{"orpha":693826,"name":"Pancreatic arteriovenous malformation","type":"Malformation syndrome","group":"Disorder","status":"active","flags":[],"synonyms":["Arteriovenous malformation of the pancreas"],"definition":"A rare visceral arteriovenous malformation of the pancreas characterized by abnormal blood flow from the arterial system directly into the venous system without passing through the capillaries. Patients may present with episodic gastrointestinal bleeding or hemoperitoneum, epigastric or abdominal pain, pancreatitis, portal hypertension, and pseudocysts. Some patients remain asymptomatic.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-693826/","orphanet_url":"https://www.orpha.net/en/disease/detail/693826","names":{"fr":{"name":"Malformation artérioveineuse pancréatique","synonyms":["MAVP","Malformation artérioveineuse du pancréas"],"definition":"Malformation artérioveineuse viscérale rare du pancréas caractérisée par un flux sanguin anormal provenant du système artériel et s'écoulant directement vers le système veineux sans passer par les capillaires. Les patients peuvent présenter des épisodes de saignements gastro-intestinaux ou un hémopéritoine, des douleurs épigastriques ou abdominales, une pancréatite, une hypertension portale et des pseudokystes. Certains patients restent asymptomatiques."},"de":{"name":"Pankreatische arteriovenöse Fehlbildung","synonyms":["Arteriovenöse Fehlbildung der Bauchspeicheldrüse"],"definition":"Eine seltene viszerale arteriovenöse Fehlbildung der Bauchspeicheldrüse, die durch einen abnormalen Blutfluss vom arteriellen System direkt in das venöse System ohne Durchlaufen der Kapillaren gekennzeichnet ist. Patienten können episodische gastrointestinale Blutungen oder Hämoperitoneum, Oberbauch- oder Bauchschmerzen, Pankreatitis, portale Hypertonie und Pseudozysten aufweisen. Einige Patienten bleiben asymptomatisch."},"es":{"name":"Malformación arteriovenosa pancreática","synonyms":["Malformación arteriovenosa del páncreas"],"definition":"Es una malformación arteriovenosa visceral poco frecuente del páncreas caracterizada por anomalías del flujo sanguíneo, que va desde el sistema arterial directamente al sistema venoso sin pasar por los capilares. Los pacientes pueden presentar hemorragia gastrointestinal episódica o hemoperitoneo, dolor epigástrico o abdominal, pancreatitis, hipertensión portal y pseudoquistes. Algunos pacientes permanecen asintomáticos."},"it":{"name":"Malformazione arterovenosa pancreatica","synonyms":["Malformazione arterovenosa del pancreas"]},"nl":{"name":"Arterioveneuze malformatie van pancreas","synonyms":["Pancreatische arterioveneuze malformatie"],"definition":"Een zeldzame viscerale arterioveneuze malformatie van pancreas, gekenmerkt door abnormale bloedstroom van het arteriële systeem rechtstreeks naar het veneuze systeem zonder door de capillairen te passeren. Patiënten kunnen zich presenteren met episodische gastro-intestinale bloeding of hemoperitoneum, epigastrische of abdominale pijn, pancreatitis, portale hypertensie, en pseudocysten. Sommige patiënten blijven asymptomatisch."},"pt":{"name":"Malformação arteriovenosa pancreática","synonyms":["Malformação arteriovenosa do pâncreas"]},"pl":{"name":"Malformacja tętniczo-żylna trzustki","synonyms":["Arteriovenous malformation of the pancreas"]},"cs":{"name":"Pancreatic arteriovenous malformation","synonyms":["Arteriovenous malformation of the pancreas"]}},"xrefs":[{"source":"ICD-10","code":"Q27.3","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"ICD-11","code":"LA90.3Y","qualifier":"NTBT","icd_relation":"attributed","validated":true,"icd11_uri":"null"}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":93890,"name":"Rare developmental defect during embryogenesis"},"parents":[{"orpha":108971,"name":"Non-syndromic visceral malformation of the liver, biliary tract, pancreas or spleen","classifications":[147,182,233]},{"orpha":693855,"name":"Visceral fast-flow vascular malformation","classifications":[147,198]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":["Not applicable"],"onset":["All ages"],"prevalence":[{"type":"Cases/families","qualification":"Case(s)","class":null,"value":100.0,"area":"Worldwide","status":"Validated","source":"ORPHANET"},{"type":"Point prevalence","qualification":"Class only","class":"<1 / 1 000 000","value":0.0,"area":"Worldwide","status":"Validated","source":"ORPHANET"}],"phenotypes":null}