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A 55-year-old Caucasian woman presented with a 1-week history of left eye redness and blurred vision. Her medical history included previous small bowel resection and ileostomy for ischaemic bowel. Ophthalmic examination revealed a left corneal ulcer requiring hospital admission for intensive topical antibiotics. Overnight she became systemically unwell and was diagnosed with urinary tract infection requiring intravenous antibiotics. Her corneal condition deteriorated resulting in corneal perforation, which required a surgical gluing procedure. Despite surgery, the cornea perforated on two further occasions. At this stage, vitamin A deficiency (VAD) was suspected, due to the corneal melting response that was occurring. VAD was subsequently confirmed by serology and had occurred in this case due to malabsorption as a result of short bowel syndrome caused by previous small bowel surgery. The patient was treated with intramuscular vitamin A and eventually made a good visual and systemic recovery.

Purpose No study has evaluated complication rates of the combined operations needed for temporary Enterostomy compared to primary anastomosis in the treatment of Jejunoileal Atresia. Therefore the aim of this study is: 1) to compare the occurrence of severe postoperative complications (defined as Clavien-Dindo ≥III within 30 days) and 2) to compare the occurrence of different short- and long-term complications following treatment for Jejunoileal atresia either by primary anastomosis or the combined Enterostomy procedures. Methods All consecutive neonates treated for Jejunoileal Atresias between January 1998 and February 2021 at our tertiary academic centres were retrospectively included. Perioperative characteristics and severity of postoperative complications (Clavien-Dindo) were extracted and evaluated, using chi-squared statistics, following each operation per treatment. Results Eighty patients were included of whom 48 (60%) received a primary anastomosis and 32 (40%) an Enterostomy. Perioperative baseline characteristics were comparable, apart from significantly more patients with a gastroschisis and significantly less patients with jejunum atresia in the Enterostomy group. Our results showed that 1) significantly (p ≤ 0.01) more CD ≥III occur following treatment by Enterostomy. 2) Both short-term (surgical site infection, wound dehiscence) and long-term (short bowel syndrome, adhesive bowel obstruction) complications occurred significantly more in those treated by Enterostomy. We showed no significant difference in anastomotic leakage/stenosis and mortality rates between both treatment strategies. Conclusion Although perioperative factors might necessitate an Enterostomy, we advise a low threshold for performing a primary anastomosis when in doubt, taking into account the double risk of major complications found in patients treated with a temporary Enterostomy.

Background Despite considerable improvements in outcomes for children with short bowel syndrome (SBS), many clinicians remain pessimistic about long-term quality of life (QoL) for this population. Methods The validated FaMM tool was used to measure parent-perceived impact of the child's condition on child and family life. Partnered disease-specific survey questions relevant to child's overall wellbeing and family function were additionally completed and reported. The cross-sectional surveys were distributed to a convenience sample of parents of children with SBS. Child and family wellbeing were described and compared across child age group and involvement of an intestinal rehabilitation program (IRP). Multivariate regression analyses investigated associations between outcomes and IRP management. Open-ended responses were analyzed to investigate perceived impact of the child's SBS on the parent. Results Seventeen parents completed both surveys; 71% perceived child QoL as higher today than what they had originally been told to expect. Child daily life and family difficulty scores suggest parents perceived both to be fairly "normal". While acknowledging effort invested in condition management, parents perceived high competence in managing their child's condition; 56% perceived personal growth resulting from their child's SBS journey. IRP management was associated with better child daily life (4.11, p = 0.015), family difficulty (-4.85, p = 0.048), and family management ability (4.28, p = 0.014) scores. Conclusions Many parents perceive child and family life with SBS to be fairly "normal", manage their child's care with great competence, and report personal growth because of their child's SBS journey. Additional research inclusive of diverse patient and parent backgrounds is warranted. Level of evidence prognosis study; Level IV.

Patients with postoperative Crohn's disease are difficult to manage because of their risk of experiencing a more severe course, multiple symptom confounders, and poor sensitivity of symptomatic remission to rule out intestinal inflammation. In this group, data are lacking on biologic therapeutic efficacy, and recommendations are lacking for those with multiple medication failures. Novel noninvasive testing can simultaneously exclude alternate causes of symptoms (serum C4, fecal fat, small intestinal bowel overgrowth breath testing) and assess intestinal inflammation (fecal calprotectin, endoscopic healing index). In addition, endoscopy-based disease activity assessment and management are required. Endoscopy should be performed within 6 months of surgery, and aggressive disease activity monitoring can be considered with colonoscopy every 1-2 years subsequently to ensure late recurrence is detected. Patients with multiple resections should be screened for short bowel syndrome. Predictive biomarkers are needed to guide medication selection in this high-risk population. Postoperative prophylactic biologic therapy is prudent for patients with preoperative biologic failure. However, there are no high-quality data to guide which agent should be selected. Selecting biologics with an alternative mechanism of action in those who had failed a biologic with adequate drug concentrations and selection of different agents in those with previous intolerance are reasonable. Significantly more study is required to assess the efficacy of therapies in this setting.

In a patient with a history of bariatric surgery, severe copper deficiency presenting with macrocytic hyperregenerative anaemia was diagnosed. Besides the impaired intestinal absorption due to a short bowel syndrome, the enteral zinc supplementation competitively decreased the intestinal copper uptake. Once the zinc supplementation was stopped, enteral copper replacement ensued and normalised haemoglobin levels with decreasing median corpuscular volume were observed during follow-up visits.

Parenteral nutrition-associated liver disease (PNALD) refers to a spectrum of conditions that can develop cholestasis, steatosis, fibrosis, and cirrhosis in the setting of parenteral nutrition (PN) use. Patient risk factors include short bowel syndrome, bacterial overgrowth and translocation, disturbance of hepatobiliary circulation, and lack of enteral feeding. A growing body of evidence suggests an intricate linkage between the gut microbiota and the pathogenesis of PNALD. In this review, we highlight current knowledge on the taxonomic and functional changes in the gut microbiota that might serve as noninvasive biomarkers. We also discuss the function of microbial metabolites and associated signaling pathways in the pathogenesis of PNALD. By providing the perspectives of microbiota-host interactions in PNALD for basic and translational research and summarizing current limitations of microbiota-based approaches, this review paves the path for developing novel and precise microbiota-based therapies in PNALD.

The literature review provides historical achievements for surgical correction and treatment of patients with acute diseases of the small intestine that have led to the appearance of a short bowel syndrome. In addition, the main directions are highlighted of the modern surgical doctrine, which is of organ-preserving and organ-substitution nature, with the pathogenetic justification of transplantation technologies. The paper proposes a classification of surgical interventions for the correction and treatment of short bowel syndrome.

Amino acid-based formulas (AAFs) are similar to most other infant kinds of milk except for one important diffe­rence. Instead of containing whole or broken down cow’s milk protein, they contain amino acids. These individual amino acids are so small that they are not recognised by the immune system as allergens and do not provoke any allergic reaction in children with cow’s milk allergy. In recent studies, AAFs were well tolerated and supported growth in otherwise healthy infants without cow’s milk protein allergy, and in those with cow’s milk protein allergy. Short bowel syndrome, a disorder characterized by diarrhea, malabsorption, fluid and electrolyte disturbances, and eventually malnutrition, is usually caused by massive intestinal resection. Even though the causes of short bowel syndrome are diverse, the management has identical pillars: parenteral nutrition and early enteral nutrition. Enteral nutrition remains an important and controllable factor. The direct trophic effect of nutrients and stimulation of gastrointestinal secretions and hormonal factors enhances intestinal adaptation. AAFs promote rapid weaning of parenteral nutrition after the introduction of an elemental formula. Besides, studies show that they reduce intestinal inflammation (the level of fecal calprotectin).

Loss of functional small bowel surface area causes short bowel syndrome (SBS), intestinal failure, and parenteral nutrition (PN) dependence. The gut adaptive response following resection may be difficult to predict, and it may take up to 2 yr to determine which patients will wean from PN. Here, we examined features of gut microbiota and bile acid (BA) metabolism in determining adaptation and ability to wean from PN. Stool and sera were collected from healthy controls and from patients with SBS (n = 52) with ileostomy, jejunostomy, ileocolonic, and jejunocolonic anastomoses fed with PN plus enteral nutrition or who were exclusively enterally fed. We undertook 16S rRNA gene sequencing, BA profiling, and 7α-hydroxy-4-cholesten-3-one (C4) quantitation with LC-MS/MS and serum amino acid analyses. Patients with SBS exhibited altered gut microbiota with reduced gut microbial diversity compared with healthy controls. We observed differences in the microbiomes of patients with SBS with ileostomy versus jejunostomy, jejunocolonic versus ileocolonic anastomoses, and PN dependence compared with those who weaned from PN. Stool and serum BA composition and C4 concentrations were also altered in patients with SBS, reflecting adaptive changes in enterohepatic BA cycling. Stools from patients who were weaned from PN were enriched in secondary BAs including deoxycholic acid and lithocholic aicd. Shifts in gut microbiota and BA metabolites may generate a favorable luminal environment in select patients with SBS, promoting the ability to wean from PN. Proadaptive microbial species and select BA may provide novel targets for patient-specific therapies for SBS. NEW & NOTEWORTHY Loss of intestinal surface area causes short bowel syndrome, intestinal failure, and parenteral nutrition dependence. We analyzed the gut microbiota and bile acid metabolome of a large cohort of short bowel syndrome adult patients with different postsurgical anatomies. We report a novel analysis of the microbiome of patients with ileostomy and jejunostomy. Enrichment of specific microbial and bile acid species may be associated with the ability to wean from parenteral nutrition.

Purpose Infants with short bowel syndrome (SBS) wean from parenteral nutrition (PN) support at variable rates. Small bowel length is a predictor, but the importance of the ileocecal valve (ICV) and colon are unclear. We aim to determine if the ICV and/or colon predict enteral autonomy. Methods Infants from a single intestinal rehabilitation program were retrospectively reviewed. Etiology of SBS, intestinal anatomy, and duration of nutritional support were collected for three years. The primary outcome was time to full enteral nutrition. ANCOVA and Cox proportional hazards model were used, with p Results 55 infants with SBS were included. After accounting for the effect of small bowel, PN duration was shorter for infants with the ICV compared to those without (mean 218 vs. 538 days, p = 0.003), and had a more significant effect on infants with ≤50% of small bowel. Increased small bowel length was a positive predictor of weaning. Patients with ≤50% of colon spent less time on PN with the ICV, compared to without (mean 220 vs 715 days, p = 0.009). Conclusions Preservation of the ICV was associated with shorter duration of PN support, while colon was not. Small bowel length is a positive predictor of enteral autonomy. Level of Evidence Level III retrospective comparative study Type of Study Retrospective review

Intestinal transplantation (ITx) is now a well-established treatment for infants and children with intestinal failure (IF), who develop complications related to longterm parenteral nutrition (PN). Transplantation of other organs such as kidney, liver and heart developed during the 1960s but did not reach widespread clinical application until the cyclosporine era of immune suppression in the early 1980s. ITx lagged behind as the intestine was found to be an organ with special problems of its own immunogenicity, its interface with the enteric lumen colonized with bacteria and transfer of a heavy load of immune competent cells of the donor at the time of engraftment. In addition, organ preservation was more fragile and consequences of ischaemic reperfusion injury more severe. At the same time, treatment of intestinal failure with parenteral nutrition (PN) had developed to give children with IF a reasonable quality of life. Long-term PN, however, has its own complications including central venous catheter related sepsis, venous thrombosis and PN associated liver disease. PN therefore is not a lifetime treatment. Sporadic cases of ITx were reported in the 1970s but all recipients died of technical complications, sepsis or rejection. Optimism for success in the cyclosporine era was short-lived as most grafts were lost to rejection. A total of 15 cases were reported between 1985 and 1990 with one long term survivor. This child received a neonatal donor intestine and has survived 19 years post transplant. Introduction of more effective tacrolimus immune suppression by Starzl in 1990 allowed for reports of 60% 1 year graft survival by 1993. Currently 90% 1 year survival is reported from some centres. Parenteral nutrition remains the current standard of care for infants and children with intestinal failure with a 54

Introduction Basidiobolomycosis is a rare fungal disease, lately appearing in the gastrointestinal system of pediatric patients. Their clinical presentation resembles that of lymphoma or granulomatous inflammations. This non-specific presentation makes Gastrointestinal Basidiobolomycosis (GIB) a diagnostic challenge. Methods We are reporting the largest series of pediatric GIB, from Saudi Arabia. 12 patients were diagnosed between January 2012 and December 2019, between the ages of 16 months and 8 years. Results The most common symptoms were fever and abdominal pain. Further examination revealed an abdominal mass. Biopsy of the mass was the mainstay of diagnosis, with histological findings of typical filamentous fungal hyphae and zygospores, surrounded by eosinophils. Conclusion Role of surgery was limited to establishing the diagnosis and dealing with complications. Antifungal medication was the cornerstone of treatment in all our patients. Three of our patients were exceptional with complications such as entero-cutaneal fistula, entero-enteric fistula and short bowel syndrome. These complications have not been previously reported. We have discussed the challenges related to their management. The diagnosis of GIB in pediatric patients with abdominal mass, needs a high index of suspicion. We believe outcome depends on the severity of disease, involvement of surrounding tissues and presence of complications at the time of diagnosis.

Background Extensive intestinal resection may lead to short bowel syndrome resulting in intestinal insufficiency or intestinal failure (IF). Intestinal insufficiency and IF involve deficiency of the proglucagon-derived hormones, GLP-1 and GLP-2. Two major problems of short bowel (SB) are epithelial surface loss and accelerated transit. Standard treatment now targets intestinal adaptation with a GLP-2 analogue, to enlarge absorptive surface area. It is possible that additional benefit can be gained from combination of GLP-1 and GLP-2 activity, with the aim to enlarge intestinal surface area and also slow intestinal transit. Methods The GLP-1 and GLP-2 specific effects of the novel dual GLP-1 and GLP-2 receptor agonist dapiglutide (rINN) were characterized in rodents. Furthermore, in a murine SB model of intestinal insufficiency with 40% ileocecal resection, the influence of dapiglutide on intestinal growth, body weight, food intake, volume status and stool water content was tested against vehicle and sham operated male mice. Results Dapiglutide significantly improves oral glucose tolerance, reduces intestinal transit time and promotes intestinal growth. In the SB mouse model, dapiglutide promotes body weight recovery, despite unchanged liquid diet intake. Dapiglutide promotes significant intestinal growth, as indicated by significantly increased villus height, as well as intestinal length. Furthermore, dapiglutide reduces stool water losses, resulting in reduced plasma aldosterone. Conclusion Dapiglutide possesses specific and potent GLP-1 and GLP-2 receptor agonist effects in rodents. In the murine SB model, combined unimolecular GLP-1R and GLP-2R stimulation with dapiglutide potently attenuates intestinal insufficiency and potentially also IF. This article is protected by copyright. All rights reserved.

Objectives Short bowel syndrome (SBS) is a complex and rare condition (incidence 1200/ 100,000 live births) that requires a multidisciplinary team approach to management. In January 2019 the first European Reference Network on Rare and Inherited Congenital Anomalies (ERNICA) Intestinal Failure (IF) workshop was held. Several questions about the strategies used in managing IF associated with short bowel syndrome (SBS) were devised. The aim of our study was to collect data on the enteral feeding strategies adopted by the ERNICA centres. Methods A questionnaire (36 questions) about strategies used to introduce enteral nutrition post-operatively and start complementary food/solids in infants with SBS associated IF was developed and sent to 24 centres in 15 countries that participated in the ERNICA-IF workshop. The answers were collated and compared with the literature. Results There was 100% response rate. In infants enteral nutrition was introduced as soon as possible, ideally within 24-48 hours post-small intestinal surgical resection. In 10/24 centres, bolus feeding was used, in 9 continuous, and in 5 a combination of both. Twenty-three centres used mothers' own milk as the first choice of feed with extensively hydrolysed feed, amino acid based feed, donor human milk or standard preterm/term formula as second choice. Although twenty-two centres introduced complementary/solid food by 6 months of age, food choice varied greatly between centres and appeared to be culturally based. Conclusions There is diversity in post-surgical enteral feeding strategies among centres in Europe. Further multi-centre studies could help to increase evidence-based medicine and management on this topic.

Teduglutide is a recombinant analog of human glucagon‐like peptide‐2 that regulates the functional and structural integrity of the cells lining the gastrointestinal tract. Teduglutide is approved for the treatment of patients with short bowel syndrome (SBS) who are dependent on parenteral support (PS). Population pharmacokinetic (PK) and exposure‐response analyses were performed to support teduglutide dosing in patients with SBS. The analysis included 219 patients with SBS (aged

BACKGROUND The glucagon-like peptide 2 analogue teduglutide is an effective drug for the treatment of short bowel syndrome patients with intestinal failure (SBS-IF). This intestinotrophic peptide improves intestinal capacity for fluid and nutrient absorption through induction of mucosal growth and reduction of gastrointestinal motility. Clinical trials demonstrated the efficacy of teduglutide in reducing the need for parenteral support (PS). This study describes an SBS-IF patient population receiving teduglutide therapy in a specialized medical care setting. METHOD A retrospective analysis was performed using data of patients experiencing nonmalignant SBS-IF. They were treated with teduglutide in a multidisciplinary SBS-IF program at a single university medical center between June 2016 and June 2020. RESULTS Thirteen patients under teduglutide treatment were included in the final analysis. Mean small bowel length was 82 ± 31 cm, with 77% of patients having their colon in continuity. Over a median follow-up of 107 weeks, all patients (13 of 13, 100%) responded to the therapy with a clinically significant reduction of PS volume. Mean PS reduction increased with therapy duration and ranged from -82.5% at week 24 (n = 13) to -100% in patients (n = 5) who were treated for 144 weeks. Enteral autonomy was achieved in 12 of 13 (92%) patients. Teduglutide therapy improved stool frequency and consistency, changed dietary habits, and reduced disease-associated sleep disruptions. CONCLUSION Integrating SBS-IF patients treated with teduglutide in a proactive and tight-meshed patient care program significantly improves the clinical outcome, leading to an increased proportion of patients reaching enteral autonomy.

This study aimed to investigate the prevalence, risk factors, and outcomes of colonic involvement in patients with acute mesenteric ischemia (AMI). CT scans from a prospective cohort of 114 AMI patients treated in an intestinal stroke center between 2009 and 2018 were blindly reviewed by two radiologists. Colon involvement was defined on CT scan by the presence of at least one of the following CT colonic features: wall thickening, pneumatosis, decreased wall enhancement, dilatation, or perforation. In addition, the clinical, biological, and radiological characteristics of patients with and without colonic involvement were compared to identify risk factors for colonic involvement on CT and its impact on morbidity and mortality. Colonic involvement was identified in 32/114 (28%) patients with AMI, the right colon being more frequently involved (n = 29/32, 91%). Wall thickening (n = 27/32) was the most common CT finding. Occlusion of the inferior mesenteric artery was the only statistically significant risk factor for colonic involvement (35% vs. 15%, p = 0.02). Patients with colonic involvement on CT vs. those without had more frequently transmural colonic necrosis (13% vs. 0%, p = 0.006), short bowel syndrome (16% vs. 4%, p = 0.04), need for long-term parenteral support (19% vs. 5%, p = 0.03), and death during follow-up (22% vs. 10%, p = 0.03). In patients with AMI, colonic involvement is associated with increased morbidity and mortality and should be carefully searched for during initial CT scan assessment. • In a prospective cohort of acute mesenteric ischemia patients from an intestinal stroke center, 28% had an associated colonic involvement on CT. • Colonic involvement on CT most commonly affected the right colon, and the occlusion of the inferior mesenteric artery was the only risk factor. • Colonic involvement on CT was associated with increased morbidity and mortality and should be carefully searched for during initial CT scan assessment.

Background: Congenital intestinal obstruction occurs in ∼1:2000 live births. Congenital duodenal atresia and duodenal stenosis are frequent causes of intestinal obstruction and occur in 1 per 5000-10,000 live births. Today, duodeno-duodeno anastomosis is still the treatment of choice, and it can be performed safely by minimally invasive surgery, although duodenojejunal anastomosis is surgically simpler and has equal results. Jejunum-ileal atresia or stenosis is a major cause of neonatal intestinal obstruction; its prevalence is 1:330-1:1500 live births. Nowadays, the ability of laparoscopic assisted identification of the atresia and repair by only exteriorization of the small bowel through the umbilicus makes this technique safe and feasible in almost every new born. Methods: This article will describe the operative technique of laparoscopic management of congenital duodenal and small bowel obstruction. Results: For congenital duodenal atresia patients, mortality rate is less than 5% and the majority are secondary to associated comorbidities. There is a low rate of anastomotic leaks, anastomotic stricture, delayed gastric emptying, and bacterial overgrowth. For small bowel atresia patients, complications include anastomotic leak, adhesions, small bowel obstruction, and short bowel syndrome in less than 5% of the patients. The rate of re-operations due to small bowel obstruction in laparoscopic assisted repair patients is less compared with laparotomy patients. Conclusions: Minimally invasive surgery for duodenal and small bowel atresia is safe and feasible and reduces the complications of open surgical procedures.

Living-donor small bowel transplant has emerged as a modality to transplant patients with short bowel syndrome without prolonged wait time, albeit at the cost of technical challenges associated with vascular anastomosis due to the small size of vessels. Suboptimal perfusion in a transplanted bowel can lead to a devastating outcome, and clinical judgment alone is not completely reliable for assessment of bowel microcirculation. Here, we report a 55-year-old female patient who underwent flow cytometric cross-match-positive living-donor bowel transplant from her daughter. Initial suboptimal perfusion prompted a revision of the arterial anastomoses. Despite normal Doppler signals over the mesenteric vessels, the bowel had a variegated appearance. The microcirculation of the bowel wall was subsequently assessed in a real-time fashion by indocyanine green fluorescence angiography, which showed improved perfusion indices with time. Hence, this simple test helped us to avoid another unnecessary exploration and revision of the anastomoses. At present, the patient is thriving on an enteral diet. This case underpins the importance of real-time intraoperative assessment of bowel per-fusion and microcirculation in difficult cases. These assessments are needed to help surgeons identify tissues at risk for ischemia and necrosis, thereby allowing for maneuvers to improve intestinal viability.

Patients with ultrashort gut have inadequate nutrient absorption. Parenteral nutrition (PN) is required to meet nutrition requirements caused by chronic intestinal failure (IF). We present a case of cachexia and IF caused by ultrashort gut following an extensive small-bowel resection caused by volvulus that was complicated by small-bowel ischemia. Targeting energy prescriptions to optimize PN using indirect calorimetry in this population with ultrashort gut has not been reported in adults. This case serves to outline the challenges in optimizing PN, including factors such as anabolic status, to meet nutrition requirements in patients with ultrashort gut and cachexia.

Background: This study characterized the gut microbiota and its diet‐related metabolic activity in children with intestinal failure (IF) on parenteral nutrition (PN) compared with healthy controls and in relation to disease characteristics. Methods: Serial fecal samples were collected from 15 IF patients (n = 68) and from 25 healthy children (n = 25). The fecal microbiota, and short chain fatty acids (SCFA) were measured. Results: The microbiota of patients with IF had a lower bacterial load (p = 0.003), Shannon diversity index (p

A 38-year-old woman with a history of Crohn’s disease, multiple bowel resections, and ileostomy placement presented to the hospital with symptoms of increased ileostomy output for 1 week. She reported that she was emptying her bag fifteen times a day as opposed to her normal 3–4 times a day. Upon workup, she was found to have an acute kidney injury (AKI), and stool studies were positive for Cryptosporidium. She was treated with nitazoxanide 500 mg p.o. BID for 3 days along with continued rehydration. The patient was discharged after creatinine (Cr) and electrolytes returned to baseline. She continued to have elevated ileostomy output, and 1 week later, she was readmitted for another AKI and worsening of symptoms. At this hospitalization, stool studies were negative for Cryptosporidium, and the gastroenterologist consult recommended evaluation for active Crohn’s and Lomotil for possible short bowel syndrome. Eventually, her laboratory results improved, and she was discharged again before the full workup was completed. The patient’s ileostomy output continued to remain high following the second hospital discharge, and she eventually returned with another AKI, her third visit in a month. The workup for active Crohn’s was completed, with fecal calprotectin, serum cortisol, and small bowel follow-through all returning to normal. At this time, postinfectious inflammatory syndrome was suspected, and she was started on 60 mg of prednisone for 2 weeks. Steroid therapy elicited a significant response with normalization of her ileostomy output and return of laboratory results to baseline. The patient was discharged without return of symptoms at follow-up.

Introducción: El linfoma de células T intestinal epiteliotrópico monomórfico es una neoplasia derivada de linfocitos intraepiteliales. La etiología es desconocida, pero se ha asociado a enfermedad celíaca o a síndrome de malabsorción, aunque en la actualidad se considera un linfoma esporádico. La histopatología se caracteriza por linfocitos de tamaño intermedio a pequeño, con mínimo pleomorfismo, que generalmente expresan CD8 y CD56. Reporte de caso: Mujer de 60 años con un cuadro de dispepsia de larga evolución, ingresada por dolor abdominal agudo, diarrea, nausea, vómito bilioso y pérdida de peso. En los estudios se determinó anemia microcítica hipocrómica y desequilibrio hidroelectrolítico. Se evidenció por tomografía de abdomen una lesión nodular esplénica y engrosamiento concéntrico segmentario de yeyuno e íleon distal asociado a la presencia de líquido libre en cavidad abdominal. Evolución: Se realizó una laparotomía exploratoria con resección de yeyuno-transverso anastomosis latero-lateral, en patología se deteminó la presencia de 3 tumores: a nivel de yeyuno de 16x8.5x6 cm, en el íleon distal de 13x6.5x3 cm y en el ciego de 5x4x2cm con histología de linfoma no Hodgkin T de alto grado, intestinal primario, de tipo monomórfico epiteliotrópico (MEITL). Fue dada de alta en mejores condiciones diez días después. Dos semanas luego del alta desarrollo sepsis de foco urinario, deshidratación por síndrome de intestino corto, hiperamonemia y neumonía de focos múltiples con derrame pleural bilateral. Falleció al sexto día de hospitalización. Conclusiones: En conclusión, el MEITL es una neoplasia agresiva primaria intestinal de reciente identificación y pobre pronóstico. No se conocen en su totalidad los mecanismos moleculares y genéticos asociados a esta entidad. El caso presentado demostró un curso clínico similar al reportado en la literatura.

Le syndrome de grêle court, conséquence d’une résection étendue de l’intestin, est la principale cause d’insuffisance intestinale, définie comme la réduction de la fonction intestinale en dessous du minimum nécessaire à l’absorption des macronutriments, de l’eau et des électrolytes. La prise en charge nécessite alors la nutrition parentérale. L’évolution du syndrome de grêle court est schématiquement scindée en trois périodes successives : 1) la période post-opératoire, d’une durée de 3 à 6 semaines ; 2) la période adaptative, d’une durée de 2 ans environ ; et 3) la période de stabilisation, dite séquellaire. Le développement d’une hyperphagie, d’une adaptation intestinale permettant l’augmentation de la surface d’absorption et de la sécrétion d’entérohormones, ainsi qu’une modification du microbiote, se produisent spontanément, améliorant l’absorption intestinale et diminuant la dépendance à la nutrition parentérale. Cet article résume les principales conséquences physiopathologiques (bénéfiques ou délétères) d’une résection étendue de l’intestin grêle et la prise en charge nutritionnelle et médicamenteuse du syndrome de grêle court chez l’adulte.

Background An open abdomen with frozen adherent bowels is classified as grade 4 in Björck’s open abdomen classification, and skin grafting after wound granulation is a typical closure option. We achieved delayed primary fascia closure for a patient who developed open abdomen with enteroatmospheric fistulas due to severe adherent small bowel obstruction. We present here the details of his management. Case presentation A 52-year-old man suffered acute abdominal pain during a flight and received an emergency laparotomy due to adhesive small bowel obstruction. Repeated laparotomies were required, and later open abdomen and proximal site jejunostomy were selected. After negative pressure wound therapy, he was transferred to our institution. Two enteroatmospheric fistulas emerged on the exposed intestine, and we diagnosed the condition as a Björck grade 4 open abdomen. After 8 months of wound care and parenteral nutrition, we decided to attempt primary wound closure because the patient required permanent oral restriction and total parenteral nutrition due to short bowel syndrome. A circular incision along the circumference of the exposed bowel allowed us to take a safe approach into the abdominal cavity. We removed the intestinal adhesions completely and resected the bowels, including the fistulas and anastomosed parts. Finally, the abdominal wall defect was reconstructed using the component separation technique, and the patient was discharged without an ostomy. Conclusions Primary fascia closure for grade 4 open abdomen is hard, but leaving a long interval before radical surgery and applying pertinent wound management may help solve this adverse situation.

Introduction. The present trial systematizes data, taken from one surgical center as an example, on treating patients with intestinal atresia and necrotizing enterocolitis with multiple intestinal anastomoses.Material and methods. The trial is a retrospective review on the treatment of 13 newborn infants who since 2010 have been put multiple intestinal anastomoses; the treatment was approved by the Hospital Ethics Committee. The average gestational age of patients was 31.2 weeks. The average age at the time of surgery – 7,9 days. Average weight - 2007 grams. The average number of anastomoses was 3.7 (range: 2-7). The average length of remained small intestine after the second surgery was 67.4 cm (range: 12-120 cm). No other surgical procedures, including gastrostomy or enterostomy, were performed. In all cases, surgical intervention ended with hermetic suturing of the abdominal cavity. Among them, there were 6 patients with the multifocal form of necrotizing enterocolitis; 6 patients had type IV atresia of the small intestine; 1 patient had the Ladd’s syndrome. Connection of intestinal segments was made by constructing several “end-to-end” anastomoses, double-row precision seam with PDS II 7/0 suture.Results. In the postoperative period, complications associated with anastomosis construction, such as leakage and narrowing, were not recorded. Transit function of the gastrointestinal tract restored on day 4, on average, after the surgery (range: 2-6 days). There were no early lethal outcomes within the first 28 days after the surgery which were associated with the surgery. 2 patients with short bowel syndrome (remained small intestine was 12 and 25 cm) and multivisceral disorders died on day 72 and 64 after the surgery. Survived patients were transferred to full enteral feeding in 56 days, in average, after the second surgery (range - 15-120 days).Conclusion. In our study, we have demonstrated potentials of a new surgical approach: one-stage formation of multiple intestinal anastomoses in case of multiple intestinal atresias as well as in case of multifocal forms of necrotizing enterocolitis. Maintaining the bowel length with multiple bowel anastomoses is very important factor for better survival of patients with the short bowel syndrome.

The term intestinal failure (IF) is understood as the transient or irreversible loss of the resorptive capacity of the bowels. This includes a multitude of diseases, some of which have anatomical causes and others functional causes. The functional capacity (absorption and motility) of the remaining digestive tract and the bacterial overgrowth and false colonization of the small bowel are of prognostic importance. After exclusion of pathological intestinal findings, such as stenosis and dilatation, initially conservative treatment is employed with the aim of intestinal adaptation. Before failure or complications, initially conservative surgery and then organ replacement by transplantation should be considered. The IF is a temporary or permanent condition. For adults a length of 100cm small bowel without the colon, 60cm still with continuity to the colon and 35cm small bowel with complete preservation of the colon including the ileocecal valve are potentially sufficient for intestinal autonomy.

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.

BACKGROUND: Short bowel syndrome is a disorder with several complications such as malnutrition and failure of drug therapy. Treatment with opioids is needed in many patients, and oral medication is preferred. However, optimal dosing is a difficult task as current guidelines are based on an intact gastrointestinal tract. Hence, the aim of this explorative case study was to assess the pharmacokinetics of orally administered oxycodone in patients with short bowel syndrome.METHODS: Six patients with short bowel syndrome were administered 10 mg oral solution oxycodone after an overnight fast. Oxycodone plasma concentrations were determined over a 6-hour period. Pharmacokinetic profiles were visually inspected. Pharmacokinetic parameters: maximum plasma concentration, time of maximum concentration and area under the curve were calculated. Data were also compared to mean values obtained in healthy participants.RESULTS: A clinically relevant concentration of oxycodone was found in all patients, although with large inter-individual variation. The absorption fraction tended to correlate positively with total intestinal length. Additionally, preservation of some or the entire colon seemed further to increase the absorption fraction. Time of maximum concentration varied from 30 min to approximately 90 min.CONCLUSIONS: Oxycodone is absorbed in a clinically relevant extent in patients with short bowel syndrome, but bioavailability varies greatly between patients, which shall be taken into consideration. Absorption is related to functional small intestinal length, but preservation of colon is also beneficial. Still, optimal therapeutic dosing must be individualized, and other factors such as those related to malnutrition and motility shall also be taken into consideration.

Short bowel syndrome is a clinical condition defined by malabsorption of nutrients and micronutrients, most commonly following extensive intestinal resection. Due to a loss of absorptive surfaces, the absorption of orally administered drugs is also often affected. The purpose of this study was to systematically review the published literature and examine the effects of short bowel syndrome on drug pharmacokinetics and clinical outcomes. Studies were identified through searches of databases MEDLINE, EMBASE, Web of Science, and SCOPUS, in addition to hand searches of studies’ reference lists. Two reviewers independently assessed studies for inclusion, yielding 50 studies involving 37 different drugs in patients with short bowel syndrome. Evidence of decreased drug absorption was observed in 29 out of 37 drugs, 6 of which lost therapeutic effect, and 14 of which continued to demonstrate clinical benefit through drug monitoring. The influence of short bowel syndrome on drug absorption appears to be drug-specific and dependent on the location and extent of resection. The presence of a colon in continuity may also influence drug bioavailability as it can contribute significantly to the absorption of drugs (e.g., metoprolol); likewise, drugs that have a wide absorption window or are known to be absorbed in the colon are least likely to be malabsorbed. Individualized dosing may be necessary to achieve therapeutic efficacy, and therapeutic drug monitoring, where available, should be considered in short bowel syndrome patients, especially for drugs with narrow therapeutic indices.

short bowel syndrome is marked by inadequate intestinal surface area to absorb nutrients. Current treatments are focused on medical management and surgical reconfiguration of the dilated intestine. We propose the use of spring-mediated distraction enterogenesis as a novel intervention to increase intestinal length. Given our previous success lengthening intestinal segments using springs with spring constant ~7 N/m that exerts 0.46 N or higher, we sought to determine the minimal force needed to lengthen porcine small intestinal segments, and to explore effects on intestine over time.Juvenile Yucatan pigs underwent laparotomy with enterotomy to introduce nitinol springs intraluminally (n = 21 springs). Bowel segments (control, spring-distracted) were retrieved on post-operative day (POD) 7 and 14, and lengths measured. Thickness of cross-sectional intestinal layers were measured using HE, and submucosal collagen fiber orientation measured using trichrome stained sections.all pigs survived to POD7 and 14. Spring constants of at least 2 N/m exerting a minimum force of 0.10 N significantly lengthened intestinal segments (p 0.0001). The stronger the spring force, the greater the induced thickness of various intestinal layers at POD7 and 14. Collagen fiber orientation was also more disordered because of stronger springs.a spring constant of approximately 2 N/m exerting 0.10 N and greater significantly lengthens intestinal segments and stimulates intestinal structural changes at POD7 and 14. This suggests a decreased force is capable of inducing spring-mediated distraction enterogenesis.

Introduction Short bowel syndrome (SBS) is a rare, highly disabling, life-threatening condition due to extensive intestinal resections, characterized by diarrhea, malabsorption, and malnutrition. SBS is the main cause of intestinal failure (SBS-IF). The primary therapy for SBS-IF is intravenous supplementation (IVS) of nutrients. The pharmacological therapy aims to improve the remnant bowel function, leading to the decrease of IVS requirement. Areas covered This review provides a safety perspective and discusses unmet clinical needs on pharmacotherapy for SBS, ranging from symptomatic agents traditionally used off-label to manage hypersecretion and diarrhea, to curative drugs with selective intestinotrophic properties. Real-world evidence on symptomatic drugs is lacking. Data on teduglutide - the first-in-class glucagon-like peptide-2 (GLP-2) receptor agonist approved in SBS - are mainly derived from clinical trials, with several unsettled safety issues, including the risk of malignancies. Expert opinion Defining the long-term safety of drugs used for SBS is a priority; a unified list of commonly used drugs with consolidated proof of effectiveness is needed to harmonize the symptomatic pharmacological approach to SBS. GLP-2 receptor agonists are a promising curative pharmaco-therapeutic approach, although long-term safety and effectiveness deserve further real-world assessment. Pharmacovigilance and global data sharing are crucial to support safe prescribing in SBS.

An arteriovenous fistula was required for permanent vascular access in a patient undergoing hemodialysis due to progressive chronic kidney disease associated with short bowel syndrome. In the present report, we discuss the case of a patient who underwent arteriovenous grafting because there was no proper native vein as a route, following which a seroma developed near the arterial anastomosis. Despite several surgical treatments, seroma not only recurred but also affected dialysis by compressing the graft. A stent was inserted into the graft to withstand the pressure from the seroma, and because one stent could not withstand the pressure, the stent overlapped where it received the most compression. Since then, the patency of graft has been well maintained for more than 2 years. Increasing the radial force of overlapping stents would be an alternative plan to help solve the problematic repeated compressible seroma despite multiple surgical treatments.

Our aim was to analyze the outcomes in children with short-bowel syndrome (SBS), parenteral nutrition dependence (PND), and intestinal failure-associated liver disease (IFALD) treated in our Intestinal Rehabilitation Program (IRP) during 2007-2018. We retrospectively reviewed charts of 135 patients with SBS-PND at the time of enrollment in IRP; of these, 89 (66%) had IFALD, defined as conjugated bilirubin (CB) of ≥2 mg/dl at enrollment and/or abnormal liver biopsy showing stage 2-4 fibrosis. Outcomes included resolution of CB, enteral autonomy, laboratory parameters (platelets, aspartate aminotransferase to platelet ratio index), growth trends, transplant rates, and mortality. Of the 89 patients, 74 had elevated CB at enrollment; the other 15 had normalized CB but had fibrosis on liver biopsy. Thirty-eight patients had liver biopsies: 36 (95%) had fibrosis, including 21/36 with bridging fibrosis/cirrhosis. The median proportion of residual small bowel was 23% (interquartile range, 13%-38%) of the expected length for age and median, daily energy requirement by PN was 100%. Two received a transplant, three died (one posttransplant), and the remaining 85 survived; 69 (81%) achieved enteral autonomy. Seventy-three (99%) of the 74 patients with hyperbilirubinemia normalized their CB with medical treatment. In a subset of eight of 89 patients with initial platelet count of

This study aimed to identify predictors and estimate time to teduglutide response among adult patients with short bowel syndrome with intestinal failure (SBS-IF) dependent on parenteral support (PS).Post-hoc analysis was performed on individual patient data from teduglutide-treated patients in the phase III teduglutide trial STEPS and the STEPS-2 extension. Response was defined as ≥20% PS volume reduction from baseline for two consecutive visits. Early responders experienced the reduction at 20 and 24 weeks during STEPS while late responders experienced the reduction during STEPS-2. Timing and predictors for response were assessed among the treated population using Cox proportional hazard model. Time to response was compared in aetiological subgroups using Kaplan-Meier analysis. Patient characteristics and time to response were compared between early vs. late responders.A total of 34 patients were included in this analysis; overall median time to response was 4.3 months. The presence of stoma predicted a positive response to teduglutide (hazard ratio [HR]: 5.6; 95% confidence interval [CI]: 1.4-21.9; p = 0.013). Vascular disease (vs. inflammatory bowel disease [IBD]) as cause of major intestinal resection (HR: 0.2; 95% CI: 0.0-0.8; p = 0.015), presence of ileocecal valve (HR: 0.1; 95% CI: 0.0-0.8; p = 0.047), and female sex (HR: 0.3; 95% CI: 0.1-1.0; p = 0.026) are negatively associated with response. In subgroup analyses, patients with IBD (vs. vascular disease), with (vs. without) a stoma, and without (vs. with) colon-in-continuity had a shorter time to response (all p 0.05). The mean times to response were 3.6 (standard deviation (SD): 1.1) months for early responders (n = 27) and 10.0 (SD: 6.1) months for late responders (n = 7). Fewer early responders had colon-in-continuity (51.9%) and ileocecal valve (0.0%) compared to late responders (100% and 28.6%, respectively; both p 0.05). Early responders had a lower mean percentage of colon remaining compared to late responders (24.6% vs. 57.1%, respectively; p = 0.016).Time to response to teduglutide depends on bowel anatomy and SBS-IF aetiology. IBD, presence of a stoma, and absence of ileocecal valve were associated with earlier response to teduglutide. These findings may enhance management of patients with SBS-IF; however, due to sample size limitations, additional studies are needed to confirm these findings.

Background Previous studies showed that type 2 short bowel syndrome (SBS) rats were accompanied by severe intestinal bacterial dysbiosis. Limited data are available for intestinal fungal dysbiosis. Moreover, no effective therapeutic drugs are available for these microbiota dysbiosis. The aims of our study were to investigate the therapeutic potential of glucagon-like peptide 2 (GLP-2) for these microbiota dysbiosis in type 2 SBS rats. Methods 8-week-old male SD rats which underwent 80% small bowel resection, ileocecum resection, partial colon resection and jejunocolostomy, were treated with saline (SBS group, n = 5) or GLP-2 (GLP2.SBS group, n = 5). The Sham group rats which underwent transection and re-anastomosis were given a saline placebo (Sham group, n = 5). 16S rRNA and ITS sequencing were applied to evaluate the colonic bacterial and fungal composition at 22 days after surgery, respectively. Results The relative abundance of Actinobacteria, Firmicutes and proinflammatory Proteobacteria increased significantly in SBS group rats, while the relative abundance of Bacteroidetes, Verrucomicrobia and Tenericutes decreased remarkably. GLP-2 treatment significantly decreased Proteus and increased Clostridium relative to the saline treated SBS rats. The diversity of intestinal fungi was significantly increased in SBS rats, accompanied with some fungi abnormally increased and some resident fungi (e.g., Penicillium) significantly decreased. GLP-2 treatment significantly decreased Debaryomyces and Meyerozyma, and increased Penicillium. Moreover, GLP-2 partially restored the bacteria-fungi interkingdom interaction network of SBS rats. Conclusion Our study confirms the bacterial and fungal dysbiosis in type 2 SBS rats, and GLP-2 partially ameliorated these microbiota dysbiosis.

Background Retroperitoneal germ cell tumors (GCTs) are rare, commonly large tumors, often diagnosed in infancy. Complete surgical resection may pose a serious challenge as encasement of major vessels and organ displacement can lead to perioperative complications. This study aims to illustrate the surgical challenges of excising retroperitoneal GCTs. Results Nine patients were included: six patients with a teratoma, two patients with a mixed GCT with a yolk sac tumor component, and one patient with a pure yolk sac tumor. Six were males and seven were younger than 1 year of age at time of presentation. In all cases, perioperative vascular or organ-related complications occurred in one patient; this resulted in short bowel syndrome. There was no recurrence or mortality during the follow-up period. Relevant literature is reviewed and described. Conclusions The anatomical relation of a retroperitoneal GCT to the major vessels and organs is unpredictable. Preoperative radiological evaluation can help to improve the understanding of the vascular anatomy and to plan accordingly. Anteriorly displaced veins may appear insignificant and arteries, such as the superior mesenteric artery may be encased. Preoperative imaging may prevent perioperative complications, leading to safer surgical procedures. Level IV evidence Therapeutic study

Summary Aim Home parenteral nutrition (HPN) is one of the most demanding medical therapies as it is the only option for patients for intestinal failure (IF). No unequivocal policy on how to start and progress with HPN has ever been presented. The IF Center at the Stanley Dudrick's Memorial Hospital in Skawina is one of the biggest centers in Poland, celebrating its twentieth birthday last year. It offered the unique chance to present how to create and grow the IF center, increasing the quality of care. The above became the aim of this study. Methods A retrospective analysis of all medical records of HPN patients from the University Hospital and Stanley Dudrick's Memorial Hospital in Skawina. Patient profile, clinical course and treatment outcomes were assessed. The evolution of all aspects of HPN is presented. A brief historical perspective has been added to better illustrate the center's growth and transformation. Results 608 patients (363 female, 245 male, mean age 55.55 year) from all over Poland were treated between December 1999 and December 2019. The most frequent indication for HPN was mechanical obstruction (277, 45.7%), followed by short bowel syndrome (SBS, 208, 34.3%) and intestinal fistula (46, 7.59%). The most common primary disease was cancer (n = 267), followed by mesenteric ischemia (n = 104), and surgical complications (n = 62). 314 patients (51.8%) died. 73 (12.04%) were successfully weaned off PN. The catheter infection rate reached 0.39/1000 catheter days. Conclusions the founding of the HPN center and its further development is possible provided that there is team of dedicated people, supported by hospital base. The real growth opportunity is guaranteed by the reimbursement. It is necessary to adapt to the changing circumstances.

OBJECTIVES: Standard parenteral nutrition (PN) solutions are safe and can meet the nutritional requirements of a significant number of pediatric patients. However, they may not always be adequate for those on long term PN. We aimed to compare the composition of individually tailored prescriptions in a pediatric population on home PN with that of available commercial PN formulations. METHODS: Retrospective analysis of the individual prescriptions of metabolically stable pediatric patients on home PN over a 1-year period (March 2019 to March 2020). These were compared with commercially available solutions with electrolytes, and replacement was considered adequate if three successive criteria were met: non-protein calorie to volume ratio (maximum variation 15%); non-protein calorie to nitrogen ratio (NPC:N) (maximum variation either 20% for long term use or 35% for possible short term use); electrolyte concentration (maximum increase 20%). RESULTS: Twenty-four patients were included (67% male; median age 7.5 years). The most common diagnosis was short bowel syndrome (58%). Replacement with a standard formulation was considered appropriate for possible short term use (maximum variation of 35% in NPC:N) in 16 (67%) patients and for long term use (maximum variation of 20% in NPC:N), the number of patients decreased to 10 (42%). CONCLUSIONS: Standard PN solutions can be adequate for a significant proportion of pediatric patients on home PN. Their use in the short term may also be appropriate in holiday periods or in settings of limited resources or restricted access to hospital facilities, such as those imposed by the COVID-19 pandemic.

Background: The utility of repeat serial transverse enteroplasty (reSTEP) has been questioned after patients failed to achieve enteral autonomy. We compared the outcomes after reSTEP to one lifetime STEP (oneSTEP), and tried to identify patients at risk for reSTEP failure. Methods: All STEPs done at our institution were reviewed. Growth, enteral autonomy, bowel-derived bloodstream infection hospital admissions, complications, and need for bowel transplantation were evaluated (p Results: 24 patients underwent 32 STEP (16 oneSTEP, 8 reSTEP). reSTEP patients were younger at first surgery (1.01±1.05 vs. oneSTEP 3.06±4.73 years, p = 0.24). Median time to reSTEP was 1.1 year (0.6–5.7). Weight-for-length z-scores improved after reSTEP (0.02±1.40 to 0.22±1.42, p = 0.81). Bowel-derived bloodstream infections decreased after surgery (oneSTEP 1.50±2.25 to 0.94±1.73, p = 0.50; reSTEP 1.88±2.10 to 1.66±2.32, p = 0.52). 37.5% (9/24) patients achieved enteral autonomy at last follow-up: 7/16 oneSTEP, 2/8 reSTEP (p = 0.37). Two complications occurred after oneSTEP (staple line ulcer, leak), none following reSTEP. Three patients (oneSTEP 1/16, reSTEP 2/8, p = 0.19) underwent bowel transplantation (all gastroschisis). Conclusion: Similar postoperative outcomes after reSTEP and single STEP (improved enteral tolerance, reduced rates of infections) support the use of reSTEP when clinically indicated, although reSTEP in young infants with a history of gastroschisis may need further evaluation.

Intestinal failure-associated liver disease is a major morbidity associated with short bowel syndrome. We sought to determine if the obesity-resistant mouse strain (129S1/SvImJ) conferred protection from liver injury after small bowel resection (SBR). Using a parenteral nutrition-independent model of resection-associated liver injury, C57BL/6J and 129S1/SvImJ mice underwent a 50% proximal SBR or sham operation. At postoperative week 10, hepatic steatosis, fibrosis, and cholestasis were assessed. Hepatic and systemic inflammatory pathways were evaluated using oxidative markers and abundance of tissue macrophages. Potential mechanisms of endotoxin resistance were also explored. Serum lipid levels were elevated in all mouse lines. Hepatic triglyceride levels were no different between mouse strains, but there was an increased accumulation of free fatty acids in the C57BL/6J mice. Histological and serum markers of hepatic fibrosis, steatosis, and cholestasis were significantly elevated in resected C57BL/6J SBR mice as well as oxidative stress markers and macrophage recruitment in both the liver and visceral white fat in C57BL/6J mice compared with sham controls and the 129S1/SvImJ mouse line. Serum endotoxin levels were significantly elevated in C57BL/6J mice with significant elevation of hepatic TLR4 and reduction in PPARα expression levels. Despite high levels of serum lipids, 129S1/SvImJ mice did not develop liver inflammation, fibrosis, or cholestasis after SBR, unlike C57BL/6J mice. These data suggest that the accumulation of hepatic free fatty acids as well as increased endotoxin-driven inflammatory pathways through PPARα and TLR4 contribute to the liver injury seen in C57BL/6J mice with short bowel syndrome. NEW & NOTEWORTHY Unlike C57BL/6 mice, the 129S1/SvImJ strain is resistant to liver inflammation and injury after small bowel resection. These disparate outcomes are likely due to the accumulation of hepatic free fatty acids as well as increased endotoxin-driven inflammatory pathways through PPARα and TLR4 in C57BL/6 mice with short bowel syndrome.

Summary Background Use of HPN in paediatrics in the UK has increased rapidly over the last 20 years but the prevalence of HPN has been challenging to define. Clinicians in the UK have noted an evolving complexity of cases and perceive improved outcomes and increased acceptability of long-term PN. These factors combined have the potential to increase the burden on existing paediatric gastroenterology services in the UK. Methods A national database was interrogated to define the prevalence of HPN in children in the UK and to explore outcomes for patients receiving HPN. Results Since 2015, 525 children were notified to the database; of these patients, mortality was In 2019, 389 children received HPN in the UK; this is nearly double the number last reported in 2012 and is a prevalence of 30 per million children. Short bowel syndrome is the largest category of these patients. However, a poorly defined group including those with multisystem disease has increased 10 fold since 2012 and is now the second largest category. Conclusions Long term HPN in childhood is safe and associated with good survival and low risk of the need for intestinal transplantation.

Background/purpose Following a serial transverse enteroplasty (STEP) procedure some children develop redilation of the small intestine leading to impaired enteral tolerance and inability to wean parenteral nutrition (PN). The benefit of a second STEP procedure (2STEP) has been controversial. Methods We performed a retrospective review of our experience (2008–2018) performing 2STEP, with comparative analysis of nutritional outcomes pre- and postsurgery. Results During this period 2STEP was performed in 23 patients (13 F:10 M) at a median (25%–75%) age of 2.2 (1.2–3.6) years. Median intestinal length was 68 (40–105) cm before and 85 (40–128) cm after 2STEP. Leading up to 2STEP, PN provided almost 75% of estimated calorie needs. By 24 weeks following 2STEP drops in mean PN percent approached statistical significance (p = 0.07) and at most recent follow up the mean PN percentage was statistically better than at the time of operation or 4 weeks prior to 2STEP, and was nearly significant compared with 12 weeks (p = 0.07) and 24 weeks (p = 0.06) prior. Thirteen children were completely off parenteral support. Conclusion When small intestine redilation occurs following a STEP procedure and where PN cannot otherwise be weaned we believe these data support performing a 2STEP. We cannot predict preoperatively which children will ultimately benefit. Level of evidence 3 (retrospective comparative study).

Adult midgut malrotation with volvulus (AMMV) is an uncommon presentation which may be found incidentally during abdominal radiologic investigations or at laparotomy. We report a case of AMMV and small bowel gangrene in a 35-year-old Gravida four, Para three at 39 weeks, 4 days gestation who presented with a short history of worsening abdominal pain, repeated vomiting and abdominal wall guarding. Emergency caesarean section performed on account of a suspected placental abruption incidentally revealed a long segment of non-viable small intestine. Subsequent midline laparotomy disclosed a midgut malrotation with volvulus and bowel gangrene. This resulted in a 4.6m resection of non-viable small bowel with Ladd’s procedure. The patient developed moderate symptoms of short bowel syndrome in the post-operative period which was successfully managed non-operatively. This case report represents a rare diagnosis, in the West-African sub-region, of an adult midgut malrotation with volvulus mimicking a third trimester obstetric emergency.

Internal hernia after gastrectomy is a rare but potentially life-threatening condition without surgical intervention. Clinical risk factors of internal hernia should, hence, be reviewed after gastrectomy. From 2008 to 2018, patients who underwent gastrectomy for gastric cancer were investigated.Abdominal computed tomography (CT) was used to screen for internal hernia, and surgical exploration was performed to confirm the diagnosis. Using retrospective statistical analysis, the incidence, characteristics, and risk factors were identified, and the characteristics of the internal hernia group were reviewed.The overall incidence of internal hernia was 0.9%. From statistical analysis, it was found that laparoscopic surgery was almost five times riskier than open gastrectomy (odds ratio [OR], 4.947; 95% confidence interval [CI], 1.308-18.710;Suspecting an internal hernia should be an important step when a patient with a history of laparoscopic gastrectomy visits for medical care. When suspected, emergent screening through CT scan and surgical intervention should be considered as soon as possible to prevent lifetime complications accordingly.

One of the primary causes of morbidity and mortality in those with short bowel syndrome (SBS) is sepsis, caused by bacterial translocation (BT). Since synbiotics can cease gut-related bacterial overgrowth, they may serve as a supportive dietary supplement-based strategy after gastrointestinal surgery. This study was conducted to determine the effects of Lactobacillus acidophilus and pectin on BT and gut adaptation after extensive small bowel resection in the rat. Forty rats were distributed in four groups. Group A suffered laparotomy, group B suffered gut transection and reanastomosis, SBS rats (group C) suffered 75% small gut resection, and finally, Group D suffered gut resection and treated with a synbiotic cocktail from day 7 before the surgery to day 14 after it. Intestinal structural changes and BT to mesenteric lymph nodes, liver, portal blood, and peripheral blood were detected on day 15 post-surgery. Treatment with a synbiotic cocktail led to a considerable reduction in bacterial translocation to liver and portal vein (degree II) compared with SBS untreated rats. Also, synbiotic administration significantly increased jejunum and ileum villus height and crypt depth, ileum villus width, and percentage of goblet cells in jejunum and ileum compared with SBS rats. In the rat model of short bowel syndrome, L. acidophilus, and pectin, as a potential synbiotic compound, could decrease the BT from the gut and improve the bowel adaptation.

Background Type II (acute) intestinal failure (IF) is usually caused by complications of abdominal surgery resulting in enteric fistulas or proximal stomas and requires parenteral nutrition (PN) for several months. This study aimed to evaluate clinical management and outcome of type II IF patients in a single center. Methods Medical records of patients referred to the Intestinal Rehabilitation Team (IRT) at Samsung Medical Center (Seoul, Korea) were retrospectively analyzed. Results From 2014 to 2019, 34 patients with IF were referred. 28 patients were type II IF and were included in the analysis. There were 17 males and 11 females. Mean age of patients was 56.7 years. Pathophysiology of IF were high-output stoma in 16 cases, extensive bowel resection (with bowel in continuity) in 7 cases, and enterocutaneous fistula in 5 cases. The catastrophic events necessitating abdominal surgery in the patients were adhesive ileus in 9 cases, superior mesenteric artery thrombosis in 8 cases, internal herniation of bowel in 5 cases, traumatic bowel injury in 3 cases, and ischemic enteritis in 3 cases. Following medical and surgical rehabilitation, 10 patients (35.7%) were weaned off PN and overall mortality was 28.5%. Deaths were related to progression of underlying malignancies in 4 cases, liver failure in 3 cases, and sepsis in 1 case. Thirteen patients underwent surgery to restore bowel continuity. Six postoperative complications occurred in 4 patients (30.7%) and there were no postoperative mortalities. Conclusion Standardized care including restorative surgery resulted in successful outcomes in type II IF patients in this cohort.