Organizational and clinical potential of the Federal Register of children with short bowel syndrome

Abstract

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.