title: ESPEN guidelines on chronic intestinal failure in adults.
abstract: 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.
authors.affiliation: Center for Chronic Intestinal Failure, Department of Digestive System, St. Orsola-Malpighi University Hospital, Bologna, Italy. Electronic address: loris.pironi@unibo.it.
authors.forename: Loris
authors.initials: L
authors.lastname: Pironi
authors.affiliation: Department of Medicine, Oncology and Hematology, University of Freiburg, Germany.
authors.forename: Jann
authors.initials: J
authors.lastname: Arends
authors.affiliation: Faculty of Medicine, University of Milan, Milan, Italy.
authors.forename: Federico
authors.initials: F
authors.lastname: Bozzetti
authors.affiliation: Nutrition Unit, Hospital General Universitario Gregorio Marañón, Madrid, Spain.
authors.forename: Cristina
authors.initials: C
authors.lastname: Cuerda
authors.affiliation: Nutrition Support Team, Auckland City Hospital, (AuSPEN) Auckland, New Zealand.
authors.forename: Lyn
authors.initials: L
authors.lastname: Gillanders
authors.affiliation: Rigshospitalet, Department of Gastroenterology, Copenhagen, Denmark.
authors.forename: Palle Bekker
authors.initials: PB
authors.lastname: Jeppesen
authors.affiliation: Centre for Intestinal Failure, Department of Gastroenterology and Nutritional Support, Hôpital Beaujon, Clichy, France.
authors.forename: Francisca
authors.initials: F
authors.lastname: Joly
authors.affiliation: Division of Gastroenterology and Hepatology, Mayo Clinic College of Medicine, Rochester, MN, USA; Oley Foundation for Home Parenteral and Enteral Nutrition, Albany, NY, USA.
authors.forename: Darlene
authors.initials: D
authors.lastname: Kelly
authors.affiliation: Intestinal Failure Unit, Salford Royal Foundation Trust, Salford, UK.
authors.forename: Simon
authors.initials: S
authors.lastname: Lal
authors.affiliation: Rigshospitalet, Department of Gastroenterology, Copenhagen, Denmark.
authors.forename: Michael
authors.initials: M
authors.lastname: Staun
authors.affiliation: General and Oncology Surgery Unit, Stanley Dudrick's Memorial Hospital, Skawina, Poland.
authors.forename: Kinga
authors.initials: K
authors.lastname: Szczepanek
authors.affiliation: Medico-Surgical Department of Gastroenterology, Hôpital Erasme, Free University of Brussels, Belgium.
authors.forename: André
authors.initials: A
authors.lastname: Van Gossum
authors.affiliation: Intestinal Failure Unit, Department of Gastroenterology and Hepatology, Radboud University Nijmegen Medical Centre, Nijmegen, The Netherlands.
authors.forename: Geert
authors.initials: G
authors.lastname: Wanten
authors.affiliation: Gastroenterology and Clinical Nutrition, CHU of Nice, University of Nice Sophia Antipolis, Nice, France.
authors.forename: Stéphane Michel
authors.initials: SM
authors.lastname: Schneider
mesh_terms: D000818:Animals; D002908:Chronic Disease; D019468:Disease Management; D004195:Disease Models, Animal; D004751:Enteritis / Q000150:complications / Q000628:therapy*; D006801:Humans; D007418:Intestinal Pseudo-Obstruction / Q000150:complications / Q000628:therapy*; D008107:Liver Diseases / Q000150:complications / Q000628:therapy; D016331:Parenteral Nutrition, Home / Q000592:standards*; D017410:Practice Guidelines as Topic; D016032:Randomized Controlled Trials as Topic; D012778:Short Bowel Syndrome / Q000150:complications / Q000628:therapy*
keywords: Guideline; Home parenteral nutrition; Intestinal failure; Intestinal pseudo-obstruction; Intestinal transplantation; Short bowel syndrome