{"orpha":583861,"name":"Isolated mesenteric vein thrombosis","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":[],"definition":"A rare intestinal disease characterized by isolated thrombosis of the mesenteric veins without involvement of the splenic or portal veins, potentially causing mesenteric ischemia. It may be primary/idiopathic or due to one or more local or systemic predisposing conditions, and may be acute/subacute or chronic, the latter being used for patients with symptoms lasting longer than 4 weeks but without bowel infarction, or patients without recent symptoms, where the thrombosis is an incidental finding. Symptoms depend on the extent and severity of thrombosis and often involve prolonged nonspecific abdominal complaints.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-583861/","orphanet_url":"https://www.orpha.net/en/disease/detail/583861","names":{"fr":{"name":"Thrombose isolée de la veine mésentérique","definition":"Maladie intestinale rare caractérisée par une thrombose isolée des veines mésentériques, sans atteinte de la veine splénique ou de la veine porte, pouvant entraîner une ischémie mésentérique. Elle peut être primitive/idiopathique ou due à un ou plus d'un facteurs prédisposants locaux ou systémiques. Elle peut également être aiguë/subaiguë ou chronique, cette dernière forme se rapportant aux patients dont les symptômes durent plus de 4 semaines sans infarctus intestinal, ou aux patients sans symptômes récents chez lesquels la découverte de la thrombose se fait de manière fortuite. Les symptômes dépendent de l'ampleur et de la sévérité de la thrombose, et les patients se plaignent souvent de douleurs abdominales non spécifiques qui durent."},"de":{"name":"Mesenterialvenenthrombose, isolierte","definition":"Eine seltene Darmerkrankung, die durch eine isolierte Thrombose der Mesenterialvenen ohne Beteiligung der Milz- oder Pfortader gekennzeichnet ist und eine mesenteriale Ischämie verursachen kann. Sie kann primär/idiopathisch oder durch eine oder mehrere lokale oder systemische prädisponierende Bedingungen bedingt sein und kann akut/subakut oder chronisch sein, wobei letzteres für Patienten mit Symptomen, die länger als 4 Wochen andauern, aber ohne Darminfarkt, oder für Patienten ohne aktuelle Symptome, bei denen die Thrombose ein Zufallsbefund ist, verwendet wird. Die Symptome hängen vom Ausmaß und der Schwere der Thrombose ab und bestehen häufig aus lang anhaltenden, unspezifischen Bauchbeschwerden."},"es":{"name":"Trombosis de la vena mesentérica aislada","definition":"Es una enfermedad intestinal poco frecuente caracterizada por trombosis aislada de las venas mesentéricas sin afectación de las venas esplénica o porta, que puede causar isquemia mesentérica. Puede ser primaria/idiopática o debida a uno o más factores de riesgo locales o sistémicos, y puede ser aguda/subaguda o crónica, refiriéndose esta última a pacientes con síntomas que perduran más de 4 semanas pero sin infarto intestinal, o a pacientes sin síntomas recientes en los que la trombosis es un hallazgo incidental. Los síntomas dependen de la extensión y gravedad de la trombosis y a menudo implican molestias abdominales prolongadas e inespecíficas."},"it":{"name":"Trombosi isolata della vena mesenterica"},"nl":{"name":"Geïsoleerde trombose van vena mesenterica","definition":"Een zeldzame intestinale aandoening, gekenmerkt door geïsoleerde trombose van vena mesenterica zonder betrokkenheid van vena splenica of vena portae, die mogelijk mesenteriale ischemie veroorzaakt. Het kan primair/idiopathisch zijn, of te wijten zijn aan een of meer lokale of systemische predisponerende aandoeningen, en kan acuut/subacuut of chronisch optreden, waarbij deze laatste verwijst naar patiënten met symptomen die langer dan 4 weken aanhouden maar zonder darminfarct, of patiënten zonder recente symptomen bij wie de trombose toevallig wordt aangetroffen. Symptomen zijn afhankelijk van de mate en de ernst van de trombose, en vaak gaat het om langdurige aspecifieke abdominale klachten."},"pt":{"name":"Trombose isolada da veia mesentérica","definition":"Doença intestinal rara caracterizada por trombose isolada das veias mesentéricas sem envolvimento das veias esplénica ou porta, podendo causar isquemia mesentérica. Pode ser primária/idiopática ou devida a uma ou mais condições predisponentes locais ou sistémicas e pode ser aguda/subaguda ou crónica, sendo este último termo utilizado para doentes com sintomas que duram mais de 4 semanas mas sem enfarte intestinal ou para doentes sem sintomas recentes, em que a trombose é um achado incidental. Os sintomas dependem da extensão e da gravidade da trombose e envolvem frequentemente queixas abdominais prolongadas e inespecíficas."},"pl":{"name":"Izolowana zakrzepica żyły krezkowej","definition":"Rzadka choroba jelit charakteryzująca się izolowaną zakrzepicą żył krezkowych bez zajęcia żył śledzionowych i wrotnych, potencjalnie powodującą niedokrwienie krezki. Może mieć charakter pierwotny/idiopatyczny lub wynikać z jednego lub większej liczby miejscowych lub ogólnoustrojowych schorzeń predysponujących, a przebieg ostry/podostry lub przewlekły, przy czym ten ostatni dotyczy pacjentów z objawami trwającymi dłużej niż 4 tygodnie, ale bez zawału jelita, lub pacjentów bez świeżych objawów , gdzie zakrzepica jest rozpoznaniem przypadkowym. Objawy zależą od rozległości i ciężkości zakrzepicy i często obejmują długotrwałe, nieswoiste dolegliwości brzuszne."},"cs":{"name":"Izolovaná trombóza mezenterické žíly"},"uk":{"name":"Ізольований тромбоз брижової вени","definition":"A rare intestinal disease characterized by isolated thrombosis of the mesenteric veins without involvement of the splenic or portal veins, potentially causing mesenteric ischemia. It may be primary/idiopathic or due to one or more local or systemic predisposing conditions, and may be acute/subacute or chronic, the latter being used for patients with symptoms lasting longer than 4 weeks but without bowel infarction, or patients without recent symptoms, where the thrombosis is an incidental finding. Symptoms depend on the extent and severity of thrombosis and often involve prolonged nonspecific abdominal complaints."},"zh":{"name":"孤立性肠系膜静脉血栓"},"tr":{"name":"İzole mezenterik ven trombozu"}},"xrefs":[{"source":"ICD-10","code":"K55.0","qualifier":"NTBT","icd_relation":"inclusion","validated":true},{"source":"ICD-11","code":"DD30.2","qualifier":"E","icd_relation":"specific","validated":true,"icd11_uri":"213868120"},{"source":"MONDO","code":"0035009","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C0267412","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":97935,"name":"Rare gastroenterologic disease"},"parents":[{"orpha":117569,"name":"Rare intestinal disease","classifications":[152]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":["Not applicable"],"onset":["Adult","Elderly"],"prevalence":[{"type":"Annual incidence","qualification":"Value and class","class":"1-9 / 100 000","value":1.6,"area":"Europe","status":"Validated","source":"18720461[PMID]_25917534[PMID]"},{"type":"Annual incidence","qualification":"Value and class","class":"1-9 / 1 000 000","value":0.5,"area":"Finland","status":"Validated","source":"25917534[PMID]"},{"type":"Annual incidence","qualification":"Value and class","class":"1-9 / 100 000","value":2.7,"area":"Sweden","status":"Validated","source":"18720461[PMID]"},{"type":"Point prevalence","qualification":"Class only","class":"Unknown","value":0.0,"area":"Europe","status":"Validated","source":"18720461[PMID]_25917534[PMID]_ORPHANET"}],"phenotypes":{"validation_status":"y","online":"y","validation_date":"2024-09-26","source":"29083683[PMID]_25755568[PMID]_26285602[PMID]","terms":[{"hpo":"HP:0002027","label":"Abdominal pain","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0030248","label":"Mesenteric venous thrombosis","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0002039","label":"Anorexia","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0002014","label":"Diarrhea","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0001974","label":"Increased total leukocyte count","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0002018","label":"Nausea","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0002013","label":"Vomiting","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0003270","label":"Abdominal distention","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0410282","label":"Abnormal circulating amylase concentration","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0001541","label":"Ascites","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0030151","label":"Cholangitis","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0001081","label":"Cholelithiasis","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0001945","label":"Fever","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0002239","label":"Gastrointestinal hemorrhage","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0002615","label":"Hypotension","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0002151","label":"Increased circulating lactate concentration","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:6000377","label":"Intramural intestinal gas","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0100806","label":"Sepsis","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0031273","label":"Shock","frequency":"Occasional (29-5%)","criteria":null}]}}