Optimal Treatment Approaches to Intestinal Behçet's Disease Complicated by Myelodysplastic Syndrome: The KASID and KSBD Multicenter Study.

PURPOSE: Studies on intestinal Behçet's disease (BD) complicated by myelodysplastic syndrome (MDS) are rare, and no established therapeutic guidelines exist. This study aimed to evaluate the clinical presentation and outcomes of patients with intestinal BD complicated by MDS (intestinal BD-MDS) and suggest a treatment strategy. MATERIALS AND METHODS: Data from patients with intestinal BD-MDS from four referral centers in Korea who were diagnosed between December 2000 and December 2022 were retrospectively analyzed. Clinical features and prognosis of intestinal BD-MDS compared with age-, sex-matched intestinal BD without MDS were investigated. RESULTS: Thirty-five patients with intestinal BD-MDS were included, and 24 (70.6%) had trisomy 8. Among the 35 patients, 23 (65.7%) were female, and the median age at diagnosis for intestinal BD was 46.0 years (range, 37.0-56.0 years). Medical treatments only benefited eight of the 32 patients, and half of the patients underwent surgery due to complications. Compared to 70 matched patients with intestinal BD alone, patients with intestinal BD-MDS underwent surgery more frequently (51.4% vs. 24.3%; p=0.010), showed a poorer response to medical and/or surgical treatment (75.0% vs. 11.4%; p<0.001), and had a higher mortality (28.6% vs. 0%; p<0.001). Seven out of 35 patients with intestinal BD-MDS underwent hematopoietic stem cell transplantation (HSCT), and four out of the seven patients had a poor response to medical treatment prior to HSCT, resulting in complete remission of both diseases. CONCLUSION: Patients with intestinal BD-MDS frequently have refractory diseases with high mortalities. HSCT can be an effective treatment modality for medically refractory patients with intestinal BD-MDS.

[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.

Catheter salvage from central line-related bloodstream infections in pediatric intestinal failure.

OBJECTIVES: Patients with intestinal failure require central venous access which puts them at risk for central line-associated bloodstream infections (CLABSI). Maintaining vascular patency is critical for this population to receive nutrition support. When CLABSIs occur line salvage can help maintain vascular access. The aim of this study is to assess factors associated with safe and successful central venous catheter salvage. METHODS: Retrospective cohort study of patients with intestinal failure at two tertiary care institutions between 2012 and 2020. The study examined the rates of attempted salvage, factors associated with successful salvage, and complications associated with salvage attempts. RESULTS: Over the study period, 76 patients with intestinal failure were include while central venous access was in place. There were a total of 94 CLABSIs. Salvage was more likely to be attempted when patients were under the direct care of an intestinal rehabilitation service (95% vs. 68%, p = 0.04). The overall successful salvage rate was 91.6% (n = 77). Gram-positive, Gram-negative, and polymicrobial infections had successful salvage rates of 97%, 92%, and 94% respectively. The successful salvage rate for fungal infections was 40%. There was no difference in 30-day complication rates for hospital readmission, intensive care unit admission, and death between patients who underwent salvage attempt and those who did not. CONCLUSIONS: Central line salvage can be safely attempted for many infections in patients with intestinal failure, leading to vascular access preservation.

Non-interventional, 5-year retrospective data of home parenteral nutrition in patients with benign chronic intestinal failure: Analysis of an Italian nurse registry (SERECARE II).

OBJECTIVE: This study is an assessment of home parenteral nutrition service performance and safety and efficacy outcomes in patients with benign chronic intestinal failure. METHODS: This is a retrospective, non-interventional, and multicenter study. Data were collected by trained nurses and recorded in a dedicated registry (SERECARE). RESULTS: From January 1, 2013 to June 30, 2018, data from a total of 683 patients with benign chronic intestinal failure were entered in the registry. Patients included 208 pediatric (53.8% male; median age = 4.0 y) and 475 adult (47.6% male; median age = 59.0 y) participants. On average, patients were visited 5.4 ± 4.5 times and received 1.4 ± 0.8 training sessions. Retraining was not common and mostly due to change of therapy or change of caregiver. Of 939 complications, 40.9% were related to the central venous catheter and were mostly infectious (n = 182) and mechanical (n = 187). The rate of infectious and mechanical complications per 1000 catheter days decreased over 5 y (0.30-0.15 and 0.33 -0.19, respectively). The rate of complications per 1000 catheter days and the mean complications per patient were higher in pediatric than in adult patients. The hospitalization rate was 1.01 per patient throughout the study period. These data were similar to those registered in a previous study period (2002-2011) (n = 1.53 per patient). Changes over time in the efficacy variables were mostly small and non-significant. CONCLUSIONS: This study confirms the importance of setting up and maintaining structured registries to monitor and improve home parenteral nutrition care. Safety outcomes have improved over the years, most likely due to the underlying efficient nursing service.

Inflammation drives pathogenesis of early intestinal failure-associated liver disease.

Patients with intestinal failure who receive long-term parenteral nutrition (PN) often develop intestinal failure-associated liver disease (IFALD). Although there are identified risk factors, the early pathogenesis is poorly understood and treatment options are limited. Here, we perform a transcriptomic analysis of liver tissue in a large animal IFALD model to generate mechanistic insights and identify therapeutic targets. Preterm Yorkshire piglets were provided PN or bottle-fed with sow-milk replacer for 14 days. Compared to bottle-fed controls, piglets receiving PN developed biochemical cholestasis by day of life 15 (total bilirubin 0.2 vs. 2.9 mg/dL, P = 0.01). RNA-Seq of liver tissue was performed. Ingenuity Pathway Analysis identified 747 differentially expressed genes (343 upregulated and 404 downregulated) with an adjusted P < 0.05 and a fold-change of > |1|. Enriched canonical pathways were identified, demonstrating broad activation of inflammatory pathways and inhibition of cell cycle progression. Potential therapeutics including infliximab, glucocorticoids, statins, and obeticholic acid were identified as predicted upstream master regulators that may reverse the PN-induced gene dysregulation. The early driver of IFALD in neonates may be inflammation with an immature liver; identified therapeutics that target the inflammatory response in the liver should be investigated as potential treatments.

The presence of enteropathy in HIV infected children on antiretroviral therapy in Malawi.

BACKGROUND: Undernutrition and malnutrition in children in low- and middle-income countries contribute to high mortality rates. Stunting, a prevalent form of malnutrition, is associated with educational and productivity losses. Environmental enteric dysfunction (EED) and human immunodeficiency virus (HIV) infection worsen these conditions. This study seeks to investigate the presence of enteropathy using EED fecal biomarkers in HIV-infected children who are stable on antiretroviral therapy (ART) across various nutritional statuses. By understanding the interplay between EED, HIV, and nutritional status, this study aims to provide insights that can inform targeted interventions to optimize nutritional outcomes in HIV infected children. METHODS/PRINCIPAL FINDINGS: This study evaluated the levels of alpha-1-antitrypsin, calprotectin and myeloperoxidase in frozen fecal samples from 61 HIV infected (mean age 9.16 ±3.08 years) and 31 HIV uninfected (6.65 ±3.41 years) children in Malawi. Anthropometric measurements and clinical data were collected. The height-for-age z-score (-1.66 vs -1.27, p = 0.040) and BMI-for-age z-score (-0.36 vs 0.01, p = 0.037) were lower in HIV infected children. Enzyme-linked immunosorbent assays were used to measure biomarker concentrations. Statistical tests were applied to compare biomarker levels based on HIV status and anthropometric parameters. Myeloperoxidase, alpha-1-antitrypsin, and calprotectin concentrations did not differ between HIV infected and HIV uninfected children of different age groups. In HIV infected children from 5-15 years, there is no difference in biomarker concentration between the stunted and non-stunted groups. CONCLUSION/SIGNIFICANCE: Our study found a higher prevalence of stunting in HIV infected children compared to uninfected children, but no significant differences in biomarker concentrations. This suggests no causal relationship between enteropathy and stunting in HIV infected children. These results contribute to the understanding of growth impairment in HIV infected children and emphasize the need for further research, particularly a longitudinal, biopsy-controlled study.

Predictive value of serum fibroblast growth factor 19 and liver stiffness for intestinal failure associated liver disease-cholestasis.

BACKGROUND: Intestinal failure associated liver disease (IFALD)-cholestasis is a common complication of long-term parenteral nutrition (PN) in patients with intestinal failure (IF). The lack of effective early identification indicators often results in poor clinical outcomes. The objective of this study was to evaluate the predictive value of serum FGF19 and liver stiffness in IFALD-cholestasis. METHODS: Eligible adults diagnosed with IF were identified from Jinling Hospital in China. Diagnostic criteria for IFALD-cholestasis: total bilirubin >1 mg/dL and conjugated bilirubin >0.3 mg/dL for ≥6 months. Fasting blood specimens were prospectively collected and serum FGF19 concentrations were determined using ELISA and liver stiffness was measured by Two-dimensional shear wave elastography. Binary logistic regression analysis identified predictors of IFALD-cholestasis. Receiver operating characteristic (ROC) curves and areas under the ROC curves (AUROC) were used to evaluate the accuracy of serum FGF19 and liver stiffness in identifying IFALD-cholestasis. RESULTS: Of 203 study patients with IF, 70 (34.5%) were diagnosed with IFALD-cholestasis. The serum FGF19 levels in those with IFALD-cholestasis were significantly decreased compared with those in patients without, and liver stiffness was significantly increased (p < 0.001). Multivariate logistic regression analyses suggested that intestinal discontinuity, dependence on PN, liver stiffness >6.5 kPa, and serum FGF19 ≤107 pg/mL were independent risk factors for IFALD-cholestasis. The AUROC for serum FGF19 and liver stiffness, which indicate the occurrence of IFALD-cholestasis, were 0.810 and 0.714, respectively. Serum FGF19 had a superior predictive performance than liver stiffness (p < 0.05). CONCLUSION: Both low circulating serum FGF19 concentration and increased liver stiffness are excellent predictors of IFALD-cholestasis, but serum FGF19 is superior to increased liver stiffness in predicting IFALD-cholestasis.

Cost-effectiveness of taurolidine-citrate in a cohort of patients with intestinal failure receiving home parenteral nutrition.

BACKGROUND: Catheter-related bloodstream infections (CRBSIs) in patients receiving home parenteral nutrition (HPN) for chronic intestinal failure (CIF) are associated with significant morbidity and financial costs. Taurolidine is associated with a reduction in bloodstream infections, with limited information on the cost-effectiveness as the primary prevention. This study aimed to determine the cost-effectiveness of using taurolidine-citrate for the primary prevention of CRBSIs within a quaternary hospital. METHODS: All patients with CIF receiving HPN were identified between January 2015 and November 2022. Data were retrospectively collected regarding patient demographics, HPN use, CRBSI diagnosis, and use of taurolidine-citrate. The direct costs associated with CRBSI-associated admissions and taurolidine-citrate use were obtained from the coding department using a bottom-up approach. An incremental cost-effective analysis was performed, with a time horizon of 4 years, to compare the costs associated with primary and secondary prevention against the outcome of cost per infection avoided. RESULTS: Forty-four patients received HPN within this period. The CRBSI rates were 3.25 infections per 1000 catheter days before the use of taurolidine-citrate and 0.35 infections per 1000 catheter days after taurolidine-citrate use. The incremental cost-effectiveness ratio indicates primary prevention is the weakly dominant intervention, with the base case value of $27.04 per CRBSI avoided. This held with one-way sensitivity analysis. CONCLUSION: Taurolidine-citrate in the primary prevention of CRBSIs in patients with CIF receiving HPN is associated with reduced hospital costs and infection rates.

Complete Resection of Necrotic Bowel Improves Survival in NEC Without Compromising Enteral Autonomy.

AIMS: Controversy persists regarding operative strategy for necrotising enterocolitis (NEC). Some surgeons advocate resecting all necrotic bowel, whilst others defunction with a stoma, leaving diseased bowel in situ to preserve bowel length. We reviewed our institutional experience of both approaches. METHODS: Neonates undergoing laparotomy for NEC May 2015-2019 were identified. Data extracted from electronic records included: demographics, neonatal Sequential Organ Failure Assessment (nSOFA) score at surgery, operative findings, and procedure performed. Neonates were assigned to two groups according to operative strategy: complete resection of necrotic bowel (CR) or necrotic bowel left in situ (LIS). Primary outcome was survival, and secondary outcome was enteral autonomy. Outcomes were compared between groups. RESULTS: Fifty neonates were identified. Six were excluded: 4 with NEC totalis and 2 with no visible necrosis or histological confirmation of NEC. Of the 44 remaining neonates, 27 were in the CR group and 17 in the LIS group. 32 neonates survived to discharge (73%). On univariate analysis, survival was associated with lower nSOFA score (P = 0.003), complete resection of necrotic bowel (OR 9.0, 95% CI [1.94-41.65]), and being born outside the surgical centre (OR 5.11 [1.23-21.28]). On Cox regression multivariate analysis, complete resection was still strongly associated with survival (OR 4.87 [1.51-15.70]). 28 of the 32 survivors (88%) achieved enteral autonomy. There was no association between operative approach and enteral autonomy (P = 0.373), or time to achieve this. CONCLUSION: Complete resection of necrotic bowel during surgery for NEC significantly improves likelihood of surviving without negatively impacting remaining bowel function. LEVEL OF EVIDENCE: III.

A comparison of health-related quality of life in chronic intestinal failure and end-stage kidney disease: A cross-sectional study.

BACKGROUND: There is inequal access to treatment and scarce evidence on how the disease burden in chronic intestinal failure (CIF) compares to other chronic nonmalignant types of organ failure. Therefore, we compared the health-related quality of life (HRQOL) of people with CIF with that of people with end-stage kidney disease (ESKD) receiving hemodialysis (HD). These groups were selected for comparison as they have similar treatment characteristics. We hypothesized that people treated with HD and people with CIF had similarly poor HRQOL. METHODS: HRQOL was evaluated and compared in a cross-sectional study of adult people with CIF and people with ESKD HD at a tertiary hospital in Denmark, using the Short-Form 36 (SF-36). RESULTS: One hundred forty-one people with CIF and 131 people with ESKD receiving HD were included in the analysis. Both groups reported low scores (<50) for HRQOL on general health, vitality, and role limitation-physical. People with ESKD receiving HD had significantly lower scores than people with CIF regarding physical functioning, general health, and vitality when adjusted for sex and age. No significant difference was found for any other SF-36 domain. CONCLUSION: HRQOL was similarly and significantly reduced in people with CIF and in people with ESKD receiving HD. People with ESKD receiving HD had significantly poorer HRQOL than people with CIF in some aspects of physical and mental health. Access to home parenteral support treatment varies among countries that typically provide HD, suggesting an inequality in healthcare based on the type of organ failure.

Advanced enteropathy-associated T cell lymphoma (EATL) presenting with severe malabsorption and concomitantly diagnosed coeliac disease (CD).

Enteropathy-associated T cell lymphoma (EATL) is an aggressive subtype of non-Hodgkin's lymphoma often associated with coeliac disease (CD). We describe a previously healthy man in his 50 s who presented with a history of abdominal pain, failure to thrive and significant weight loss over a 3-month period. Investigations revealed a positive coeliac serology, diffuse duodenal atrophy with multiple duodenal and jejunal ulcers on endoscopy and mesenteric lymphadenopathy on CT imaging. Duodenal tissue biopsy confirmed a diagnosis of EATL Stage IVB. Chemotherapy with cyclophosphamide, doxorubicin, vincristine and prednisone regimen was initiated. This case highlights the need for greater awareness and consideration of EATL in individuals with worsening malabsorption and abdominal pain, irrespective of coeliac history.

[Small intestinal mucormycosis associated with acute myeloid leukemia: a case report].

The patient was a man in his 70s. During the treatment for acute myeloid leukemia, abdominal pain and bloody stools appeared. A diagnosis of small intestinal ileus was made by computed tomography scan. Treatment with an ileus tube did not improve his condition, and enteroscopy revealed the presence of ileal ulcers. Based on histological examination, small intestinal mucormycosis was suspected, and thus, antifungal drugs were administered. However, the patient developed perforated peritonitis and underwent small intestine resection. He was finally diagnosed with small intestinal mucormycosis with the help of the resected specimen. The gastrointestinal form of mucormycosis rarely occurs, and small intestinal lesions are very rare. Enteroscopy was helpful in its diagnosis and treatment.

Thalidomide for Recurrent Bleeding Due to Small-Intestinal Angiodysplasia.

BACKGROUND: Recurrent bleeding from the small intestine accounts for 5 to 10% of cases of gastrointestinal bleeding and remains a therapeutic challenge. Thalidomide has been evaluated for the treatment of recurrent bleeding due to small-intestinal angiodysplasia (SIA), but confirmatory trials are lacking. METHODS: We conducted a multicenter, double-blind, randomized, placebo-controlled trial to investigate the efficacy and safety of thalidomide for the treatment of recurrent bleeding due to SIA. Eligible patients with recurrent bleeding (at least four episodes of bleeding during the previous year) due to SIA were randomly assigned to receive thalidomide at an oral daily dose of 100 mg or 50 mg or placebo for 4 months. Patients were followed for at least 1 year after the end of the 4-month treatment period. The primary end point was effective response, which was defined as a reduction of at least 50% in the number of bleeding episodes that occurred during the year after the end of thalidomide treatment as compared with the number that occurred during the year before treatment. Key secondary end points were cessation of bleeding without rebleeding, blood transfusion, hospitalization because of bleeding, duration of bleeding, and hemoglobin levels. RESULTS: Overall, 150 patients underwent randomization: 51 to the 100-mg thalidomide group, 49 to the 50-mg thalidomide group, and 50 to the placebo group. The percentages of patients with an effective response in the 100-mg thalidomide group, 50-mg thalidomide group, and placebo group were 68.6%, 51.0%, and 16.0%, respectively (P<0.001 for simultaneous comparison across the three groups). The results of the analyses of the secondary end points supported those of the primary end point. Adverse events were more common in the thalidomide groups than in the placebo group overall; specific events included constipation, somnolence, limb numbness, peripheral edema, dizziness, and elevated liver-enzyme levels. CONCLUSIONS: In this placebo-controlled trial, treatment with thalidomide resulted in a reduction in bleeding in patients with recurrent bleeding due to SIA. (Funded by the National Natural Science Foundation of China and the Shanghai Municipal Education Commission, Gaofeng Clinical Medicine; ClinicalTrials.gov number, NCT02707484.).

Risk factors for catheter-related bloodstream infections associated with home parental nutrition in children with intestinal failure: A prospective cohort study.

BACKGROUND & AIMS: Catheter-related bloodstream infection (CRBSI) is the most common, potentially life-threatening complication of long-term parenteral nutrition (PN). We prospectively assessed the incidence and risk factors for CRBSI in children receiving long-term home PN (HPN) for intestinal failure (IF) in a single IF rehabilitation center. METHODS: Data regarding episodes and potential risk factors for CRBSI in children on HPN were prospectively recorded. RESULTS: Forty-one of 75 children were diagnosed with CRBSI. The overall CRBSI rate was 1.61 per 1000 catheter days. The indications for HPN were gastrointestinal motility disorders in 35%, short bowel syndrome (SBS) in 28% graft versus host disease (GvHD) post bone marrow transplant in 17%, congenital enteropathy in 15%, and severe neurodevelopmental impairment in 5%. Gastrointestinal motility disorders had significantly higher CRBSI rate compared to other groups (p < 0.0005; 2.74 in motility group vs 1.54 in GvHD group vs 0.52 in congenital enteropathies vs 0.36 in SBS group vs 0.67 in severe neurodevelopmental delay). Multivariate analysis revealed that enterocutaneous distal stoma (ileostomy or colostomy) (HR 3.35 [95% CI, 1.63-6.86]; p < 0.001), age <2 years (HR 0.28 [95% CI, 0.15-0.53]; p < 0.0001), male sex (HR 2.28 [95% CI, 1.51-3.43]; p < 0.0001), non-use of taurolidine citrate lock (HR 2.70 [95% CI, 1.72-4.11]; p < 0.0001) and gastrointestinal motility disorder (HR 3.02 [95% CI, 1.81-4.91]; p < 0.001) were independent risk factors for developing CRBSI. CONCLUSIONS: Extra care in managing PN connections and disconnections should be taken in children with an underlying gastrointestinal motility disorder, distal enterocutaneous stoma, male sex and those aged <2 years since they are at a significantly higher risk of CRBSI. Early introduction of taurolidine lock should be considered.

Cutoffs and Characteristics of Abnormal Bowel Dilatation in Pediatric Short Bowel Syndrome.

OBJECTIVES: Although excessive intestinal dilatation associates with worsened outcomes in pediatric short bowel syndrome (SBS), little is known about the natural history and definition of pathological dilatation. We addressed fore-, mid-, and hind-gut dilatation in children with SBS, who had not undergone autologous intestinal reconstructive (AIR) surgery, in relation to controls. METHODS: SBS children without history of AIR surgery (n = 59) and age-matched controls without any disclosed intestinal pathology (n = 140) were included. Maximum diameter of duodenum, small bowel (SB), and colon were measured in each intestinal contrast series during 2002 to 2020 and expressed as diameter ratio (DR) to L5 vertebrae height. Predictive ability of DR for weaning off parenteral nutrition (PN) was analyzed with Cox proportional hazards regression models using multiple cutoffs. RESULTS: Duodenum (DDR), SB (SBDR), and colon (CDR) DR were 53%, 183%, and 23% higher in SBS patients compared to controls ( P < 0.01 for all). The maximal DDR and SBDR measured during follow-up is associated with current PN dependence and young age. DDR correlated with SBDR ( r = 0.586, P < 0.01). Patients with maximal DDR less than 1.5, which was also the 99th percentile for controls, were 2.5-fold more likely to wean off PN ( P = 0.005), whereas SBDR and CDR were not predictive for PN duration. CONCLUSIONS: All segments of remaining bowel, especially SB, dilate above normal levels in children with SBS. In SBS children without AIR surgery, PN dependence and young age is associated with duodenal and small intestinal dilatation, while duodenal dilatation also predicted prolonged PN.

The gut microbiome and intestinal failure-associated liver disease.

Intestinal failure-associated liver disease (IFALD) is a common hepatobiliary complication resulting from long-term parenteral nutrition (PN) in patients with intestinal failure. The spectrum of IFALD ranges from cholestasis, steatosis, portal fibrosis, to cirrhosis. Development of IFALD is a multifactorial process, in which gut dysbiosis plays a critical role in its initiation and progression in conjunction with increased intestinal permeability, activation of hepatic immune responses, and administration of lipid emulsion. Gut microbiota manipulation including pre/probiotics, fecal microbiota transplantation, and antibiotics has been studied in IFALD with varying success. In this review, we summarize current knowledge on the taxonomic and functional changes of gut microbiota in preclinical and clinical studies of IFALD. We also review the function of microbial metabolites and associated signalings in the context of IFALD. By providing microbiota-targeted interventions aiming to optimize PN-induced liver injury, our review provides perspectives for future basic and translational investigations in the field.

Current Insights Regarding Intestinal Failure-Associated Liver Disease (IFALD): A Narrative Review.

Intestinal failure-associated liver disease (IFALD) is a spectrum of liver disease including cholestasis, biliary cirrhosis, steatohepatitis, and gallbladder disease in patients with intestinal failure (IF). The prevalence of IFALD varies considerably, with ranges of 40-60% in the pediatric population, up to 85% in neonates, and between 15-40% in the adult population. IFALD has a complex and multifactorial etiology; the risk factors can be parenteral nutrition-related or patient-related. Because of this, the approach to managing IFALD is multidisciplinary and tailored to each patient based on the etiology. This review summarizes the current knowledge on the etiology and pathophysiology of IFALD and examines the latest evidence regarding preventative measures, diagnostic approaches, and treatment strategies for IFALD and its associated complications.

Pediatric intestine and multivisceral transplant.

PURPOSE OF REVIEW: Intestinal and multivisceral transplantation (ITx, MVTx) is the cornerstone in treatment of irreversible intestinal failure (IF) and complications related to parenteral nutrition. This review aims to highlight the unique aspects of the subject in pediatrics. RECENT FINDINGS: Etiology of intestinal failure (IF) in children shares some similarity with adults but several unique considerations when being evaluated for transplantation will be discussed. Owing to significant advancement in IF management and home parenteral nutrition (PN), indication criteria for pediatric transplantation continues to be updated. Outcomes have continued to improve with current long-term patient and graft survival in multicenter registry reports reported at 66.1% and 48.8% at 5 years, respectively. Pediatric specific surgical challenges such abdominal closure, post transplantation outcomes, and quality of life are discussed in this review. SUMMARY: ITx and MVTx remain lifesaving treatment for many children with IF. However long-term graft function is still a major challenge.