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.

BACKGROUND: Despite the presence of ganglion cells in the rectum, some patients have symptoms similar to those of Hirschsprung\'s disease. A consensus has yet to be established regarding the terminology for these diseases. We defined this group of diseases as \"allied disorders of Hirschsprung\'s disease\" and compiled these guidelines to facilitate accurate clinician diagnosis and provide appropriate treatment strategies for each disease.
METHODS: These guidelines were developed using the methodologies in the Medical Information Network Distribution System (MINDS). Of seven allied disorders, isolated hypoganglionosis; megacystis-microcolon-intestinal hypoperistalsis syndrome; and chronic idiopathic intestinal pseudo-obstruction were selected as targets of clinical questions (CQ). In a comprehensive search of the Japanese- and English-language articles in PubMed and Ichu-Shi Web, 836 pieces of evidence related to the CQ were extracted from 288 articles; these pieces of evidence were summarized in an evidence table.
RESULTS: We herein outline the newly established Japanese clinical practice guidelines for allied disorders of Hirschsprung\'s disease. Given that the target diseases are rare and intractable, most evidence was drawn from case reports and case series. In the CQ, the diagnosis, medication, nutritional support, surgical therapy, and prognosis for each disease are given. We emphasize the importance of full-thickness intestinal biopsy specimens for the histopathological evaluation of enteric ganglia. Considering the practicality of the guidelines, the recommendations for each CQ were created with protracted discussions among specialists.
CONCLUSIONS: Clinical practice recommendations for allied disorders of Hirschprung\'s disease are given for each CQ, along with an assessment of the current evidence. We hope that the information will be helpful in daily practice and future studies.

OBJECTIVE: Chronic intestinal pseudo-obstructive (CIPO) conditions are considered the most severe disorders of gut motility. They continue to present significant challenges in clinical care despite considerable recent progress in our understanding of pathophysiology, resulting in unacceptable levels of morbidity and mortality. Major contributors to the disappointing lack of progress in paediatric CIPO include a dearth of clarity and uniformity across all aspects of clinical care from definition and diagnosis to management. In order to assist medical care providers in identifying, evaluating, and managing children with CIPO, experts in this condition within the European Society for Pediatric Gastroenterology, Hepatology, and Nutrition as well as selected external experts, were charged with the task of developing a uniform document of evidence- and consensus-based recommendations.
METHODS: Ten clinically relevant questions addressing terminology, diagnostic, therapeutic, and prognostic topics were formulated. A systematic literature search was performed from inception to June 2017 using a number of established electronic databases as well as repositories. The approach of the Grading of Recommendations Assessment, Development and Evaluation (GRADE) was applied to evaluate outcome measures for the research questions. Levels of evidence and quality of evidence were assessed using the classification system of the Oxford Centre for Evidence-Based Medicine (diagnosis) and the GRADE system (treatment). Each of the recommendations were discussed, finalized, and voted upon using the nominal voting technique to obtain consensus.
RESULTS: This evidence- and consensus-based position paper provides recommendations specifically for chronic intestinal pseudo-obstruction in infants and children. It proposes these be termed paediatric intestinal pseudo-obstructive (PIPO) disorders to distinguish them from adult onset CIPO. The manuscript provides guidance on the diagnosis, evaluation, and treatment of children with PIPO in an effort to standardise the quality of clinical care and improve short- and long-term outcomes. Key recommendations include the development of specific diagnostic criteria for PIPO, red flags to alert clinicians to the diagnosis and guidance on the use of available investigative modalities. The group advocates early collaboration with expert centres where structured diagnosis and management is guided by a multi-disciplinary team, and include targeted nutritional, medical, and surgical interventions as well as transition to adult services.
CONCLUSIONS: This document is intended to be used in daily practice from the time of first presentation and definitive diagnosis PIPO through to the complex management and treatment interventions such as intestinal transplantation. Significant challenges remain to be addressed through collaborative clinical and research interactions.

BACKGROUND: Intestinal obstructions/pseudo-obstruction of the small/large bowel are frequent conditions but their management could be challenging. Moreover, a general agreement in this field is currently lacking, thus SICUT Society designed a consensus study aimed to define their optimal workout.
METHODS: The Delphi methodology was used to reach consensus among 47 Italian surgical experts in two study rounds. Consensus was defined as an agreement of 75.0% or greater. Four main topic areas included nosology, diagnosis, management and treatment.
RESULTS: A bowel obstruction was defined as an obstacle to the progression of intestinal contents and fluids generally beginning with a sudden onset. The panel identified four major criteria of diagnosis including absence of flatus, presence of >3.5 cm ileal levels or >6 cm colon dilatation and abdominal distension. Panel also recommended a surgical admission, a multidisciplinary approach, and a gastrografin swallow for patients presenting occlusions. Criteria for immediate surgery included: presence of strangulated hernia, a >10 cm cecal dilatation, signs of vascular pedicles obstructions and persistence of metabolic acidosis. Moreover, rules for non-operative management (to be conducted for maximum 72 hours) included a naso-gastric drainage placement and clinical and laboratory controls each 12 hours. Non-operative treatment should be suspended if any suspects of intra-abdominal complications, high level of lactates, leukocytosis (>18.000/mm3 or Neutrophils >85%) or a doubling of creatinine level comparing admission. Conversely, consensus was not reached regarding the exact timing of CT scan and the appropriateness of colonic stenting.
CONCLUSIONS: This consensus is in line with current international strategies and guidelines, and it could be a useful tool in the safe basic daily management of these common and peculiar diseases.
BACKGROUND: Delphi study, Intestinal obstruction, Large bowel obstruction, Pseudo-obstruction, Small bowel.
Le ostruzioni e pseudo-ostruzioni del piccolo/ grande intestino sono condizioni frequenti, ma la loro gestione risulta spesso difficile per cui è necessario avere un adeguato e condiviso percorso gestionale. A tal proposito la Società Italiana di Chirurgia d’Urgenza e del Trauma (SICUT) ha disegnato uno studio volto a definire il management ottimale dei pazienti con occlusione intestinale.
Lo studio è stato condotto utilizzando la metodologia Delphi coinvolgendo un panel di 47 chirurghi italiani con maturata esperienza clinica. Il consenso è stato definito come un accordo uguale o superiore al 75.0%. Lo studio si è focalizzato su quattro aree tematiche comprendenti la nosologia, la diagnosi, la gestione e il trattamento delle ostruzioni e delle pseudo-ostruzioni intestinali. Un occlusione intestinale è stata definita come un ostacolo alla progressione del contenuto intestinale. RISULTATI: in genere ad esordio improvviso. Il panel ha identificato quattro principali criteri di diagnosi, tra i quali l’assenza di canalizzazione ai gas, la presenza di livelli ileali > 3.5 cm o di una dilatazione del colon> 6 cm nonché di una distensione addominale. Viene inoltre consigliato il ricovero in chirurgia, un approccio multidisciplinare e un transito con Gastrografin per tutti i pazienti che presentano un’occlusione. Tra i criteri per un intervento chirurgico immediato sono inclusi: la presenza di un’ernia strozzata, una dilatazione del cieco> 10 cm, segni di ostruzione del peduncolo vascolare e la persistenza di acidosi metabolica. La gestione non operatoria dei pazienti (la cui durata massima dovrebbe essere di 72 ore) include il posizionamento di un sondino nasogastrico e la necessità di effettuare controlli clinici e laboratoristici ogni 12 ore. Il trattamento non operatorio deve essere sospeso in caso insorga il sospetto di complicanze intra-addominali, compaia un alto livello di lattati, una leucocitosi (> 18.000 / mm3 o neutrofili> 85%) o un raddoppiamento del livello di creatinina rispetto ai valori basali al ricovero. Al contrario il panel non ha raggiunto alcun consenso riguardo l’esatta tempistica della TAC e l’opportunità di stenting del colon come intervento palliativo in attesa di chirurgia definitiva.
Le raccomandazioni stabilite sono in linea con le strategie e le linee guida internazionali, e potrebbero essere uno strumento utile per la gestione del paziente affetto da occlusione intestinale.

Over the last 20 years, there has been increasing focus on the development of novel stem cell based therapies for the treatment of disorders and diseases affecting the enteric nervous system (ENS) of the gastrointestinal tract (so-called enteric neuropathies). Here, the idea is that ENS progenitor/stem cells could be transplanted into the gut wall to replace the damaged or absent neurons and glia of the ENS. This White Paper sets out experts\' views on the commonly used methods and approaches to identify, isolate, purify, expand and optimize ENS stem cells, transplant them into the bowel, and assess transplant success, including restoration of gut function. We also highlight obstacles that must be overcome in order to progress from successful preclinical studies in animal models to ENS stem cell therapies in the clinic.

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.

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Allogeneic hematopoietic SCT (HSCT) has been proposed as a treatment for patients with mitochondrial neurogastrointestinal encephalomyopathy (MNGIE). HSCT has been performed in nine patients using different protocols with varying success. Based on this preliminary experience, participants of the first consensus conference propose a common approach to allogeneic HSCT in MNGIE. Standardization of the transplant protocol and the clinical and biochemical assessments will allow evaluation of the safety and efficacy of HSCT as well as optimization of therapy for patients with MNGIE.

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