Pediatric short bowel syndrome: state of the problem and current treatment in the Russian Federation

Никонов, Е. Л.; Чубарова, А. И.; Аверьянова, Ю В; Полевиченко, Е. В.; Skvortsova, T. A.; Vitkovskaya, I.P.; Bogdanov, I I; Popova, Milen; Хорошева, Е. Ю.

doi:10.17116/dokgastro202090315

Dok. gastroenterol.

2020

DOI: 10.17116/dokgastro202090315

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Pediatric short bowel syndrome: state of the problem and current treatment in the Russian Federation

Е. Л. Никонов¹,

А. И. Чубарова²,

Ю В Аверьянова³

et al.

Abstract: РЕЗЮМЕ Цель обзора. Представить результаты совета экспертов на тему «Синдром короткой кишки у пациентов детского возраста. Текущее состояние проблемы и лечения пациентов в России», на котором обсуждалась современная практика оказания и пути дальнейшего совершенствования медицинской помощи детям с синдромом короткой кишки (СКК). Основные положения. СКК является тяжелым заболеванием, требующим привлечения широкого круга медицинских специалистов и ресурсов. По данным регистра, число детей с СКК в РФ составляет ок… Show more

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Regional experience in the treatment of children with short bowel syndrome

Камалова

Подшивалин²,

Sageeva³

et al. 2022

Ross. vestn. perinatol. pediatr.

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Short bowel syndrome is a rare, chronic, debilitating, and potentially life-threatening condition in which loss of intestinal absorption capacity deprives children of the ability to absorb sufficient nutrients from the food they consumed. The authors describe regional experience of treating children with short bowel syndrome, which includes conservative and surgical approach, with an analysis of the leading causes, prognostic factors, timing of achieving intestinal autonomy, complications and catamnesis.Purpose. The study aims at analyzing cases of short bowel syndrome in children in order to improve the provision of medical care for children in the Republic of Tatarstan and the nearest regions.Material and methods. Single-center, retrospective study was conducted of 22 cases of children with short bowel syndrome who received treatment in the surgical department for young children in the State Autonomous Healthcare Institution Children’s Republican Clinical Hospital of the Ministry of Health of the Republic of Tatarstan for the period September 2014 till November 2021.Results. The most common cause of intestinal resection in the study group were: congenital malformations in 7 children (32%), necrotizing enterocolitis in 5 children (23%), small intestine malrotation (Ladd’s bands) in 4 children (18%), mesenteric thrombosis in 4 (18%), Hirschsprung’s disease in 2 children (9%). By the end of the first year after diagnosis, intestine self-regulation was achieved in 17 children. The mortality rate was 9%.

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Regional experience in the treatment of children with short bowel syndrome

Камалова

Подшивалин²,

Sageeva³

et al. 2022

Ross. vestn. perinatol. pediatr.

View full text Add to dashboard Cite

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Clinical significance of determining the level of citrulline in children who have undergone intestinal resection during the neonatal period

Druzhkova,

Kamalova,

Sageeva

et al. 2024

Ross. vestn. perinatol. pediatr.

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Citrulline is an amino acid that is not found in protein and does not enter the body through food, enteral or parenteral nutrition. It is instead synthesized exclusively by enterocytes. Therefore, citrulline concentrations in the blood can reflect the process of biosynthesis in the intestines and are directly related to changes in the function of these cells.Purpose. The aim of the study was to assess citrulline levels in children who had undergone intestinal resection during the neonatal period.Material and methods. The study included 22 children with short bowel syndrome, diagnosed by a doctor (main group; average age 3.7 months). We also included 20 children who had undergone surgery to remove less than 50% of their intestine but did not have short bowel syndrome (comparison group; average age 3 months 1 day). The control group consisted of 23 healthy children who had never had surgery on their gastrointestinal tract and did not have any congenital malformations or clinical signs of enteropathy (average age 5 months 3 days). High performance liquid chromatography-mass spectrometry (HPLC-MS) was used to measure the level of citrulline.Results. The average level of citrulline in the blood of children in the main group was 18 μmol/l (95% CI 10–22.8), in the comparison group – 17.5 μmol/l (95% CI 16–21), in the control group – 26 μmol/l (95% CI 22–3). Citrulline levels in patients who underwent surgery in the neonatal period were significantly different from those of children in the control group There were no differences in the content of citrulline between the main and comparison groups, depending on whether the colon was preserved or not and whether the ileocecal valve was present (p>0.05).Conclusion. An increase in citrulline level in the blood of children receiving therapy with a glucagon-like peptide-2 analog indicates the possibility of using this marker to monitor changes in intestinal absorption associated with physiological or pharmacological adaptation of the intestine.

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Thrombophilia in a patient with short bowel syndrome

et al. 2023

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This article presents a clinical case of thrombophilia in a patient with short bowel syndrome. Thrombophilia is characterized by a disorder of the blood clotting system, in which the risk of thrombosis increases. Thrombophilia, local vascular insufficiency, intestinal obstruction with fistula formation requiring intestinal resection play a significant role in the pathogenesis of short bowel syndrome. The patient was admitted with the diagnosis “thrombophilia, resection of the small intestine (residual small intestine 17 cm), ileostoma”. Condition after multiple surgical interventions on the abdominal cavity. Surgeries performed: atypical gastric resection, resection of the greater omentum, jejunostomy, resection of the cardial and antral parts of the stomach, derivation of duodenostomy, esophagostomy, derivation of loop jejunostomy, Braun enteroanastomosis, perforation of the colon, suturing, derivation of colostomy, cholecystectomy, GI reconstruction, Roux esophagoenteroanastomosis, resection of the sigmoid colon, small intestine, bearing intestinal fistulas, removal of double-barrel ileostomy, abdominal cavity drainage, relaparotomy, closure of ileal perforation, retrograde intubation of the small intestine, determination of indications for intestinal transplantation. The following tests were performed: CT-enterography, colonoscopy with subsequent morphological examination, abdominal ultrasound, enterogastroscopy, clinical and biochemical blood tests, PCR, coagulogram, urinalysis, percussion and auscultation of the chest. Morphological examination showed fragments of the wall of the colon – with erosion, fragments of fatty tissue – with immature granulation tissue, fragment of stroma covered with multilayer flat keratinized epithelium with the focus of epithelized slit-like ulcer. Clexane was administered as part of the treatment complex, nutritional support additionally included Micrazyme capsules 25000 units, teduglutide, remabipid. Rebamipid and Teduglutide allowed to stabilize the patient's condition and improve the condition of the perioperative area tissues. In case of ineffectiveness of the therapy used the indications for liver transplantation are determined.

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Pediatric short bowel syndrome: state of the problem and current treatment in the Russian Federation

Cited by 5 publications

References 3 publications

Regional experience in the treatment of children with short bowel syndrome

Regional experience in the treatment of children with short bowel syndrome

Clinical significance of determining the level of citrulline in children who have undergone intestinal resection during the neonatal period

Thrombophilia in a patient with short bowel syndrome

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