{"orpha":583856,"name":"Isolated splenic vein thrombosis","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":[],"definition":"A rare gastroenterologic disease characterized by isolated thrombotic obstruction of the splenic vein, typically due to pancreatic disorders, in particular acute or chronic pancreatitis or pancreatic neoplasms. It is the main cause of left-sided portal hypertension. Patients may be asymptomatic or present with abdominal pain, gastrointestinal bleeding, nausea, and vomiting, as well as signs of portal hypertension.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-583856/","orphanet_url":"https://www.orpha.net/en/disease/detail/583856","names":{"fr":{"name":"Thrombose isolée de la veine splénique","definition":"Maladie gastroentérologique rare caractérisée par l'obstruction thrombotique isolée de la veine splénique, généralement due à des troubles pancréatiques, en particulier à une pancréatite aiguë ou chronique ou à des néoplasmes du pancréas. Il s'agit de la première cause d'hypertension portale gauche. Les patients peuvent être asymptomatiques ou présenter des douleurs abdominales, des saignements gastro-intestinaux, des nausées et des vomissements, ainsi que des signes d'hypertension portale."},"de":{"name":"Milzvenenthrombose, isolierte","definition":"Eine seltene gastroenterologische Krankheit, die durch eine isolierte thrombotische Obstruktion der Milzvene gekennzeichnet ist, die typischerweise auf Erkrankungen der Bauchspeicheldrüse, insbesondere auf eine akute oder chronische Pankreatitis oder Neoplasien der Bauchspeicheldrüse, zurückzuführen ist. Sie ist die Hauptursache für eine linksseitige portale Hypertension. Die Patienten können asymptomatisch sein oder unter Bauchschmerzen, gastrointestinalen Blutungen, Übelkeit und Erbrechen sowie Anzeichen einer portalen Hypertension leiden."},"es":{"name":"Trombosis de la vena esplénica aislada","synonyms":["Trombosis venosa esplénica aislada"],"definition":"Es una enfermedad digestiva poco frecuente caracterizada por una obstrucción trombótica aislada de la vena esplénica, normalmente debida a trastornos pancreáticos, en particular pancreatitis aguda o crónica o neoplasias pancreáticas. Es la principal causa de hipertensión portal izquierda. Los pacientes pueden permanecer asintomáticos o presentar dolor abdominal, hemorragia digestiva, náuseas y vómitos, así como signos de hipertensión portal."},"it":{"name":"Trombosi isolata della vana splenica"},"nl":{"name":"Geïsoleerde trombose van vena splenica","synonyms":["Geïsoleerde trombose van miltader","Geïsoleerde trombose van vena lienalis"],"definition":"Een zeldzame gastro-enterologische aandoening, gekenmerkt door geïsoleerde trombotische obstructie van vena splenica (miltader), doorgaans als gevolg van aandoeningen van de pancreas, in het bijzonder acute of chronische pancreatitis of neoplasmata van pancreas. Het is de voornaamste oorzaak van linkszijdige portale hypertensie. Patiënten kunnen asymptomatisch zijn, of zich presenteren met abdominale pijn, gastro-intestinale bloeding, misselijkheid, en braken, alsook met verschijnselen van portale hypertensie."},"pt":{"name":"Trombose isolada da veia esplénica","definition":"Doença gastroenterológica rara caracterizada por obstrução trombótica isolada da veia esplénica, geralmente devida a doenças pancreáticas, em particular pancreatite aguda ou crónica ou neoplasias pancreáticas. É a principal causa de hipertensão portal do lado esquerdo. Os doentes podem ser assintomáticos ou apresentar dor abdominal, hemorragia gastrointestinal, náuseas e vómitos, bem como sinais de hipertensão portal."},"pl":{"name":"Izolowana zakrzepica żył śledzionowych","definition":"Rzadka choroba gastroenterologiczna charakteryzująca się izolowaną zakrzepową niedrożnością żyły śledzionowej, zwykle spowodowaną chorobami trzustki, w szczególności ostrym lub przewlekłym zapaleniem lub nowotworami trzustki. Jest główną przyczyną lewostronnego nadciśnienia wrotnego. U pacjentów może przebiegać bezobjawowo lub manifestować się bólem brzucha, krwawieniem z przewodu pokarmowego, nudnościami i wymiotami oraz objawami nadciśnienia wrotnego."},"cs":{"name":"Izolovaná trombóza slezinné žíly"},"uk":{"name":"Ізольований тромбоз селезінкової вени","definition":"A rare gastroenterologic disease characterized by isolated thrombotic obstruction of the splenic vein, typically due to pancreatic disorders, in particular acute or chronic pancreatitis or pancreatic neoplasms. It is the main cause of left-sided portal hypertension. Patients may be asymptomatic or present with abdominal pain, gastrointestinal bleeding, nausea, and vomiting, as well as signs of portal hypertension."},"zh":{"name":"孤立性脾静脉血栓"},"tr":{"name":"İzole dalak ven trombozu"}},"xrefs":[{"source":"ICD-10","code":"I82.8","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"ICD-11","code":"DB98.4","qualifier":"E","icd_relation":"specific","validated":true,"icd11_uri":"121618859"},{"source":"MONDO","code":"0035008","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C0272416","qualifier":"E","icd_relation":null,"validated":true},{"source":"MedDRA","code":"10041659","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":97935,"name":"Rare gastroenterologic disease"},"parents":[{"orpha":97935,"name":"Rare gastroenterologic disease","classifications":[152]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":["Not applicable"],"onset":["Adult","Elderly"],"prevalence":[{"type":"Annual incidence","qualification":"Class only","class":"Unknown","value":0.0,"area":"Worldwide","status":"Validated","source":"ORPHANET"},{"type":"Point prevalence","qualification":"Class only","class":"Unknown","value":0.0,"area":"Worldwide","status":"Validated","source":"ORPHANET"}],"phenotypes":null}