Achieving Digestive Autonomy and Gastrointestinal Continuity in a Patient with Short Bowel Syndrome Secondary to Concomitant Jejunal Atresia and Small Intestinal Hirschsprung's Disease.

Concomitant presentation of jejunal atresia and Hirschsprung's disease is rare and places children at high risk for developing short bowel syndrome and parenteral nutrition dependence, which can affect the feasibility/timing of pull-through. A patient was born with jejunal atresia with a delayed diagnosis of Hirschsprung's disease. After several procedures and bowel resections, the patient was ultimately left with an end jejunostomy and long Hartman's pouch with short bowel syndrome, dependent on parenteral nutrition. The patient initially presented to our institution at age 2 with failure to thrive secondary to an obstructed/dilated jejunostomy and mild enterocolitis of their defunctionalized segment. The patient subsequently underwent completion of subtotal colectomy and revision of jejunostomy utilizing a serial transverse enteroplasty to manage the dilated bowel and gain length. The patient was able to wean off parenteral nutrition and achieve nutritional autonomy by age 5. Following this, the patient was able to undergo an ileoanal pull-through. After the pull-through, the patient was able to pass stool independently and suffered no major complications to date. Serial transverse enteroplasty can be successfully utilized in patients with a history of Hirschsprung's disease and jejunal atresia to achieve nutritional autonomy and ultimately reestablish gastrointestinal continuity with pull-through.

When parenteral nutrition is the answer: The case of pediatric intestinal rehabilitation.

In pediatric patients with intestinal failure, parenteral nutrition is lifesaving but also has several associated risks. The goals of intestinal rehabilitation include promoting growth, minimizing complications associated with intestinal failure, and reaching enteral autonomy, if possible. Pediatric intestinal rehabilitation programs are interdisciplinary teams that strive to provide optimal care for children dependent on parenteral nutrition. The provision of parenteral nutrition requires close monitoring of patients' growth, nutrition concerns, clinical status, and laboratory parameters. Recent advances in the field of intestinal rehabilitation include new lipid emulsions, considerations regarding enteral feeding, advances in micronutrient provision, and central venous catheter preservation techniques. Challenges in the field remain, including improving overall quality of life with home parenteral nutrition administration and preventing recently recognized complications such as chronic intestinal inflammation.

Quality of life from childhood to adulthood: Perspectives from adult patients with pediatric-onset intestinal failure.

Adult patients with pediatric onset short bowel syndrome (SBS) or intestinal failure (IF) have been described as a distinct population warranting further research. The aim of this exploratory study aimed was to offer initial insights into this population's navigation of childhood, adolescence, and transition into adulthood. Both quantitative and qualitative data were collected from a convenience sample of adults with pediatric-onset SBS/IF using a disease-specific pilot survey; 14 questionnaires were completed. Responses indicated childhood and adulthood were complex and marked by joys and trials, while adolescence was experienced by many as a particularly challenging time. As adults, numerous patients experienced barriers to accessing the medical care they desired and described difficulties finding experienced and knowledgeable providers who listened and offered individualized care. This study highlights the importance of further studying this unique patient population, suggesting it can offer critical insights to inform the development of interventions and transition programs.

Ileal lengthening through internal distraction: A novel procedure for ultrashort bowel syndrome.

Purpose: Ultrashort bowel syndrome is a rare, but morbid surgical problem without effective treatment. Recent clinical analysis has demonstrated the critical influence of ileal length on ultimate enteral autonomy. Surgical techniques to increase ileal length in nondilated bowel do not exist. We describe a novel technique to lengthen ileum in children with ultrashort bowel syndrome. Methods: Beginning in May 2021 prospective candidate children were identified. Candidacy for ileal tube lengthening included diagnosis of ultrashort bowel syndrome, intact ileocecal valve with remnant ileum, and proximal intestinal stoma or draining gastrostomy. Informed consent was obtained. Following laparoscopic lysis of adhesions, a balloon catheter was inserted through a left flank stab incision and into the lumen of the remnant ileum around a purse string suture. Cecopexy was performed in the right-lower quadrant. Clips were used to mark the cecum and the proximal extent of ileum. The catheter length was fixed externally at the completion of the procedure. Serial x-rays were used to measure distraction effect while increasing tension was applied to the catheter over the subsequent weeks. Ileal tube lengthening was performed until the end of the catheter was reached or the tube was dislodged. A contrast study was performed at the completion of lengthening. Intestinal length at time of restoration of continuity and clinical outcomes were recorded. Results: Four infants were enrolled from May 2021-July 2023. Diagnoses leading to ultrashort bowel syndrome were mesenteric teratoma, necrotizing enterocolitis, and multiple intestinal atresia. At the time of restoration of intestinal continuity, a median of 1.75 cm (45 %) additional ileal length was achieved at a median of 25.5 days. There were no serious complications following ileal tube lengthening and no additional operative interventions were required. Conclusions: Ileal lengthening through internal distraction is a feasible surgical intervention to salvage ileum for infants with ultrashort bowel syndrome. Ileal tube lengthening may result in distraction enterogenesis, providing a novel intervention to increase intestinal length. Level of evidence: IV (Case series without comparison group).

Micronutrient supplementation in patients with malabsorptive conditions.

Patients with malabsorptive conditions can develop micronutrient deficiencies, even if they receive vitamins, minerals, or trace elements through their enteral or parenteral nutrition. Additionally, clinicians have faced challenges with micronutrient supplementation during parenteral product shortages and when transitioning patients from parenteral to enteral/oral nutrition. Evaluating micronutrient deficiencies through laboratory markers has various limitations, including that many are acute phase reactants, may not reflect storage status, or may not be readily available in clinical practice. Furthermore, clinicians can become overwhelmed with the variety of vitamin and mineral products available, the differences in dosages and ingredients in these products, and lastly, the inherent challenges associated with an impaired gastrointestinal tract. The current review will discuss some challenges clinicians may encounter in clinical practice during the evaluation, assessment, and prescription of micronutrient supplementation in patients with malabsorptive conditions.

Totally thoracoscopic atrial fibrillation surgery following massive small bowel resection due to superior mesenteric artery embolization: report of two cases.

BACKGROUND: Thromboembolic occlusion of the superior mesenteric artery (SMA) is a grave complication in individuals diagnosed with atrial fibrillation (AF). This condition often necessitates extensive bowel resection, culminating in short bowel syndrome, which presents challenges for anticoagulant administration and/or antiarrhythmic therapy. CASE PRESENTATION: Presented here are findings of two patients, aged 78 and 72 years, respectively, who underwent comprehensive thoracoscopic AF surgery subsequent to extensive small bowel resection following SMA embolization. In each, onset of AF precipitated an embolic event, while the concurrent presence of short bowel syndrome complicated anticoagulation management. Total thoracoscopic AF surgery, comprised stapler-closure of the left atrial appendage (LAA) and bilateral epicardial clamp-isolation of the pulmonary veins, an operative modality aimed at addressing AF rhythm control and mitigating embolic events such as cerebral infarction, led to favorable outcomes in both cases. Additionally, computed tomography (CT) conducted one month post-surgery revealed the absence of residual tissue in the LAA, with the left atrium demonstrating a well-rounded, spherical shape. At the time of writing, the patients have remained asymptomatic following surgery regarding thromboembolic and arrhythmic manifestations for 29 and 10 months, respectively, notwithstanding the absence of anticoagulant or antiarrhythmic pharmacotherapy. Additionally, electrocardiographic surveillance has revealed persistent sinus rhythm. CONCLUSIONS: The present findings underscore the feasibility and efficacy of a total thoracoscopic AF surgery procedure for patients presented with short bowel syndrome complicating SMA embolization, thus warranting consideration for its broader clinical application.

A Systematic Review of Quality of Life in Patients with Short Bowel Syndrome and Their Caregivers.

Purpose: Understanding the quality of life and the factors that influence it for patients with short bowel syndrome (SBS) and their caregivers is of utmost importance in order to enhance their well-being. Therefore, This study aimed to provide a comprehensive understanding of the impact of SBS on patients and their caregivers, as well as its associated factors, by synthesizing the available evidence. Methods: A systematic review of the literature was done using PubMed, Embase databases, CNKI, and ISPOR conference papers. Included articles were manually searched to identify any other relevant studies. Quality was assessed using appropriate Joanna Briggs Institute critical appraisal tools. Results: This review included 16 studies, comprising 15 observational studies and 1 randomized controlled trial. The findings revealed that the QoL of patients with SBS was lower than that of the general population regarding physical functioning and psychological domain. Meanwhile, caregivers experienced challenges in maintaining their QoL. The QoL of SBS patients was found to be influenced by various factors such as treatment, age, sex, stoma, and small intestine length. Among them, the treatment is the most noteworthy factor that can be effectively improved through external interventions. Conclusion: While numerous studies have provided insights into the compromised QoL experienced by individuals with SBS and their caregivers, there remains a scarcity of large-sample quantitative investigations examining the determinants of QoL. The existing body of literature on caregivers is also notably deficient.

Obeticholic acid attenuates the intestinal barrier disruption in a rat model of short bowel syndrome.

BACKGROUND: Short bowel syndrome (SBS) features nutrients malabsorption and impaired intestinal barrier. Patients with SBS are prone to sepsis, intestinal flora dysbiosis and intestinal failure associated liver disease. Protecting intestinal barrier and preventing complications are potential strategies for SBS treatment. This study aims to investigate the effects of farnesoid X receptor (FXR) agonist, obeticholic acid (OCA), have on intestinal barrier and ecological environment in SBS. METHODS AND RESULTS: Through testing the small intestine and serum samples of patients with SBS, impaired intestinal barrier was verified, as evidenced by reduced expressions of intestinal tight junction proteins (TJPs), increased levels of apoptosis and epithelial cell damage. The intestinal expressions of FXR and related downstream molecules were decreased in SBS patients. Then, global FXR activator OCA was used to further dissect the potential role of the FXR in a rat model of SBS. Low expressions of FXR-related molecules were observed on the small intestine of SBS rats, along with increased proinflammatory factors and damaged barrier function. Furthermore, SBS rats possessed significantly decreased body weight and elevated death rate. Supplementation with OCA mitigated the damaged intestinal barrier and increased proinflammatory factors in SBS rats, accompanied by activated FXR-related molecules. Using 16S rDNA sequencing, the regulatory role of OCA on gut microbiota in SBS rats was witnessed. LPS stimulation to Caco-2 cells induced apoptosis and overexpression of proinflammatory factors in vitro. OCA incubation of LPS-pretreated Caco-2 cells activated FXR-related molecules, increased the expressions of TJPs, ameliorated apoptosis and inhibited overexpression of proinflammatory factors. CONCLUSIONS: OCA supplementation could effectively ameliorate the intestinal barrier disruption and inhibit overexpression of proinflammatory factors in a rat model of SBS and LPS-pretreated Caco-2 cells. As a selective activator of FXR, OCA might realize its protective function through FXR activation.

Emerging drugs for the treatment of short bowel syndrome.

INTRODUCTION: SBS is a rare and disabling condition. The standard management is based on diet optimization with parenteral supplementation. In addition, glucagon-like peptide-2 (GLP-2)analogs, have shown promising results as disease-modifying therapies for SBS. AREAS COVERED: Short bowel syndrome (SBS) is defined as a reduction in functional intestinal length to less than 200 cm, leading to intestinal failure (IF) leading to malnutrition and parenteral support dependency. This review discusses the current management of SBS-CIFpatients, the place of GLP-2 analog treatment in terms of efficacy, safety and availability, and the new perspectives opened by the use of enterohormones. EXPERT OPINION: Clinical trials and real-world experience demonstrated that Teduglutide   reduces dependence on parenteral support and has a place in the management of patients with SBS-CIF.  The use of Teduglutide should be discussed in patients stabilized after resection and its introduction requires the advice of an expert center capable of assessing the benefit-risk ratio. The complex, individualized management of SBS-C IF requires theexpertise of a specialized IF center which a multidisciplinary approach. The arrival of new treatments will call for new therapeutic strategies, and the question of how to introduce and monitor them will represent a new therapeutic challenge.

Effects of glutamine and omega-3 fatty acids on intestinal neomucosa formation on colon serosa in rats.

Background/aim: Intestinal neomucosa formation is a technique defined for the treatment of short bowel syndrome. This study evaluates the effect of glutamine and omega-3 fatty acids on the growth of intestinal neomucosa on the colonic serosal surface has been evaluated. Materials and methods: Thirty-two adult male Sprague-Dawley rats were randomly divided into 4 groups: sham, control, glutamine, and omega-3. Laparotomy was performed on all groups. For rats other than the sham group, a 1-cm full-thickness incision was made 4 cm proximal to the ileocecal valve, and colonic serosal surface was sutured as a serosal patch over these openings. By using the oral gavage technique, the glutamine group was ingested with 200 mg/kg/day of glutamine, and the omega-3 group was ingested with 100 mg/kg/day of omega-3 fatty acids. At the end of 14 days, the rats were euthanized, blood specimens were collected, and intestinal segments, including serosal patches, were excised. Results: Transforming growth factor-beta was significantly lower in the glutamine group compared to the control group. Similarly, fibroblast growth factor-2 was significantly lower in the glutamine group compared to the sham group. Intestinal neomucosa formation was observed in 100% of rats in the glutamine group. In the control and omega-3 groups, intestinal neomucosa formation was observed in 57.1% and 60% of rats, respectively. The inflammatory response, granulation tissue formation, and fibroblastic activity were more severe in the rats of the glutamine and omega-3 groups. Conclusion: The intestinal neomucosa formation is an experimental technique, and both glutamine and omega-3 fatty acids have the potential to positively affect inflammatory response, granulation tissue formation, and fibroblastic activity. Specifically, glutamine has a favorable effect on intestinal neomucosa formation.

Life on the line - Incidence and management of central venous catheter complications in intestinal failure.

BACKGROUND & AIMS: Loss of venous access is threatening for patients with intestinal failure (IF) under long-term parenteral nutrition (PN). We aimed to identify the incidence of central venous catheter (CVC) complications, compare different devices, and analyze interventional recanalizing procedures to restore the patency of occluded CVCs. METHODS: For this retrospective cohort study, patient data from a prospective IF database spanning 16 years was analyzed at a tertiary referral center. Catheter dwell times (CDTs) were distinguished by Kaplan-Meier survival analysis and subgroup analyses were performed for different CVC types (tunneled/port catheters). Specific complications (occlusion, catheter-related infection (CRI), displacement, and material defect) were analyzed. Explantation rates and CDTs were compared. RESULTS: Overall, 193 CVCs in 77 patients with IF under PN could be enrolled (62.524 "CVC-days"). Broviac type "B" was found to be significantly superior to type "A" regarding occlusion, CRI, and material defects (log-rank test: p = 0.05; p = 0.026; p = 0.005 respectively). Port catheters were displaying the highest incidence of CRI (2.13 events/1000 catheter days). Interventional catheter recanalization was performed 91 times and significantly increased the CDT from a median of 131 days (IQR: 62; 258) to 389 days (IQR: 262; 731) (Mann-Whitney-U-test: p= <0.001) without increasing complications. CONCLUSIONS: Different complication rates and CDT were seen depending on CVC type. Tunneled catheters were significantly superior concerning CRI. Interventional catheter recanalization is a viable alternative to fibrinolytics to restore CVC patency, but long-term patency data is scarce.

Quality of Life of Children with Short Bowel Syndrome from Patients' and Parents' Points of View.

Despite limited research, existing studies using generic quality of life (QOL) tools indicate decreased physical health and compromised emotional functioning in children with IF. This study investigates QOL in children with short bowel syndrome (SBS) and its determinants. The study included 57 pediatric patients with SBS treated at Mannheim's University Hospital between 1998 and 2014. To evaluate QOL, the KINDL questionnaire was used. Three age-specific questionnaire variants were employed, and parental proxy reports were collected. Most patients underwent intestinal lengthening procedures, with varying primary diagnoses. A comparison with healthy children from the patient's perspective revealed no difference but from the parent's perspective showed lower QOL in SBS patients, especially regarding physical and mental well-being. QOL varied with age, with 7-10-year-olds reporting the lowest scores. Several factors, including independence from parenteral nutrition and the presence of a complete colon, positively influenced QOL. The independence of parenteral nutrition and the presence of a complete colon positively influenced QOL. The Bianchi technique for intestinal lengthening has also shown promise but needs further research. The observation sample in this study is too small to generalize about the whole population of SBS patients. However, this study shows that many health and treatment factors affect QOL, and a large multicenter study is necessary. Our findings underline the importance of appropriate psychological support for children with SBS and their families.

Cholecystitis following the initiation of glucagon-like peptide-2 analogue for short bowel syndrome: A case report.

Patients with short bowel syndrome (SBS) have a risk for cholelithiasis and cholecystitis, particularly those who have received long-term parenteral nutrition (PN). Teduglutide (Revestive), a glucagon-like peptide-2 (GLP-2) analogue, is the first effective therapy approved for treating patients with SBS via self-subcutaneous injection. It also pharmacologically inhibits gallbladder contraction, which may increase the risks for cholelithiasis and cholecystitis. Here, we report a case of cholecystitis occurring after the introduction of a GLP-2 analogue in a patient with SBS and cholelithiasis. A 16-year-old girl, with a residual intestinal anatomy of 5 cm jejunum and left colon, was referred to our hospital for further treatment of SBS. She underwent jejunocolic anastomosis 2 months later. After that, she received PN for 2.5 years. Teduglutide treatment was initiated to reduce PN dependence. Several asymptomatic gallbladder stones were found during a routine ultrasound examination before drug initiation. On day 31 of teduglutide treatment, right subcostal pain with fever occurred, and the patient was diagnosed with acute cholecystitis. GLP-2 analogue treatment was temporarily discontinued. The patient underwent gallbladder drainage followed by cholecystectomy 3 weeks later. Histopathological findings illustrated mucosal hyperplasia of the gallbladder. Her postoperative course was uneventful, and teduglutide was restarted 2 weeks postoperatively. GLP-2 analogues promote gallbladder refilling and epithelial hyperplasia, which may be a risk factor for cholecystitis in patients with cholelithiasis, as observed in our patient. Based on our experience, patients with SBS and established asymptomatic cholelithiasis may be considered for prophylactic cholecystectomy before the administration of GLP-2 analogues.

Gut instinct: Navigating the landscape of parenteral support in short bowel syndrome.

Depending on the remaining bowel anatomy and the degree of bowel adaptation, patients with short bowel syndrome (SBS) may require parenteral nutrition (PN) and/or intravenous fluid support, sometimes temporarily and sometimes permanently. Although the use of parenteral support in SBS is often lifesaving, it is not without its limitations. Herein, we undertake a focused review of several issues related to use of parenteral support in patients with SBS, including initiation of parenteral support, considerations when formulating PN, select complications, short-term and long-term nutrition monitoring, and weaning strategies.

Strategies to Promote Success in Oral Feedings in Infants and Children with Intestinal Failure due to Short Bowel Syndrome.

Infants and children with intestinal failure are at risk for pediatric feeding disorders, which challenge their oral feeding development. This article explores these challenges and offers several practical strategies that can be used by multidisciplinary care teams and at-home caregivers to help support the development of oral feeding in these children and eventually lead to their attaining enteral autonomy.

No safe time window in malrotation and volvulus: A consecutive cohort study.

BACKGROUND: Malrotation and volvulus classically present with bilious vomiting. It is more common earlier in life, but there are other causes of bile-stained vomiting. This leads some clinicians to 'watch and wait'. In the presence of a volvulus, this is potentially a fatal decision. It is not clear from the literature if there is a safe time window in which children can be observed in the hope of avoiding transfers or radiological investigations. AIM: To determine whether time to identification and management of midgut volvulus correlated with morbidity and mortality; and whether there were patterns to transition of care. METHODS: Multicentre, retrospective review of all children with malrotation ± volvulus at two tertiary children's hospitals in Brisbane from 2000 to 2012. Data collected included age at presentation, timing between symptom onset and presentation, radiological findings, and definitive surgical management. Outcomes included patient length of stay (LOS), total parenteral nutrition (TPN) duration, re-operations and death. RESULTS: There were 96 cases of malrotation identified, with 23 excluded (elective operation, insufficient data). Neonates made up 66% of included cases. Only 14% of cases were over 12 months old. Bilious vomiting or bile-stained aspirates were the presenting symptoms in 71% (52). Overall mortality was 5.56%. Time from symptom onset to presentation or management was not significantly associated with morbidity or mortality. More than half (53%, 39/73) of patients received total parenteral nutrition; 20/39 for more than 10 days. Neonates and infants had a significantly higher rate of TPN compared with older children (P < 0.001). Those requiring TPN post-operatively had a significantly higher mortality compared with those who did not (P = 0.02). Time from symptom onset to presentation or definitive management was not significantly associated with LOS, TPN duration, or need for re-operation. CONCLUSION: Malrotation remains a time-critical diagnosis to secure and treat. Even a short duration of symptoms can be associated with high morbidity or mortality. There is no place for 'watch and wait' for such patients, and malrotation/volvulus should be emergently actively excluded with contrast studies.

Translation into Brazilian Portuguese and transcultural adaptation and validation of the treatment specific questionnaire Home Parenteral Nutrition- Quality of Life (HPN-QOL©).

BACKGROUND & AIMS: Home Parenteral Nutrition (HPN) is the main treatment for patients with chronic intestinal failure. It is commonly prescribed for nutritional recovery, survival increase and, whenever possible, improvement of quality of life. As there are no validated instruments in Brazilian Portuguese to be used in these patients, the objective of this study was to carry out the transcultural adaptation and validation of Home Parenteral Nutrition - Quality of life (HPN-QOL©) into Brazilian Portuguese. METHODS: This observational and cross-sectional study was conducted at the multidisciplinary short-bowel syndrome clinic (AMULSIC) of the Hospital das Clínicas of the University of São Paulo Medical School (HC-FMUSP). A five-stage protocol was adopted for the transcultural adaptation: initial translation; synthesis; reverse translation; experts committee and pre-test. The adapted questionnaire was applied to a convenience (representative) sample (n = 16) and Cronbach's Alpha Coefficient, Intraclass Correlation Coefficient (ICC), and Bland-Altman Test were submitted for the analysis of the internal consistency and intraobserver and interobserver reproducibility. RESULTS: The transcultural adaptation was considered excellent (Content Validity Index = 100%). The internal consistency was satisfactory for most of the scales (16/19), and α > 0.70 was 84.21%. ICC values revealed high intraobserver and interobserver reproducibility in most of the scales. No significant difference was observed between intraobservers and interobservers in any of the questions (p > 0.05). CONCLUSIONS: The questionnaire was shown as adapted and valid for use in Brazil. Future trials with a higher sample are yet to be developed to shed light on specific scales that were inconsistent. It's expected that this would contribute for the usual quality of life assessment for individuals treated with HPN in Brazil.

Factores asociados al desenlace en niños con mal rotación intestinal manejados en el Hospital Infantil de San Vicente Fundación, Medellín, Colombia / Factors associated with outcome in children with intestinal malrotation managed at the Hospital Infantil of San Vicente Fundación, Medellín, Colombia

Introducción. El intestino primitivo rota durante la vida embrionaria. Cuando ocurre de forma inadecuada aparece la malrotación intestinal que puede llevar a la obstrucción o al vólvulo del intestino medio. La incidencia disminuye con el aumento de la edad. La malrotación intestinal es una de las principales causas de complicaciones del tracto gastrointestinal en la edad pediátrica. Métodos. Estudio retrospectivo, observacional, transversal y analítico, de la experiencia durante 10 años en pacientes menores de 15 años con diagnóstico de malrotación intestinal, tratados en el Hospital Infantil de San Vicente Fundación, en Medellín, Colombia. Se buscó la asociación entre variables demográficas, clínicas e imagenológicas con los desenlaces. Resultados. Se obtuvieron 58 pacientes con malrotación intestinal, 65 % menores de 1 año. En 29,3 % de los pacientes se hizo el diagnóstico con la presentación clínica; los síntomas predominantes fueron distensión abdominal y emesis. En el 24,1 % se confirmó el diagnóstico con imágenes. Las variables con una diferencia estadísticamente significativa a favor de encontrar una malrotación complicada fueron choque séptico (OR=11,7), síndrome de respuesta inflamatoria sistémica (OR=8,4) y deshidratación (OR=5,18). Conclusiones. La malrotación intestinal tiene complicaciones como perforación, peritonitis, vólvulo e intestino corto. El vólvulo se acompaña de shock y sepsis, con mortalidad hasta del 50 %. Las imágenes diagnósticas son una ayuda, pero no se puede basar la conducta médica en estas porque ninguna imagen garantiza el diagnóstico definitivo. Los signos de alarma son poco específicos. En menores de un año con emesis, distensión y dolor abdominal se debe sospechar malrotación intestinal.

Introduction. The primitive intestine rotates during embryonic life. When it occurs inappropriately, intestinal malrotation appears, which can lead to obstruction or midgut volvulus. The incidence decreases when age increases. Intestinal malrotation is one of the main causes of complications of the gastrointestinal tract in pediatric age. Method. Retrospective, observational, cross-sectional and analytical study of the experience over 10 years in patients under 15 years of age with a diagnosis of intestinal malrotation, treated at Hospital Infantil of San Vicente Fundación, in Medellín, Colombia. The association between demographic, clinical and imaging variables with the outcomes was sought. Results. There were 58 patients with intestinal malrotation, 65% under one year of age. In 29.3% of patients, intestinal malrotation was diagnosed clinically. The predominant symptoms were abdominal distension and emesis. In 24.1% the diagnosis was confirmed with imaging. The variables with a statistically significant difference in favor of finding a complicated malrotation were septic shock (OR=11.7), systemic inflammatory response syndrome (OR-8.4), and dehydration (OR=5.18). Conclusions. Malrotation has complications such as perforation, peritonitis, volvulus, and short bowel. Volvulus is accompanied by shock and sepsis, with mortality of up to 50%. Diagnostic images are helpful, but medical conduct cannot be based on them because no image guarantees a definitive diagnosis. The warning signs are not very specific. In children under one year of age with emesis, distension and abdominal pain, intestinal malrotation should be suspected.

Evaluation of the Effectiveness of Teduglutide Treatment in Patients with Short Bowel Syndrome in Slovakia-Multicenter Real-World Study.

(1) Background: We present the first real-world-data study on teduglutide-treated SBS patients in the Slovak Republic and the first study to enable the comparison of the effects of teduglutide treatment between the adult and pediatric populations. (2) Methods: This was a non-interventional retrospective cohort study of adult and pediatric SBS patients treated with teduglutide. Primary and secondary endpoints were the results of teduglutide use at 12 weeks and 6 months after the initiation of treatment, compared to baseline. (3) Results: Teduglutide treatment led to a statistically significant reduction in the volume of intravenous hydration, HPN caloric intake, HPN and intravenous hydration applications per week and to increased urine output in adult patients. The results in the pediatric population were similar, but not statistically significant. A complete weaning off HPN was achieved in 57.14% of all patients (50.00% of children; 62.50% of adults) after a median of 0.99 years of teduglutide treatment (1.07 and 0.98 years for children and adults, respectively). (4) Conclusions: Teduglutide treatment in SBS patients leads to considerable reduction in or even weaning off PN in both pediatric and adult patients.

The gut microbiota in adults with chronic intestinal failure.

OBJECTIVE: Fecal microbiota was investigated in adult patients with chronic intestinal failure (CIF) due to short bowel syndrome (SBS) with jejunocolonic anastomosis (SBS-2). Few or no data are available on SBS with jejunostomy (SBS-1) and CIF due to intestinal dysmotility (DYS) or mucosal disease (MD). We profiled the fecal microbiota of various pathophysiological mechanisms of CIF. METHODS: Cross-sectional study on 61 adults with CIF (SBS-1 30, SBS-2 17, DYS 8, MD 6). Fecal samples were collected and profiled by 16S rRNA amplicon sequencing. Healthy controls (HC) were selected from pre-existing cohorts, matched with patients by sex and age. RESULTS: Compared to HC, SBS-1, SBS-2 and MD patients showed lower alpha diversity; no difference was found for DYS. In beta diversity analysis, SBS-1, SBS-2 and DYS groups segregated from HC and from each other. Taxonomically, the CIF groups differed from HC even at the phylum level. In particular, CIF patients' microbiota was dominated by Lactobacillaceae and Enterobacteriaceae, while depleted in typical health-associated taxa belonging to Lachnospiraceae and Ruminococcaceae. Notably, compositional peculiarities of the CIF groups emerged. Furthermore, in the SBS groups, the microbiota profile differed according to the amount of parenteral nutrition required and the duration of CIF. CONCLUSIONS: CIF patients showed marked intestinal dysbiosis with microbial signatures specific to the pathophysiological mechanism of CIF as well as to the severity and duration of SBS.