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Background and Aims: Home parenteral nutrition (HPN) is essential in the management of chronic intestinal failure (CIF) and malignant bowel obstruction (MBO), particularly in cases where enteral feeding is not feasible. This review examines the evidence from 34 studies to evaluate the impact of HPN on survival and quality of life (QoL) in patients with MBO, CIF, and advanced cancer, as well as to identify clinical predictors of survival and address psychosocial challenges. Methods: A comprehensive review was conducted of 34 studies, focusing on the use of HPN in patients with MBO, CIF, and advanced cancer. Data were analyzed for survival outcomes, QoL metrics, and predictors of survival, including performance status, albumin levels, and the continuation of chemotherapy. The psychosocial aspects of HPN therapy were also assessed, particularly its influence on patient’s daily lives and mental health. Results: Key predictors of improved survival included good performance status, higher albumin levels, and the ability to continue chemotherapy. While HPN extended survival in many cases, its impact on QoL varied significantly. Psychosocial challenges, including lifestyle disruption and mental health strain, were common among long-term HPN patients, underscoring the need for comprehensive patient support. Conclusions: HPN can be a life-sustaining therapy for patients with CIF, MBO, and advanced cancer, but its success depends on careful patient selection and management. Identifying predictors of survival helps optimize outcomes, while addressing psychosocial challenges is crucial to minimizing the negative impact on QoL. This review highlights the need for a balanced approach to maximize the benefits of HPN.

Background/Objectives: Patients who undergo gastrectomy for gastric adenocarcinoma and subsequently develop chronic intestinal failure requiring long-term home parenteral nutrition (HPN) represent a clinically vulnerable cohort in whom survival is shaped by profound nutritional depletion and systemic inflammation. Immuno-nutritional biomarkers may support improved risk stratification in this setting. Methods: This retrospective study included adults who underwent gastrectomy for gastric cancer and developed malignant chronic intestinal failure requiring HPN. Immuno-nutritional status at HPN qualification was evaluated using the Controlling Nutritional Status (CONUT) score and the lymphocyte-to-monocyte ratio (LMR). Overall survival was analysed using Cox proportional hazards models. LMR discrimination was assessed using receiver operating characteristic (ROC) analysis with a Youden-derived cut-off, and differences in AUC were tested using DeLong’s method. Results: Ninety-seven patients met the inclusion criteria. Median overall survival was 176 days. In multivariable analysis, CONUT and LMR were the only independent predictors of survival. Each one-point increase in CONUT was associated with an approximately 70% increase in mortality risk. LMR demonstrated good discriminative ability (AUC 0.795), and a cut-off of 2.083 differentiated survival trajectories. The combined CONUT–LMR model improved prognostic classification, and DeLong’s test confirmed a significant AUC difference compared with single-marker models. Kaplan–Meier curves showed clear separation across CONUT and LMR strata (log-rank p < 0.001). Conclusions: Among patients requiring long-term HPN after gastrectomy for gastric cancer, CONUT and LMR provide complementary prognostic information. Their combined use enhances survival stratification and may support earlier identification of patients with high-risk trajectories.

The paucity of data on the epidemiology of chronic intestinal failure (CIF) in pediatric patients is a matter of particular concern. The objective of this article is to provide a comprehensive description of the epidemiology of CIF in Spain, encompassing its incidence, geographical distribution, underlying causes, and demographic and clinical characteristics. These findings are based on data collected from the multicentre REPAFI registry. This is a national, multicentre, ambispective cohort study including patients who initiated home parenteral nutrition (HPN) between January 2015 and January 2025. The data collected encompassed various demographic details, underlying diagnoses, the type of HPN utilized, and the nutritional status of the subjects at the commencement of HPN treatment. The study included 163 patients (55.2% male) from 10 hospitals. The principal cause of CIF was short bowel syndrome (SBS) in 77.3% of cases, followed by severe motility disorders (12.9%), congenital enteropathies (CE) (5.5%), and other causes (4.3%). Among patients diagnosed with SBS, necrotizing enterocolitis was identified as the most prevalent underlying cause (32.5%). The most prevalent anatomical configuration was identified as type 2 (jejuno-colic anastomosis). A significant proportion, amounting to 62.7%, exhibited a lack of an ileocecal valve (ICV), while 23% demonstrated a residual bowel length (RBL) of less than 15 centimetres. The median RBL was 35 cm (IQR: 15.7-52.5). Patients diagnosed with SBS exhibited a lower gestational age and birthweight compared with the other groups ( < 0.05). Patients diagnosed with SBS and CE exhibited a lower mean age at the onset of HPN ( < 0.05). Furthermore, patients with CE exhibited the lowest weight-for-age Z-score at the initiation of HPN ( < 0.05). The present study provides the first epidemiological data on the state of pediatric CIF in Spain. The most prevalent cause of CIF was SBS, with a younger age at the initiation of HPN in comparison to other published studies. Patients with CE exhibited the most severe degree of malnutrition at the initiation of HPN.

Patients with chronic intestinal failure (CIF) often experience reduced quality of life due to the psychosocial constraints of home parenteral nutrition (HPN) and/or fluid infusions. Despite its major impact on daily life, adherence to these treatments remains poorly documented. To assess adherence to HPN and/or fluid infusions in adult CIF patients and identify associated factors. This multicenter, cross-sectional study included CIF patients from six French HPN centers. Eligible patients were ≥18 years old, on HPN and/or fluid infusions for ≥6 months with a stable regimen for ≥3 months. Adherence was assessed via self-reported questionnaires, physician and coordinating nurse evaluations, and phone interviews. Anxiety and depression were evaluated using the Hospital Anxiety and Depression Scale, and the Wake Forest Physician Trust Scale measured trust in the physician. A total of 126 patients were included; 82% had their infusions administered by nurses. Overall, 24% of patients exhibited poor adherence, with 9.5% negotiating treatment and 18% missing infusions. Poor adherence was associated with low weekly infusion volume (<7750 mL, odds ratio [OR] 3.31, 95% confidence interval [CI] [1.09–11.2], P = 0.041), high anxiety scores (OR 5.29, 95% CI [1.58–19.2], P = 0.008), and low trust in physicians (Wake Forest Physician Trust Scale, OR 0.90, 95% CI [0.82–0.98], P = 0.018). Coordinating nurses’ perception strongly predicted adherence. One in four CIF patients showed poor adherence. Those with low infusion volumes, high anxiety, or low physician trust require close monitoring. Coordinating nurses play a key role in identifying non-adherence. •One in four patients with chronic intestinal failure has poor adherence to HPN.•Low HPN volume, anxiety, and low trust in physicians are key risk factors for poor adherence.•Coordinating nurses’ perception is a strong predictor of patient adherence to HPN.•Patients who negotiate their treatment are more likely to miss prescribed HPN infusions.•Multidisciplinary teams are essential to improve adherence in HPN patients.

Short-bowel syndrome (SBS) is defined as a state of malabsorption after resection or loss of a major portion of the bowel due to congenital or acquired factors. This article presents an overview on the recent management of pediatric SBS. The pediatric SBS population is very heterogeneous. The incidence of SBS is estimated to be 24.5 per 100,000 live births. The nutritional, medical, and surgical therapies available require a comprehensive evaluation. Thus, multidisciplinary intestinal rehabilitation programs (IRPs) are necessary for the management of these complex patients. The key points of focus in IRP management are hepato-protective strategies to minimize intestinal failure-associated liver disease; the aggressive prevention of catheter-related bloodstream infections; strategic nutritional supply to optimize the absorption of enteral calories; and the management and prevention of small bowel bacterial overgrowth, nephrocalcinosis, and metabolic bone disease. As the survival rate of children with SBS currently exceeds 90%, the application of small bowel transplantation has been evolving. The introduction of innovative treatments, such as combined therapy of intestinotrophic hormones, including glucagon-like peptide-2, may lead to further improvements in patients’ quality of life.

Children with intestinal failure suffer liver damage associated with parenteral nutrition: a condition known as intestinal failure-associated liver disease (IFALD), which requires transplantation of both liver and intestine. In many countries, simultaneous transplantation of these two organs is performed using grafts from a deceased donor, but there have been no such cases in Japan, and the details of the procedure are not clear. Recently, we performed simultaneous split liver and intestinal transplantation in two premature infants with IFALD, using organs from identical deceased donors and achieved good results. These are the first two cases of this procedure being performed in Japan. We report these cases and discuss the important aspects of the surgical and perioperative management.

Background: Chronic intestinal failure (CIF) is a heterogeneous disease that affects pediatric and adult populations worldwide and requires complex multidisciplinary management. In recent years, many advances in intravenous supplementation support, surgical techniques, pharmacological management, and intestinal transplants have been published. Based on these advances, international societies have published multiple recommendations and guidelines for the management of these patients. The purpose of this paper is to show the differences that currently exist between the recommendations (ideal life) and the experiences published by different programs around the world. Methods: A review of the literature in PubMed from 1980 to 2024 was carried out using the following terms: intestinal failure, CIF, home parenteral nutrition, short bowel syndrome, chronic intestinal pseudo-obstruction, intestinal transplant, enterohormones, and glucagon-like peptide-2. Conclusions: There is a difference between what is recommended in the guidelines and consensus and what is applied in real life. Most of the world’s countries are not able to offer all of the steps needed to treat this pathology. The development of cooperative networks between countries is necessary to ensure access to comprehensive treatment for most patients on all continents, but especially in low-income countries.

Purpose To identify the prognostic factors for pediatric severe intestinal motility disorder (IMD). Methods We reviewed the medical records of patients with severe IMD, who required total parenteral nutrition (TPN) for ≥ 60 days at our institution between April, 1984 and March, 2023, examining their characteristics to identify prognostic factors. Results The types of IMD in the 14 patients enrolled in this study were as follows: isolated hypoganglionosis (IHG, n  = 6), extensive aganglionosis (EAG: n  = 6), and chronic idiopathic intestinal pseudo-obstruction (CIIP, n  = 2). There was no significant difference in mortality among the three types of severe IMD. Weaning-off TPN and the use of the colon were not significant prognostic factors, but cholestasis was a significant prognostic factor ( p  = 0.005). There was a high mortality rate (50%), with the major causes of death being intestinal failure-associated liver disease (IFALD) following hepatic failure, and catheter-related blood stream infection (CRBSI). One IHG patient underwent small bowel transplantation but died of acute rejection. Conclusion Severe IMD is still associated with a high mortality rate and cholestasis predicts the prognosis. Thus, preventing or improving IFALD and CRBSI caused by long-term TPN is important for reducing the mortality rate.

•Mortality and weaning rates of patients with chronic intestinal failure in home parenteral nutrition differ widely among cohorts, because these outcomes were often considered independent—rather than competing—events, leading to biased estimates•We analyzed through a competing risk analysis the rates and predictors of mortality and weaning separately in patients with and without short bowel syndrome•In patients with short bowel syndrome, reconstructive surgery reduced mortality and dependence from parenteral nutrition•In patients without short bowel syndrome, presence of a stoma was associated with reduced mortality and dependence from parenteral nutrition•Surgical procedures strongly affected mortality and weaning risk in patients with chronic intestinal failure. Home parenteral nutrition (HPN) is the standard treatment for patients with chronic intestinal failure (CIF). Mortality and weaning rates of these patients differ widely among cohorts; however, these outcomes were often considered independent—rather than competing—events, leading to an upward bias of the retrieved estimates. The aim of this retrospective cohort study was to evaluate, evaluating through a competing risk analysis, the rates and predictors of mortality and weaning in CIF patients from an Italian referral center. All adult patients with CIF receiving > 3 mo HPN from 1985 until 2016 were enrolled. Clinical information was collected from the database of the Intestinal Failure Unit of Torino, Italy. Patients were stratified according to the presence or not of short bowel syndrome (SBS). The cumulative incidences of death and weaning were 27.3% and 32.3% and 39.0% and 33.7% at 5 and 10 y from HPN initiation, respectively. At multivariable competing risk analyses, mortality was predicted by age (sub-distribution hazard ratio [SHR] = 1.65 per 10-y increase; 95% CI, 1.35–2.01), type 3 SBS (SHR = 0.38; 0.15–0.94), small bowel length ≥ 100 cm (SHR = 0.42; 0.22–0.83), and reconstructive surgery (SHR = 0.11; 0.02–0.64) in SBS patients, and by age (SHR = 1.38 per 10-y increase; 1.16–1.64) and presence of stoma (SHR = 0.30; 0.12–0.78) in non-SBS patients. In the same model, weaning was predicted by type 3 SBS (SHR = 6.86; 3.10–15.16), small bowel length ≥ 100 cm (SHR = 3.54; 1.99–6.30), and reconstructive surgery (SHR = 2.86; 1.44–5.71) in SBS patients, and by age (SHR = 0.79 per 10-y increase; 0.66–0.94) and presence of stoma (SHR = 2.64; 1.38–5.07) in non-SBS patients. Surgical procedures strongly affected mortality and weaning risk in CIF patients.

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.