{"orpha":104008,"name":"Short bowel syndrome","type":"Clinical group","group":"Group of disorders","status":"active","flags":[],"synonyms":[],"definition":"Short bowel syndrome is an intestinal failure due to either a congenital defect, intestinal infarction or extensive surgical resection of the intestinal tract that results in a functional small intestine of less than 200cm in length and is characterized by diarrhea, nutrient malabsoption, bowel dilation and dysmobility.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-104008/","orphanet_url":"https://www.orpha.net/en/disease/detail/104008","names":{"fr":{"name":"Syndrome du grêle court","synonyms":["Syndrome de l'intestin court"],"definition":"Le syndrome de l'intestin court est une défaillance intestinale causée soit par une anomalie congénitale, un infarctus intestinal ou une résection chirurgicale étendue du tractus intestinal qui engendre un intestin court fonctionnel de moins de 200 cm de long. Ill est caractérisé par une diarrhée, une malabsorption des nutriments, une dilatation des intestins et une dysmotilité."},"de":{"name":"Kurzdarm-Syndrom","definition":"Das Kurzdarmsyndrom ist eine intestinale Funktionsstörung und tritt als angeborener Defekt, nach Darminfarkt oder nach ausgiebiger chirurgischer Resektion des Darmtraktes auf, wenn die Restlänge funtionellen Dünndarms weniger als 200 cm beträgt. Symptome sind Diarrhoe, Malabsorption der Nährstoffe, Ausweitung des Darms und gestörte Darmmotilität"},"es":{"name":"Síndrome del intestino corto","definition":"El síndrome del intestino corto es un fallo intestinal ya sea debido a un defecto congénito, a un infarto intestinal o una resección quirúrgica extensiva intestinal, que da lugar a un intestino funcional pequeño de menos de 200 cm de longitud y que se caracteriza por diarrea, malabsorción de nutrientes, dilatación intestinal y dismovilidad."},"it":{"name":"Sindrome dell'intestino corto","definition":"La sindrome dell'intestino corto è un'insufficienza intestinale da difetto congenito, infarto intestinale o resezione chirurgica estesa dell'intestino, che dà origine a un intestino tenue funzionante di lunghezza inferiore ai 200 cm, che si caratterizza per diarrea, malassorbimento delle sostanze nutritive, dilatazione dell'intestino e alterata motilità."},"nl":{"name":"Kortedarmsyndroom","definition":"Een darmfalen als gevolg van hetzij een congenitaal defect, darminfarct of uitgebreide chirurgische excisie van darmkanaal die resulteert in een functionele dunne darm met een lengte van minder dan 200cm, gekenmerkt door diarree, malabsorptie van voedingsstoffen, dilatatie van darm, en motiliteitsstoornis."},"pt":{"name":"Síndrome do intestino curto"},"pl":{"name":"Zespół krótkiego jelita"},"cs":{"name":"Syndrom krátkého střeva"},"uk":{"name":"Синдром короткої кишки","definition":"Short bowel syndrome is an intestinal failure due to either a congenital defect, intestinal infarction or extensive surgical resection of the intestinal tract that results in a functional small intestine of less than 200cm in length and is characterized by diarrhea, nutrient malabsoption, bowel dilation and dysmobility."},"zh":{"name":"短肠综合征","definition":"短肠综合征是一种由先天性缺陷、肠梗死或广泛手术切除引起的肠功能衰竭，其特征为腹泻、营养物质吸收不良、肠道扩张和运动障碍。"},"tr":{"name":"Kısa bağırsak sendromu"}},"xrefs":[{"source":"ICD-11","code":"DA96.04","qualifier":"E","icd_relation":"specific","validated":true,"icd11_uri":"780637678"},{"source":"MONDO","code":"0015183","qualifier":"E","icd_relation":null,"validated":true},{"source":"MONDO","code":"15183","qualifier":"E","icd_relation":null,"validated":true},{"source":"MeSH","code":"D012778","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C0036992","qualifier":"E","icd_relation":null,"validated":true},{"source":"GARD","code":"1502","qualifier":"E","icd_relation":null,"validated":true},{"source":"MedDRA","code":"10049416","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":[{"orpha":365563,"name":"Primary short bowel syndrome","type":"Clinical group"},{"orpha":95427,"name":"Secondary short bowel syndrome","type":"Disease"}],"genes":[],"genes_via_subtypes":[],"inheritance":[],"onset":[],"prevalence":[{"type":"Point prevalence","qualification":"Value and class","class":"1-9 / 100 000","value":2.0,"area":"Europe","status":"Validated","source":"European Medicines Agency 2019[OTHER]"}],"phenotypes":null}