Short Bowel Syndrome In Children Research Articles

Teduglutide therapy in a child with short bowel syndrome

Introduction. Short bowel syndrome (SBS) in children is a rare disease. One of the most common etiological factors for the development of SBS in children is atresia of the small intestine. After surgical correction of the congenital anomaly, the remaining intestine attempts to increase absorption to restore homeostasis, and the process of intestinal adaptation begins. This process of adaptation can be assisted with analogues of endogenous growth factors of the intestine, such as teduglutide. Case outline. This report presents ? girl, aged two years and eight months, who had an estimated 20 cm of small intestine after surgical correction of congenital small bowel atresia and clinical signs of SBS. She was repeatedly hospitalized due to frequent need for parenteral correction of fluid, electrolyte, and nutrient imbalances. Stagnation in body weight and slow growth in body height were accompanied by weakened gross motor strength and slowed psychophysical development. After exploit conservative treatment measures, stimulation of intestinal adaptation was initiated with the drug teduglutide. After six months of drug therapy, progress was observed in body parameters, as well as an increase in intelligence quotient and motor abilities. Conclusion. SBS is a challenging entity for every clinician, and its previous therapy has mainly consisted of parenteral substitution of nutrients, fluids, and electrolytes. Surgical treatment carries the risk of loss of the remaining bowel and lifelong immunosuppression. The pharmacological possibilities of promoting intestinal adaptation using drugs such as teduglutide represent a light at the end of the tunnel for patients with SBS.

Intestinal Bowel Lengthening within the First 6 Months of Life: Institutional Experience and Review of the Literature.

Management of short bowel syndrome in children has been surrounded by much debate with timing of the lengthening procedure still controversial. Early bowel lengthening procedure (EBLP) has been defined as any bowel lengthening procedure performed before 6 months of age. The purpose of this paper is to report the institutional experience in EBLP and to review the literature on this subject to identify common indications. An institutional retrospective analysis of all the intestinal lengthening procedures was performed. Furthermore, an Ovid/Embase search regarding children who underwent bowel lengthening in the past 38 years was conducted. Primary diagnosis, age at procedure, type of procedure, indication, and outcome were analyzed. Ten EBLP were performed in Manchester from 2006 to 2017. Median age at surgery was 121 days (102-140), preoperative small bowel (SB) length was 30 cm (20-49) while postoperative SB length was 54 cm (40-70), with a median increased bowel length of 80%. Ninety-seven papers were reviewed, with more than 399 lengthening procedures performed. Twenty-nine papers matched criteria with more than 60 EBLP were observed of which 10 were performed in a single center from 2006 to 2017. EBLP was performed due to SB atresia, to excessive bowel dilatation or failure to enteral feeds, at a median age of 60 days (1-90). Serial transverse enteroplasty was the most frequent procedure used lengthening the bowel from 40 cm (29-62.5) to 63 cm (49-85), with a median increased bowel length of 57%. This study confirms that no clear consensus on indication or timing to perform early SB lengthening is reported. According to the gathered data, EBLP should be considered, only in cases of actual necessity after review of qualified intestinal failure center.

Nutritional Management of Intestinal Failure due to Short Bowel Syndrome in Children.

The most common cause of intestinal failure (IF) in childhood remains short bowel syndrome (SBS), where bowel mass is significantly reduced due to a congenital atresia or resection and parenteral nutrition (PN) needed. Home PN has improved outcome and quality of life, but the long-term therapeutic goal is to achieve enteral autonomy whilst avoiding long term complications. This paper is aimed at discussing nutritional strategies available to clinicians caring for these patients. A literature search was performed from 1992 to 2022 using Pubmed, MEDLINE and Cochrane Database of Systematic Reviews, and recent guidelines were reviewed. In the absence of evidence, recommendations reflect the authors' expert opinion. Consensus on the best possible way of feeding children with IF-SBS is lacking and practice varies widely between centres. Feeding should commence as soon as possible following surgery. Oral feeding is the preferred route and breast milk (BM) the first milk of choice in infants. Donor BM, standard preterm or term formula are alternatives in the absence of maternal BM. Extensively hydrolysed or amino acid-based feeds are used when these are not tolerated. Solids should be introduced as soon as clinically appropriate. Children are encouraged to eat by mouth and experience different tastes and textures to avoid oral aversion. Aggressive weaning of PN and tube (over-) feeding are now discouraged. To date, uniform agreement on the optimal type of feed, timing of food introduction and feeding regime used is lacking and great difference in practice remains. There is need for more research to establish common treatment protocols.

Regional experience in the treatment of children with short bowel syndrome

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%.

Short bowel syndrome in children: results of the conference

The paper provides information on the scientific and practical conference on the treatment of children with short bowel syndrome. The conference was attended by 188 Russian and 15 foreign specialists. The speakers shared their own experience on various aspects of treatment, answered numerous questions.

Трансплантация кишечной микробиоты. Перспективы применения в педиатрии

The aim of this review is to summarize data on the experience of using fecal microbiota transplantation (FMT) as a method of treating intestinal diseases, in particular in pediatric practice. FMT is the process of transferring fecal material from a healthy donor to the recipient’s gastrointestinal tract in order to alter gut microbial composition. To date, the most amount of research has been conducted in the field of treatment for Clostridium difficile infection. The mortality rate among children with this pathology is 1–5%. Randomized clinical trials show a higher efficacy of FMT compared to the use of antibiotics (efficacy rates of 90% and 26%, respectively) in the treatment for C. difficile infection. The effectiveness of FMT as a treatment for other diseases has been discussed. Cases of the successful use of FMT as a treatment for short bowel syndrome in children have been described, and studies on the effectiveness of this technique in patients with irritable bowel syndrome and inflammatory bowel diseases have been conducted. Most studies have been carried out on small samples of patients, and the results vary. The potential of using FMT as a treatment of children with severe bowel diseases is high, which makes it necessary to conduct further research in pediatric practice. Key words: inflammatory bowel disease, Clostridium difficile infection, short bowel syndrome, irritable bowel syndrome, fecal microbiota transplantation

Double barrel enteroplasty for the management of short bowel syndrome in children.

Currently, there are two well-established methods of bowel lengthening in patients with short bowel syndrome (SBS)-longitudinal intestinal lengthening and tailoring (LILT) and serial transverse enteroplasty (STEP) [1-4]. Both procedures may carry a high reported morbidity and mortality of 30.2% and 14.4%, respectively [5]. We report the outcomes of a novel technique: double barrel enteroplasty (DBE) for autologous intestinal reconstruction. We performed a retrospective review of all ten patients who underwent DBE at our institution since 2011. All patients have SBS and were dependent on parenteral nutrition (PN) at the time of surgery. Etiologies were gastroschisis (n = 4), bowel atresia (n = 3), necrotising enterocolitis (n = 1), volvulus (n = 1), and near-total intestinal aganglionosis (n = 1). Patient survival, complications, and subsequent enteral autonomy were evaluated. All patients are alive with normal liver function. Five children achieved enteral autonomy, while the remaining are on weaning PN. There was no bleeding, anastomotic leak, perforation, infective complications, or intestinal necrosis. No patient has required a liver and/or intestinal transplant. Double barrel enteroplasty is technically feasible and safe. It has similar efficacy and may have fewer complications when compared with other methods of autologous intestinal reconstruction.

Citrulline as a biomarker of short bowel syndrome in children

Purpose. To determine the diagnostic significance of blood citrulline level when estimating the short bowel syndrome (SBS) in children under 5 years old. To determine the blood citrulline level in children of different age. Methods. The examined group (N=31) included children under 5 who underwent small or small and large intestine resection and developed (15 children) or not developed (16 children) clinical signs of SBS. The control group included children who underwent no surgery on the GIT and had no congenital malformations and clinical signs of enteropathy (N=42). To determine the level of citrulline, overpressured high performance liquid column chromatography was used. Statistical analysis of the results was done with MedCalc® version 18.11.3. Results. Mean level of blood citrulline is 28.9 µmol/l in the examined group (95% CI 23.2–34.6) and 30.5 µmol/l in the control group (95% CI 25.0–35.1). Reliable data of the ratio between blood level citrulline and age of the child were obtained both among operated and non-operated children. The odds ratio of determining citrulline in blood was below 24.4 µmol/l in development of SBS clinical picture and amounted to 4.08 р < 0,05. Additional use of ROC analysis confirms the presence of interrelation between citrulline level and absence or presence of SBS clinical signs with 100% specificity and 43% sensitivity. The area under curve (categorical variable showing whether SBS is present or lacking) is 0,746, р = 0,005. Conclusion. Determination of serum citrulline as a diagnostic marker of short bowel syndrome in children under 5 found low sensitivity but high specificity. Thus, blood citrulline index can be used as a biomarker to diagnose SBS, to describe the course of SBS in detail and determine the risk of severe forms of this pathology in children under 5.

Advances in tissue engineering in the treatment of short bowel syndrome in children

Short bowel syndrome(SBS) is a multi-system disease caused by congenital or acquired intestinal diseases, which is the main cause of intestinal failure in children and has high morbidity and mortality in children.Despite decades of experience in the management of short bowel syndrome, current therapy is primarily supportive.Definitive treatment often requires intestinal transplantation, However, bowel transplantation brings new complications and a higher mortality rate.In order to develop novel approaches to the treatment of short bowel syndrome, artificial intestines have come under scrutiny by placing intestinal stem cells on a bioscaffold that has an absorptive surface resembling native intestine and taking advantage of neovascularization to develop a blood supply.This review will explore recent advances in functional epithelium and mesenchymal niche, biomaterials, vascularization, highlighting both success and ongoing challenges in the field. Key words: Tissue-engineering; Artificial intestine; Short bowel syndrome; Children

Non-transplant Surgical Management of Short Bowel Syndrome in Children: An Overview.

Management of severe Short Bowel Syndrome (SBS) is still one of the largest challenges of the medicine. Vast majority of the short bowel patients are children, the conditions that lead to this possible outcome most often are necrotizing enterocolitis (NEC), small intestinal volvulus as a result of intestinal malrotation, gastroschisis and the "apple peel" syndrome. Therefore, paediatricians and paediatric surgeons face this challenge most often. The nontransplant treatment appears to be effective using surgical procedure to increase absorptive surface and to reduce the transit time, but in some cases these procedures are enough to weaning of TPN. The aim of this review was to summarize the modern non-stransplant surgical management of short bowel syndrome.

The short bowel syndrome in children: peculiarities of etiopathogenesis, clinical signs and treatment (analysis of literature and own observations)

The short bowel syndrome in children: peculiarities of etiopathogenesis, clinical signs and treatment (analysis of literature and own observations)

P3B.16: The Overload Gut Syndrome

Introduction: Short bowel syndrome (SBS) is the main cause for intestinal failure in children. Parenteral nutrition allows survival and growth while physiological intestinal adaptation occurs. Most patients can be weaned off PN after two to three years. In some cases, although adaptation was successful and PN was weaned without difficulty, intestinal symptoms such as abdominal pain, discomfort, abdominal distension and diarrhea occur, along with a decrease in growth rate requiring the resumption of PN - defining the overload gut syndrome (OGS). The aim of this study was to describe the natural history, the clinical and biological characteristics and the management of OGS. Methods: this retrospective study included all children referred to our center with a history of SBS weaned off PN who needed to return to PN between 2013 and 2018. All children were evaluated in our ward before resuming PN. Clinical history, examination and biological testings, including plasma citrulline levels were collected. A stool balance analysis was performed using the duplicate meal technique and bomb calorimetry. Results: Nine children were included in the study. Mean age at PN resumption was 10 years and 10 months. PN discontinuation lasted six and a half years (mean). All patients presented with stunting which was more important on height than on weight (loss of 1.5 SD and 1.3 SD respectively). All patients were hyperphagic, five of them received tube feeding on top of oral feeding, with mean ingested calories of 2336 kcalories/day, with a ratio of ingested calories over resting energy expenditure of 205%. Mean number of stools was 4.4 per day, with a mean stool weight of 1400g/day (52g/kg/d). One patient presented with recurrent episodes of D-lactic acidosis. Four patients had bicarbonates concentration under 21mmol/l. Mean total absorption rate was 69%. After PN start, the intestinal symptoms resolved, weight increased by 1.4 SD and height by 0.8 SD at 12 months follow-up. Biological parameters all improved. Mean citrulline plasma levels remained stable (26mmol/l at PN re-start - 24mmol/l at 12 months follow-up). Conclusion: Overload gut syndrome is a rare complication of SBS in children. It occurs in children with high enteral intake and low absorption rate: the remnant intestine cannot face the calorie load and becomes overwhelmed. Symptoms should be recognized early in order to resume PN in time to avoid consequences on final stature.

MORPHOLOGICAL CHANGES OF THE SMALL INTESTINE IN ACUTE STRANGULATIVE INTESTINAL OBSTRUCTION IN THE STAGE OF DECOMPOSITION IN CHILDREN

Purpose. The purpose was to determine the depth and prevalence of pathomorphological changes in the small intestine of children at the distance from the visible border of necrosis with decompensated acute strangulated intestinal obstruction (ASIO) to determine the minimum possible length of intestinal resection.Material and methods. Morphological studies of a resected fragment of the small intestine were performed in 24 children aged 3 days to 18 years who had undergone a surgery for ASIO with intestinal necrosis. Strangulated adhesive obstruction was found in 17 children, five children had congenital intestine torsion and two children faced small intestinal strangulation in the congenital mesenteric defect. The postsurgical material was studied morphologically using histological stains with the distance of 3 cm between the section.Results. The depth, prevalence of the morphological changes in the small intestinal wall, intensity and length of morphological changes in children with decompensated ASIO were determined.Conclusion. A differentiated approach to the selection of resection scope in the proximal and distal directions from the visible border of ASIO-related necrosis is necessary. The distances are twice shorter in children as compared to adults. Refusal from extensive resections will enable to reduce the rate of short bowel syndrome in children.

Tissue engineering for the treatment of short bowel syndrome in children.

Short bowel syndrome is a major cause of morbidity and mortality in children. Despite decades of experience in the management of short bowel syndrome, current therapy is primarily supportive. Definitive treatment often requires intestinal transplantation, which is associated with significant morbidity and mortality. In order to develop novel approaches to the treatment of short bowel syndrome, we and others have focused on the development of an artificial intestine, by placing intestinal stem cells on a bioscaffold that has an absorptive surface resembling native intestine, and taking advantage of neovascularization to develop a blood supply. This review will explore recent advances in biomaterials, vascularization, and progress towards development of a functional epithelium and mesenchymal niche, highlighting both success and ongoing challenges in the field.

Синдром короткої кишки у дітей як хірургічна, загальномедична та соціальна проблема

Синдром короткої кишки у дітей як хірургічна, загальномедична та соціальна проблема

PD-011 短腸症候群に対する栄養戦略 : 小児の場合(短腸症候群の治療方針・栄養管理法, 第44回日本小児外科学会学術集会)

PD-011 短腸症候群に対する栄養戦略 : 小児の場合(短腸症候群の治療方針・栄養管理法, 第44回日本小児外科学会学術集会)

Scalability of an endoluminal spring for distraction enterogenesis

IntroductionTechniques of distraction enterogenesis have been explored to provide increased intestinal length to treat short bowel syndrome (SBS). Self-expanding, polycaprolactone (PCL) springs have been shown to lengthen bowel in small animal models. Their feasibility in larger animal models is a critical step before clinical use. MethodsJuvenile mini-Yucatan pigs underwent jejunal isolation or blind ending Roux-en-y jejunojejunostomy with insertion of either a PCL spring or a sham PCL tube. Extrapolated from our spring characteristics in rodents, proportional increases in spring constant and size were made for porcine intestine. ResultsJejunal segments with 7mm springs with k between 9 and 15N/m demonstrated significantly increased lengthening in isolated segment and Roux-en-y models. Complications were noted in only two animals, both using high spring constant k>17N/m. Histologically, lengthened segments in the isolated and Roux models demonstrated significantly increased muscularis thickness and crypt depth. Restoration of lengthened, isolated segments back into continuity was technically feasible after 6weeks. ConclusionSelf-expanding, endoluminal PCL springs, which exert up to 0.6N force, safely achieve significant intestinal lengthening in a translatable, large-animal model. These spring characteristics may provide a scalable model for the treatment of SBS in children.

Treatment of short bowel syndrome in children. Value of the Intestinal Rehabilitation Program.

The main cause of acute intestinal failure is short bowel syndrome, generally as a result of resection of extensive segments of small intestine. As a result, the main symptoms are watery diarrhea, malabsorption syndrome, chronic malnutrition, and death, if the patient is not properly treated. If the length of the remaining intestine is greater than 30 cm, complete adaptation is possible and the patient may not require parenteral nutrition. The currently recommended treatment includes the use of prolonged parenteral nutrition and enteral nutrition, always aimed at constant weight gain, in conjunction with surgeries aimed at elongating the dilated bowel. This set of procedures constitutes what is called an Intestinal Rehabilitation Program. This therapy was used in 16 children in periods ranging from 8 months to 7.5 years, with survival in 75% of the cases. Finally, the last resort to be used in children with complete resection of the small bowel is an intestinal transplant. However, to date there is no record of a Brazilian child that has survived this procedure, despite it being attempted in seven patients. We conclude that the results of the intestinal rehabilitation program are encouraging for the continuation of this type of treatment and stimulate the creation of the program in other pediatric care institutions.

Синдром короткої кишки у дітей: термінологія, сучасні аспекти патоґенезу, нові підходи до діагностики та лікування (огляд літератури та власний клінічний досвід)

Синдром короткої кишки у дітей: термінологія, сучасні аспекти патоґенезу, нові підходи до діагностики та лікування (огляд літератури та власний клінічний досвід)

Short bowel syndrome in children: Surgical and medical perspectives

The main cause of intestinal failure in children is due to short bowel syndrome (SBS) resulting from congenital or acquired intestinal lesions. From the first lengthening procedure introduced by Bianchi, the last three decades have seen lengthening procedures established as fundamental components of multidisciplinary intestinal rehabilitation programs. Debate on indications and timing of the procedures is still open leaving SBS surgical treatment a great challenge. However, enteral autonomy is possible only with an individualized approach remembering that each SBS patient is unique. Current literature on autologous gastrointestinal reconstruction technique was reviewed aiming to assess a comprehensive pathway in SBS non-transplant management.