From intestinal failure to transplantation: Review on the current need for transplant indications under multidisciplinary transplant programs worldwide.

INTRODUCTION: Intestinal failure, defined as the loss of gastrointestinal function to the point where nutrition cannot be maintained by enteral intake alone, presents numerous challenges in children, not least the timing of consideration of intestine transplantation. OBJECTIVES: To describe the evolution of care of infants and children with intestinal failure including parenteral nutrition, intestine transplantation, and contemporary intestinal failure care. METHODS: The review is based on the authors' experience supported by an in-depth review of the published literature. RESULTS: The history of parenteral nutrition, including out-patient (home) administration, and intestine transplantation are reviewed along with the complications of intestinal failure that may become indications for consideration of intestine transplantation. Current management strategies for children with intestinal failure are discussed along with changes in need for intestine transplantation, recognizing the difficulty in generalizing recommendations due to the high level of heterogeneity of intestinal pathology and residual bowel anatomy and function. DISCUSSION: Advances in the medical and surgical care of children with intestinal failure have resulted in improved transplant-free survival and a significant fall in demand for transplantation. Despite these improvements a number of children continue to fail rehabilitative care and require intestine transplantation as life-saving therapy or when the burden on ongoing parenteral nutrition becomes too great to bear.

Parenteral nutrition: a life-saving intervention for 4 months in short bowel syndrome-a case report and review of the literature.

BACKGROUND: Short bowel syndrome (SBS) in adults is defined as having less than 180 to 200 cm of remaining small bowel. Many literature sources do not provide precise epidemiological data, and challenges in estimating the prevalence of SBS include its multifactorial etiology and varying definitions. The most common pathologies leading to SBS include Crohn disease, mesenteric ischemia, radiation enteritis, post-surgical adhesions, and post-operative complications. CASE PRESENTATION: This article presents a clinical case of a 76-year-old Lithuanian patient who underwent parenteral nutrition for four months due to SBS. Before the following diagnosis, the patient had undergone two surgeries. During the hospitalization, life-threatening conditions such as stercoral peritonitis, septic shock, and acute respiratory failure, were observed and treated. As a result of SBS, hypoproteinemia and hypoalbuminemia developed, leading to the prescription of full parenteral nutrition. After correcting the malnutrition, a third surgery was performed, resulting in the discontinuation of parenteral nutrition and the resumption of a regular diet. CONCLUSIONS: Parenteral nutrition is the sole effective method for preserving the lives of patients with a short segment of the intestine. While on parenteral nutrition, patients can be prepared for reconstructive surgery.

[Nutritional support treatment for short bowel syndrome related intestinal failure].

Intestinal failure is a syndrome characterized by a diminished intestinal function that is inadequate to maintain normal digestion and absorption, leading to systemic metabolic disorder and requiring long-term nutritional supplementation to sustain health and growth. Short bowel syndrome (SBS) is one of the primary causes of intestinal failure. Given the significant differences among SBS patients, nutritional treatment strategies should emphasize individualization. This review focuses on SBS, combining its anatomical and pathological characteristics, to introduce nutritional support treatment plans and experiences for patients with intestinal failure.

Usefulness and description of the intestinal bypass technique in children with short bowel syndrome: report of a Mexican cohort.

BACKGROUND: Short bowel syndrome (SBS) is one of the most frequent causes of intestinal failure, needing parenteral nutrition to maintain an energy-protein and water-electrolyte balance. At the Hospital Infantil de México Federico Gómez (HIMFG), the formation of two stomas is a technique used for intestinal rehabilitation, where the use of residue through the bypass technique (BT) helps to maintain gastrointestinal functionality, water-electrolyte, and nutritional stability. This study aimed to describe the technique of using intestinal residue through BT as a treatment strategy in intestinal rehabilitation and its effect on the biochemical and nutritional status of pediatric patients with SBS. METHODS: An analytical and retrospective cross-sectional study was performed in patients hospitalized at HIMFG with SBS who underwent BT during their hospital stay between 2019 and 2020 and then followed up for 8 weeks. RESULTS: A total of 10 patients were included in this study, with a mean age of 24 months; 50% were female. BT was able to reduce the inflammatory process in the liver caused by the continuous use of parenteral nutrition; enteral caloric intake increased from 25.32 kcal/kg/day to 72.94 kcal/kg/day, but it was insufficient to improve their nutritional status. CONCLUSIONS: BT is a safe and effective alternative in intestinal rehabilitation in patients with SBS to stimulate trophism and intestinal functionality, allowing a progression of enteral feeding and a decrease in the hepatic inflammatory process that occurs in these patients with prolonged parenteral nutrition.

INTRODUCCIÓN: El síndrome de intestino corto (SIC) es una de las causas más frecuentes de insuficiencia intestinal que requiere del uso de nutrición parenteral para mantener un balance energético-proteico e hidroelectrolítico. En el Hospital Infantil de México Federico Gómez (HIMFG) la formación de dos estomas es una técnica empleada para la rehabilitación intestinal, donde con el aprovechamiento de residuo mediante la técnica de puenteo (TP) se ayuda a mantener la funcionalidad gastrointestinal, equilibrio hidro-electrolítico y estabilidad nutricional. El objetivo de este estudio fue describir la técnica del aprovechamiento de residuo intestinal mediante TP como estrategia de tratamiento en la rehabilitación intestinal y su efecto en el estado bioquímico y nutricional de pacientes pediátricos con SIC. MÉTODOS: Se llevó a cabo un estudio transversal analítico y retrospectivo en pacientes hospitalizados en el HIMFG con SIC en quienes se realizó la TP durante su estancia intrahospitalaria entre 2019 y 2020. RESULTADOS: Se incluyeron 10 pacientes en este estudio, con una edad promedio de 24 meses, y el 50% de sexo femenino. La TP logró disminuir el proceso inflamatorio hepático ocasionado por el uso continuo de nutrición parenteral; la ingesta calórica por vía enteral incrementó de 25.32 kcal/kg/día a 72.94 kcal/kg/día, pero fue insuficiente para mejorar el estado nutricional. CONCLUSIONES: La TP es una alternativa segura y efectiva en la rehabilitación intestinal en pacientes con SIC para estimular el trofismo y funcionalidad intestinal, permitiendo una progresión de la alimentación enteral y disminución del proceso inflamatorio hepático que se presentan en estos pacientes con nutrición parenteral prolongada.

A case report of lactobacillus bacteremia in a patient on chronic parenteral nutrition.

BACKGROUND AND AIMS: Short bowel syndrome is a malabsorption disorder typically caused by the physical loss of a portion of the intestine, whereafter the body is unable to adequately absorb nutrients, fluids, and electrolytes. Many patients with short bowel syndrome are reliant on home parenteral nutrition through a tunneled or peripherally inserted central catheter to ensure sufficient hydration and nutrition. Central venous catheters are a nidus for bacteria, and patients are at risk for infections associated with high levels of morbidity and mortality. Lactobacillus is a ubiquitous microorganism that most frequently colonizes mucosal surfaces such as the gastrointestinal tract. Lactobacillus bacteremia is rare, with limited occurrence in current medical literature. METHODS: Our patient is a 60-year-old female with a past medical history significant for multiple abdominal surgeries resulting in short bowel syndrome, with subsequent dependence on home parenteral nutrition via peripherally inserted central catheter. She had type III chronic intestinal failure, category D2, and stage 1 moderate malnutrition. She was originally admitted to the hospital for a presumed pulmonary embolism and was found to have a deep vein thrombosis in the setting of her peripherally inserted central catheter. On admission her abdominal exam was unremarkable, she denied abdominal pain, and her only gastrointestinal complaint was chronic stable diarrhea. During the hospitalization she developed severe left lower quadrant abdominal pain and noted decreased frequency of her bowel movements. A computed tomography scan of her abdomen revealed chronic stable intestinal distension and was concerning for obstruction. Clinically she remained without symptoms of acute obstruction or ileus. During the admission she became febrile, with blood cultures from her peripherally inserted central catheter and peripheral IV growing out gram negative rods determined to be lactobacillus bacteremia. The infectious disease team recommended removal of her peripherally inserted central catheter given their concern for a line infection. RESULTS: The patient was treated with broad-spectrum antibiotics, did well clinically, and was ultimately discharged following reinsertion of her peripherally inserted central catheter after negative repeat blood cultures. Though she initially did well in the outpatient setting, she ultimately passed away ten months later after re-presenting to the hospital in septic shock, secondary to bowel ischemia and suspected fungemia of her peripherally inserted central catheter. CONCLUSIONS: In this case report, we describe an unusual case of a patient with short bowel syndrome on chronic parenteral nutrition who developed catheter-associated lactobacillus bacteremia - the first reported case in an adult patient on parenteral nutrition.

Assessment of clinical outcome after sudden alteration of home parenteral nutrition program from customised to commercially premixed admixtures. Experience with short bowel syndrome treatment.

BACKGROUND & AIMS: Disruptions in the home parenteral nutrition (HPN) process may lead to failure to achieve the intended treatment purposes. This study aimed to evaluate the mid-term clinical outcome in a group of patients with short bowel syndrome (SBS) after a sudden change in the type of home parenteral nutrition programs from customized to commercially premixed admixtures. METHODS: The study conducted in 2020 identified 51 patients with SBS: 27 (53%) women and 24 (47%) men. The SBS were classified as SBS with end jejunostomy 23 (45%) patients, with jejuno-colon anastomosis 23 (45%) patients, with jejuno-ileo anastomosis and total colon in continuity 5 (10%) patients. The following therapeutic program-related data were analysed: changes in nutritional status, body mass index (BMI), Controlling Nutritional Status (CONUT) score, and biochemical assessment. For statistical analysis, the Wilcoxon rank-sum and signed-rank paired tests with continuity corrections were used to compare the results. A p-value of <0.001 was considered statistically significant. RESULTS: There was no statistically significant difference between the analysed groups in total energy, amino acid concentrations, and intravenous volume supplementation. BMI and CONUT assessments of nutritional status and selected biochemical parameters were stable during the study period. CONCLUSIONS: The study demonstrated that a sudden change in the HPN therapy program from parenteral admixtures, tailored to meet individual patients' needs, to commercially premixed admixtures had no significant impact on the mid-term clinical condition of patients with SBS.

[Indications and results of intestinal transplantation for short bowel syndrome after mesenteric ischemia]. / Indikation und Ergebnisse der Darmtransplantation bei Kurzdarmsyndrom nach mesenterialer Ischämie.

BACKGROUND: Intestinal transplantation (ITx) is the only causal treatment for complicated chronic intestinal failure after mesenteric ischemia and impending failure of parenteral supplementation. Isolated or combined ITx with the inclusion of the intestine is associated with demanding immunological, perioperative and infection associated challenges. AIM: The characterization of chronic intestinal failure, the indications, transplant survival, transplantation techniques and success rates. MATERIAL AND METHODS: Collection, summary and critical appraisal of international guidelines, the guidelines of the German Medical Chamber, and the international literature. RESULTS: The first successful ITx were performed in 1987 and 1988 at the University of Kiel Germany and the University of Pittsburgh, USA. The number of ITx rose continuously but in phases from the end of the 1990s to over 200 per year but has currently decreased to 100-150 per year due to optimized intestinal rehabilitation. While the 1­year and 3­year transplant survival rates were 30% and 20% before 1991, they increased in phases up to 60% and 50%, respectively, after 1995 and have now achieved almost 80% and 70%, respectively. CONCLUSION: The substantial improvement in the results of ITx can be partly explained by progress in operative techniques, intensive care medicine and a better understanding of mucosal immunity; however, optimized strategies in immunosuppression as well as prevention of infectious diseases and malignancies have also made decisive contributions.

Skipped Aganglionic Lengthening Transposition (SALT) is highly effective to achieve enteral autonomy in selected patients with intestinal failure secondary to total intestinal aganglionosis.

BACKGROUND: Intestinal Failure, parenteral nutrition (PN) dependence, and subsequent liver disease are the most challenging and life-threatening complications of short bowel syndrome experienced by patients with total intestinal aganglionosis. Skipped Aganglionic Lengthening Transposition (SALT) showed to be a promising procedure to overcome such problems. We herein report the results of two patients who underwent SALT at the Umberto Bosio Center for Digestive Diseases. PATIENTS AND METHODS: Between November 2019 and July 2022, 2 patients with total intestinal aganglionosis underwent SALT as autologous intestinal lengthening procedure. Perioperative data and long-term outcomes are reported. Patient #1-A 18 month-old male (PN dependant) with 30 cm of ganglionated bowel at birth experienced a 35% increase of intestinal length after SALT (from 43 to 58 cm) thanks to three 5 cm interposed aganglionic loops. Postoperative course was uneventful and he was totally weaned by PN after 28 months postoperatively. He is without PN only receiving enteric feeding 53 months after the procedure. Patient #2-A 11 year-old female (PN dependant) with 100 cm of ganglionated jejunum underwent SALT at 11 years and experienced a 19% increase of bowel length thanks to four 5 to 7 cm interposed aganglionic loops. Postoperatively she required excision of two out of the four loops due to severe strictures and inadequate perfusion with a subsequent overall 10% increase of length after SALT. Of note, she improved significantly with a progressive reduction of PN that has been stopped after 18 months. CONCLUSION: Skipped aganglionic lengthening transposition (SALT) seems to be very effective in improving nutrients absorption in patients with total intestinal aganglionosis by increasing absorptive bowel surface and decelerating intestinal flow for a longer and more effective contact of enteric material with ileal mucosa. Provided these impressive results are confirmed in the very long-term, SALT could become a valid alternative for the treatment of patients with total intestinal aganglionosis carrying at birth at least 20 to 30 cm of ganglionated jejunum.

Fat malabsorption in short bowel syndrome: A review of pathophysiology and management.

Fat malabsorption is central to the pathophysiology of short bowel syndrome (SBS). It occurs in patients with insufficient intestinal surface area and/or function to maintain metabolic and growth demands. Rapid intestinal transit and impaired bile acid recycling further contribute to fat malabsorption. A significant portion of patients require parenteral nutrition (PN) for their survival but may develop sepsis and liver dysfunction as a result. Despite advancements in the treatment of SBS, fat malabsorption remains a chronic issue for this vulnerable patient population. Peer-reviewed literature was assessed on the topic of fat malabsorption in SBS. Current management of patients with SBS involves dietary considerations, PN management, antidiarrheals, glucagon-like peptide 2 agonists, and multidisciplinary teams. Clinical trials have focused on improving intestinal fat absorption by facilitating fat digestion with pancreatic enzymes. Targeting fat malabsorption in SBS is a potential pathway to improving lifestyle and reducing morbidity and mortality in this rare disease.

Magnifying Endoscopy With Narrow Band Imaging for Graft Failure and Disease Recurrence in Patients With Crohn Disease After Intestinal Transplantation: 2 Case Reports.

Crohn disease (CD) is one of the most common causes of short bowel syndrome and intestinal failure. Intestinal transplantation (IT) is sometimes needed for patients with CD who develop intestinal failure after multiple intestinal resections resulting from CD-related complications, such as uncontrollable bleeding and penetrating diseases. However, there have been few case reports concerning the endoscopic surveillance of patients with CD after IT. In this article, we present 2 patients with CD who underwent IT because of short bowel syndrome with intestinal failure. We administered posttransplantation immunosuppressants and conducted regular follow-up magnifying endoscopy with narrow-band imaging (ME-NBI). Both cases demonstrated favorable outcomes after surveillance with ME-NBI. In this report, we outline our post-IT follow-up strategies applying the VENCH scoring system, which is based on endoscopic features using ME-NBI to predict graft rejection. Our approach could effectively distinguish between acute cellular rejection and non-rejection, particularly disease recurrence of underlying CD. This study was approved by the institutional review board of Far Eastern Memorial Hospital (FEMH-105023-F). The patients provided written informed consent for publication.

Factors associated with D-lactic acidosis in pediatric intestinal failure: A case-control study.

BACKGROUND: D-lactic acidosis (DLA) is a serious complication of short bowel syndrome (SBS) in children with intestinal failure (IF). Malabsorbed carbohydrates are metabolized by bacteria in the intestine to D-lactate which can lead to metabolic acidosis and neurologic symptoms. METHODS: A retrospective chart review was performed in children ≤18 years old with SBS who had one of the following criteria: unexplained metabolic acidosis, neurologic signs or symptoms, history of antibiotic therapy for small bowel bacterial overgrowth, or high clinical suspicion of DLA. Cases had serum D-lactate concentration >0.25 mmol/L; controls with concentrations ≤0.25 mmol/L. RESULTS: Of forty-six children, median age was 3.16 (interquartile range (IQR): 1.98, 5.82) years, and median residual bowel length was 40 (IQR: 25, 59) cm. There were 23 cases and 23 controls. Univariate analysis showed that cases had significantly lower median bicarbonate (19 vs. 24 mEq/L, p = 0.001), higher anion gap (17 vs. 14 mEq/L, p < 0.001) and were less likely to be receiving parenteral nutrition, compared with children without DLA. Multivariable analysis identified midgut volvulus, history of intestinal lengthening procedure, and anion gap as significant independent risk factors. Midgut volvulus was the strongest independent factor associated with DLA (adjusted odds ratio = 17.1, 95% CI: 2.21, 133, p = 0.007). CONCLUSION: DLA is an important complication of pediatric IF due to SBS. Patients with IF, particularly those with history of midgut volvulus, having undergone intestinal lengthening, or with anion gap acidosis, should be closely monitored for DLA.

Dietary polyamines promote intestinal adaptation in an experimental model of short bowel syndrome.

Intestinal adaptation does not necessarily recover absorptive capacity in short bowel syndrome (SBS), sometimes resulting in intestinal failure-associated liver disease (IFALD). Additionally, its therapeutic options remain limited. Polyamines (spermidine and spermine) are known as one of the autophagy inducers and play important roles in promoting the weaning process; however, their impact on intestinal adaptation is unknown. The aim of this study was to investigate the impact of polyamines ingestion on adaptation and hepatic lipid metabolism in SBS. We performed resection of two-thirds of the small intestine in male Lewis rats as an SBS model. They were allocated into three groups and fed different polyamine content diets (0%, 0.01%, 0.1%) for 30 days. Polyamines were confirmed to distribute to remnant intestine, whole blood, and liver. Villous height and number of Ki-67-positive cells in the crypt area increased with the high polyamine diet. Polyamines increased secretory IgA and mucin content in feces, and enhanced tissue Claudin-3 expression. In contrast, polyamines augmented albumin synthesis, mitochondrial DNA copy number, and ATP storage in the liver. Moreover, polyamines promoted autophagy flux and activated AMP-activated protein kinase with suppression of lipogenic gene expression. Polyamines ingestion may provide a new therapeutic option for SBS with IFALD.

Expenditure and survival of adult patients with intestinal failure due to short bowel syndrome: real-world evidence from Southern Finland.

OBJECTIVES: Comprehensive follow-up data from the largest hospital district in Finland was used to assess hospital-based healthcare resource utilization (HCRU) and expenses, incidence and prevalence, survival, and effect of comorbidities/complications on survival of adult patients with intestinal failure due to short bowel syndrome (SBS-IF). METHODS: This study utilized electronic healthcare data covering all ≥18-year-old patients with SBS-IF at the Hospital District of Helsinki and Uusimaa in Finland between 2010 and 2019. Patients were followed from SBS-IF onset until the end of 2020 or death and compared to birth year and sex-matched control patients without SBS-IF. RESULTS: The study included 77 patients with SBS-IF (cases) and 363 controls. Cases had high HCRU; the cumulative expenses were about tenfold compared to the controls, at the end of the study (€123,000 vs. €14,000 per patient). The expenses were highest during the first year after SBS-IF onset (€53,000 per patient). Of the cases with a median age 62.5 years, 51.9% died during study time. The median survival was 4.4 years from SBS-IF onset and cases died 13.5 times more likely during the follow-up compared to controls. Mortality risk was lower in female cases (hazard ratio (HR) 0.46; 95% confidence intervals (CI) 0.24, 0.9) and higher with presence of comorbidities (Charlson comorbidity index HR 1.55; 95% CI 1.2, 2.0) and mesenteric infarction (HR 4.5; 95% CI 1.95, 10.36). The incidence of adult SBS-IF was 0.6 per 100,000 adults. CONCLUSION: Our study demonstrates a high demand for healthcare support and elevated mortality in adult SBS-IF-patients. Our results suggest that the presence of comorbidities is a key driver for mortality.

AGA Clinical Practice Update on Diet and Nutritional Therapies in Patients With Inflammatory Bowel Disease: Expert Review.

DESCRIPTION: Diet plays a critical role in human health, but especially for patients with inflammatory bowel disease (IBD). Guidance about diet for patients with IBD are often controversial and a source of uncertainty for many physicians and patients. The role of diet has been investigated as a risk factor for IBD etiopathogenesis and as a therapy for active disease. Dietary restrictions, along with the clinical complications of IBD, can result in malnutrition, an underrecognized condition among this patient population. The aim of this American Gastroenterological Association (AGA) Clinical Practice Update (CPU) is to provide best practice advice statements, primarily to clinical gastroenterologists, covering the topics of diet and nutritional therapies in the management of IBD, while emphasizing identification and treatment of malnutrition in these patients. We provide guidance for tailored dietary approaches during IBD remission, active disease, and intestinal failure. A healthy Mediterranean diet will benefit patients with IBD, but may require accommodations for food texture in the setting of intestinal strictures or obstructions. New data in Crohn's disease supports the use of enteral liquid nutrition to help induce remission and correct malnutrition in patients heading for surgery. Parenteral nutrition plays a critical role in patients with IBD facing acute and/or chronic intestinal failure. Registered dietitians are an essential part of the interdisciplinary team approach for optimal nutrition assessment and management in the patient population with IBD. METHODS: This expert review was commissioned and approved by the AGA Clinical Practice Updates Committee and the AGA Governing Board to provide timely guidance on a topic of high clinical importance to the AGA membership and underwent internal peer review by the CPU Committee and external peer review through standard procedures of Gastroenterology. The best practice advice statements were drawn from reviewing existing literature combined with expert opinion to provide practical advice on the role of diet and nutritional therapies in patients with IBD. Because this was not a systematic review, formal rating of the quality of evidence or strength of the presented considerations was not performed. Best Practice Advice Statements BEST PRACTICE ADVICE 1: Unless there is a contraindication, all patients with IBD should be advised to follow a Mediterranean diet rich in a variety of fresh fruits and vegetables, monounsaturated fats, complex carbohydrates, and lean proteins and low in ultraprocessed foods, added sugar, and salt for their overall health and general well-being. No diet has consistently been found to decrease the rate of flares in adults with IBD. A diet low in red and processed meat may reduce ulcerative colitis flares, but has not been found to reduce relapse in Crohn's disease. BEST PRACTICE ADVICE 2: Patients with IBD who have symptomatic intestinal strictures may not tolerate fibrous, plant-based foods (ie, raw fruits and vegetables) due to their texture. An emphasis on careful chewing and cooking and processing of fruits and vegetables to a soft, less fibrinous consistency may help patients with IBD who have concomitant intestinal strictures incorporate a wider variety of plant-based foods and fiber in their diets. BEST PRACTICE ADVICE 3: Exclusive enteral nutrition using liquid nutrition formulations is an effective therapy for induction of clinical remission and endoscopic response in Crohn's disease, with stronger evidence in children than adults. Exclusive enteral nutrition may be considered as a steroid-sparing bridge therapy for patients with Crohn's disease. BEST PRACTICE ADVICE 4: Crohn's disease exclusion diet, a type of partial enteral nutrition therapy, may be an effective therapy for induction of clinical remission and endoscopic response in mild to moderate Crohn's disease of relatively short duration. BEST PRACTICE ADVICE 5: Exclusive enteral nutrition may be an effective therapy in malnourished patients before undergoing elective surgery for Crohn's disease to optimize nutritional status and reduce postoperative complications. BEST PRACTICE ADVICE 6: In patients with IBD who have an intra-abdominal abscess and/or phlegmonous inflammation that limits ability to achieve optimal nutrition via the digestive tract, short-term parenteral nutrition may be used to provide bowel rest in the preoperative phase to decrease infection and inflammation as a bridge to definitive surgical management and to optimize surgical outcomes. BEST PRACTICE ADVICE 7: We suggest the use of parenteral nutrition for high-output gastrointestinal fistula, prolonged ileus, short bowel syndrome, and for patients with IBD with severe malnutrition when oral and enteral nutrition has been trialed and failed or when enteral access is not feasible or contraindicated. BEST PRACTICE ADVICE 8: In patients with IBD and short bowel syndrome, long-term parenteral nutrition should be transitioned to customized hydration management (ie, intravenous electrolyte support and/or oral rehydration solutions) and oral intake whenever possible to decrease the risk of developing long-term complications. Treatment with glucagon-like peptide-2 agonists can facilitate this transition. BEST PRACTICE ADVICE 9: All patients with IBD warrant regular screening for malnutrition by their provider by means of assessing signs and symptoms, including unintended weight loss, edema and fluid retention, and fat and muscle mass loss. When observed, more complete evaluation for malnutrition by a registered dietitian is indicated. Serum proteins are no longer recommended for the identification and diagnosis of malnutrition due to their lack of specificity for nutritional status and high sensitivity to inflammation. BEST PRACTICE ADVICE 10: All patients with IBD should be monitored for vitamin D and iron deficiency. Patients with extensive ileal disease or prior ileal surgery (resection or ileal pouch) should be monitored for vitamin B12 deficiency. BEST PRACTICE ADVICE 11: All outpatients and inpatients with complicated IBD warrant co-management with a registered dietitian, especially those who have malnutrition, short bowel syndrome, enterocutaneous fistula, and/or are requiring more complex nutrition therapies (eg, parenteral nutrition, enteral nutrition, or exclusive enteral nutrition), or those on a Crohn's disease exclusion diet. We suggest that all newly diagnosed patients with IBD have access to a registered dietitian. BEST PRACTICE ADVICE 12: Breastfeeding is associated with a lower risk for diagnosis of IBD during childhood. A healthy, balanced, Mediterranean diet rich in a variety of fruits and vegetables and decreased intake of ultraprocessed foods have been associated with a lower risk of developing IBD.

A Narrative Review of Patient-Reported Outcome Measures and Their Application in Recent Pediatric Surgical Research: Advancing Knowledge and Offering New Perspectives to the Field.

INTRODUCTION: Patient-reported outcome measures (PROMs) can be employed in both research and clinical care to enhance our understanding of outcomes that matter to patients. This narrative review aims to describe PROM use in recent pediatric surgical research, identify and describe psychometrically robust PROMs, providing an overview of those derived from pediatric patient input, and make recommendations for future research. MATERIALS AND METHODS: A search was conducted to identify articles published from 2021 to August 2023 describing the availability and/or use of at least one valid or reliable PROM in children with conditions including anorectal malformations, biliary atresia, congenital diaphragmatic hernia, duodenal atresia, esophageal atresia, abdominal wall defects, Hirschsprung's disease, sacrococcygeal teratoma, and short bowel syndrome. Articles were categorized based on their objectives in applying PROMs. Psychometrically robust PROMs were identified and described. RESULTS: Out of the 345 articles identified, 49 met the inclusion criteria. Seventeen focused on esophageal atresia and 14 on Hirschsprung's disease. Twenty-nine PROMs were identified, with 12 deemed psychometrically robust. Seven psychometrically robust PROMs were developed using patient input in the primary item generation. Most PROMs were applied to advance understanding of conditions and/or treatment and fewer were developed or psychometrically evaluated. No PROMs were assessed for their impact or incorporated into an implementation study. CONCLUSIONS: This review reveals gaps in the application of PROMs in recent pediatric surgical research. Emphasis should be placed on the development and utilization of psychometrically robust PROMs, broadening the scope of covered diseases, conducting impact assessments, and evaluating implementation strategies.

Iron overload in patients with Crohn's disease with short bowel syndrome who received long-term parenteral nutrition with trace elements.

Crohn's disease patients often need regular home parenteral nutrition (HPN) for intestinal failure due to multiple intestinal resections. Trace elements are necessary for long-term HPN but the requirement volume of iron is undetermined. We describe three patients with Crohn's disease with short bowel syndrome (SBS) who had iron overload as a result of long-term HPN including iron. Serum ferritin level was significantly decreased through depleting intravenous iron administration in all cases. One patient needed regular insulin injection and phlebotomy for diabetes mellitus due to hemochromatosis, and intravenous iron administration had a significant impact on the patient's health. Long-term routine intravenous iron administration should be cautious in SBS patients to avoid the overload.

Exploring the impact of pediatric short bowel syndrome on parent well-being using a disease-specific pilot survey.

BACKGROUND: Children with short bowel syndrome (SBS) have complex care needs, most of which are met in the home by family caregivers who may experience a range of stressors unique to this experience. Prior research suggests that parents of children with SBS have poorer health-related quality of life than peers parenting children without health needs, but the mechanisms shaping parent outcomes are understudied. METHODS: A pilot survey was developed using a community-driven research design to measure the impact of disease-specific items on parent-perceived well-being. The cross-sectional survey, which included both closed-ended and open-ended items, was distributed to a convenience sample of parents of children with SBS. Quantitative and qualitative data were integrated for a mixed-methods analysis of how individual items impacted parent well-being. RESULTS: Twenty parents completed the survey. Sleep interruptions, lack of support and resources, and psychological stressors and their mental health implications were more frequently reported as stressors than logistics related to caregiving (e.g., managing therapies and preparing specialized meals). CONCLUSION: The impact of a child's SBS on parent well-being may stem mainly from three interconnected domains: poor sleep and its consequences, lack of access to support and resources, and a range of psychological stressors that affect parent mental health. Understanding the mechanisms through which SBS shapes parent well-being is a necessary first step for developing targeted interventions to support parents and provide family-centered care.

Neighborhood Income Is Associated with Health Care Use in Pediatric Short Bowel Syndrome.

OBJECTIVE: To evaluate associations between neighborhood income and burden of hospitalizations for children with short bowel syndrome (SBS). STUDY DESIGN: We used the Pediatric Health Information System (PHIS) database to evaluate associations between neighborhood income and hospital readmissions, readmissions for central line-associated bloodstream infections (CLABSI), and hospital length of stay (LOS) for patients <18 years with SBS hospitalized between January 1, 2006, and October 1, 2015. We analyzed readmissions with recurrent event analysis and analyzed LOS with linear mixed effects modeling. We used a conceptual model to guide our multivariable analyses, adjusting for race, ethnicity, and insurance status. RESULTS: We included 4289 children with 16 347 hospitalizations from 43 institutions. Fifty-seven percent of the children were male, 21% were Black, 19% were Hispanic, and 67% had public insurance. In univariable analysis, children from low-income neighborhoods had a 38% increased risk for all-cause hospitalizations (rate ratio [RR] 1.38, 95% CI 1.10-1.72, P = .01), an 83% increased risk for CLABSI hospitalizations (RR 1.83, 95% CI 1.37-2.44, P < .001), and increased hospital LOS (ß 0.15, 95% CI 0.01-0.29, P = .04). In multivariable analysis, the association between low-income neighborhoods and elevated risk for CLABSI hospitalizations persisted (RR 1.70, 95% CI 1.23-2.35, P < .01, respectively). CONCLUSIONS: Children with SBS from low-income neighborhoods are at increased risk for hospitalizations due to CLABSI. Examination of specific household- and neighborhood-level factors contributing to this disparity may inform equity-based interventions.

Therapeutic drug monitoring of osimertinib in non-small cell lung cancer and short bowel syndrome: A case report.

Short bowel syndrome (SBS) following extensive intestinal resection is often characterized by impaired absorption of orally administered drugs, including tyrosine kinase inhibitors (TKI). We report the case of a patient with EGFR-mutated non-small cell lung carcinoma treated with 80 mg/day of the TKI osimertinib who achieved partial response of the tumour, but was subsequently subjected to a double-barrelled jejunostomy due to ileus. Due to the development of SBS after the bypass surgery, plasma concentrations of osimertinib were monitored using mass spectrometry. The therapeutic drug monitoring confirmed a malabsorption of osimertinib in the patient (108 ng/mL, which is below the 5th percentile of the expected plasma concentration) and was useful to guide adjustments of TKI dosing in order to achieve adequate blood levels (161 ng/mL after increase of the dose to 120 mg/day) in order to maintain tumour control.

Sustained elimination of parenteral support in adult patients with under 60 cm of small intestine: A case series.

Patients with short bowel syndrome (SBS) are often managed by expert multidisciplinary teams. One of the main goals in the management of SBS is the weaning of parenteral support (PS). Weaning of PS removes the risks associated with long-term central line placement and eliminates the need for intestinal transplant. Whereas several papers detail the ongoing care and management of patients with SBS who are dependent on PS, there are few reports discussing the successful weaning of PS in this patient population. We present five case studies examining weaning of PS in adult patients with <60-cm small bowel and partial or full colon who were enrolled in a multidisciplinary adult intestinal rehabilitation program. The case studies demonstrate weaning can occur >2 years after initial assessment and enrollment in an expert program. The ability of the program to focus on individualized care and frequent patient communication is key to PS weaning in patients with SBS.