This article presents an analysis of current clinical guidelines for the management of patients with eosinophilic esophagitis (EoE) — a chronic immune-mediated disease of the esophagus characterized by eosinophilic infiltration of the mucosa (≥ 15 eos/hpf) and clinical manifestations of esophageal dysfunction. The review highlights key changes in diagnostic approaches and discusses contemporary treatment strategies, including dietary therapy (starting with the least restrictive diets), pharmacological treatment (proton pump inhibitors, topical steroids, biologic therapy), and endoscopic dilation for strictures. The authors emphasize the need for comprehensive monitoring (clinical, endoscopic, and histological) to assess treatment response and for long-term maintenance therapy given the chronic nature of the disease. Special attention is paid to pediatric aspects of patient management.

The authors present current data on the etiology, pathogenesis, clinical manifestations of celiac disease in children of different ages, associated diseases, laboratory and instrumental diagnostics, diet therapy, additional treatment methods, vaccination, and follow-up of children with celiac disease based on the developed clinical guidelines “Celiac Disease. Children”.

BACKGROUND: Small intestinal bacterial overgrowth (SIBO) is defined as an overgrowth of microorganisms more than 103 microbial bodies in 1 ml and non-specific symptoms (abdominal pain, flatulence, unstable stool, diarrhea or constipation). AIM: The aim of the study is to based on the study of the prevalence and age characteristics of various variants of the course of the syndrome of excessive bacterial growth of the small intestine in children with gastroenterological and allergic pathology, to increase the quality of diagnosis and therapeutic approaches to this pathological syndrome. MATERIALS AND METHODS: The study included 102 patients (55 female and 47 male), median age: 10.0 [7.2–12.0], with functional dyspepsia (n = 32), functional constipation (n = 20), chronic diseases of the upper digestive tract (n = 13), atopic dermatitis (n = 21) and allergic diseases of the respiratory system (n = 16). All patients underwent gastroenterological and allergological examination including hydrogen breath test on the Lactophan apparatus and hydrogen-methane test on the GastroCheck Gastrolyzer apparatus to diagnose of hydrogenogenic (H2-SIBO) and methanogenic SIBO (CH4-SIBO). RESULTS: Inclusion of methane level in exhaled air into the diagnostic algorithm increased the frequency of SIBO diagnosis depending on pathology by 10–30%. In older age groups, there was a tendency to decrease the frequency of H2-SIBO, with a significant difference between preschool and high school age (18/75% and 12/42.9 %, p = 0.018), and a tendency to increase CH4-SIBO from 4.2 % to 17.9%. The frequency of H2-SIBO was significantly higher in children with functional versus chronic diseases of the upper digestive tract (22/71% и 4/30,8 %, p = 0,02). The highest frequency of SIBO was found in children with functional dyspepsia (89.8 %), functional constipation (87.9%) and atopic dermatitis (94.5%). CH4-SIBO was predominant in children with functional constipation (33%). CONCLUSIONS: Parallel identification of hydrogen and methane in exhaled air increased the frequency of SIBO diagnosis in children of different age groups with gastroenterological and allergic pathology, which is relevant for further examination and therapy. CH4-SIBO flow was more typical for older children and H2-SIBO for younger age groups.

Macroamylasemia is a rare disorder characterized by the formation of an amylase-globulin complex that is too large to be easily cleared by the kidneys, resulting in elevated serum amylase levels. It is a benign condition with no serious signs or symptoms and requires no treatment. This article presents a case of long-term asymptomatic hyperamylasemia in a 10-year-old child. The child was admitted to the gastroenterology department with complaints of abdominal pain. Since the age of 8, there have been unexplained increases in serum amylase to 235 U/L and pancreatic amylase to 63.5 U/L, without clinical manifestations of pancreatitis, with normal levels of urine amylase and coprological elastase. An examination was performed, during which pancreatic pathology was excluded. The ratio of amylase and creatinine clearances was 0.754%. This ratio turned out to be less than 1%, which is more likely to indicate macroamysalemia. After further examination, type 1 macroamylasemia was diagnosed, which was the cause of hyperamylasemia — a non-life-threatening biochemical anomaly that requires a detailed examination, broad differential diagnostics, but does not require treatment. However, macroamylasemia is a diagnostic challenge because it must be differentiated from other causes of hyperamylasemia to avoid unnecessary testing and treatment. Hyperamylasemia is one of the main diagnostic criteria for pancreatitis, requiring extensive additional testing and treatment, often in a hospital setting. Therefore, it is important that physicians can recognize and diagnose macroamylasemia at an early stage to avoid unnecessary diagnostic and therapeutic measures. In this regard, we hope that the clinical example we have provided will be interesting and useful for physicians of various specialties.

Authors represent the Russia’s first clinical case description of a pediatric patient with ulcerative colitis whose diagnosis was combined with Lambert-Eaton myasthenic syndrome (LEMS), a rare autoimmune disorder of neuromuscular junction transmission. The observed case draws specialists’ attention to the complexity of diagnosis and the importance of a multidisciplinary approach in the diagnosis and management of such patients. Another purpose was to increase physicians’ awareness of LEMS thereby reducing the period needed to establishing of the diagnosis, which in its turn improves the prognosis of the course of the disease for patients.

The study is devoted to the comparison of the results of rapid urease tests (RUT) and the data obtained in histological examination. For this purpose, the semi-quantitative assessment of urease activity of two BUTs was compared: the semi-quantitative Pronto Dry (GASTREX, France) widely used in Europe and the AMA RUT Expert M test registered in Russia (AMA LLC, Russia) with the data obtained by histological and PCR analyses. High correlation of semi-quantitative indices of urease activity according to the results of both tests as well as correlation of these indices with the data obtained by histological examination and PCR analysis was established.

The article provides an analysis of changes in ideas about pathogenetic mechanisms, approaches to the diagnosis and treatment of irritable bowel syndrome in children. Current data on the pathophysiological mechanisms of IBS are presented. Algorithms for the management of children with this pathology are presented, based on the new positions of the Rome IV criteria and Russian recommendations for the diagnosis and treatment of functional disorders of the digestive system in children.

Eosinophilic esophagitis (EoE) is characterized clinically by typical endoscopic features and the presence of more than 15 eosinophils visible per high-power field in esophageal mucosal biopsy samples. Due to deep chronic eosinophilic inflammation, it can lead to complications, such as esophageal stenosis and rarely perforation. Intramural esophageal dissection (IED) is a previously undescribed complication of EoE in children. This article presents a clinical case of IED in a 15-year-old boy with clinical debut as sudden epigastric pain and nausea against the background of acute respiratory viral infection. Endoscopy, computed tomography, and X-ray examination revealed a double-barrel esophagus with two cavities, the esophagus itself and a blind diverticulum (pouch or “pocket”), in which granulation tissue and active inflammation with many neutrophils were detected. Initial histological assessment showed no significant eosinophilic infiltration in the esophagus. After 6 months, endoscopy revealed characteristic signs of EoE: longitudinal grooves and erosions, transverse rings, narrowing of the esophageal lumen. In the middle third, the picture of double-barrel esophagus remained unchanged, but without signs of acute detachment and with a dense septum between two esophageal cavities. Histologically, the infiltrate contained more than 50 eosinophils per high-power field. The diagnosis of EoE complicated by IED was established. A strict hypoallergenic diet and an oral gel budesonide were prescribed. Endoscopic dissection of the esophageal septum was performed, its patency was restored. In 2 months, during a re-examination after a course of pharmacotherapy and diet, there were no complaints, radiological evaluation of esophageal patency showed no abnormalities. Endoscopically, the esophagus was freely passable, there was a small fold at the surgical site, longitudinal grooves and small erosions in the lower third maintained. Histologically, the number of eosinophils decreased significantly. The therapy was continued. This clinical case demonstrates the possibility of IED development as a complication of EoE. The course of EoE can be asymptomatic, while IED can serve as the first manifestation of the disease. The endoscopic presentation of double-barrel esophagus is a consequence of dissection. The combination of a hypoallergenic diet, topical steroids, and endoscopic septectomy is effective in the treatment of EoE with IED. Key words: eosinophilic esophagitis, intramural esophageal dissection, double-barrel esophagus

Autoimmune pancreatitis is a specific peculiar and rather new form of chronic pancreatitis (ChP) that has been intensively studied by pancreatology specialists of the whole world for the last years. Autoimmune pancreatitis does not have any individual specific clinical features. This condition was described for the first time in adults and it often mimics pancreas cancer. Autoimmune pancreatitis can involve some organs, synchronously or metachronously, and is rarely identified in children. Autoimmune pancreatitis is a systemic fiber-inflammatory condition with poorly understood pathophysiological mechanism, affecting different parts of the body. In case of the treatment absence the disease can lead to fibrosis and irreversible impairment of the organs. However, the combination of the findings of serologic, histologic, and instrumental methods of investigation characteristic for аutoimmune pancreatitis allows to identify its presence. IgG4-associated disorder has been mainly described in adults, therefore pediatricians usually do not know much about it. We underline the complexity of the diagnosis of autoimmune pancreatitis / IgG4-associated disorder, in children. The article presents two clinical cases of children diagnosed with autoimmune pancreatitis. Both children had the symptoms of abdominal pain and/or mechanical jaundice combined with focal pancreas enlargement, roughness of the basic pancreas duct and distal narrowing. According to our data, the diagnosis of autoimmune pancreatitis in children can be made on the basis of the combination of specific clinical symptoms at admission and various results of imaging. Both timely made diagnosis and administered treatment provide a favorable prognosis for further course of the disease and complication development.

There are many studies devoted to the study of the intestinal microbiota. They prove the leading role of microorganisms in the formation of the immune response of the host. Intestinal microbiota contributes to the switch of preferential differentiation of Th-lymphocytes from Th2-type, typical for newborns, to the formation of Tr-cells (regulatory) with a corresponding increase in the formation of TGF-β and IL-10. Consequently, immunological tolerance is formed under the influence of the intestinal microbiota. This process occurs in the first months of life and has a long-term effect, setting the features of the body’s immune response to external infectious and food antigens, which determines the predisposition to the development of food allergies later in life. This article describes the factors that influence the colonization of the baby’s gut. Maternal gut microbiota, maternal health, mode of delivery (natural childbirth or cesarean section), type of feeding (breastfeeding or formula feeding) - all these factors affect the colonization of the gut and the formation of an immune response. In addition, lactobacilli and bifidobacteria play a critical role in the formation of immunological tolerance. Probiotics can modulate the immune response similar to the intestinal microbiota. Lactobacillus and bifidobacterium infant strains are optimal. Clinical studies have proven the effectiveness of perinatal and early administration of probiotics for the prevention of food allergies. A balanced combination of Lactobacillus rhamnosus and Bifidobacterium longum is a symbiosis of bacteria that enhances the effectiveness of each other, which promotes the optimal establishment of the intestinal microbiocenosis from birth and is the prevention of both infectious diseases and food allergies. A combination probiotic containing both Lactobacillus rhamnosus and Bifidobacterium longum seems to be the optimal choice.

In recent years, due to the widespread introduction of molecular genetic methods for the study of the intestinal microbiome and the proof of its role in the development of many socially significant diseases, strategies for influencing human health by correcting the microbiota and its functions have become especially in demand. For this purpose, probiotics are commonly used, which are living microorganisms that improve the health of the host. They have certain indications and, in general, good tolerability. But extremely rarely, their reception can be complicated by the development of bacteremia, especially in immunocompromissive patients. The condition for the preservation of live functionally active bacteria is not always achievable. This prompts the search for possibilities for the use of non-living bacterial cells, called paraprobiotics, or components of microbial cells and their metabolites - postbiotics. Studies show that paraprobiotics in terms of effects on the immune system and intestinal barrier are not inferior to the effect of probiotic, but are better and safe. Postbiotics also approach the action of probiotics in many respects and are more stable. However, optimal technologies for the production of paraprobiotics and postbiotics, as well as indications for them, have not yet been clearly developed. This requires further research.

The authors present a clinical case of asymptomatic spontaneous emphysema in a child with a severe course of Crohn's disease, which is a type of inflammatory bowel disease (IBD). The emphysema was diagnosed on the occasion, during the CT scan prior to the start of the immunobiological (Anti-TNF-α) therapy. The etiopathogenetic causes for this condition, its diagnosis and relationship with the severity of the underlying disease are discussed in the paper. A retrospective analysis of the prevalence of this pathology is also given. Conclusion: in the absence of an obvious cause (perforation), the pneumomediastinum can be explained by retroperitoneal air leak through microperforations in severe colitis, which is an unfavorable prognostic criterion for a severe course of the Crohn's disease and increases the risk of surgical treatment, colectomy as usual.

A clinical case of mitochondrial neurogastrointestinal encephalopathy (MNGIE), a rare autosomal recessive multisystem disease caused by TYMP gene mutations and thymidine phosphorylase (TP) deficiency, is presented. Authors draw the specialists’ attention to the complexity of making a diagnosis and the importance of an interdisciplinary approach in the diagnosis and management of such patients with the purpose of increasing the pediatric practitioners’ MNGIE awareness thereby reducing the time to diagnosis, which in its turn improves the prognosis for the course of the disease for the patients.

The clinical guidelines for the diagnosis and treatment of the3 functional disorders of the digestive system in children were prepared by a Group of Experts, domestic leading specialists in the field of the pediatric gastroenterology, who generalized the foreign guidelines and domestic experience, suggesting the tactics for the pediatrician actions in the everyday practice. Part 3 of the Guidelines discusses the billiard tract dysfunctions and functional constipations. There are no biliary tract dysfunctions in the Pediatric Sections of Rome Consensus IV; however, the Russian pediatric school of thought has always considered them as important in terms of one of the causes for abdominal pain in children. This attitude was supported by the experts, and it is maintained in these Guidelines. The functional constipations are common in the children of different ages, and they present not only a medical problem, but also a serious social one for both children and their parents. That is why the considerable attention has been paid to this pathology considering the psychosocial aspects of the correction.

A group of experts, leading specialists in the field of pediatric gastroenterology have prepared clinical recommendations for the diagnosis and treatment of functional digestive disorders in children; they have summarized the international recommendations and domestic experience, suggesting the tactics of in everyday pediatrician’s practice. The second part of the guidelines discusses functional disorders with abdominal pain: functional nausea and vomiting, functional dyspepsia, irritable bowel syndrome, functional abdominal pain. The authors have clarified the definitions, classification and criteria for the diagnosis of functional dyspepsia and irritable bowel syndrome taking into account actual concepts. They also have defined current therapeutic principles. The diagnosis of functional nausea and vomiting, as well as functional abdominal pain, is rarely found in our pediatric practice, and therefore the detailed information provided on these diseases is of particular importance for a practitioner.

There is ample evidence that insulin resistance, hyperinsulinemia, and obesity are at the heart of the development of non-alcoholic fatty liver disease (NAFLD). The disease is now considered as the hepatic component of metabolic syndrome (MS).64 children with NAFLD were assessed for metabolic syndrome stigma. An analysis was also made on the state of the problem according to the literature on the general links of the pathogenesis of these conditions, methods of diagnosis and treatment of NAFLD.All components of MS are observed with different frequencies in patients with NAFD. This disease, together with type 2 diabetes mellitus, becomes very common diseases in childhood. The incidence of NAFLD in children is constantly growing, it has begun to occur in infants, an outcome in cirrhosis of the liver is possible within childhood, although the prognosis for NAFLD remains definitely uncertain. Weight loss with a low glycemic index diet, regular exercise, and other lifestyle changes are the mainstay of NAFLD treatment, but not yet very effective for various reasons. In these conditions, it is necessary to increase the role of primary prevention of MS and NAFLD.

A group of leading experts in the field of pediatric gastroenterology prepared clinical recommendations for the diagnosis and treatment of functional digestive disorders (FDD) in children; they have summarized international recommendations and domestic experience and suggested the tactics for a pediatrician in everyday practice. The first part of the recommendations discusses modern views on functional digestive disorders, their development mechanisms and terminology. The article considers the biopsychosocial model of functional digestive disorders, as well as an updated classification based on the Rome criteria IV, including domestic views and experience. The article discusses the diagnostic criteria and principles of treatment of functional digestive disorders in infants (infant colic, infant regurgitation, Infant dyshesia), as well as cyclic vomiting syndrome. In all cases the authors provide a deflnition, classification (if it is developed), ICD-10 codes, an examination and treatment plan at various levels of the healthcare system, and indications for hospitalization of the children.