means of the barium enema allowing to define the provision of a caecum. For research of a small bowel baric weight should be entered in a large number. Signs of widespread malabsorption, in particular Gee's diseases, consider inhomogeneity of distribution of baric weight, expansion of a gut and a thickening of folds of a mucous membrane, however they are not specific and have no great diagnostic value.
Scanning with use of radioactive technetium ("The hardware) allows to find precisely a malrelated mucous membrane of gastric type in a duplikatura or Mekkel's diverticulum, but these states seldom are followed by absorption disturbances.
Ultrasonic scanning is capable to reveal changes of tissue of pancreas, anomaly of development and concrements of bilious ways even at the newborns suffering from absorption disturbances.
Administration of contrast medium in biliary and pancreatic channels by means of the endoscope (a retrograde nankreatokholangiografiya) is necessary in those exceptional and difficult cases when separate studying of the specified channels is required.
Biopsy of a small bowel. The peroral aspiration biopsy of a mucous membrane of a small bowel became an important diagnostic method at inspection of children with a syndrome of the broken absorption. A necessary condition of diagnosis of a Gee's disease is detection of typical diffusion changes of a mucous membrane; characteristic changes come to light at an abetalipoproteinemiya. The microscopic structure of a mucous membrane is changed at a lambliasis, a lymphangiectasis, deficit of gamma-globulins, viral enteritis, tropical to a spr, intolerance of proteins of milk and soy, and also at idiopathic diffusion damage of a mucous membrane.
In bioptata it is possible to define activity of disaccharidases. Diffusion damage of a mucous membrane is followed by decrease of the activity of these enzymes. For more exact diagnosis of a Gee's disease of a bioptata of a small bowel in the form of an organ culture treat to action of in vitro gluten.
The diseases causing system disturbances of digestion or absorption, a mucoviscidosis, Shvakhman's syndrome — Daymonda (a pancreas hypoplasia with a neutropenia) see in appropriate sections.

{ mospagebreak title= the Digestive tract at chronic insufficiency
food }

12:46. THE DIGESTIVE TRACT AT CHRONIC INSUFFICIENCY OF FOOD
The exocrine part of a pancreas is much more sensitive to proteinaceous and power insufficiency, than intestines; at chronic malnutrition secretion of digestive enzymes is suppressed rather early (see section 12.78). In the developed countries where malnutrition meets seldom, chronic diseases of digestive organs and an effect of their treatment are the serious reasons of insufficiency of food; at the same time, undoubtedly, function of a pancreas suffers. This phenomenon is especially dangerous to children as reserves of nutrients at them are rather small. Hroyichesky malnutrition only through a long time leads to emergence of clinical signs of disturbance of digestion as the last arises if more than 90% of exocrine tissue of pancreas are damaged. Nevertheless at heavy chronic malnutrition vneshnesekretorny insufficiency of a pancreas is most often caused by it, but not primary disease of gland.
Intestines are very steady against proteinaceous and power insufficiency. At a kwasiorkor the considerable flattening of fibers of a small bowel is noted, but it most likely speaks accompanying infectious or a parasitic disease. At a nutritional dystrophy the structure of fibers is kept, but the submicroscopy reveals changes in cells and microvillis. Chronic malnutrition can lead to immunity easing (section 12.78) and to change of structure of intestinal microflora.
The data obtained recently in experiments on animals have a certain value for clinic. If oral administration of food is completely stopped, then lump mucous a cover and its vsasyvatelny function decrease even at preservation of balance of nutrients by means of their intravenous administration. These changes disappear at oral administration even of a small amount of food. Thus, advantage of administration of nutrients in the natural way, but not intravenously is proved.
Besides, at chronic malnutrition regeneration of an intestinal epithelium therefore recovery after acute intestinal diseases (for example, viral enteritis) in such cases drags on can be slowed down.
Data on changes in intestines and pancreas at deficit of separate nutrients are quite poor; it is known that the lack of potassium can become the reason of paralytic impassability, and heavy dehydration causes a lock. Available facts demonstrate that the lack of other components of food is shown by insignificant clinical symptoms. Low content of iron in food leads to the increased absorption it a mucous membrane; in some heavy
cases the mucous membrane is flattened. Insufficient consumption of B12 vitamin and folic acid leads to change of a form of erythrocytes, but it is not followed by serious functional frustration. Some gipokaltsiyemichesky states can be followed by a steatorrhea, even with loss of iron and water but this insufficiently studied dependence is shown changeably.



 
"Diseases of bodies of an urinary system at children   Diseases of the lacrimal bodies"