{"orpha":854,"name":"Non-malignant and non-cirrhotic portal vein thrombosis","type":"Clinical syndrome","group":"Disorder","status":"active","flags":[],"synonyms":["Non-cirrhotic and non-tumoral portal vein thrombosis","Non-malignant non-cirrhotic PVT"],"definition":"Portal vein thrombosis (PVT) is associated with acute (recent) or chronic (long-standing) thrombosis of the portal system.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-854/","orphanet_url":"https://www.orpha.net/en/disease/detail/854","names":{"fr":{"name":"Thrombose portale non-maligne and non-cirrhotique","synonyms":["Thrombose de la veine porte non cirrhotique et non tumorale","Thrombose portale non cirrhotique et non maligne"],"definition":"La thrombose portale est caractérisée par un thrombus d'apparition récente ou ancienne."},"de":{"name":"Pfortaderthrombose, nicht-maligne und nicht-zirrhotische","synonyms":["Nicht-maligne und nicht-zirrhotische PVT","Pfortaderthrombose, nicht-zirrhotische"],"definition":"Die Pfortaderthrombose (PVT) ist eine akute (erst kurze Zeit bestehende) oder chronische Thrombose des Pfortadersystems."},"es":{"name":"Trombosis venosa portal no maligna y no cirrótica","synonyms":["TVP no maligna y no cirrótica","Trombosis venosa portal no cirrótica y no tumoral"],"definition":"La trombosis venosa portal (TVP) está asociada a una trombosis aguda (reciente) o crónica (de larga duración) del sistema portal."},"it":{"name":"Trombosi non maligna e non cirrotica della vena porta","synonyms":["Trombosi non cirrotica e non neoplastica della vena porta","PVT non maligna non cirrotica"]},"nl":{"name":"Niet-maligne en niet-cirrotische poortadertrombose","synonyms":["Niet-cirrotische en niet-tumorale poortadertrombose","Niet-maligne en niet-cirrotische trombose van vena portae","Niet-maligne niet-cirrotische VPT"],"definition":"Poortadertrombose (PVT) is geassocieerd met acute (recente) of chronische (langdurige) trombose van poortaderstelsel."},"pt":{"name":"Trombose da veia porta não-maligna e não-cirrótica","synonyms":["TVP não-maligna e não-cirrótica","Trombose da veia porta não-cirrótica e não-tumoral"],"definition":"A trombose da veia porta (PVT) está associada com trombose aguda (recente) ou crónica (longa duração) do sistema porta."},"pl":{"name":"Pierwotna zakrzepica żyły wrotnej","synonyms":["Non-cirrhotic and non-tumoral portal vein thrombosis","Non-malignant non-cirrhotic PVT"]},"cs":{"name":"Trombóza portální žíly","synonyms":["Non-cirrhotic and non-tumoral portal vein thrombosis","Non-malignant non-cirrhotic PVT"]},"uk":{"name":"Первинний тромбоз воротної вени","synonyms":["Нециротичний тромбоз портальної вени"],"definition":"Portal vein thrombosis (PVT) is associated with acute (recent) or chronic (long-standing) thrombosis of the portal system."},"zh":{"name":"原发性门静脉血栓形成","synonyms":["非肝硬化门静脉血栓"]},"tr":{"name":"Primitif portal ven trombozu","synonyms":["Sirotik olmayan portal ven trombozu"]}},"xrefs":[{"source":"ICD-10","code":"I81","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"ICD-11","code":"DB98.3","qualifier":"NTBT","icd_relation":"specific","validated":true,"icd11_uri":"866276949"},{"source":"MONDO","code":"0019431","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C0155773","qualifier":"E","icd_relation":null,"validated":true},{"source":"MedDRA","code":"10036206","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":57146,"name":"Rare hepatic disease"},"parents":[{"orpha":101938,"name":"Rare vascular liver disease","classifications":[183]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":["Not applicable"],"onset":["All ages"],"prevalence":[{"type":"Point prevalence","qualification":"Value and class","class":"1-9 / 100 000","value":3.7,"area":"Sweden","status":"Validated","source":"21039677[PMID]"},{"type":"Annual incidence","qualification":"Value and class","class":"1-9 / 1 000 000","value":0.7,"area":"Sweden","status":"Validated","source":"21039677[PMID]"},{"type":"Point prevalence","qualification":"Class only","class":"Unknown","value":0.0,"area":"Europe","status":"Validated","source":"ORPHANET"},{"type":"Annual incidence","qualification":"Value and class","class":null,"value":2.75,"area":"Italy","status":"Validated","source":"28180235[PMID]"},{"type":"Annual incidence","qualification":"Value and class","class":"1-9 / 100 000","value":1.72,"area":"Europe","status":"Validated","source":"21039677[PMID]_28180235[PMID]"}],"phenotypes":null}