GEE'S DISEASE (coeliakia; Greek koilia-kos intestinal, suffering from disorder of intestines; synonym: S. Gee's disease — Gertera — Geybnera, intestinal infantility, tseliakichesky to a spr, an idiopathic steatorrhea) — the disease of digestive tract which is characterized by disturbance of processes of absorption in intestines as a result of intolerance of protein of a gluten of cereals (gliadine) and leading to exhaustion.
The disease is for the first time described in 1888 Mr. of S. Gee. Frequency of a disease makes 1:6000, is most widespread in areas where cereals (wheat, rye, oats, etc.) are the main food (Western Europe, Australia, North America).
Distinguish primary (inborn) and secondary it is whole and and to a cue. which can arise at any age.
Etiology and pathogeny. Earlier major importance in development of a disease was attached to toxic action on intestines of gliadine (see) and, in particular, one of its fractions — and - gliadine. At the same time assumed that own layer of a mucous membrane of intestines is exposed to initial damage, and changes in cells of an epithelium appear later. However the theory of toxic impact of gliadine on an organism completely did not explain development of C., and the mechanism of toxic action remained not clear. In a crust, time of C. consider as an enzymopathy, at a cut insufficiency of cellular enzymes (specific aminopeptidases) providing process of deamination of gliadine in intestines that leads to accumulation in an organism of its toxic metabolites is noted. The disease has hereditary character. As confirmation to it serve cases of development of a disease in unioval twins, and also identification of antibodies to gliadine at the immediate family of patients, at to-rykh a wedge, symptoms of a disease are absent. Genetically caused susceptibility to a disease was confirmed in 1972 with researches of R. L. Stokes and his colleagues in England, and irrespective of them Folchek (Z. M of Falchuk) and W. Stro-ber in the USA, to-rye found out that antigen of fabric histocompatability HLA-B8 occurs at 80 — 90% of the teenagers sick with a Gee's disease. In 1974 Mac-Nill (L. To. McNeill) with sotr. noted that the persons having HLA-B8 antigen are ten times more inclined to a disease of C. in comparison with persons, at to-rykh this antigen is absent.
152 GEE'S DISEASE
Development of C. connect also with immune disturbances. So, in blood serum and a secret of a small bowel of patients find antibodies of the class IgM to gliadine of wheat and to other proteins which are contained in food; at early stages of a disease the maintenance of IgA increases, the dysgammaglobulinemia is noted. In the material received at a biopsy of a mucous membrane of a small bowel plasmocytes and T lymphocytes come to light. Thus, at C. genetically caused disturbance of an immune response of an organism on introduction to food of gliadine is observed. Confirmation of this assumption is emergence secondary C., developing as a result of manifestation of the latent defect at influence of adverse factors (long persistent intestinal infection, parasitic diseases, malformations went. - kish. a path, a state after an enterectomy, use of nek-ry medicines, etc.).
According to a number of the researchers who found deposits of a complement in own layer of a mucous membrane of intestines, reaction antigen — an antibody is the cornerstone of a disease (see. And ntigen — an antibody reaction), and the process developing at the same time is similar to Artyus's phenomenon (see Artyus a phenomenon) that is confirmed by local reaction of binding complement (see). However against the allergic nature of a disease negative skin tests (see), and also lack of parallelism between extent of immune disturbances and disease severity testify.
Pathological anatomy. Changes of a mucous membrane of intestines at C. are similar to changes at enteritis of other etiology (see Enteritis), but are considerably more expressed. At a Gee's disease diffusion injury of a mucous membrane of a small bowel, shortening and deformation of fibers, sharp expansion of crypts is noted. At gistol. a research the atrophy of a ferruterous epithelium, disturbance or disappearance of a border of epithelial cells with a border comes to light; at their electronic microscopic examination shortening and reduction of quantity of microvillis, emergence in cells of the vacuoles containing lipidopodobny substance is defined. In enterocytes swelling of mitochondrions, fragmentation of an endoplasmic reticulum, and further disturbance of integrity of a cellular membrane with an exit of organellas from cells is noted. In process of improvement of a condition of the patient against the background of an agliadinovy diet there is gradual (within
3 — 24 months) a recovery of a mucous membrane of intestines. At premature inclusion in food of the products containing gliadine, changes in a mucous membrane of a small bowel appear again.
Clinical picture. Primary Gee's disease is usually shown at the age of 6 months — 142 years. More prematurity of a disease is promoted by artificial feeding. The disease is characterized by persistent ponosa after consumption of the products containing gliadine (bread, pasta, semolina and gerkulesovy porridges, cookies and other flour confectionery, pastes, sausages, sausage, meat soups, meat sauces). In process of progressing of a disease the disturbance of absorption which is followed by loss of appetite, lag in the weight, growth and other symptoms (see Maljab-sorbtsii a syndrome), with the subsequent frustration of a metabolism, exhaustion and dehydration of an organism develops. Children are sluggish, adynamic, irritable, badly adapt to a surrounding situation, frustration of a dream is noted. Intestines are blown up. At a palpation of a stomach capotement and fluctuation (pseudo-ascites) is defined. Kal is plentiful, foamy, whitish, with a greasy luster. Weight the calla reaches per day 1 — 1,5 kg and more (at norm of 80 — 100 g). In a koprogramma prevail fat to - you and washed. At C. the phenomena of a secondary exudative enteropathy — an exit and loss of proteins of plasma through an intestinal wall, a hypoproteinemia and development of an edematous syndrome are observed (see Hypostasis). Together with proteins of plasma the child loses a significant amount fat to - t that is followed by disturbance of a lipometabolism, malabsorption of fats and fat-soluble vitamins. Absorption of vitamin D is more often broken owing to what at the child rakhitopodobny changes of a skeleton, osteoporosis, caries of teeth develop; spontaneous fractures of bones of the lower extremities are observed. In blood decrease in level of calcium and phosphorus, reduction of activity of an alkaline phosphatase is noted. At C. there is a disturbance of carbohydrate metabolism what low level of glucose in blood on an empty stomach, and also a flat sugar curve testifies during the carrying out a glucose tolerance test to (see a diabetes mellitus). Owing to insufficient absorption of iron at patients hypochromia anemia develops. Vneshnesekretorny function of a pancreas at C. suffers a little and unlike a mucoviscidosis decrease of the activity of its enzymes is observed only in the period of an aggravation.
C. proceeds wavy, the periods of an aggravation alternate with the periods of nek-ry improvement of a state.
The diagnosis is made on the basis by a wedge, pictures, data of radiological and biochemical researches. One of characteristic diagnostic characters is development of symptoms of a disease after consumption of the products containing gliadine. At rentgenol. a research uneven expansion of loops of a small bowel, alternation of sites of a spasm with sites of an atony is noted; during the carrying out a research multiple horizontal fluid levels, change or total absence of a relief, uneven, more often accelerated, passing of a baric suspension on an empty stomach are determined by a small bowel.
At a biochemical blood analysis extremely low level of the general lipids is defined, it is not etherified - nykh unsaturated polyester to - t, cholesterol, in Calais a large number free fat to - t, in intestinal contents availability of the proteins of plasma revealed by means of an immunoelectrophoresis (see) and immunoprecipitations (see Precipitation) filtrates a calla in an agar with a normal human antiserum.
For identification of C. apply also test with loading gliadine. For this purpose a small amount of flour is mixed with a large amount of water, then excess of water is deleted, and to the test add alcohol. Spirit extract contains gliadine, and in draft there is an insoluble glutenin. Extract is evaporated, and the gliadine received thus is given to the child inside at the rate of 300 — 350 mg/kg, it is better with sugar in mix with milk or kefir. In 3 — 5 hours determine the content of gliadine in plasma by a chromatography kro-
vi. At healthy children the content of gliadine after loading increases no more than by 40% of initial level, at C. — more than for 100%.
For diagnosis of a disease and assessment of vsasyvatelny function of a small bowel, and also apply test with loading to assessment of efficiency of treatment by D-xylose. D - the Xy-rod (see Xylose) is well dissolved in water, actively soaked up in a small bowel, to 40% it the most part — during the 5th is removed hour with urine in not changed look. By amount of the removed D-xylose it is possible to judge its absorption in a small bowel and weight of process. The largest content of D-xylose in urine is noted in 60 min. after loading. At healthy children this size makes 30 — 35% of the entered quantity, at C. — on average 20%. During the conducting test to children of early age D-xylose appoint in a dose 0,3 g to 1 kg of the weight (weight) of a body. At the body weight of 17 kg and above loading D-xylose makes 5 g. Definition of D-xylose in urine is carried out by Rowe and Rice's method (J. N of Roe, E. W. Rice, 1948). Method transformation of D-xylose during the processing is the cornerstone of it ice acetic to - that in the furfural giving about parabromine-aniline in the presence of thiourea red coloring, intensity to-rogo it is proportional to the content of D-xylose in urine.
Differential diagnosis carry out with a mucoviscidosis (see), primary exudative enteropathy (see the Enteropathy exudative), intolerance of disaccharides (see Maljab-sorbtsii a syndrome), with a disease of Girsh-sprunga (see Megacolon), tuberculosis mezenterialny limf, nodes (see Tuberculosis out of pulmonary). It is based on data of a multilateral research and includes definition of sodium and chlorine in stalemate liquid and nail plates, and also degrees of manifestation of a steatorrhea (for an exception of a mucoviscidosis), a koprologichesky research and identification of serum proteins and a calla (for the purpose of an exception of an exudative enteropathy), various loading tests, with the help to-rykh reveal intolerance of disaccharides, a X-ray and histologic inspection of intestines (at differentiation with a disease of Girshsprunga), tuberkulinovy diagnosis (for an exception of tuberculosis), etc. Improvement of a condition of the child at purpose of an agliadinovy diet is of great importance for differential diagnosis.
Treatment. In the main C in treatment. purpose of a diet with an exception of a diet of the products containing gliadine (see above) is. Rice, corn, soy, potatoes, carrots and other vegetables, milk, cheese, fresh meat, fish, eggs, vegetable and butter, fruit, sugar, jam, honey do not contain gliadine. The diet appointed to the child shall be high-calorific. Content of proteins should be increased by 20% in comparison with age norm. Due to the insufficiency of a lipase and disaccharidases developing for the second time, it is necessary to replace fats of animal origin with not refined seed fats, milk — fermented milk products.
Appoint fermental drugs (panzinorm, abomin, Pancreatinum, Polyzimum, festal, pankurmen, etc.) courses on 3 — 4 weeks; at an intestinal dysbiosis — biological drugs (bacteriophage). Depending on biochemical indicators carry out correction of metabolic disturbances — pour a blood plasma, administer the proteinaceous drugs (albumine, Neocompensanum, gamma-globulin, polyglobulin). Appoint vitamins A, B, C, D, iron preparations. Attempts of use of corticosteroids did not yield visible results.
For children, sick C., dispensary observation is established: at primary C. before the period of puberty, at secondary — within not less than five years after detection of a disease. At the same time watch the weight and growth of the child, conduct a research a calla (koprologichesky), blood (the general, clinical and biochemical), urine, intestinal microflora. Expansion of a diet (inclusion of the products containing gliadine) is carried out during the entire period of growth, but not earlier than in
1V2 — 2 from an initiation of treatment and after conducting test with D-xylose. The issue of preventive inoculations is resolved strictly individually, however inoculations can be begun not earlier than through 1 — lV2 of year after permanent remission.
The forecast at timely treatment favorable.
Prevention is not developed.
See also Gluten disease.
Bibliography: And l and m about in and M. M. and P y - to about in a JI. A. The test with d-/-f/-xylose in diagnosis of a Gee's disease at children, Laborat. business, No. 8, page 487, 1976; Badalyan L. O.,
T and about l and V. A. N to an iveltishchevyu.a. Hereditary diseases at children, page 244, M., 1971; Diseases of digestive organs at children, under the editorship of A. V. Mazurin, page 486, M., 1984; M and with l about in M. S. Lectures on faculty pediatrics, p. 2, page 90, L., 1960; X-ray inspection of digestive organs at children, under the editorship of T. A. Osipkova, page 92, L., 1974; The Guide to gastrointestinal diseases, under the editorship of N. I. Nisevich, page 115, M., 1969; Titova L. S., etc. The research of the soaking-up function of intestines by means of D-xylose at children early a cart -
a rasta is normal also at chronic frustration of food, Pediatrics, No. 10, page 27, 1974; Filipgtkin M. A., M e N of the h and nanosecond to and I am L. V. ifridmane. S. K to radiological diagnosis of a syndrome of a Gee's disease at children, Vestn. rentgenol. and radio-gramophones., No. 6, page 64, 1971; G e e S. J. On the coeliac affection, St. Barth. Hosp. Rep., v. 24, p. 17, 1888; Hamilton J. R. a. M with N e i! I L. K. Childhood celiac disease, response of treated patients to a small uniform daily dose of wheat gluten, J. Pedicit., y. 81, p. 885, 1972; J e a n R. e. a. Aspects actuels de la maladie coeliaque de l’enfant, Rev. Pediat., t. 7, p. 563, 1971; J o s J. e. a. Etude histochimique de la muqueuse duodeno-jejunale dans la maladie coeliaque, Pediat. Res., t. 1, p. 27, 1967; Nelson texbook of pediatrics, ed. by R. E. Behram a. V. C. Vanghan, Philadelphia, 1983; P about 1 a k J. M. a. o. Secretin cells in coeliac disease, Gut, v. 14, p.870, 1973; SavilahtiE. Intestinal immu-nolobulins in children with coeliac disease, ibid., v. 13, p. 958, 1972; Weigel W. Die Duodenalbiopsie ais Methode der Diagnostik und der Verlaufskontrolle bei Kindern mit Coliakie, Mschr. Kinderheilk., Bd 117, S. 481, 1969. V. A. Tabolin.