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ObjectiveTo evaluate associations between neighborhood income and burden of hospitalizations for children with short bowel syndrome (SBS).Study designWe 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

IntroductionSevere forms of short bowel syndrome (SBS) resulting in chronic intestinal failure (IF) have limited therapeutic options, all of which are associated with significant morbidities. Spring-mediated distraction enterogenesis (SMDE) uses an intraluminal self-expanding spring to generate mechanical force to induce intestinal stretching and sustained axial growth, providing a promising novel approach for patients with SBS. Previous studies have established this method to be safe and effective in small and large animal models. However, SMDE has previously not been implemented in a large, clinically relevant animal model.MethodsJuvenile mini-Yucatan pigs with 75% of their small intestine resected had intraluminal springs placed after an initial adaptive period. Morphological and histological assessments were performed on SMDE segments compared to the control region of the intestine undergoing normal adaptive responses to resection.ResultsWhile the initial histologic adaptive response observed following resection was attenuated after a month, the SMDE segments instead augmented these adaptive changes. Specifically, intestinal length increased 2-fold in SMDE segments, and the widths of the epithelial, muscularis, and serosal layers were enhanced in SMDE compared with control segments of the same animal. This data suggests that morphologic intestinal adaptation may be enhanced with SMDE in the setting of SBS.DiscussionHere we demonstrate the successful and reproducible implementation of SMDE in a large animal model in the setting of prior intestinal resection, making SMDE a viable and novel approach for SBS to be explored further.

Purpose: This study investigated the anti-inflammatory effect of clodronate, a vesicular nucleotide transporter (VNUT) inhibitor, on intestinal-failure-associated liver disease (IFALD) in a rat model of short bowel syndrome (SBS). Methods: The rats underwent jugular vein catheterization for continuous total parenteral nutrition (TPN) and 90% small bowel resection. The animals were divided into the following groups: TPN/SBS (Control group), TPN/SBS/intravenous administration of low-dose clodronate (20 mg/kg twice per week; Low group), or TPN/SBS/intravenous administration of high-dose clodronate (60 mg/kg twice per week; High group). On day 7, the rats were euthanized. Hepatic steatosis and hepatocellular injury were also assessed. Results: Hepatic steatosis and lobular inflammation in the liver were observed in all groups. The High group showed histologically reduced hepatic steatosis compared with the Control group. IL-6 and Nlrp3 expression in the High group was significantly suppressed compared to that in the Control group. The expression of other inflammatory cytokines tended to be lower in the High dose group than in the control group. The lipid metabolism gene expression in the liver specimens showed no significant differences among the groups. Conclusion: The high-dose administration of clodronate may, therefore, inhibit hepatic steatosis and inflammation associated with IFALD in patients with SBS. [ABSTRACT FROM AUTHOR]

Background: This study aimed to gain real-world insights from healthcare professionals (HCPs) regarding the management of adult patients with short bowel syndrome and intestinal failure (SBS-IF) who received teduglutide and achieved parenteral support (PS) independence or PS volume stability for ≥12 months. Methods: This cross-sectional survey was conducted in five European countries and Canada via a self-reported questionnaire (November 2022–March 2023) among HCPs who manage patients with SBS-IF and who had prescribed teduglutide to ≥5 patients with SBS-IF receiving PS. Results: Of the 70 HCPs who completed the survey, almost all reported managing patients with SBS-IF who achieved PS independence or PS volume stability (99%, 69/70 and 97%, 68/70, respectively) and maintained the standard teduglutide dose, without changes. A total of 52 HCPs managed patients who achieved PS independence and discontinued teduglutide. Of these HCPs, 73% (38/52) anticipated that these patients would remain PS-independent, not requiring PS reintroduction. Of the remainder, 79% (11/14) estimated that ≤40% of these patients would require PS reintroduction. While many HCPs (81%, 42/52) would reintroduce teduglutide in patients who discontinued its use after achieving PS independence, none would do so for patients who discontinued teduglutide after achieving PS volume stability if a patient's condition worsened. Conclusions: This survey found that patients with SBS-IF can achieve PS independence or PS volume stability with teduglutide treatment. However, some HCPs (27%, 14/52) believe that a proportion of patients discontinuing teduglutide after achieving PS independence will require PS reintroduction. This survey suggests that teduglutide treatment should continue uninterrupted, unless clinically indicated, but this requires confirmation in future studies. [ABSTRACT FROM AUTHOR]

Background: Short bowel syndrome is considered a low prevalence disease. The scant information available about intestinal failure in Latin America was the driving force to expand this registry. Methods: A prospective, multicenter observational registry was created for patients with chronic intestinal failure short bowel at specialized centers in Latin America. Demographics, clinical characteristics, nutrition assessment, parenteral nutrition management, intestinal rehabilitation, related complications, clinical outcome, and survival were analyzed. Results: From May 2020 to July 2023, 167 patients (115 adults, 52 children) from 20 centers were enrolled. For the adults, the mean age was 37.2 ± 18 years, 48% were female, and the mean follow‐up was 22.6 ± 18.3 months. The main etiology was surgical resections (postsurgical complications: 37%; ischemia: 25%); the mean intestinal length was 73 ± 55 cm. The complications were as follows: infections: 0.4/1000 catheter‐days; thrombosis: 0.24/1000 catheter‐days; liver disease: 2.6%. The outcomes were as follows: 28% were rehabilitated, 15% died, 9.6% were lost to follow‐up, 0.9% underwent transplant, and 45.6% continued follow‐up. For the children, the mean age 48 ± 52 months, 48% were female, 52% were premature. The mean follow‐up was 17.2 ± 5.6 months; the mean remaining intestinal length was 38 ± 45 cm. The leading etiologies were atresia (25%), NEC (23%), and gastroschisis (21%). The complication were as follows: infections: 2/1000 catheter‐days; thrombosis: 2.22/1000 catheter‐day; 25% developed liver disease. The outcomes were as follows: 7.7% died, 3.8% were rehabilitated, and 88.5% continued follow‐up. Conclusion: The RESTORE amendment served as a registry and educational tool for the participating teams. The aspiration is to objectively show current aspects of intestinal failure in the region and carry them to international standards. Including all Latin American countries and etiologies of chronic intestinal failure besides short gut would serve to complete this registry. [ABSTRACT FROM AUTHOR]

Background: Serial transverse enteroplasty is used to treat patients with chronic intestinal failure owing to short bowel syndrome. Current literature lacks discussion of its role for other etiologies of intestinal failure and its impact on adult patients' nutrition support needs and quality of life. Methods: We performed a case series on adults with parenteral nutrition (PN) dependence who underwent serial transverse enteroplasty at Emory University Hospital, a quaternary referral center between 2011 and 2022. Data collected included demographics, operative technique, and preoperative and postoperative PN requirements. A phone survey was administered to evaluate the impact of PN and the operation on quality of life. Results: Ten patients underwent the procedure of interest during the study period. Indications included short bowel syndrome following multiple abdominal operations or intra‐abdominal catastrophe and chronic partial bowel obstruction with dysmotility. Bowel length increased by a median of 83%. All patients were discharged home after a median hospital stay of 21 days. At 1‐year follow‐up, survival was 100%, two (20%) patients fully weaned from PN, three others (30%) reduced PN frequency, and six (60%) decreased their daily parenteral energy requirement. Two additional patients fully weaned from PN by 18 months postoperatively. Conclusion: This represents one of the largest case series of serial transverse enteroplasty in adults. Small intestinal length nearly doubled, and PN dependence was reduced in most patients. Given the low morbidity and good quality of life observed in this series, this procedure should be more widely investigated for patients with chronic intestinal failure. [ABSTRACT FROM AUTHOR]

Purpose: This study aimed to investigate the effectiveness of dipeptidyl peptidase IV inhibitors (DPP4-I) against liver damage, especially fibrosis and lipid accumulation, in a rat intestinal failure-associated liver disease (IFALD) model. Methods: SD rats were divided into two groups: the Control (n = 7; normal saline + IFALD model) and DPP4-I (n = 7; DPP4-I + IFALD model; short bowel syndrome (SBS) + total parenteral nutrition) groups. All rats were euthanized 21 days postoperatively to obtain tissue samples. Liver fibrosis was evaluated by Sirius Red and α-SMA staining. Liver damage was assessed using the steatosis, activity, and fibrosis score. Inflammation cytokines were examined by ELISA. Results: The survival rate was comparatively different, being 87.5% in the DPP4-I group and 70.0% in the Control group. Two rats of the Control group showed progressive liver fibrosis in the periportal area with fibrous streaks. Further, the mean area percentage of α-SMA immune-positive cells was significantly lower in the DPP4-I group than in the Control group. TGF-β levels were significantly lower in the DPP4-I group than in the Control group. Conclusion: DPP4-I administration reduced liver fibrosis in IFALD, possibly by inhibiting DPP4-I-induced adipogenesis and suppressing TGF-β. These results may contribute to elucidating the mechanism of IFALD. [ABSTRACT FROM AUTHOR]

Introduction: Broviac catheter is a type of central venous catheter (CVC) used for long‐term parenteral nutrition in specific patients, e.g., diagnosed with intestinal failure as short bowel syndrome (SBS). The way of the catheter insertion is conceived to minimalize the risk of infections. However, CVC‐related blood stream infections (CVC‐BSIs), including infective endocarditis (IE), remain most important complications associated with Broviac catheter. Staphylococcus epidermidis stands out as a prevalent pathogen. The increasing number of CVCs results in an increased incidence of healthcare‐associated IE. Complete parenteral treatment is an independent risk that increases the likelihood of IE. Treatment of IE is mainly based on antibiotic therapy, but in certain cases, surgical treatment is needed. Presentation of Case: A 71‐year‐old female with SBS who had been receiving total parenteral nutrition through the Broviac catheter for several months was admitted in a serious condition with significant weakness, increasing shortness of breath, deteriorating cough, fever, low blood pressure, and heart palpitations. Echocardiography revealed severe aortic valve IE with a large, longitudinal, highly mobile vegetation (up to 40 mm) and massive aortic regurgitation with pulmonary edema. Fast pathogen detection in the patients' blood (S. epidermidis) was obtained using PCR‐based multiplex test. Due to life‐threatening conditions, emergency surgery with aortic valve replacement was performed. Consistent rehabilitation resulted in good condition achievement. Follow‐up echocardiography showed normal function of the aortic valve bioprosthesis. Conclusion: The use of CVC, including Broviac catheter, is associated with an increased risk of infections, including IE. Treatment‐resistant severe HF complicating IE requires emergency surgery. [ABSTRACT FROM AUTHOR]

Short bowel syndrome (SBS) leads to severe morbidity and mortality. Intestinal adaptation is crucial in improving outcomes. To understand the human gene pathways associated with adaptation, we perform single-cell transcriptomic analysis of human small intestinal organoids explanted from mice with experimental SBS. We show that transmembrane ion pathways, specifically the transepithelial zinc transport pathway genes SLC39A4 and SLC39A5, are upregulated in SBS. This discovery is corroborated by an external dataset, bulk RT-qPCR, and Western blots. Oral zinc supplementation is shown to improve survival and weight gain of SBS mice and increase the proliferation of intestinal crypt cells in vitro. Finally, we identify the upregulation of SLC39A5 and associated transcription factor KLF5 in biopsied intestinal tissue specimens from patients with SBS. Thus, we identify zinc supplementation as a potential therapy for SBS and describe a xenotransplantation model that provides a platform for discovery in other intestinal diseases. SBS leads to severe morbidity and mortality. Here, using a xenotransplantation model, the authors uncover the role of zinc transport pathways in intestinal adaptation in SBS, highlighting the potential of zinc supplementation as a therapeutic approach. [ABSTRACT FROM AUTHOR]

Intestinal failure (IF) is a debilitating condition characterized by the insufficient function of the gastrointestinal tract to absorb nutrients and fluids essential for life. This review consolidates recent advancements and challenges in managing IF among adult and pediatric populations, highlighting differences in etiology, management, and outcomes. Over the recent years, significant strides have been made in the nutritional and medical management of IF, significantly reducing mortality rates and improving the quality of life for patients. Key advancements include the development and availability of glucagon-like peptide-2 (GLP-2) analogs, improved formulations of parenteral nutrition, and the establishment of specialized interdisciplinary centers. Short bowel syndrome (SBS) remains the predominant cause of IF globally. The pediatric segment is increasingly surviving into adulthood, presenting unique long-term management challenges that differ from adult-onset IF. These include the need for tailored nutritional support, management of IF-associated liver disease, and addressing growth and neurodevelopmental outcomes. The therapeutic landscape for IF continues to evolve with the development of new treatment modalities and better understanding of the condition's pathophysiology. However, disparities in treatment outcomes between children and adults suggest the need for age-specific management strategies. This review underscores the importance of a nuanced approach to IF, incorporating advancements in medical science with a deep understanding of the distinct needs. [ABSTRACT FROM AUTHOR]

Joel Habener, Svetlana Mojsov, and Lotte Bjerre Knudsen have been awarded the 2024 Lasker-DeBakey Clinical Medical Research Award for their work on GLP-1 receptor agonists and their applications to obesity. GLP-1 receptor agonists have revolutionized the treatment of metabolic disorders like diabetes and obesity, and have shown potential for treating other disorders as well. These drugs have been studied in cardiovascular trials and have shown significant reductions in cardiovascular outcomes, as well as beneficial effects on blood pressure, lipoproteins, and inflammation. However, they do come with side effects such as nausea, vomiting, pancreatitis, and injection site reactions. Longer-acting forms of GLP-1 receptor agonists have been developed to improve convenience for patients. [Extracted from the article]

Short bowel syndrome is the most common reversible cause of intestinal failure. Most of the children are started on parenteral nutrition (PN) after surgery to enable growth and allow time for intestinal adaptation. This is a process whereby the shorter length of bowel is able to achieve complete function as if the entire length of bowel is present. With management advances, most children with short bowel syndrome can discontinue PN and establish full enteral feeds. This article mainly focuses on the complications of short bowel syndrome that need to be avoided for the child to achieve intestinal adaptation, establish on enteral feeds/oral diet and achieve enteral autonomy. [ABSTRACT FROM AUTHOR]

Background: Pediatric patients with intestinal failure require long‐term parenteral nutrition owing to impaired enteral nutrition absorption. A potential complication is essential fatty acid deficiency (EFAD), resulting from decreased linoleic and α‐linolenic acid concentrations and defined by an increased triene:tetraene ratio (TTR; Mead acid:arachidonic acid). Historically, soybean oil lipid emulsion (SOLE) was the only commercially available parenteral lipid in the United States. Recently, a composite lipid emulsion (CLE) and fish oil lipid emulsion (FOLE) received US Food and Drug Administration approval. This study investigated whether lipid emulsion regimen impacts EFAD incidence in pediatric patients with intestinal failure. Methods: This study was a 10‐year retrospective cohort study of pediatric patients with intestinal failure who received parenteral SOLE, CLE, or FOLE. The primary outcome was EFAD incidence, defined as a TTR ≥ 0.2. Secondary outcomes included TTR ≥ 0.05, cholestasis incidence, lipid dose effect on EFAD incidence, and fatty acid parameter differences. Results: A total of 144 fatty acid profiles from 47 patients were reviewed. EFAD did not occur in any lipid emulsion group. There were no differences in the incidence of TTR ≥ 0.05 or cholestasis. The effect of dose could not be evaluated because of no EFAD incidence. Lastly, although each group had varied fatty acid parameters, none saw decreased essential fatty acid levels. Conclusion: This study found that, with close monitoring, the lipid emulsion regimen did not impact EFAD incidence. This suggests that FOLE and CLE do not increase EFAD risk compared with SOLE in pediatric patients with intestinal failure. [ABSTRACT FROM AUTHOR]

Background: Teduglutide, the first glucagon-like peptide 2 analogue, has been demonstrated to facilitate the absorption of gut nutrient and lessen the need for parenteral assistance in patients with Short Bowel Syndrome (SBS). However, its adverse drug events (AEs) are primarily documented in clinical trials, with a deficit in real-world data. This study evaluates the AEs profile of teduglutide based on Food and Drug Administration (FDA) Adverse Event Reporting System (FAERS) data. Method: A disproportionality analysis of FAERS data from Quarter 1 (Q1) 2013 to Quarter 3 (Q3) 2023 was conducted to examine the association between teduglutide and adverse events, employing Reporting Odds Ratio (ROR), Proportional Reporting Ratio (PRR), Bayesian Confidence Propagation Neural Network (BCPNN), and Empirical Bayesian Geometric Mean (EBGM) methods. Results: Out of 13,809,302 reports in the FAERS database, 10,114 reports identified teduglutide as the "primary suspect" in AEs identification. During the dosing observation period, the median occurrence of adverse events was 393 days (interquartile range [IQR] 97-996 days). Teduglutide-associated AEs occurred in 27 System Organ Classes (SOC), of which renal and urinary disorders is not mentioned in the specification. Based on the four algorithms, a total of 260 major disproportionality preferred terms (PTs) were filtered out, including previously unreported AEs including weight decreased (n = 805), vascular device infection (n = 683), dehydration (n = 596) and nephrolithiasis (n = 146). Conclusion: Our findings corroborate the AEs listed in the teduglutide prescribing information and additionally unveil new adverse reaction signals such as nephrolithiasis. These discoveries could aid in clinical monitoring and risk identification for teduglutide. [ABSTRACT FROM AUTHOR]

With advancements in antiretroviral therapy (ART), the average lifespan of people with human immunodeficiency virus (HIV) is increasing, as is the number of older adults with HIV. Accordingly, the number of patients with HIV who undergo surgery or require critical care for various reasons is increasing. Since the prognosis of people with HIV depends on the continuous and effective maintenance of ART, there is a need to consider effectively maintaining ART in people with HIV in these conditions. This case involved a 55-year-old patient with well-controlled HIV who received ART and presented to the emergency room with acute abdominal pain. He was diagnosed with extensive bowel infarction and panperitonitis and received critical care in the intensive care unit, including mechanical ventilation and continuous renal replacement therapy. The patient was administered enteral nutrition via a nasogastric tube. The patient subsequently underwent extensive small bowel resection and developed short bowel syndrome. The patient maintained ART during that period. A literature review related to the use of ART under these conditions is included in this study. This case was discussed at the [Exploring Difficult Cases in HIV Clinics] of the Korean Society for AIDS Conference held in 2023. [ABSTRACT FROM AUTHOR]

Background: Surgeons often encounter patients with intestinal failure due to inadequate intestinal length ("short bowel syndrome"/SBS). Treatment in these patients remains challenging and the process of physiologic adaptation may take years to complete, which frequently requires parenteral nutrition. We propose a proof-of-concept mechanical bowel elongation approach using a self-expanding prototype of an intestinal expansion sleeve (IES) for use in SBS to accelerate the adaptation process. Methods: IESs were deployed in the small intestines of Sprague Dawley rats. Mechanical characterization of these prototypes was performed. IES length–tension relationships and post-implant bowel expansion were measured ex vivo. Bowel histology before and after implantation was evaluated. Results: IES mechanical studies demonstrated decreasing expansive force with elongation. The deployment of IES devices produced an immediate 21 ± 8% increase in bowel length (p < 0.001, n = 11). Mechanical load testing data showed that the IESs expressed maximum expansive forces at 50% compression of the initial pre-contracted length. The small-intestine failure load in the rats was 1.88 ± 21 N. Intestinal histology post deployment of the IES showed significant expansive changes compared to unstretched bowel tissue. Conclusions: IES devices were scalable to the rat intestinal model in our study. The failure load of the rat small intestine was many times higher than the force exerted by the contraction of the IES. Histology demonstrated preservation of intestinal structure with some mucosal erosion. Future in vivo rat studies on distraction enterogenesis with this IES should help to define this organogenesis phenomenon. [ABSTRACT FROM AUTHOR]

The destruction of red cells during blood collection or with the processing of the sample continues to occur at a high rate, especially among emergency department (ED) patients. This can produce pre-analytical laboratory errors, particularly for potassium. We determined the incidence of hemolyzed samples and discuss the potential medical impact for hypokalemic patients who potassium level is artificially normal (pseudoeukalemia). Potassium results were obtained for a 6-month period. Using a measured hemolysis index (HI), hemolysis was present in 3.1 % for all potassium ordered (n=94,783) and 7.5 % for ED orders (n=22,770). Most of these samples were reported as having high normal result or were hyperkalemic. There were 22 hemolytic samples with a potassium of <3.5 mmol/L, and 57 hemolytic samples with a potassium in lower limit of normal (3.5–3.8 mmol/L). From this group, we examined the medical histories of 8 selected patients whose initially normal potassium levels were subsequently confirmed to have a potassium values that were below, at, or just above the lower limit of normal due to hemolysis. The primary complaint for these patients were: necrotizing soft tissue infection, pancreatitis, volume overload from heart failure with reduced ejection fraction, hypertension treated with hydrochlorothiazide, and presence of a short bowel syndrome. A subsequent non-hemolyzed sample was collected demonstrating hypokalemia in all of these patients. Within these cases, there was a potential for harm had hemolysis detection not been performed. We demonstrate the medical importance of detecting hemolysis for patients who have pseudoeukalemia. This is relevant because the HI cannot be obtained when electrolytes are tested using whole blood samples, and a normal potassium may lead to inappropriate patient management. [ABSTRACT FROM AUTHOR]

Purpose of Review: Intestinal failure (IF), a rare organic dysfunction, is characterized by a reduction in intestinal function, necessitating intravenous supplementation to sustain health and/or growth due to inadequate absorption of macro and micronutrients, water, and electrolytes. This condition is distinct from "Intestinal Insufficiency," where intravenous replacement is not immediately necessary. The purpose of this review is to evaluate the literature published in the last 5 years on drug treatment in the management of patients with intestinal failure (IF) and short bowel syndrome (SBS) and to identify strategies to minimize fluid and electrolyte loss by increasing nutrient-enterocyte contact time, improving intestinal transit time, replenishing micronutrients, and enhancing nutrient absorption. Recent Findings: We describe current treatments for rehabilitation aimed at optimizing absorption of the intestinal mucosa. We also outline recommended pharmacological adjustments of some drugs used in the treatment of commonly found comorbidities in these patients. Recent articles have demonstrated that GLP-2 analogs have become an important tool in the treatment of SBS, improving intestinal secretory control, gastrointestinal transit time, nutrient absorption, and, most importantly, positively impacting quality of life. Summary: IF is a rare disease defined by need for intravenous hydration and nutrients delivery and associated with malnutrition and high mortality rate. Medical management and treatment of IF can increase survival time and quality of life. [ABSTRACT FROM AUTHOR]