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This study investigated the prevalence, characteristics, and management of patients with chronic intestinal failure (CIF) in the United States in 2012-2020, based on parenteral support (PS) prescription claims and healthcare utilization.Patients with CIF were identified from the Integrated DataVerse® claims database if they had at least two PS prescriptions within 6 months and a relevant diagnosis. Analysis included prevalence and characteristics of patients with CIF, their travel distance to receive PS prescriptions, and the distribution of PS providers and their prescribing history.Up to 24,048 patients with CIF were identified, equivalent to 75 patients per million. CIF affected people of all ages, being more prevalent in women than in men. Many providers signed PS orders for small patient groups over short time periods, whereas few providers signed PS orders for large patient groups long term, indicating a lack of centralization. The distribution of PS providers suggested a disparity in healthcare coverage in rural vs urban areas, leading to patients traveling considerable distances to receive PS prescriptions. This may be exacerbated by a decline of providers with expertise in CIF and nutrition.Healthcare disparities for patients with CIF have likely been obscured by the lack of CIF-specific diagnostic and procedure codes, obliging providers to code for their patients under other codes. Effective policy changes, including centralized care, revision of reimbursement models, and expansion of nutrition-focused education in addition to the newly introduced International Classification of Diseases codes, are needed to provide the best care for patients.

BACKGROUND Outcomes of left lateral segment (LLS) grafts in pediatric recipients were compared between living (LD-LLS) and deceased donor (DD-LLS) grafts. METHODS 195 LLS grafts (99DD-LLS-96LD-LLS) were analyzed with a median follow-up of 9.1years. The primary endpoints were overall patient/graft survival. RESULTS LD-LLS grafts were younger (0.9vs.1.4years, p = 0.039), more likely to have a fulminant liver failure (17.9%vs.5.3%,p = 0.002), less likely to have a metabolic disorder (6.3%vs.25.5%,p = 0.002), and less likely to be undergoing retransplantation (5.3% vs.16.2%,p = 0.015). There was a trend toward decreased hepatic artery thrombosis in LD-LLS grafts (6.6% vs. 15.5%,p = 0.054). No differences in the overall biliary complications occurred. The LD-LLS group had prolonged survival compared to the DD-LLS group with 10-year survival rates of 81%, and 74% (p = 0.005), respectively. LD-LLS grafts had longer graft survival compared to DD-LLS grafts (10-year graft survival 85%vs.67%,p = 0.005). Recipient age >1year (HR 2.39,p = 0.026), aortic reconstruction (HR 2.12,p = 0.046) and vascular complication (HR 3.12,p

Liver allocation was updated on February 4, 2020, replacing a Donor Service Area (DSA) with acuity circles (AC). The impact on waitlist outcomes for patients listed for combined liver-intestine transplantation (multivisceral transplantation [MVT]) remains unknown. The Organ Procurement and Transplantation Network/United Network for Organ Sharing database was used to identify all candidates listed for both liver and intestine between January 1, 2018 and March 5, 2021. Two eras were defined: pre-AC (2018–2020) and post-AC (2020–2021). Outcomes included 90-day waitlist mortality and transplant probability. A total of 127 adult and 104 pediatric MVT listings were identified. In adults, the 90-day waitlist mortality was not statistically significantly different, but transplant probability was lower post-AC. After risk-adjustment, post-AC was associated with a higher albeit not statistically significantly different mortality hazard (sub-distribution hazard ratio[sHR]: 8.45, 95% CI: 0.96–74.05; p = .054), but a significantly lower transplant probability (sHR: 0.33, 95% CI: 0.15–0.75; p = .008). For pediatric patients, waitlist mortality and transplant probability were similar between eras. The proportion of patients who underwent transplant with exception points was lower post-AC both in adult (44% to 9%; p = .04) and pediatric recipients (65% to 15%; p = .002). A lower transplant probability observed in adults listed for MVT may ultimately result in increased waitlist mortality. Efforts should be taken to ensure equitable organ allocation in this vulnerable patient population.

The presence of preformed donor-specific antibodies in recipient serum against anti-human leukocyte antigen is a significant risk factor that negatively affects the outcomes of intestinal transplantation. Avoiding high-risk intestinal transplantation by physical and virtual cross matches has had limited success due to time constraints and ineffective correlation, respectively.We developed a guideline to improve the association between physical and virtual cross matches using the retrospective data of 56 consecutive primary adult isolated intestinal transplantations from a single center.The mean fluorescence intensity of 2,000 for positive donor-specific antibodies revealed the best association between physical and virtual cross matches among different cut-off values, but with an unacceptable false positive rate of 54%. An enhanced virtual cross match with the summation of the mean fluorescence intensity of each anti-human leukocyte antigen improved the association between physical and virtual cross matches, with a sensitivity of 83% and specificity of 98%.This enhanced virtual cross match more effectively predicts high-risk intestinal transplantation and is a better substitute for physical cross-match than the current virtual cross match. It also helps to avoid ill-considered abandonment of intestinal transplantation that is unnecessarily deemed high risk based on a simple virtual cross match.

International audience; We study a parafermion Josephson junction comprising a pair of counterpropagating edge modes of two quantum Hall systems, proximitized by an s-wave superconductor. We show that the difference between the lengths (which can be controlled by external gates) of the two counterpropagating chiral edges at the Josephson junction, can act as a source of spontaneous phase bias. For the Laughlin filling fractions, ν=1/m, m∈2Z+1, this leads to an electrical control of either Majorana (m=1) or parafermion (m≠1) zero modes.

Candida auris is an urgent antimicrobial resistance threat due to its global emergence, high mortality, and persistent transmissions. Nearly half of C. auris clinical and surveillance cases in the United States are from the New York and New Jersey Metropolitan area. We performed genome, and drug-resistance analysis of C. auris isolates from a patient who underwent multi-visceral transplantation. Whole-genome comparisons of 19 isolates, collected over 72 days, revealed closed similarity (Average Nucleotide Identity > 0.9996; Aligned Percentage > 0.9764) and a distinct subcluster of NY C. auris South Asia Clade I. All isolates had azole-linked resistance in ERG11(K143R) and CDR1(V704L). Echinocandin resistance first appeared with FKS1(S639Y) mutation and then a unique FKS1(F635C) mutation. Flucytosine-resistant isolates had mutations in FCY1, FUR1, and ADE17. Two pan-drug-resistant C. auris isolates had uracil phosphoribosyltransferase deletion (FUR1[1Δ33]) and the elimination of FUR1 expression, confirmed by a qPCR test developed in this study. Besides ERG11 mutations, four amphotericin B-resistant isolates showed no distinct nonsynonymous variants suggesting unknown genetic elements driving the resistance. Pan-drug-resistant C. auris isolates were not susceptible to two-drug antifungal combinations tested by checkerboard, Etest, and time-kill methods. The fungal population pattern, discerned from SNP phylogenetic analysis, was consistent with in-hospital or inpatient evolution of C. auris isolates circulating locally and not indicative of a recent introduction from elsewhere. The emergence of pan-drug-resistance to four major classes of antifungals in C. auris is alarming. Patients at high risk for drug-resistant C. auris might require novel therapeutic strategies and targeted pre-and/or posttransplant surveillance.

BACKGROUND Chronic intestinal failure (CIF) is an ultrarare disease, with an estimated national prevalence of ∼25,000 cases. There is a suspicion of widespread lack of expertise in CIF care, but no formal assessment tool or data exist. We developed and validated a knowledge test in CIF and now report our preliminary results from testing CIF knowledge in a cohort of US gastroenterologists. METHOD We developed a 20-question knowledge test in CIF, covering four key components of IF. After internal testing, refinement, and revision, we administered the test to a convenience sample of experts and nonexperts in IF. We then deployed the validated test to a cohort of 100 US gastroenterologists. RESULTS The test had a Cronbach alpha of 0.74, suggesting a reliable test, with a threshold score to discriminate experts and nonexperts of 13.4 (maximum 20) and with a sensitivity of 81.3% and specificity of 86.4%. The overall mean score of 8.2 for the 100 US gastroenterologists was at the level of nonexperts in our convenience sample. CONCLUSION The preliminary results of our validated knowledge test in IF among a broad group of US gastroenterologists demonstrate lack of knowledge in IF.

Intestinal failure (IF) is a rare chronic disease requiring intravenous (IV) fluids or parenteral nutrition (PN) dependency for optimal patient health and sustenance. The complex care is best managed by specialized multidisciplinary teams. Patients who have limited access to intestinal rehabilitation centers often receive IV/PN care from clinicians lacking specialty expertise. An innovative videoconferencing project was launched in May 2019 to provide online telementoring and case-based learning in IF. The Extension for Community Healthcare Outcomes (ECHO) model was adopted to provide education and virtual support via the Learn Intestinal Failure Tele-ECHO (LIFT-ECHO) project. Online clinics include patient case presentations, moderated discussion, best-practice recommendations, and didactic continuing education lectures on IF- and PN-related topics. Participation is interprofessional and international. Via knowledge dissemination and specialty mentorship, LIFT-ECHO is expected to improve healthcare for patients with IF and transform care delivery by overcoming the limitations in access to expertise.

Intestinal transplantation (ITx) is the treatment of choice for patients with intestinal failure who have developed life-threatening complications related to long-term parenteral nutrition. Patients may also undergo ITx as part of a combined liver-intestine or multivisceral transplant for a variety of indications, most commonly intestinal failure-associated liver disease or porto-mesenteric thrombosis. Endoscopy plays a critical role in the posttransplant management of these patients, most commonly in the diagnosis and management of rejection, which occurs in up to 30-40% of patients within the first-year posttransplant. With a lack of noninvasive biomarkers to identify the presence of rejection, endoscopy and biopsy remain the gold standard for its diagnosis. Endoscopic evaluation of the graft is also important in the identification of other complications post-ITx, including posttransplant lymphoproliferative disorder, graft-versus-host disease, and enteric infections. Each patient's posttransplant anatomy may be slightly different, making endoscopy sometimes technically challenging and necessitating clear and frequent communication with the surgical team in order to help identify the highest yield approach. Herein, we review the most common pathologies found endoscopically in the post-ITx patient and describe some of the unique challenges the endoscopist faces when evaluating these complex patients.

Recent data suggest that frequent endoscopy and biopsy without evidence of graft dysfunction does not appear to confer survival advantage after intestinal transplantation. After abandoning protocol surveillance, endoscopic examination was decreased significantly at our center. These observations led us to question the need for stoma creation in intestinal transplantation. Herein, we report clinical outcomes of intestinal transplantation without stoma, compared to conventional transplant with stoma. Data analysis was limited to adult intestinal transplantation without liver allograft between 2015 and 2018. We compared patient and graft survival, frequency of endoscopic evaluation, episodes of acute rejection, nutritional therapy, and renal function between "Control group (with stoma)," n = 18 grafts in 16 patients and "Study group (without stoma)," n = 16 grafts in 15 patients. Overall outcome was similar between the 2 groups with respect to graft and patient survival, episodes of acute rejection, and its response to treatment. Nutritional outcomes were similar in both groups. Fewer antidiarrheal medications were required in the study group, but this did not translate into demonstrable gains in preservation of renal function, despite an apparent trend to improvement. Intestinal transplantation without stoma appears to be an acceptable practice model without obvious adverse impact on outcome.

Human leukocyte antigen allosensitization prior to transplant can increase the risk of early graft loss and prolong waitlist times for intestinal transplant candidates. Desensitization offers a potential therapeutic option to reduce the quantity of preformed antibodies prior to organ allocation and facilitate transplantation with a more immunologically compatible donor allograft. However, there remains a paucity of data to guide the use of desensitization in the setting of intestinal transplantation. As a result, in this review we evaluate the existing literature supporting the role of desensitization therapy in intestinal transplant, describe our own experience with the implementation of a risk-stratified desensitization protocol, and finally explore barriers and unanswered questions that continue to limit the widespread adoption of desensitization as a management strategy for highly sensitized intestinal transplant candidates.

BACKGROUND Trough-adjusted tacrolimus is commonly prescribed following intestinal transplantation to prevent allograft rejection. Despite established practice, there remains limited direct evidence linking tacrolimus levels with improved clinical outcomes. METHODS This was a single-center review of all adult non-liver containing intestinal allograft recipients from 2011 to 2018. Patients received lymphocyte depleting induction and maintenance immunosuppression consisting of tacrolimus and a corticosteroid taper. Tacrolimus time-in-therapeutic range (TAC-TTR) was calculated for all patients from the date of transplant until 1-year post-transplant using Rosendaal's method. Cox-Proportional hazards modeling was utilized to assess freedom from acute rejection and graft failure stratified by TAC-TTR quartile. RESULTS 47 patients were included in the review. Mean TAC-TTR for the cohort was 30.2% ± 11.4. Fifteen episodes of acute rejection were observed, 8 of which were severe. Patients in the highest TAC-TTR quartile >36% had a lower incidence of acute rejection and graft failure relative to patients with a TAC-TTR

BACKGROUND Intestinal failure (IF) is defined as an ultrarare disease, with an estimated prevalence of ∼25,000 cases in the US. There is a suspicion of disparities in outcomes in IF care, likely related to widespread lack of expertise. The Extension for Community Healthcare Outcomes (ECHO) model originally described by Dr Sanjeev Arora has been used to disseminate knowledge and best practices in many chronic diseases to improve outcomes. We examined our initial experience with using the ECHO model to disseminate learning in IF. METHOD This is a retrospective review of the launch, growth, and geographic reach of the Learn Intestinal Failure TeleECHO (LIFT-ECHO) program using prospectively collected data. RESULTS The LIFT-ECHO program has achieved significant geographic reach and clinician engagement. The program has reached close to two-thirds of the states in the US and several countries outside. Clinician engagement in the learning program appears to be growing exponentially. CONCLUSION It is feasible to use the ECHO model to disseminate knowledge in managing a rare disease like IF while maintaining fidelity to the proven model. Studies are underway to demonstrate direct benefit to patients.

Purpose of review There has been a striking decline in the annual volumes of adult intestinal transplants performed in the United States from a peak in 2008, reaching its lowest volume in 2019. The current review examines the pattern and potential reasons for the decline. Recent findings We observe that while improvements in intestinal rehabilitation may be contributing to some of the decline, movements of key personnel and the paucity of experts in a rarefied field may also be contributing to declining volumes. Summary We suggest that the decline in volumes of adult intestinal transplants are likely to be multifactorial. At a time of improving transplant outcomes, the indications for intestinal transplant suggested by the Centers for Medicare and Medicaid Services, may be outdated and worthy of revision.

BACKGROUND The ideal donor in intestinal transplantation (ITX) is generally considered to be 50% to 70% of recipient body weight. This may be due to concerns for "small for size" syndrome as seen in liver transplantation. We report our experience using smaller donors (donor-recipient weight ratio [DRWR], < 50%) in ITX recipients. METHODS We studied a group of ITX recipients with DRWR of 50% or less to unmatched controls who received intestinal allografts with DRWR greater than 50%. We examined patient and graft survival and enteral autonomy from parenteral nutrition as surrogate markers for safety of using smaller donors and ease of abdominal wall closure between groups to determine the value. RESULTS There was no difference in overall patient and graft survival, time to enteral autonomy from parenteral nutrition, and weight gain after ITX over time between groups. The need for complicated abdominal closure techniques was significantly more frequent in the control group than in the study group (34.6% vs 6.9%, P = 0.01). Secondary abdominal closure occurred more frequently in the control group (15.4% vs 0%, P = 0.014). Wound revisions also occurred more frequently in the control group (15.4% vs 0%, P = 0.028). CONCLUSIONS Our data suggest that ITX using smaller donors (DRWR ≤ 50%) seems to be an acceptable practice without adverse impact on surgical complications, nutritional autonomy, and patient and graft survival. Abdominal wall closure seems easier in recipients of smaller donors and "small for size" syndrome as described in liver transplantation does not occur with intestinal allografts.

Background:In multiple clinical studies, teduglutide reduced parenteral support (PS) with a consistent safety profile in adults with short bowel syndrome–associated intestinal failure (SBS–IF). The objective of this study was to assess adverse events (AEs) from a pooled data set.Methods:Safety data from four prospective clinical trials of teduglutide in patients with SBS–IF were assimilated. AEs were evaluated in patient groups based on treatment received in each study and in populations stratified to create distinct subgroups based on aetiology, bowel anatomy and baseline PS volume requirements.Results:Safety data are reported for up to 2.5 years, totalling 222 person-years exposure to teduglutide. In most patients, AEs were reported as mild or moderate in severity in all patient groups and occurred at comparable rates between patients who received teduglutide or placebo. Several common gastrointestinal AEs, including abdominal pain, nausea and abdominal distension, were reported more frequently earlier in the course of treatment, with their frequency declining over time. Fewer gastrointestinal AEs were reported in patients with vascular causes of SBS–IF and patients with most of their colon-in-continuity than in other patient subgroups. Across the patient stratification subgroups, the predominant treatment-emergent AEs for which patients receiving teduglutide had a significantly increased relative risk were abdominal distension and gastrointestinal stoma complication compared with patients receiving placebo.Conclusions:Teduglutide had a safety profile consistent with prior adult data and no new safety concerns were identified. The most frequently reported AEs were gastrointestinal in origin, consistent with the underlying disease condition and intestinotrophic actions of teduglutide.Clinical Trial Registry information:NCT00081458/EudraCT, 2004-000438-35; NCT00798967/EudraCT, 2008-006193-15; NCT00172185/EudraCT, 2004-000439-27; NCT00930644/EudraCT, 2009-011679-65

Insufficient absorptive mucosal surface is the fundamental problem in the short bowel state. Intestinal adaptation has been well studied, and it is well recognized that it may lead to dilatation of the bowel with increased thickness of the bowel wall, resulting from both mucosal hypertrophy and hyperplasia. Autologous reconstructive surgery exploits bowel dilatation in short bowel syndrome and maximizes the absorptive potential of the available mucosal surface. Indeed, autologous gastrointestinal reconstructive procedures may be better viewed as optimizing bowel diameter rather than focusing on length, thus allowing better prograde peristalsis and improved contact between luminal nutrients and mucosa, ultimately enhancing absorption.

Small bowel obstruction in a pediatric patient following liver transplant often results from adhesions, hernias, or post-transplant lymphoproliferative disease. Here, we present an unusual and previously unreported entity - Roux-en-Y intussusception in an eight-yr-old female several years after liver transplantation. Although a rare complication, Roux-en-Y intussusception should be considered as a potential etiology in the patient presenting with bowel obstruction, with specific attention to acute presentation accompanying jaundice.

Intestinal transplantation is a successful treatment for children with irreversible intestinal failure who experience life-threatening complications of parenteral nutrition. Total or near-total intestinal aganglionosis is a rare form of Hirschprung’s disease (HD) with high morbidity and mortality and near-certain dependence on parenteral nutrition. Advances in surgical techniques, immunosuppressive regimens, and posttransplant surveillance and management have led to improvements in patient and graft survival following intestinal transplantation. Intestinal transplantation may provide a life-saving treatment option for children with total or near-total intestinal aganglionosis.

Surgery in NCPH is reserved for patients with PH complications who fail medical and endoscopic treatments. Surgery aims to disrupt esophagogastric collateral circulation through devascularization procedures or to decrease portal pressure by creating portoportal or PSS. Devascularization procedures are useful in patients with esophageal varices and unfavorable anatomy for a shunt. The only portoportal shunt is the mesenterico‐left portal vein bypass (Rex shunt) and it restores the normal portal flow to the liver in EPVO. Decompressive PSS (including nonselective, selective, and partial) prevent rebleeding from esophageal and gastric varices but they may complicate with portosystemic encephalopathy, occlusion, and subsequent rebleeding. Results after shunt surgery depend on the specific disease (with EPVO showing the best long‐term results), preoperative liver function status, and postoperative patency of the shunt.

In developed countries, infections with Cryptosporidium species are rare and are often associated with waterborne or food-borne outbreaks. There is increasing evidence that solid organ transplant recipients are at increased risk of debilitating cryptosporidiosis in the setting of exogenous immunosuppression. We report a case of cryptosporidiosis, likely acquired through travel, in an intestinal transplant recipient who was successfully treated with nitazoxanide. This case highlights the unique challenges in diagnosis and treatment of immunocompromised patients with cryptosporidiosis, specifically in intestinal transplantation.

EBV-SMT are a rare entity following organ transplantation. Given the rarity of the tumor, there is no standard approach to diagnosis and treatment. A literature search identified 28 reported cases of EBV-SMT in addition to our own experience with one case. The aim of this review is to summarize the existing data regarding pathogenesis, diagnosis, and treatment.

Long-term parenteral nutrition (PN) may be complicated by PN-associated liver disease (PNALD), and some studies suggest an association between the use of soy-based fat emulsions and PNALD development. Patients' liver function typically improves and PNALD resolves after reducing or stopping a soy-based fat emulsion, and thus lipid minimization has been the primary strategy for managing PNALD in many intestinal rehabilitation programs. However, fat emulsions often cannot be stopped entirely, leading some patients to develop PNALD even after lipid reduction strategies have been implemented. Smoflipid emulsion (Kabi-Fresenius, Bad Homburg, Germany), a balanced mixture of soybean oil, medium-chain triglycerides (MCTs), olive oil, and fish oil, was recently approved by the Food and Drug Administration for use in the United States as an equivalent alternative to Intralipid (Baxter Healthcare Corporation, Deerfield, IL). In several pediatric studies, patients who received Smoflipid had significantly lower serum bilirubin levels than those who received Intralipid. In this case report, we present a patient who developed severe PNALD with subsequent resolution after 20 weeks on Smoflipid.

INTRODUCTION Intestinal failure-associated liver disease (IFALD) occurs commonly in intestinal transplant (ITx) candidates receiving parenteral nutrition (PN). The aim of this study is to establish the prevalence and risk factors for advanced liver fibrosis in adults at the time of ITx. METHODS Retrospective chart review of all ITx was performed in adults between January 2000 and May 2014. Advanced liver fibrosis was defined as stage 3 or stage 4 fibrosis. RESULTS Fifty-three patients met the inclusion criteria. The mean age was 50.6 ± 10.9 years, and the majority were female (60.4%) and Caucasian (67.9%). The mean body mass index was 21.7 ± 3.8 kg/m2 and the median duration of PN was 402 (interquartile range: 529) days. Advanced liver fibrosis at the time of ITx was found in 13 patients (24.5%). The multivariate analysis revealed that female gender and white race were significant predictors of advanced liver fibrosis. A total bilirubin >3.0 mg/dL for > a month prior to ITx was associated with an odds ratio of 8.9 for advanced fibrosis at the time of ITx but did not reach statistical significance (P = 0.055). CONCLUSION Close to one-quarter of the ITx recipients had advanced liver fibrosis. In the current era of improved PN management, our data suggests that previously reported risk factors for IFALD, such as extreme short gut syndrome and PN duration, may have a lesser impact on development of liver fibrosis. A prolonged duration of bilirubin elevation may be associated with advanced liver fibrosis in patients with IFALD, but this requires validation in a larger cohort.

Background Intestinal transplantation (ITx) is the definitive therapy for patients suffering from intestinal failure. Previous published reports suggest that these cases should be managed peri-operatively with the same intensive monitors and techniques as in liver transplantation. Methods We retrospectively reviewed the anesthetic management of sixty-seven isolated intestinal, intestinal-pancreas and intestinal-kidney transplants over the previous decade (2005-2015) in our tertiary care institution. Results Patients were typically managed with a single arterial line, a single central venous catheter and rarely intensive modalities such as a pulmonary artery catheter, a transesophageal echocardiography, a second arterial catheter or central venous catheter, a rapid infusion system, a cell salvage device or viscoelastic testing. Significant hemodynamic derangements were rare and the rate of post reperfusion syndrome (PRS) was 8.96%. Our fluid administration type and volume and transfusion type and volume were similar to previous reports in which more intensive anesthetic management was employed. Conclusion We demonstrate that intestinal transplantation can safely occur without utilizing the intensive resources requisite for a liver transplant. This article is protected by copyright. All rights reserved.

Background Teduglutide is a glucagon-like peptide 2 (GLP-2) analog that has been approved for the treatment of adult short bowel syndrome (SBS)-associated intestinal failure (IF; SBS-IF). Teduglutide increases villus height and crypt depth in the small bowel mucosa, promoting nutrition absorption and enteral independence from parenteral nutrition (PN). We aim to report our single-center experience with teduglutide in adult patients with SBS to provide real-world context to its use. Method We conducted a retrospective analysis on patients managed within our tertiary-level intestinal rehabilitation program to identify patients with SBS-IF treated with teduglutide from 2009-2015. The current report includes all patients at our center who had any exposure to teduglutide, including those who received commercial drug after approval by the Food and Drug Administration (FDA) and outside the scope of clinical trials. Results A total of 18 patients were treated with teduglutide. Eleven patients (61%) achieved complete enteral independence from PN and/or intravenous fluids (IV) at a median time of 10 months (range: 3-36 months). PN/IV volume requirement was reduced in all patients except two. Ten of the 11 patients (91%) who achieved enteral autonomy had colon. All patients off PN/IV required additional oral vitamins and electrolyte supplementations. Conclusion Our preliminary experience is consistent with prior reports of successful partial or complete weaning from PN/IV with teduglutide treatment in adult patients with SBS. The presence of colon appears to be favorable in obtaining enteral independence from PN/IV, regardless of residual small bowel length. Patients on teduglutide may remain at high risk of micronutrient deficiencies.

To evaluate the efficacy of nontransplant surgery for pediatric cholestasis, 58 clinically diagnosed children, including 20 with Alagille syndrome (ALGS), 16 with familial intrahepatic cholestasis-1 (FIC1), 18 with bile salt export pump (BSEP) disease, and 4 others with low γ-glutamyl transpeptidase disease (levels100 U/L), were identified across 14 Childhood Liver Disease Research Network (ChiLDReN) centers. Data were collected retrospectively from individuals who collectively had 39 partial external biliary diversions (PEBDs), 11 ileal exclusions (IEs), and seven gallbladder-to-colon (GBC) diversions. Serum total bilirubin decreased after PEBD in FIC1 (8.1 ± 4.0 vs. 2.9 ± 4.1 mg/dL, preoperatively vs. 12-24 months postoperatively, respectively; P = 0.02), but not in ALGS or BSEP. Total serum cholesterol decreased after PEBD in ALGS patients (695 ± 465 vs. 457 ± 319 mg/dL, preoperatively vs. 12-24 months postoperatively, respectively; P = 0.0001). Alanine aminotransferase levels increased in ALGS after PEBD (182 ± 70 vs. 260 ± 73 IU/L, preoperatively vs. 24 months; P = 0.03), but not in FIC1 or BSEP. ALGS, FIC1, and BSEP patients experienced less severely scored pruritus after PEBD (ALGS, 100% vs. 9% severe; FIC1, 64% vs. 10%; BSEP, 50% vs. 20%, preoperatively vs.24 months postoperatively, respectively; P0.001). ALGS patients experienced a trend toward greater freedom from xanthomata after PEBD. There was a trend toward decreased pruritus in FIC1 after IE and GBC. Vitamin K supplementation increased in ALGS after PEBD (33% vs. 77%; P = 0.03). Overall, there were 15 major complications after surgery. Twelve patients (3 ALGS, 3 FIC1, and 6 BSEP) subsequently underwent liver transplantation.This was a multicenter analysis of nontransplant surgical approaches to intrahepatic cholestasis. Approaches vary, are well tolerated, and generally, although not uniformly, result in improvement of pruritus and cholestasis. (Hepatology 2017;65:1645-1654).

Biliary atresia (BA) is the most common liver disease leading to liver transplantation (LT) during childhood. The optimal timing of listing and LT for children with failed portoenterostomy (PE) is not clear. The aim of our study was to determine the optimal timing of LT for children with BA and failed PE by using a Markov model simulation analysis.A Markov model was constructed presenting the progression of the severity of liver status for patients with BA who underwent PE before 60 days old. Three treatment strategies were compared: LT for moderate liver disease (MLD), LT for severe liver disease (SLD), and no LT, and 10,000 patients were simulated in each strategy. Health states were defined as LT, early repeat LT (≤30 days after LT), late repeat LT (30 days after LT), status post-LT (period after first LT), and death.For patients with an available liver for transplantation (living donors), LT at MLD was associated with an increase of 17.4% additional expected life-years (LY) as compared with LT at SLD. Patient survival rates after 10 years were 84.7% and 75.5% in the MLD and SLD strategies, respectively. For the patients with no LT, the survival rate after 10 years was 48.1%. When the probability of deceased donor LT was lower than 50% from time of listing at 3 months, there was no increase in expected LY of MLD strategy. "No LT" resulted in approximately 60% reduction of expected LY compared with LT for patients with SLD.Our model suggests that early listing and transplantation is beneficial in patients with an available liver for transplantation. For patients in whom the probability for LT is low, there appears to be no advantage to early listing. A validation of the present model in a "real" cohort of patients with BA is needed.

Gastric variceal bleeding is associated with high morbidity and mortality. Balloon-occluded retrograde transvenous obliteration is a relatively new treatment used to control bleeding gastric varices that involves transvenous sclerosis of gastric varices through a spontaneous gastrorenal shunt. Here, we report on a 14-yr-old patient that underwent balloon-occluded retrograde transvenous obliteration for refractory bleeding fundal varices in the setting of esophageal varices and cirrhosis, which did not respond to medical management or endoscopic injection. This case report serves as a reminder that balloon-occluded retrograde transvenous obliteration can successfully control fundal variceal bleeding in pediatric patients and may serve as a bridge to liver transplantation.

For patients with short bowel syndrome (SBS), surgery can play an important role in preventing, mitigating, and, in some cases, reversing intestinal failure (IF). During intestinal resection, bowel length should be conserved to the fullest extent possible to avoid dependence on parenteral nutrition (PN). Bowel salvage may be improved by initially preserving tissue of questionable viability and later reevaluating during "second-look" procedures. Once the patient is stabilized, ostomy reversal and recruitment of distal unused bowel should be prioritized whenever feasible. Following progression to IF, surgical management of SBS depends on the symptoms and anatomical characteristics of the individual patient. For carefully selected patients with rapid intestinal transit and dilated bowel, longitudinal intestinal lengthening and tailoring (LILT) and serial transverse enteroplasty (STEP) procedures may provide benefit. Outcomes following STEP and LILT are generally similar, and the choice between these procedures may rest on surgeon preference. For patients with rapid intestinal transit in the absence of bowel dilation, segmental reversal of the small bowel may reduce PN requirements. Intestinal transplantation is the standard of care for patients in whom intestinal rehabilitation attempts have failed and who are at risk of life-threatening complications of PN. Because patients awaiting isolated intestine transplant show increased survival compared with patients awaiting combined intestine-liver transplant, early referral of appropriate patients, before the development of advanced liver disease, is critical to enhancing patient outcomes.

Pre-emptive transplantation is a well-established practice for certain types of end-organ failure such as in the use of kidney transplantation. For irreversible intestinal failure, total parenteral nutrition (TPN) remains the gold standard, due to the suboptimal long-term results of intestinal transplantation. As such, the only role for pre-emptive transplantation, if at all, will be for patients identified to be at high risk of complications and mortality while on definitive long-term TPN. In these patients, the timing of early listing and transplantation could become life-saving, taking into account that mortality on the waiting list is still the highest for intestinal candidates. The development of simulation models or pre-transplant scoring systems could help in selecting patients based on potential outcome on TPN or with transplantation, and recent reports from high-volume centers identify few underlying pathologic conditions and some TPN complications as at higher risk of increased morbidity and mortality. A pre-emptive transplant could be used as a rehabilitative procedure in a well-selected case-by-case scenario, among TPN patients at risk of liver failure, repeated central line infections, mesenteric infarction, short bowel syndrome (SBS)