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The article presents a clinical case of a 23-year-old patient with an extremely severe congenital form of chronic intestinal pseudoobstruction coupled with a neuromyopathy,colon malrotation, malabsorption, bacterial overgrowth syndrome, cholelithiasis and gastrostasis, which excluded bowel transplantation. Long-term treatment in the intensive care unit with combined, mainly parenteral nutrition for 6 months, using antibiotics, prokinetics, intestinal decompression allowed to achieve partial stabilization of the patients condition and transfer to home treatment with the continuation of adequate complex therapy.Представлено клиническое наблюдение больной 23 лет с крайне тяжелой врожденной формой хронической интестинальной псевдообструкции с нейромиопатией. мальротацией толстой кишки, мальабсорбцией, синдромом избыточного бактериального роста, холелитиазом, гастростазом, исключавшим выполнение трансплантации кишечника. Длительное лечение в отделении интенсивной терапии с комбинированным, преимущественно парентеральным питанием в течение 6 мес, применением антибиотиков, прокинетиков, кишечной декомпрессии позволило добиться частичной стабилизации состояния и перевода пациентки на домашнее лечение с продолжением адекватной комплексной терапии.

Aim. To determine the frequency of celiac disease (CD) among gastroenterological patients and criteria for its active detection. Materials and methods. 1.358 patients referred for gastroenterologist consultation from 2016 to 2019 was conducted, of which 140 had CD (339 males 24.9%; 1019 females 75.1%). The average age was 40.415.4 (1886 years). All patients were determined anti-TTG IgA, IgG, and analyzed the clinical symptoms and analysis. The results were subjected to statistical processing Statistica 13.3 (StatSoft Inc., USA). Results. In patients without CD (1218 people), high level of anti-TTG IgA and IgG was observed in 59 (4.8%), an increase in anti-TTG IgA in 54 (4.4%), and anti-TTG IgG in 38 patients (3.1%). The CD diagnosis confirmed in 51 patients (4.2%). The main symptoms were diarrhea (88%), abdominal pain (60.7%), bloating (73.8%), nausea (40.3%), weight loss (44.3%). Anemia was determined in 31.6%, serum iron 33%, hypoproteinemia 12.6%, hypoalbuminemia 12%, hypokalemia 5.48%, hypocalcemia 21.9%. An increase in the level of AST 14.5%, ALT 14.6%. Comparative analysis showed that in the group with newly detected CD, anemia, malabsorption syndrome, increase AST, ALT were significantly more frequent than in patients with normal antibodies, which confirms the need to detect CD among patients with these laboratory abnormalities. Conclusion. The incidence of CD among patients with a gastroenterological symptoms was 4.2%. Analysis of clinical and laboratory data has shown that a comprehensive analysis of clinical symptoms and laboratory indicators at the stage of primary treatment will allow timely identification of CD patients and prescribe GFD.

Chronic intestinal pseudo-obstruction a rare violation of the motor skills of the gastrointestinal complex, similar to mechanical obstruction, but without a mechanical obstacle. The development of chronic intestinal pseudo-obstruction is caused by a disturbance on the part of the smooth muscles and the nervous system of the gastrointestinal system. Common symptoms include constipation, abdominal pain, nausea, vomiting, bloating. Violation of peristalsis leads to food stagnation in the hinges of the small intestine, their dilation, the development of bacterial insemination syndrome. Eating disorders, bacterial contamination syndrome (CDDs) lead to impaired suction syndrome, cahexia. Treatment is aimed at providing adequate nutrition, the use of drugs that activate motor skills, suppress the growth of microbes in the small intestine, the implementation of intestinal decompression. Surgical treatment: resection of the affected segment of the gut. In the refractory course of the disease intestinal transplantation.

Hypogammaglobulinemic sprue (HGGS) belongs to orphan (rare) diseases. Its basis is a concurrence of primary immunodeficiency and celiac disease. The clinical picture of HGGS is characterized by recurrent bacterial infection foci, chronic diarrhea, and severe malabsorption syndrome (MAS). Immunological changes are exhibited by a decrease in the serum levels of γ-globulins (GG), immunoglobulins (Ig) A and G, and, to a lesser extent, IgM and B lymphocytes. The peculiarity of the morphological pattern of HGGS is the atrophy of the gastric mucosa and small bowel mucosal villi, which is accompanied by lymphocytic infiltration, formation of lymphoid follicles in the proper mucosal plate, and a drastic drop in plasma cells. It is precisely these signs that serve as a basis for identifying HGGS as an independent nosological entity. Its management is to be on a life-long gluten-free diet (GFD), to administer GG or Ig monthly, to correct metabolic disturbances, and to treat focal infections. The paper describes a case history of a female patient with HGGS. The disease was characterized by recurrent lung and kidney inflammatory foci concurrent with chronic diarrhea and grade 3 MAS. There was a dramatic reduction in GG, IgG, and IgA levels. Clinical remission and small intestinal mucosal structural recovery occurred due to GFD.Аннотация. Гипогаммаглобулинемическая спру (ГГГС) относится к орфанным (редким) заболеваниям. В ее основе лежит сочетание врожденного иммунодефицита с целиакией. Клиническая картина ГГГС характеризуется рецидивирующими очагами бактериальной инфекции, хронической диареей, тяжелым синдромом нарушенного всасывания (СНВ). Изменения иммунологического статуса проявляются снижением в сыворотке крови уровня γ-глобулинов (ГГ), иммуноглобулинов (ИГ) А и G , в меньшей степени - ИГ М и В-лимфоцитов. Особенностью морфологической картины ГГГС является атрофия слизистой оболочки (СО) желудка и ворсинок СО тонкой кишки, сочетающаяся с лимфоцитарной инфильтрацией и образованием лимфоидных фолликулов в собственной пластинке, резким уменьшением плазматических клеток. Именно эти признаки служат основанием для выделения ГГГС в самостоятельную нозологическую форму. Лечение ГГГС заключается в пожизненном соблюдении аглютеновой диеты (АГД), ежемесячном введении препаратов ГГ или ИГ, коррекции метаболических нарушений и лечении очаговых инфекций. Приведена история болезни больной ГГГС. Течение заболевания характеризовалось рецидивирующими воспалительными очагами в легких, почках, сочетавшихся с хронической диареей и СНВ III степени тяжести. Отмечалось резкое снижение ГГ и ИГ G и А. Под влиянием АГД наступила клиническая ремиссия и частичное восстановление структуры слизистой оболочки тонкой кишки.

A clinical observation of a patient with celiac disease, aged 23, with severe form of osteoporosis, accompanied by atraumatic fractures bones, lameness, late onset of menstruation and pathology of pregnancy is described. It is emphasized that only a timely diagnosis celiac disease and timely prescribed treatment make it possible to avoid severe complications.

The article presents the results of examination of 32 patients with common variable immune deficiency (barn) with involvement in the pathological process of the digestive system. The features of the clinical picture, the content of immunoglobulins in the blood serum, morphological structure of the mucosa of small intestine as well as treatment. Special attention is paid to the small intestine in the pathogenesis of the barn.

Protein-losing enteropathy (PLE) is a rare complication of intestinal diseases. Its main manifestation is hypoproteinemic edema. The diagnosis of PLE is based on the verification of protein loss into the intestinal lumen, by determining fecal α1-antitrypsin concentration and clearance. The localization of the affected colonic segment is clarified using radiologic and endoscopic techniques. The mainstay of treatment for PLE is a fat-free diet enriched with medium-chain triglycerides. Surgical resection of the affected segment of the colon may be the treatment of choice for severe hypoproteinemia resistant to drug therapy.Энтеропатия с потерей белка (ЭПБ) — редкое осложнение болезней кишечника. Основным ее проявлением служат гипопротеинемические отеки. Диагностика ЭПБ основана на верификации потери белка в просвет кишки посредством определения в кале α1-антитрипсина, его клиренса. Локализацию пораженного сегмента кишки уточняют с помощью лучевых и эндоскопических методов. Основу лечения ЭПБ составляет безжировая диета, обогащенная среднецепочечными триглицеридами. При тяжелой гипопротеинемии, резистентной к медикаментозной терапии, методом выбора может быть хирургическое лечение — резекция сегмента пораженной кишки.

The paper describes a rare case of celiac disease in the absence of serum anti-tissue transglutaminase (anti-tTG) antibodies. A 51-year-old patient has been suffering from diarrheas for 20 years. He has lost 15 kg gradually; weakness progressed; muscle cramps, leg edemas, and signs of dynamic pseudoobstruction appeared. Morphological examination revealed small intestinal mucosal (SIM) villous atrophy (Marsh IIIC stage). IgA anti-tTG and IgG anti-tTG antibodies were 0.086 and 0.178, respectively. The patient was prescribed a gluten-free diet, water electrolyte solutions to correct metabolic disturbances, and prednisolone. During a control examination after 6 months, the patient had no complaints and gained 22 kg, and the SIM villus height was increased. The specific feature of the case is specific negative serological tests for celiac disease.Представлен редкий случай целиакии в отсутствие антител к тканевой трансглутаминазе (АтТГ) в сыворотке крови. Больной 51 года в течение 20 лет страдал поносами. Постепенно масса тела снизилась на 15 кг, нарастала слабость, появились судороги мышц, отеки голеней и признаки динамической псевдообструкции. При морфологическом исследовании выявлена атрофия ворсинок слизистой оболочки тонкой кишки (СОТК), стадия Marsh IIIC. АтТГ IgA 0,086, АтТГ IgG 0,178. Больному назначены аглютеновая диета, водно-электролитные растворы для коррекции метаболических нарушений и преднизолон. При контрольном обследовании через 6 мес жалоб не предъявлял, масса тела увеличилась на 22 кг, увеличилась высота ворсинок СОТК. Особенностью случая является отрицательные серологические тесты, специфичные для целиакии.

The article presents a clinical case of a 33-year-old Armenian man, who suffered from two rare diseases: Familial Mediterranean fever and celiac. The diagnosis of Familial Mediterranean fever: abdominal-feverish form, is confirmed by genetic markers. The morphological study of duodenal mucosa's specimens confirms the celiac.

Chronic diarrhea syndrome results from excess colonic bile acids. Fibroblast growth factor 19 (FGF19), produced in the ileum is response to bile acid absorption, regulates hepatic bile acids synthesis. Bile acid malabsorption can be found in various gastrointestinal conditions: the greatest losses bile acids with excrements were marked at patients with surgical resection of terminal ileum. The maintenance in daily colonic excess bile at patients with postholecystectomy exceed more than in three times the indicators noted at control subjects, but at the patients with irritable bowel sindrom did not exceed the norm. Effective remedies in treatment chologenic diarrhea were bile acid sequestran.

Violation of reproductive function in patients with celiac disease can manifest as delayed puberty, infertility, amenorrhea, premature menopause, spontaneous abortion, low birth weight. The aim of the study was to establish the frequency and nature of reproductive function violation in patients with CD in the Russian Federation.The study included 132 women (average age 38,5 +/- 1,17 years) with CD observed in CSRIG in the period from 2000 to 2010. Comparison group consisted 105 women (average age 38,7 +/- 1,6 years) with predominantly functional bowel disorders (irritable bowel syndrome, functional constipation, functional bloating, inert colon). Were took into account information regarding obstetric history, physical and laboratory signs of malabsorption syndrome (MS) study of antibodies to alpha-gliadin immunoglobulin (IG) A class (AGA) and tissue transglutaminase (AtTG).The average age of onset of menses was 14,3 +/- 1,4 years, and in the control group - 13,0 +/- 1,3 years (p0.05), half of patients with C (43.9%) had menstruation begun at age 15 years and older, while 7.6% of the women--aged 17 and older. In the comparison group menses beginning after 15 years was only at 13.3% of women. In 61.3% of patients with CD was irregular menstrual cycle while in the comparison group such violations were noted in 13.3% (p0.001). Prolonged periods of amenorrhea we observed in women with newly diagnosed GC 3 times more likely than the comparison group: 43.9% and 11.4% respectively (p0.01). They also had nearly 3 times more likely to occur spontaneous miscarriage: at 46.9% and 14.3% respectively (p0.01). The frequency of dead children birth was about the same: 2.3% and 1.9% respectively (p0.05). The frequency of reproductive disorders increased with the growth of the severity of MS. In 43% of women after 6-8 months of strict adherence to a gluten-free diet (GFD) had disappeared amenorrhea and there were regular menses. Three women of childbearing age, strictly abided the GFD and had a history of repeated spontaneous abortion during the year managed to get pregnant and give birth to healthy full-term baby.Reproductive disorders in women with celiac disease are significantly more likely than at women with functional bowel disease. One of the reasons of reproductive disorders in patients with CD can be malabsorption of necessary nutrients in the small intestine. The presence of reproductive disorders should be considered as a risk factor for celiac disease, so these women should be screened for celiac disease.

The aim--to study fatty acid composition of lipids in blood serum of patients with celiac disease, their role in metabolic processes and methods of therapy.The study included 28 patients with celiac disease with malabsorption syndrome III degree: 20 women and 8 men aged 17 to 45 years. Was determined: steatorrhea with help of chemical quantitative method of Van de Kamer, fatty acids in blood serum lipids by thin layer and gas chromatography.There was a substantial steatorrhea in patients with celiac disease with malabsorption syndrome III in severity. Most patients with celiac disease were found to decrease the essential fatty acids, the most significant--arachidonic acid, which leads to disruption of the synthesis of prostaglandins, prostacyclin, a tendency to thrombosis. Parenteral administration of the drug essentiale helps normalize the fatty acid composition of serum.

We described a patient 40 years old, admitted to the clinic with periodic attacks of pain in the right upper quadrant. With ultrasound it was confirmed chronic acalculous cholecystitis, and at endoscopy and multiple biopsies revealed atrophy of the mucosa of the duodenum (DM), corresponding to celiac disease (stage III in the Marsh classification). Titer of antibodies to gliadin (AGA) and tissue transglutaminase (AtTG) were higher: 60 and 110 units/ml, respectively, at a rate of 10 units/ml. The patient was assigned a lifetime adherence to a gluten-free diet, serologic test and a control endoscopy with biopsy at 6 months. The important role of the doctor-endoscopist in the diagnosis of latent forms of celiac disease. The significance of DM atrophy in the pathogenesis of patients with chronic cholecystitis.

In this article presented results of examination and treatment of 207 patients with glutensensitive celiac disease. And also you can find comprehensive method for the treatment of patients with varying severity of disease. The fundamental method of therapy glutensensitive celiac disease is strict lifelong adherence to a gluten-free diet (GFD), which eliminated protein cereal--gluten contained in wheat, rye and barley. Recently, there is large number of products which include the "hidden gluten". Its elimination from the diet of GFD is mandatory for patients with celiac disease. In the presence of diarrhea and malabsorption syndrome are used adsorbents, astringents, enzymes, intestinal antiseptic, probiotics. With the purpose of correction of metabolic disorders intravenous electrolyte mixture containing potassium, calcium and magnesium. To eliminate protein deficiency drugs used solid protein mixture of pure amino acids, gluten-free mixes for enteral feeding. Clinical examination of patients with celiac disease is aimed at monitoring compliance with GFD, early detection of cancer, autoimmune and other related diseases.

To study condition of the stomach, gall-bladder, pancreas, liver in celiac disease (CD) and contribution of their dysfunction to clinical presentation of CD symptoms.A total of 215 CD patients entered the study (191 females and 24 males) aged under 20 years (n=100), 20 to 50 (n=74) and over 50 years (n=41). The control group consisted of 25 healthy volunteers. Acid-forming function of the stomach, blood serum gastrin level were studied. Bile for biochemical test was obtained at duodenal intubation using 40 ml of 40% glucose solution or 25% magnesium solution as food stimulators, and intravenous injection of cholecystokininpancreosimin. Cholic acid was assayed in bile portions B and C. Two-channel probe was used to obtain duodenal content before meal and after intravenous injection of pancreosimin for tripsin, amylase and lipase assay. Clinical and biochemical blood tests were made as well as puncture biopsy of the liver with histological study of biopsy material.CD patients were found to have high basal and stimulated acid-forming function, high gastrin concentration in the blood. The morphological examination detected lymphocytic gastritis. There were an inert type of pancreatic enzyme secretion, gall-bladder hypokinesia or atony. Gall-bladder contracted only after intravenous injection of cholecystokinin. Changes in the liver were characterized by hypertransaminasemia, steatohepatitis.Changes in the stomach in patients with new-onset CD promote formation of ulcer. Decline in excretory pancreatic function, slow enzyme secretion, marked hypokinesia of the gall-bladder, hyperenzymemia and steatohepatitis as manifestations of hepatic pathology result in dramatic disorder of digestion and absorption of food substances.

To study the mechanisms of chronic diarrhea in patients with celiac disease and the syndrome of short small intestine.The study included 24 patients: 9 with celiac disease with malabsorption syndrome of II and III degree, 15 patients with the syndrome of short small intestine and 9 healthy volunteers. Study of microflora in the small and large intestine was performed by seeding serial dilutions of the contents of upper small intestine and feces, followed by qualitative and quantitative view of selected microorganisms. Value of free and conjugated bile acids were determined by electrophoresis followed by densitometry.2/3 of the examined patients revealed bacterial colonization of the small intestine. Were inoculated staphylococci, E. coli, enterococci. Was found a significant change in the ratio of bile acids: a significant increase in free, damaging the mucous, and a decrease in conjugated involved in the digestion of lipids. Unabsorbed lipids were one of the causes of diarrhea in the examined groups of patients. Sequential antibiotic drugs and probiotic promoted the normalization of the stool, microflora of the small and large intestine.

Gluten-sensitive enteropathy (Celiac Disease)--is an autoimmune inflammatory disease, with hyper Regenerative atrophy of the mucous membrane of the small intestine, that is precipitated by the ingestion of gluten, a component of wheat rye, barley, protein. Exclusion of dietary gluten results in healing of the mucosa of the small intestine. The main method of treatment and prevention of complications of celiac disease is strict lifelong adherence to gluten-free diet (AGD), which reduces the quality of life of patients. The labeling of all foods and greater variety of gluten-free products, reducing their cost, psychological support doctors and nutritionists will improve their quality of life.

It is established that a heightened nitrogen balance is defined by a dysabsorbtion syndrom. Proved that the optimal quota of protein at albuminous insufficiency is about 135 g/day. Parenteral introduction of aminoacidic mixes (Infesol) led to clinical improvement, normalization of an aminoacidic spectrum of blood. Use of mixes for an enteral nutrition (Nutridrink) provides a reduction of symptoms of albuminous deficiency.

High concentration of Ab Gl, Ab tTG, Ab Em and Ab Rt and increased amount of immunocompetent cells in the SIM in coeliac disease is an index of the developing specific immune response to gliadin and tissue antigens. High specificity and sensitivity of Ab tTG and Ab Em were determined in celiac disease.

To review diagnostic approaches in chronic diseases of the small intestine.A total of 1096 patients with chronic diseases of the small intestine were admitted to the clinic of the Central Research Institute of Gastroenterological Diseases in 1987-2006.Most of the patients (90.5%) had celiac disease, hypolactasia and other types of disaccharidase deficiency, yersiniosis ileitis, Krohn's disease, postresection syndrome of a short small intestine, mesenterial ischemia and endocrine enteropathy. Rare diseases (general variable hypogammaglobulinemia, lymphoma, Wipple's disease and diverticulosis of the small intestine) were diagnosed in 5.8% cases. Primary amyloidosis of the small intestine, eosinophilic gastroenteritis, arteriomesenterial obstruction, primary intestinal pseudoobstruction, hypogammaglobulinemic spru, primary intestinal lymphangiectasia, tuberculosis, total polyposis, Peutz-Eggers and Cronkhite-Canada syndromes, collagenic sprue, erosive-ulcerative jejunoileitis, adenocarcinoma and heavy alpha-chain disease were detected in 3.7% examinees. These diseases were encountered in one to 5 cases for the latest 20 years.Clinical diagnosis of small intestinal diseases is based on the syndromes of chronic diarrhea, defective absorption, enteral protein loss, small intestinal obstruction and intestinal hemorrhage. Differential diagnosis of the nosological entities employs x-ray, endoscopic, histological, immunological and other methods. Most of the small intestinal diseases including rare can be diagnosed in any gastroentorological department.

The article describes the clinical picture of different options of celiac disease. The disease has mostly a subdued and oligosymptomatic form and is associated with different autoimmune diseases. The screening celiac disease diagnostics (detection of gliadin, endomysium and tissue transglutaminase anti-antibodies) is used to detect the celiac disease in patients belonging to the risk group. The morphologic study of the small intestine mucous membrane is the most reliable diagnostics method. A strict life-term gluten-free diet is the key method for celiac disease treatment. In case of severe malabsorption syndrome, metabolic disorders are corrected.

To examine incidence of celiac disease in patients with iron deficiency anemia (IDA) of unclear origin.A total of 331 IDA patients were examined for celiac disease (CD). The diagnosis was made on the basis of histological findings upon examination of the biopsy of small intestinal mucosa, detection of antibodies to alpha-gliadine (AGA) and tissue transglutaminase (TTG).The cause of anemia was revealed in 270 (81.5%) of 331 patients. The rest 61 patients (18.4%) had anemia of unknown cause. A rise of AGA was detected in 33 (54.0%) of 61 patients, TTG--in 29 (47.5%) patients. CD was diagnosed in 29 (47.5%) patients. Four of 61 patients (6.5%) had no symptoms of malabsorption.All the patients with IDA of unclear genesis must undergo serological tests for CD. In high titers of antibodies to AGA and TTG, retrobulbar duodenal biopsy is indicated with histological study of small intestinal mucosa.