BACKGROUND: Short bowel syndrome is a medical and social problem characterized by high mortality and disability rates, controversial tactics, resource-intensive therapy, and lack of legal support. In 2016, the Russian Association of Pediatric Surgeons initiated the creation of the Federal Register of Children with Short Bowel Syndrome. AIM: This study conducts an audit of the Register to identify the prevalence of the pathological syndrome, establish the causes of its formation and trends in transforming the therapeutic paradigm, determine the pattern, nature, and frequency of various complications, establish factors limiting its informativeness involving representatives of implementors and users, and justify changes increasing database organization and clinical significance. MATERIALS AND METHODS: The Register has 334 patients registered. Of these, 23 children died, and five were removed from the Register at 18. The Quinta software package was used to collect, process, and manage geographically distributed data with information about patients data, identification of medical organizations, diagnosis, the content of therapeutic measures, and the structure of complications. RESULTS: The Register contains data on patients from 71 regions of Russia. Bowel resections prevailed among the initial interventions due to necrotizing enterocolitis (18.0%) and intestinal atresia (17.4%). Reconstructive interventions (125), including serial transverse enteroplasty and spiral intestinal lengthening and tailoring techniques, were implemented in 37 and 24 cases. One hundred and eighty patients needed parenteral nutrition: 18 patients required total parenteral nutrition, and 162 patients needed partial parenteral nutrition. Enteral feeding was used in 193 cases, meeting the needs of 31 children and supplementing parenteral therapy for 162 children. The structural flaw of the Register is the absence of a section on palliative care. CONCLUSIONS: The identified limitations of the Register indicate the need to improve the provided primary information, form a platform for supporting medical decisions, create a palliative care unit, and integrate with state health development programs.

The article presents a case description of a 1.9-year-old girl who was admitted on an emergency basis with a clinical picture of peritonitis due to the intake of magnetic foreign bodies. During the operation, fistulas were found between the intestinal loops and intestinal perforations, which were divided and sutured. On the 3rd postoperative day, against the background of the development of infectious complications, symptoms of multiple organ failure were manifested. Contrast-enhanced computed tomography revealed thrombosis of the superior mesenteric and right femoral arteries. Relaparotomy wаs performed – subtotal resection of the small intestine with preservation of the ileocecal angle and fasciotomy of the leg muscles. Positive dynamics was achieved in the postoperative period. The patient was transferred to a specialized medical institution for the treatment of children with short bowel syndrome. In the follow-up observation after 1.5 years, against the background of complex therapy, there was an increase in body weight by 2.8 kg and in height by 4 cm. Independent enteral feeding with restrictions on the use of lipids and carbohydrates was commenced. Parenteral nutrition continues up to 12 hours a day with a tendency to reduce the volume of the parenteral component. Chair was 2 times a day, mushy. The child moves independently, leads an active lifestyle. Conclusion: an analysis of the causes that led to the occurrence of complications atypical for magnetic foreign bodies was carried out. In the domestic and foreign literature, we did not find such complications, which served as the basis for the publication of a clinical observation.