{"orpha":294422,"name":"Chronic intestinal failure","type":"Clinical syndrome","group":"Disorder","status":"active","flags":[],"synonyms":["CIF"],"definition":"Chronic intestinal failure (CIF) is a chronic type of intestinal failure characterized by a nonfunctioning small bowel (that may be reversible or irreversible) where the body is unable to maintain energy and nutritional needs through absorption of food or nutrients via the intestinal tract (despite being metabolically stable) and which therefore necessitates long-term parenteral feeding. CIF may be the result of congenital digestive diseases (such as gastroschisis, atresia of small intestine), short bowel syndrome, intra-abdominal or pelvic cancer, or progressive and devastating gastrointestinal or systemic benign diseases (such as Crohn disease).","note":null,"url":"https://raremap.alftech.space/d/ORPHA-294422/","orphanet_url":"https://www.orpha.net/en/disease/detail/294422","names":{"fr":{"name":"Insuffisance intestinale chronique","synonyms":["CIF","IHC"],"definition":"L'insuffisance intestinale chronique est un type chronique d'insuffisance intestinale caractérisé par un intestin grêle non fonctionnel (de manière réversible ou irréversible). Le corps ne peut pas maintenir l'énergie et ses besoins nutritionnels par l'absorption des aliments ou des nutriments via le tractus intestinal (même s'il est stable métaboliquement), ce qui rend nécessaire une alimentation parentérale sur le long terme. L'insuffisance intestinale chronique peut être la conséquence de maladies digestives congénitales (telles que la gastroschisie, l'atrésie de l'intestin grêle), du syndrome du grêle court, d'un cancer intra-abdominal ou pelvien, ou de maladies bénignes systémiques (telles que la maladie de Crohn) ou de maladies gastro-intestinales progressives et dévastatrices."},"de":{"name":"Darmversagen, chronisches","synonyms":["CIF","Chronische intestinale Insuffizienz"],"definition":"Die chronische intestinale Insuffizienz (CIF) ist eine chronische Form von Darmversagen, die durch eine (reversible oder irreversible) Dünndarminsuffizienz gekennzeichnet ist, bei der der Körper nicht in der Lage ist, seinen Energie- und Nährstoffbedarf durch die Aufnahme von Nahrung oder Nährstoffen über den Darmtrakt zu decken (obwohl der Stoffwechsel stabil ist) und daher eine langfristige parenterale Ernährung erfordert. CIF kann die Folge von angeborenen Verdauungsstörungen (z. B. Gastroschisis, Dünndarmatresie), Kurzdarmsyndrom, intraabdominalem oder pelvinem Krebs oder von fortschreitenden und zerstörerischen gastrointestinalen oder systemischen gutartigen Erkrankungen (z. B. Morbus Crohn) sein."},"es":{"name":"Insuficiencia intestinal crónica","synonyms":["CIF"],"definition":"Es un tipo crónico de insuficiencia intestinal caracterizada por un intestino delgado no funcionante (que puede ser reversible o irreversible) en la que el organismo es incapaz de cubrir sus necesidades energéticas y nutricionales mediante la absorción de alimentos o nutrientes en el tracto intestinal (a pesar de ser metabólicamente estable) y que, por lo tanto, requiere alimentación parenteral a largo plazo. La insuficiencia intestinal puede ser el resultado de enfermedades digestivas congénitas (tales como gastrosquisis, atresia del intestino delgado), síndrome del intestino corto, cáncer intraabdominal o pélvico, o enfermedades gastrointestinales progresivas y devastadoras o benignas sistémicas (como la enfermedad de Crohn)."},"it":{"name":"Insufficienza intestinale cronica","synonyms":["CIF"],"definition":"L'insufficienza intestinale cronica (IIC) è una forma cronica di insufficienza intestinale caratterizzata dalla disfunzione dell'intestino tenue, che può essere reversibile o irreversibile, con conseguente incapacità dell'organismo a trattenere l'energia e a soddisfare le esigenze nutrizionali attraverso l'assorbimento del cibo e dei nutrienti con l'intestino (anche se esso rimane stabile dal punto di vista metabolico), necessitando perciò della nutrizione parenterale a lungo termine. L'insufficienza intestinale cronica può essere causata da malattie congenite del tubo digerente (gastroschisi, atresia dell'intestino tenue), dalla sindrome dell'intestino corto, dai tumori intra-addominali o pelvici, oppure dalle malattie progressive e devastanti del tratto gastrointestinale o dalle malattie sistemiche benigne (ad esempio il morbo di Crohn)."},"nl":{"name":"Chronisch darmfalen","synonyms":["CIF"],"definition":"Chronisch darmfalen (CIF) is een chronisch type van darmfalen dat gekarakteriseerd wordt door een niet functionerende dunne darm (omkeerbaar of onomkeerbaar), waardoor het lichaam niet in staat is om de nodige energie en voedingsstoffen te voorzien door absorptie van voedsel of nutriënten via het darmstelsel (ondanks metabole stabiliteit), en dus parenterale voeding op lange termijn vereist is. CIF kan het resultaat zijn van congenitale digestieve ziekten (zoals gastroschisis, atresie van de dunne darm), korte-darmsyndroom, intra-abdominale kanker of kanker in het bekken, of progressieve en destructieve gastro-intestinale of systemische goedaardige ziekten (zoals ziekte van Crohn)."},"pt":{"name":"Insuficiência intestinal crónica","synonyms":["CIF"]},"pl":{"name":"Przewlekła niewydolność jelit","synonyms":["CIF"]},"cs":{"name":"Chronické selhání střev","synonyms":["CIF"]},"uk":{"name":"Хронічна кишкова недостатність","synonyms":["CIF"],"definition":"Chronic intestinal failure (CIF) is a chronic type of intestinal failure characterized by a nonfunctioning small bowel (that may be reversible or irreversal) where the body is unable to maintain energy and nutritional needs through absorption of food or nutrients via the intestinal tract (despite being metabolically stable) and which therefore necessitates long-term parenteral feeding. CIF may be the result of congenital digestive diseases (such as gastroschisis, atresia of small intestine), short bowel syndrome, intra-abdominal or pelvic cancer, or progressive and devastating gastrointestinal or systemic benign diseases (such as Crohn disease)."},"zh":{"name":"慢性肠衰竭","synonyms":["慢性肠衰竭"],"definition":"慢性肠功能不全（CIF）是一种慢性肠功能衰竭，其特征是小肠功能不全（可为可逆或不可逆），机体无法通过肠道吸收食物或营养物质来维持能量和营养需求（尽管代谢稳定），因此需要长期肠外喂养。先天性消化系统疾病（如腹裂、小肠闭锁）、短肠综合征、腹腔内或盆腔癌，或进行性和破坏性胃肠道或系统性良性疾病（如克罗恩病）可导致CIF。"},"tr":{"name":"Kronik bağırsak yetmezliği","synonyms":["CIF"]}},"xrefs":[{"source":"ICD-10","code":"K63.8","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"ICD-11","code":"DA96.05","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"MONDO","code":"0017418","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C4274352","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":97935,"name":"Rare gastroenterologic disease"},"parents":[{"orpha":506216,"name":"Rare disorder potentially indicated for bowel transplant","classifications":[233]},{"orpha":117569,"name":"Rare intestinal disease","classifications":[152]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":["Not applicable"],"onset":["All ages"],"prevalence":[{"type":"Prevalence at birth","qualification":"Value and class","class":"6-9 / 10 000","value":86.0,"area":"Italy","status":"Validated","source":"18589446[PMID]"},{"type":"Point prevalence","qualification":"Value and class","class":"1-5 / 10 000","value":42.4691,"area":"Italy","status":"Validated","source":"36215864[PMID]_ORPHANET"}],"phenotypes":null}