{"orpha":95427,"name":"Secondary short bowel syndrome","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":[],"definition":"Secondary short bowel syndrome is an intestinal failure caused by any condition that results in a functional small intestine of less than 200 cm in length and is characterized by diarrhea, nutrient malabsoption, bowel dilation and dysmobility.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-95427/","orphanet_url":"https://www.orpha.net/en/disease/detail/95427","names":{"fr":{"name":"Syndrome du grêle court secondaire","synonyms":["SGC secondaire","Syndrome de l'intestin court secondaire"],"definition":"Le syndrome de l'intestin court secondaire est une défaillance intestinale due à un intestin court fonctionnel de moins de 200 cm de long. Il est caractérisé par une diarrhée, une malabsorption des nutriments, une dilatation intestinale et une dysmotilité."},"de":{"name":"Kurzdarm-Syndrom, sekundäres","synonyms":["Kurzdarm-Syndrom durch nekrotisierende Enterokolitis","Kurzdarm-Syndrom durch operative Resektion","Kurzdarm-Syndrom durch totale oder subtotale Aganglionose","Kurzdarm-Syndrom durch Thrombose","Kurzdarm-Syndrom durch Trauma","Kurzdarm-Syndrom durch Volvulus"],"definition":"Das Sekundäre Kurzdarmsyndrom ist eine Funktionsstörung des Darms, die immer auftritt, 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 secundario del intestino corto","definition":"El síndrome secundario del intestino corto es un fallo intestinal causado por cualquier condición que dé lugar a un intestino funcional pequeño de menos de 200 cm de longitud, y se caracteriza por diarrea, malabsorción de nutrientes, dilatación intestinal y dismovilidad."},"it":{"name":"Sindrome secondaria dell'intestino corto","definition":"La sindrome secondaria dell'intestino corto è un'insufficienza intestinale causata dalle malattie associate ad un intestino tenue funzionale di lunghezza inferiore ai 200 cm, che si caratterizzano per diarrea, malassorbimento dei cibi, dilatazione dell'intestino e alterata motilità."},"nl":{"name":"Secundair kortedarmsyndroom","synonyms":["Kortedarmsyndroom als gevolg van chirurgische resectie","Kortedarmsyndroom als gevolg van necrotiserende enterocolitis","Kortedarmsyndroom als gevolg van totale of subtotale aganglionose","Kortedarmsyndroom als gevolg van trauma","Kortedarmsyndroom als gevolg van trombose","Kortedarmsyndroom als gevolg van volvulus"],"definition":"Een darmfalen als gevolg van eender welke aandoening die resulteert in een functionele dunne darm met een lengte van minder dan 200 cm, gekenmerkt door diarree, malabsorptie van voedingsstoffen, dilatatie van darm, en motiliteitsstoornis."},"pt":{"name":"Síndrome do intestino curto secundário"},"pl":{"name":"Wtórny zespół krótkiego jelita"},"cs":{"name":"Sekundární syndrom krátkého střeva"},"uk":{"name":"Вторинний синдром короткої кишки","definition":"Secondary short bowel syndrome is an intestinal failure caused by any condition that results in a functional small intestine of less than 200 cm in length and is characterized by diarrhea, nutrient malabsoption, bowel dilation and dysmobility."},"zh":{"name":"继发性短肠综合征","definition":"继发性短肠综合征是一种由任何导致有功能的小肠长度小于200厘米的疾病引起的肠衰竭，其特征是腹泻、营养不良、肠扩张和运动障碍。"},"tr":{"name":"Sekonder kısa bağırsak sendromu"}},"xrefs":[{"source":"ICD-10","code":"K91.2","qualifier":"BTNT","icd_relation":"attributed","validated":true},{"source":"ICD-11","code":"DA96.04","qualifier":"NTBT","icd_relation":"index","validated":true,"icd11_uri":"780637678"},{"source":"MONDO","code":"0019802","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C5681579","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":97935,"name":"Rare gastroenterologic disease"},"parents":[{"orpha":104008,"name":"Short bowel syndrome","classifications":[152,233]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":["Not applicable"],"onset":["All ages"],"prevalence":[{"type":"Annual incidence","qualification":"Class only","class":"Unknown","value":0.0,"area":"Worldwide","status":"Validated","source":"30725620[PMID]_ORPHANET"},{"type":"Point prevalence","qualification":"Class only","class":"Unknown","value":0.0,"area":"Worldwide","status":"Validated","source":"30725620[PMID]_ORPHANET"}],"phenotypes":{"validation_status":"y","online":"y","validation_date":"2019-06-17","source":"30037012[PMID]_30613802[PMID]_30624508[PMID]_30725620[PMID]_28394957[PMID]_28827911[PMID]","terms":[{"hpo":"HP:0002244","label":"Abnormality of the small intestine","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0100508","label":"Abnormality of vitamin metabolism","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0002024","label":"Malabsorption","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0004395","label":"Malnutrition","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0001824","label":"Weight loss","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0003270","label":"Abdominal distention","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0003111","label":"Abnormal blood ion concentration","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0001977","label":"Abnormal thrombosis","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0002621","label":"Atherosclerosis","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0001396","label":"Cholestasis","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0002019","label":"Constipation","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0001944","label":"Dehydration","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0002014","label":"Diarrhea","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0004387","label":"Enterocolitis","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0001508","label":"Failure to thrive","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0001543","label":"Gastroschisis","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0001510","label":"Growth delay","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0003572","label":"Low plasma citrulline","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0030248","label":"Mesenteric venous thrombosis","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0002591","label":"Polyphagia","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0000832","label":"Primary hypothyroidism","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0012850","label":"Small intestinal dysmotility","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0030247","label":"Splanchnic vein thrombosis","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0002570","label":"Steatorrhea","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0011473","label":"Villous atrophy","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0002580","label":"Volvulus","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0002013","label":"Vomiting","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0002251","label":"Aganglionic megacolon","frequency":"Very rare (<4-1%)","criteria":null},{"hpo":"HP:0011787","label":"Central hypothyroidism","frequency":"Very rare (<4-1%)","criteria":null},{"hpo":"HP:0001265","label":"Hyporeflexia","frequency":"Very rare (<4-1%)","criteria":null},{"hpo":"HP:0011100","label":"Intestinal atresia","frequency":"Very rare (<4-1%)","criteria":null},{"hpo":"HP:0100806","label":"Sepsis","frequency":"Very rare (<4-1%)","criteria":null}]}}