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Chronic intestinal failure

MedGen UID:
909276
•Concept ID:
C4274352
•
Disease or Syndrome
Synonyms: Chronic failure of small intestine; chronic intestinal failure; CIF; Type III chronic intestinal failure
SNOMED CT: Chronic intestinal failure (716665002); Chronic failure of small intestine (716665002); Type III chronic intestinal failure (716665002)
Modes of inheritance:
Not genetically inherited
MedGen UID:
988794
•Concept ID:
CN307044
•
Finding
Source: Orphanet
clinical entity without genetic inheritance.
See: This record
Not genetically inherited (Orphanet)
 
Monarch Initiative: MONDO:0017418
Orphanet: ORPHA294422

Definition

A chronic type of intestinal failure with characteristics of a nonfunctioning small bowel that may be reversible or irreversible. The body is unable to maintain energy and nutritional needs through absorption of food or nutrients via the intestinal tract despite being metabolically stable. This necessitates long-term parenteral feeding. [from SNOMEDCT_US]

Professional guidelines

PubMed

AGA Clinical Practice Update on Diet and Nutritional Therapies in Patients With Inflammatory Bowel Disease: Expert Review.
Hashash JG, Elkins J, Lewis JD, Binion DG
Gastroenterology 2024 Mar;166(3):521-532. Epub 2024 Jan 23 doi: 10.1053/j.gastro.2023.11.303. PMID: 38276922
ESPEN practical guideline: Clinical nutrition in chronic intestinal failure.
Cuerda C, Pironi L, Arends J, Bozzetti F, Gillanders L, Jeppesen PB, Joly F, Kelly D, Lal S, Staun M, Szczepanek K, Van Gossum A, Wanten G, Schneider SM, Bischoff SC; Home Artificial Nutrition & Chronic Intestinal Failure Special Interest Group of ESPEN
Clin Nutr 2021 Sep;40(9):5196-5220. Epub 2021 Aug 2 doi: 10.1016/j.clnu.2021.07.002. PMID: 34479179
Advances in chronic intestinal failure management and therapies.
Deutsch L, Cloutier A, Lal S
Curr Opin Gastroenterol 2020 May;36(3):223-229. doi: 10.1097/MOG.0000000000000631. PMID: 32073503

Recent clinical studies

Etiology

ESPEN micronutrient guideline.
Berger MM, Shenkin A, Schweinlin A, Amrein K, Augsburger M, Biesalski HK, Bischoff SC, Casaer MP, Gundogan K, Lepp HL, de Man AME, Muscogiuri G, Pietka M, Pironi L, Rezzi S, Cuerda C
Clin Nutr 2022 Jun;41(6):1357-1424. Epub 2022 Feb 26 doi: 10.1016/j.clnu.2022.02.015. PMID: 35365361
ESPEN practical guideline: Home enteral nutrition.
Bischoff SC, Austin P, Boeykens K, Chourdakis M, Cuerda C, Jonkers-Schuitema C, Lichota M, Nyulasi I, Schneider SM, Stanga Z, Pironi L
Clin Nutr 2022 Feb;41(2):468-488. Epub 2021 Nov 24 doi: 10.1016/j.clnu.2021.10.018. PMID: 35007816
ESPEN guideline on home parenteral nutrition.
Pironi L, Boeykens K, Bozzetti F, Joly F, Klek S, Lal S, Lichota M, Mühlebach S, Van Gossum A, Wanten G, Wheatley C, Bischoff SC
Clin Nutr 2020 Jun;39(6):1645-1666. Epub 2020 Apr 18 doi: 10.1016/j.clnu.2020.03.005. PMID: 32359933
ESPEN guidelines on chronic intestinal failure in adults.
Pironi L, Arends J, Bozzetti F, Cuerda C, Gillanders L, Jeppesen PB, Joly F, Kelly D, Lal S, Staun M, Szczepanek K, Van Gossum A, Wanten G, Schneider SM; Home Artificial Nutrition & Chronic Intestinal Failure Special Interest Group of ESPEN
Clin Nutr 2016 Apr;35(2):247-307. Epub 2016 Feb 8 doi: 10.1016/j.clnu.2016.01.020. PMID: 26944585
Cholestasis induced by total parenteral nutrition.
Guglielmi FW, Regano N, Mazzuoli S, Fregnan S, Leogrande G, Guglielmi A, Merli M, Pironi L, Penco JM, Francavilla A
Clin Liver Dis 2008 Feb;12(1):97-110, viii. doi: 10.1016/j.cld.2007.11.004. PMID: 18242499

Diagnosis

AGA Clinical Practice Update on Diet and Nutritional Therapies in Patients With Inflammatory Bowel Disease: Expert Review.
Hashash JG, Elkins J, Lewis JD, Binion DG
Gastroenterology 2024 Mar;166(3):521-532. Epub 2024 Jan 23 doi: 10.1053/j.gastro.2023.11.303. PMID: 38276922
ESPEN micronutrient guideline.
Berger MM, Shenkin A, Schweinlin A, Amrein K, Augsburger M, Biesalski HK, Bischoff SC, Casaer MP, Gundogan K, Lepp HL, de Man AME, Muscogiuri G, Pietka M, Pironi L, Rezzi S, Cuerda C
Clin Nutr 2022 Jun;41(6):1357-1424. Epub 2022 Feb 26 doi: 10.1016/j.clnu.2022.02.015. PMID: 35365361
Chronic intestinal failure and short bowel syndrome in Crohn's disease.
Aksan A, Farrag K, Blumenstein I, Schröder O, Dignass AU, Stein J
World J Gastroenterol 2021 Jun 28;27(24):3440-3465. doi: 10.3748/wjg.v27.i24.3440. PMID: 34239262Free PMC Article
Mitochondrial neurogastrointestinal encephalomyopathy (MNGIE): Position paper on diagnosis, prognosis, and treatment by the MNGIE International Network.
Hirano M, Carelli V, De Giorgio R, Pironi L, Accarino A, Cenacchi G, D'Alessandro R, Filosto M, Martí R, Nonino F, Pinna AD, Baldin E, Bax BE, Bolletta A, Bolletta R, Boschetti E, Cescon M, D'Angelo R, Dotti MT, Giordano C, Gramegna LL, Levene M, Lodi R, Mandel H, Morelli MC, Musumeci O, Pugliese A, Scarpelli M, Siniscalchi A, Spinazzola A, Tal G, Torres-Torronteras J, Vignatelli L, Zaidman I, Zoller H, Rinaldi R, Zeviani M
J Inherit Metab Dis 2021 Mar;44(2):376-387. Epub 2020 Sep 8 doi: 10.1002/jimd.12300. PMID: 32898308Free PMC Article
ESPEN endorsed recommendations. Definition and classification of intestinal failure in adults.
Pironi L, Arends J, Baxter J, Bozzetti F, Peláez RB, Cuerda C, Forbes A, Gabe S, Gillanders L, Holst M, Jeppesen PB, Joly F, Kelly D, Klek S, Irtun Ø, Olde Damink SW, Panisic M, Rasmussen HH, Staun M, Szczepanek K, Van Gossum A, Wanten G, Schneider SM, Shaffer J; Home Artificial Nutrition & Chronic Intestinal Failure; Acute Intestinal Failure Special Interest Groups of ESPEN
Clin Nutr 2015 Apr;34(2):171-80. Epub 2014 Sep 21 doi: 10.1016/j.clnu.2014.08.017. PMID: 25311444

Therapy

ESPEN micronutrient guideline.
Berger MM, Shenkin A, Schweinlin A, Amrein K, Augsburger M, Biesalski HK, Bischoff SC, Casaer MP, Gundogan K, Lepp HL, de Man AME, Muscogiuri G, Pietka M, Pironi L, Rezzi S, Cuerda C
Clin Nutr 2022 Jun;41(6):1357-1424. Epub 2022 Feb 26 doi: 10.1016/j.clnu.2022.02.015. PMID: 35365361
Chronic intestinal failure and short bowel syndrome in Crohn's disease.
Aksan A, Farrag K, Blumenstein I, Schröder O, Dignass AU, Stein J
World J Gastroenterol 2021 Jun 28;27(24):3440-3465. doi: 10.3748/wjg.v27.i24.3440. PMID: 34239262Free PMC Article
ESPEN guideline on home parenteral nutrition.
Pironi L, Boeykens K, Bozzetti F, Joly F, Klek S, Lal S, Lichota M, Mühlebach S, Van Gossum A, Wanten G, Wheatley C, Bischoff SC
Clin Nutr 2020 Jun;39(6):1645-1666. Epub 2020 Apr 18 doi: 10.1016/j.clnu.2020.03.005. PMID: 32359933
ESPEN guidelines on chronic intestinal failure in adults.
Pironi L, Arends J, Bozzetti F, Cuerda C, Gillanders L, Jeppesen PB, Joly F, Kelly D, Lal S, Staun M, Szczepanek K, Van Gossum A, Wanten G, Schneider SM; Home Artificial Nutrition & Chronic Intestinal Failure Special Interest Group of ESPEN
Clin Nutr 2016 Apr;35(2):247-307. Epub 2016 Feb 8 doi: 10.1016/j.clnu.2016.01.020. PMID: 26944585
Cholestasis induced by total parenteral nutrition.
Guglielmi FW, Regano N, Mazzuoli S, Fregnan S, Leogrande G, Guglielmi A, Merli M, Pironi L, Penco JM, Francavilla A
Clin Liver Dis 2008 Feb;12(1):97-110, viii. doi: 10.1016/j.cld.2007.11.004. PMID: 18242499

Prognosis

Efficacy of Teduglutide for Parenteral Support Reduction in Patients with Short Bowel Syndrome: A Systematic Review and Meta-Analysis.
Bioletto F, D'Eusebio C, Merlo FD, Aimasso U, Ossola M, Pellegrini M, Ponzo V, Chiarotto A, De Francesco A, Ghigo E, Bo S
Nutrients 2022 Feb 14;14(4) doi: 10.3390/nu14040796. PMID: 35215445Free PMC Article
Mitochondrial neurogastrointestinal encephalomyopathy (MNGIE): Position paper on diagnosis, prognosis, and treatment by the MNGIE International Network.
Hirano M, Carelli V, De Giorgio R, Pironi L, Accarino A, Cenacchi G, D'Alessandro R, Filosto M, Martí R, Nonino F, Pinna AD, Baldin E, Bax BE, Bolletta A, Bolletta R, Boschetti E, Cescon M, D'Angelo R, Dotti MT, Giordano C, Gramegna LL, Levene M, Lodi R, Mandel H, Morelli MC, Musumeci O, Pugliese A, Scarpelli M, Siniscalchi A, Spinazzola A, Tal G, Torres-Torronteras J, Vignatelli L, Zaidman I, Zoller H, Rinaldi R, Zeviani M
J Inherit Metab Dis 2021 Mar;44(2):376-387. Epub 2020 Sep 8 doi: 10.1002/jimd.12300. PMID: 32898308Free PMC Article
Review article: diagnosis and management of intestinal failure-associated liver disease in adults.
Bond A, Huijbers A, Pironi L, Schneider SM, Wanten G, Lal S
Aliment Pharmacol Ther 2019 Sep;50(6):640-653. Epub 2019 Jul 25 doi: 10.1111/apt.15432. PMID: 31342540
Dysmotility disorders: a nutritional approach.
Billiauws L, Corcos O, Joly F
Curr Opin Clin Nutr Metab Care 2014 Sep;17(5):483-8. doi: 10.1097/MCO.0000000000000095. PMID: 25023191
Cholestasis induced by total parenteral nutrition.
Guglielmi FW, Regano N, Mazzuoli S, Fregnan S, Leogrande G, Guglielmi A, Merli M, Pironi L, Penco JM, Francavilla A
Clin Liver Dis 2008 Feb;12(1):97-110, viii. doi: 10.1016/j.cld.2007.11.004. PMID: 18242499

Clinical prediction guides

ESPEN practical short micronutrient guideline.
Berger MM, Shenkin A, Dizdar OS, Amrein K, Augsburger M, Biesalski HK, Bischoff SC, Casaer MP, Gundogan K, Lepp HL, de Man AME, Muscogiuri G, Pietka M, Pironi L, Rezzi S, Schweinlin A, Cuerda C
Clin Nutr 2024 Mar;43(3):825-857. Epub 2024 Jan 30 doi: 10.1016/j.clnu.2024.01.030. PMID: 38350290
AGA Clinical Practice Update on Diet and Nutritional Therapies in Patients With Inflammatory Bowel Disease: Expert Review.
Hashash JG, Elkins J, Lewis JD, Binion DG
Gastroenterology 2024 Mar;166(3):521-532. Epub 2024 Jan 23 doi: 10.1053/j.gastro.2023.11.303. PMID: 38276922
ESPEN micronutrient guideline.
Berger MM, Shenkin A, Schweinlin A, Amrein K, Augsburger M, Biesalski HK, Bischoff SC, Casaer MP, Gundogan K, Lepp HL, de Man AME, Muscogiuri G, Pietka M, Pironi L, Rezzi S, Cuerda C
Clin Nutr 2022 Jun;41(6):1357-1424. Epub 2022 Feb 26 doi: 10.1016/j.clnu.2022.02.015. PMID: 35365361
Efficacy of Teduglutide for Parenteral Support Reduction in Patients with Short Bowel Syndrome: A Systematic Review and Meta-Analysis.
Bioletto F, D'Eusebio C, Merlo FD, Aimasso U, Ossola M, Pellegrini M, Ponzo V, Chiarotto A, De Francesco A, Ghigo E, Bo S
Nutrients 2022 Feb 14;14(4) doi: 10.3390/nu14040796. PMID: 35215445Free PMC Article
Mitochondrial neurogastrointestinal encephalomyopathy (MNGIE): Position paper on diagnosis, prognosis, and treatment by the MNGIE International Network.
Hirano M, Carelli V, De Giorgio R, Pironi L, Accarino A, Cenacchi G, D'Alessandro R, Filosto M, Martí R, Nonino F, Pinna AD, Baldin E, Bax BE, Bolletta A, Bolletta R, Boschetti E, Cescon M, D'Angelo R, Dotti MT, Giordano C, Gramegna LL, Levene M, Lodi R, Mandel H, Morelli MC, Musumeci O, Pugliese A, Scarpelli M, Siniscalchi A, Spinazzola A, Tal G, Torres-Torronteras J, Vignatelli L, Zaidman I, Zoller H, Rinaldi R, Zeviani M
J Inherit Metab Dis 2021 Mar;44(2):376-387. Epub 2020 Sep 8 doi: 10.1002/jimd.12300. PMID: 32898308Free PMC Article

Recent systematic reviews

Immunonutrition in patients who underwent major abdominal surgery: A comprehensive systematic review and component network metanalysis using GRADE and CINeMA approaches.
Ricci C, Serbassi F, Alberici L, Ingaldi C, Gaetani L, De Raffele E, Pironi L, Sasdelli AS, Mosconi C, Di Marco MC, Casadei R
Surgery 2023 Dec;174(6):1401-1409. Epub 2023 Sep 27 doi: 10.1016/j.surg.2023.08.005. PMID: 37775395
Efficacy of Teduglutide for Parenteral Support Reduction in Patients with Short Bowel Syndrome: A Systematic Review and Meta-Analysis.
Bioletto F, D'Eusebio C, Merlo FD, Aimasso U, Ossola M, Pellegrini M, Ponzo V, Chiarotto A, De Francesco A, Ghigo E, Bo S
Nutrients 2022 Feb 14;14(4) doi: 10.3390/nu14040796. PMID: 35215445Free PMC Article
Reporting on outcome measures in pediatric chronic intestinal failure: A systematic review.
Nagelkerke SCJ, Mager DJ, Benninga MA, Tabbers MM
Clin Nutr 2020 Jul;39(7):1992-2000. Epub 2019 Sep 10 doi: 10.1016/j.clnu.2019.08.027. PMID: 31551169
Systematic Review: Noninvasive Assessments of Intestinal Failure-Associated Liver Disease in the Adult Population.
Huijbers A, Koggel LM, Bronkhorst C, Verheij J, Wanten GJA
JPEN J Parenter Enteral Nutr 2019 Jul;43(5):615-626. Epub 2019 Apr 1 doi: 10.1002/jpen.1524. PMID: 30937931
Refeeding syndrome in the gastroenterology practice: how concerned should we be?
Nunes G, Brito M, Santos CA, Fonseca J
Eur J Gastroenterol Hepatol 2018 Nov;30(11):1270-1276. doi: 10.1097/MEG.0000000000001202. PMID: 29994872

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