In 2016, ESPEN published the guideline for Chronic Intestinal Failure (CIF) in adults. An updated version of ESPEN guidelines on CIF due to benign disease in adults was devised in order to incorporate new evidence since the publication of the previous ESPEN guidelines.
The grading system of the Scottish Intercollegiate Guidelines Network (SIGN) was used to grade the literature. Recommendations were graded according to the levels of evidence available as A (strong), B (conditional), 0 (weak) and Good practice points (GPP). The recommendations of the 2016 guideline (graded using the GRADE system) which were still valid, because no studies supporting an update were retrieved, were reworded and re-graded accordingly.
The recommendations of the 2016 guideline were reviewed, particularly focusing on definitions, and new chapters were included to devise recommendations on IF centers, chronic enterocutaneous fistulas, costs of IF, caring for CIF patients during pregnancy, transition of patients from pediatric to adult centers. The new guideline consist of 149 recommendations and 16 statements which were voted for consensus by ESPEN members, online in July 2022 and at conference during the annual Congress in September 2022. The Grade of recommendation is GPP for 96 (64.4%) of the recommendations, 0 for 29 (19.5%), B for 19 (12.7%), and A for only five (3.4%). The grade of consensus is \"strong consensus\" for 148 (99.3%) and \"consensus\" for one (0.7%) recommendation. The grade of consensus for the statements is \"strong consensus\" for 14 (87.5%) and \"consensus\" for two (12.5%).
It is confirmed that CIF management requires complex technologies, multidisciplinary and multiprofessional activity, and expertise to care for the underlying gastrointestinal disease and to provide HPN support. Most of the recommendations were graded as GPP, but almost all received a strong consensus.

An international, multidisciplinary management working group (MWG) convened to review clinically useful short bowel syndrome (SBS) literature and identify gaps and inconsistencies in the management of adults with SBS.
Using nominal group technique for literature review, key publications were identified, discussed, and ranked by importance related to management of SBS. Gaps in management recommendations for SBS were identified upon critical review of the selected publications.
Five guidelines, seven review articles, one series of six articles, and one single center series were selected and prioritized for their importance to SBS management. Evaluation of the articles by the MWG identified ten gaps and opportunities to standardize and improve SBS management.
The main practice areas in need of more definitive guidelines are the management of high stool output and strategies to improve absorption of medications, nutrients, and fluids. An understanding of current real-world clinical practices related to these gaps could allow for development of best practice standards and improve patient-focused care.

Intestinal failure secondary to short bowel syndrome in pediatrics, is a rare condition with high morbimortality. A follow up multidisciplinary team is necessary to minimize complications and optimize the intestinal rehabilitation. There are no gold standard guidelines for the management of this group of complex patients. The development of clinical guidelines may contribute for an adequate management of patients with intestinal failure and short bowel syndrome. This Clinical Guideline for the Management was developed by 16 experts based on modified Delphi methodology. The meetings were held at the Argentinian Association of Enteral and Parenteral Nutrition (Asociación Argentina de Nutrición Enteral y Parenteral); the topics analyzed were definitions, epidemiology, enteral and parenteral nutrition, pharmacological and surgical treatments, and criteria for referring patients to intestinal rehabilitation centers. The document is aimed to provide basic scientific knowledge for medical institutions, health providers, healthcare providers, patients and families.
La falla intestinal secundaria a síndrome de intestino corto en pediatría es una entidad poco frecuente, de alta morbimortalidad. Requiere de un equipo interdisciplinario para su abordaje, lo cual ha demostrado que disminuye la morbimortalidad y aumenta la posibilidad de que los pacientes logren la autonomía intestinal. Existe una falta de evidencia científica en diferentes abordajes de la patología. Consideramos necesario el desarrollo de esta Guía para el Manejo Clínico construida sobre la base de la metodología Delphi modificada, en la Asociación Argentina de Nutrición Enteral y Parenteral, por 16 expertos que se reunieron para discutir y consensuar los principales aspectos de tratamiento clínico. Se analizaron 4 aspectos: definiciones y epidemiología; nutrición enteral, nutrición parenteral; tratamientos farmacológicos y quirúrgicos, y criterios de derivación a centros de alta complejidad. Sin duda este documento será de utilidad para los pacientes, los profesionales y las instituciones, así como para los diferentes financiadores del sistema de salud.

This practical guideline is based on the ESPEN Guidelines on Chronic Intestinal Failure in Adults.
ESPEN guidelines have been shortened and transformed into flow charts for easier use in clinical practice. The practical guideline is dedicated to all professionals including physicians, dieticians, nutritionists, and nurses working with patients with chronic intestinal failure.
This practical guideline consists of 112 recommendations with short commentaries for the management and treatment of benign chronic intestinal failure, including home parenteral nutrition and its complications, intestinal rehabilitation, and intestinal transplantation.
This practical guideline gives guidance to health care providers involved in the management of patients with chronic intestinal failure.

Nutritional management of patients with intestinal failure often includes the use of oligomeric formulas. Implementing the use of oligomeric formulas in surgical patients with maldigestion or malabsorption could be a nutritional strategy to be included in clinical protocols. We aim to generate knowledge from a survey focused on the effectiveness of nutritional therapy with oligomeric formulas with Delphi methodology. Each statement that reached an agreement consensus among participants was defined as a median consensus score ≥7 and as an interquartile range ≤3. The use of oligomeric formulas in surgical patients, starting enteral nutrition in the post-operative phase in short bowel syndrome and in nonspecific diarrhea after surgical procedures, could improve nutritional therapy implementation. Stakeholders agreed that early jejunal enteral nutrition with oligomeric formula is more effective compared to intravenous fluid therapy and it is useful in patients undergoing upper gastro-intestinal tract major surgery when malabsorption or maldigestion is suspected. Finally, oligomeric formulas may be useful when a feeding tube is placed distally to the duodenum. This study shows a practical approach to the use of oligomeric formulas in surgical patients with intestinal disorders and malabsorption, and it helps clinicians in the decision-making process.

We have recently established an intestinal rehabilitation unit (IRU) in Abu Ali Sina transplantation center affiliated to Shiraz University of Medical Sciences, Iran. Our intestinal failure rehabilitation and transplant program aims to provide state-of-the-art care for adult patients with different degrees of intestinal insufficiency and failure. In the IRU, we aimed to design an algorithmic approach to patients with small bowel ischemia and short bowel syndrome (SBS) based on our institutional experience in our country and based on other pioneering studies from other regions of the world.

To assess the effectiveness of postoperative feeding guidelines in reducing the incidence and severity of intestinal failure-associated liver disease (IFALD) among infants.
Two cohorts of infants <6 months old undergoing intestinal surgery were compared: preguideline (retrospective data from 2007 to 2013; n = 83) and postguideline (prospective data from 2013 to 2016; n = 81). The guidelines included greater initial enteral nutrition volumes of 20 mL/kg/d and daily feeding advancement if tolerated. The primary outcomes were incidence of IFALD (peak direct bilirubin [DB] >2 mg/dL) and severity (DB >5 mg/dL for moderate-severe). Multiple logistic regression was used to determine the odds of developing IFALD. Other outcomes were time to reach 50% and 100% goal calories from enteral nutrition and the incidence of necrotizing enterocolitis after feeding.
The incidence of IFALD decreased from 71% to 51% (P = .031), and median peak DB decreased from 5.7 to 2.4 mg/dL (P = .001). After adjusting for diagnosis and prematurity, the odds of developing IFALD of any severity were reduced by 60% (OR 0.40, 95% CI 0.20-0.85), and the odds of developing moderate-to-severe IFALD were reduced by 72% (OR 0.28, 95% CI 0.13-0.58) with guideline use. Time to reach 50% enteral nutrition decreased from a median of 10 to 6 days (P = .020) and time to reach 100% enteral nutrition decreased from 35 to 21 days (P = .035) with guideline use. The incidence of necrotizing enterocolitis after initiating enteral nutrition did not change (5% vs 9%, P = .346).
Implementation of feeding guidelines reduced time to reach feeding goals, significantly reducing IFALD incidence and severity.

Home parenteral nutrition (HPN) is a technique that has allowed the survival in the community of those patients with serious diseases resulting in an intestinal failure that made their nutrition impossible by other methods. It is indicated if there is a documented intestinal failure (understood by the reduction of the intestinal function to the minimum to the point that intravenous supplementation is required to maintain health and/or growth) with impossibility for oral or enteral exclusive nutrition, provided that there is the possibility of managing the patient at home and that there is no short-term survival expectancy. It requires taking into account the patient\'s quality of life, family environment and the capacity of the patient and/or their caregivers to be trained for HPN therapy. In low prevalence health topics, as intestinal failure, where the available scientific evidence is of poor quality, consensus documents add value in decision-making. Furthermore, HPN is a complex process and, although there is extensive experience in its application and even clinical practice guidelines, in daily practice there are uncertainties about its suitability, usefulness, rational use and associated costs. For this reason, this document of consensus has been carried out, using the GRADE method. With this document we intend to define our position with regard to the current use of HPN in our country and answer several controversial questions related to this treatment.
La nutrición parenteral en domicilio (NPD) es una técnica que ha permitido la supervivencia en la comunidad de aquellos pacientes con enfermedades graves resultantes en un fallo intestinal que hacía imposible su nutrición por otros métodos. Esta está indicada si existe un fallo intestinal documentado (entendido como la reducción de la función intestinal al mínimo hasta el punto de que se requiere suplementación intravenosa para mantener la salud y/o el crecimiento) con imposibilidad para la nutrición exclusiva por vía oral/enteral, posibilidad de manejo del enfermo en el domicilio y que no exista una expectativa corta de supervivencia, teniendo en cuenta la calidad de vida del paciente, el entorno familiar y la capacidad del paciente y/o de sus cuidadores de entrenamiento para la terapia de NPD.En los problemas de salud poco prevalentes, como es el fallo intestinal, en los cuales la evidencia científica disponible es de baja calidad, los documentos de consenso/expertos aportan valor en la toma de decisiones. Se suma a ello que la NPD es un proceso muy complejo y, a pesar de que existe una amplia experiencia en su aplicación e incluso guías de práctica clínica, en la práctica diaria se plantean incertidumbres acerca de su conveniencia, utilidad, uso racional y costes asociados. Por este motivo se ha realizado este documento de consenso, utilizando el método GRADE, con el que pretendemos definir nuestra posición con respecto al uso actual de la NPD en nuestro país y dar respuesta a una serie de preguntas que generan controversia en relación a este tratamiento.

OBJECTIVE: Teduglutide is an active, glucagon-like peptide (GLP)-2 analog with proven clinical efficacy regarding intestinal adaptation in patients with short bowel syndrome (SBS). There are two factors that preclude its reimbursement, and thereby, its availability: its cost (reaching ∼$300,000/y)-which significantly exceeds the cost of home parenteral nutrition (HPN) in most countries-and the lack of clear guidelines. The aim of this study was to create evidence-based working criteria for the use of teduglutide that could be used in clinical settings.
METHODS: Experts from the Polish Network of Intestinal Failure Centers analyzed available research and considered experience on the topic of HPN and intestinal failure to create guidelines.
RESULTS: Experts agreed that there are two groups of HPN patients who can benefit from therapy with a GLP-2 analog: those with a good prognosis (in whom complete weaning from HPN may be possible) and those with a poor prognosis (the therapy would be lifesaving). Patient criteria comprise the following: inclusion and exclusion criteria, parameters that can be used for monitoring, outcome measures, and the rationale for the termination of the treatment.
CONCLUSIONS: It was possible to describe inclusion criteria for both patient groups that justify the use of teduglutide from medical and economic perspectives.

OBJECTIVE: Chronic Intestinal Failure (CIF) is the long-lasting reduction of gut function, below the minimum necessary for the absorption of macronutrients and/or water and electrolytes, such that intravenous supplementation is required to maintain health and/or growth. CIF is the rarest organ failure. Home parenteral nutrition (HPN) is the primary treatment for CIF. No guidelines (GLs) have been developed that address the global management of CIF. These GLs have been devised to generate comprehensive recommendations for safe and effective management of adult patients with CIF.
METHODS: The GLs were developed by the Home Artificial Nutrition & Chronic Intestinal Failure Special Interest Group of ESPEN. The GRADE system was used for assigning strength of evidence. Recommendations were discussed, submitted to Delphi rounds, and accepted in an online survey of ESPEN members.
RESULTS: The following topics were addressed: management of HPN; parenteral nutrition formulation; intestinal rehabilitation, medical therapies, and non-transplant surgery, for short bowel syndrome, chronic intestinal pseudo-obstruction, and radiation enteritis; intestinal transplantation; prevention/treatment of CVC-related infection, CVC-related occlusion/thrombosis; intestinal failure-associated liver disease, gallbladder sludge and stones, renal failure and metabolic bone disease. Literature search provided 623 full papers. Only 12% were controlled studies or meta-analyses. A total of 112 recommendations are given: grade of evidence, very low for 51%, low for 39%, moderate for 8%, and high for 2%; strength of recommendation: strong for 63%, weak for 37%.
CONCLUSIONS: CIF management requires complex technologies, multidisciplinary and multiprofessional activity, and expertise to care for both the underlying gastrointestinal disease and to provide HPN support. The rarity of the condition impairs the development of RCTs. As a consequence, most of the recommendations have a low or very low grade of evidence. However, two-thirds of the recommendations are considered strong. Specialized management and organization underpin these recommendations.