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

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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.

About the authors

Vladimir M. Rozinov

Veltishchev Research and Clinical Institute for Pediatrics of the Pirogov Russian National Research Medical University

Email: rozinov@inbox.ru
ORCID iD: 0000-0002-9491-967X
SPIN-code: 2770-3752

Dr. Sci. (Med.) Professor, Deputy Director of Veltishchev Research Clinical Institute for Pediatrics and Pediatric Surgery

Russian Federation, 2, Taldomskaya st., Moscow, 125412

Fatima B. Ampar

Veltishchev Research and Clinical Institute for Pediatrics of the Pirogov Russian National Research Medical University

Email: fatampar@mail.ru
ORCID iD: 0000-0002-4594-7025
SPIN-code: 9902-4290

Resident Pediatric Surgeon

Russian Federation, 2, Taldomskaya st., Moscow, 125412

Mariya V. Samorokovskaya

Veltishchev Research and Clinical Institute for Pediatrics of the Pirogov Russian National Research Medical University; Speransky Children’s l Hospital No. 9

Email: masha-sam94@mail.ru
ORCID iD: 0000-0002-8897-5886
SPIN-code: 1665-6929

Researcher, Pediatric Surgeon

Russian Federation, 2, Taldomskaya st., Moscow, 125412; Moscow

Mikhail A. Isakov

Joint-Stock Company “Aston Consulting”

Author for correspondence.
Email: m.isakov@aston-health.com
ORCID iD: 0000-0001-9760-1117
SPIN-code: 5870-8933

Cand. Sci. (Biol.), Head of the therapeutic direction

Russian Federation, 2, Taldomskaya st., Moscow, 125412

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Supplementary files

Supplementary Files
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2. Fig. 1. Dynamics of inclusion in the Register of children with short bowel syndrome (by date of registration, n = 334)

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3. Fig. 2. Distribution of patients by number of surgeries

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4. Fig. 3. Distribution of patients according to the residual length of the small intestine, n = 303

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5. Fig. 4. Distribution of patients according to the residual length of the colon, n = 105

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