{"orpha":521219,"name":"Mirizzi syndrome","type":"Clinical syndrome","group":"Disorder","status":"active","flags":[],"synonyms":["Extrinsic biliary compression syndrome"],"definition":"A rare biliary tract disease characterized by external compression and subsequent obstruction of an extrahepatic biliary duct by one or more gallstones in the cystic duct or the gallbladder. Patients may present with acute or chronic cholecystitis with right upper abdominal pain, nausea, and vomiting, jaundice, or cholangitis. Cholecystobiliary or -enteric fistulae can arise due to chronic inflammation and ulceration.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-521219/","orphanet_url":"https://www.orpha.net/en/disease/detail/521219","names":{"fr":{"name":"Syndrome de Mirizzi","synonyms":["Syndrome de compression extrinsèque de la voie biliaire principale"],"definition":"Maladie rare des voies biliaires caractérisée par la compression extrinsèque et, par la suite, l'obstruction de la voie biliaire extra-hépatique par un ou plusieurs calculs biliaires enclavés dans le canal cystique ou la vésicule biliaire. Les patients peuvent manifester une cholécystite aiguë ou chronique et des douleurs dans la partie supérieure droite de l'abdomen, des nausées et des vomissements, un ictère ou une cholangite. Des fistules cholécysto-biliaires ou entériques peuvent se développer en raison d'une inflammation et d'une ulcération chronique."},"de":{"name":"Mirizzi-Syndrom","synonyms":["Extrinsische Gallenwegskompression"],"definition":"Eine seltene Erkrankung der Gallenwege, die durch eine externe Kompression und anschließende Obstruktion eines extrahepatischen Gallengangs durch einen oder mehrere Gallensteine im Ductus cysticus oder in der Gallenblase gekennzeichnet ist. Die Patienten können eine akute oder chronische Cholezystitis mit Schmerzen im rechten Oberbauch, Übelkeit und Erbrechen, Gelbsucht oder Cholangitis aufweisen. Durch chronische Entzündungen und Ulzerationen können cholezystobiliäre oder -enterische Fisteln entstehen."},"es":{"name":"Síndrome de Mirizzi","synonyms":["Síndrome de compresión biliar extrínseca"],"definition":"Es una enfermedad de las vías biliares poco frecuente caracterizada por compresión externa y posterior obstrucción de un conducto biliar extrahepático por uno o más cálculos biliares en el conducto cístico o en la vesícula biliar. Los pacientes pueden presentar colecistitis aguda o crónica con dolor abdominal superior derecho, náusea y vómitos, ictericia o colangitis. Pueden surgir fístulas colecistobiliares o entéricas debido a la inflamación crónica y ulceración."},"it":{"name":"Sindrome di Mirizzi","definition":"Si tratta di una malattia rara delle vie biliari, caratterizzata da una compressione esterna, e dalla conseguente ostruzione, di una via biliare extraepatica da parte di uno o più calcoli biliari nel dotto cistico o nella cistifellea. I pazienti presentano colecistite acuta o cronica associata al dolore nella parte superiore destra dell'addome, alla nausea, al vomito, all'ittero o alla colangite. L'infiammazione e la presenza di ulcere possono causare la formazione di fistole colecisto-biliari o enteriche."},"nl":{"name":"Syndroom van Mirizzi","synonyms":["Extrinsieke biliaire compressie-syndroom"],"definition":"Een zeldzame aandoening van galwegen, gekarakteriseerd door externe compressie en daaropvolgende obstructie van een extrahepatische galweg door een of meer galstenen in de galblaasafvoergang of de galblaas. Patiënten kunnen zich presenteren met acute of chronische cholecystitis (galblaasontsteking) met rechtsboven abdominale pijn, misselijkheid, en braken, geelzucht, of cholangitis. Cholecystobiliaire of cholecysto-enterale fistels kunnen ontstaan ten gevolge van chronische inflammatie en ulceratie."},"pt":{"name":"Síndrome Mirizzi"},"pl":{"name":"Zespół Mirizziego","synonyms":["Extrinsic biliary compression syndrome"]},"cs":{"name":"Mirizziho syndrom","synonyms":["Extrinsic biliary compression syndrome"]},"uk":{"name":"Синдром Міріцці","synonyms":["Синдром зовнішньої компресії жовчних шляхів"],"definition":"A rare biliary tract disease characterized by external compression and subsequent obstruction of an extrahepatic biliary duct by one or more gallstones in the cystic duct or the gallbladder. Patients may present with acute or chronic cholecystitis with right upper abdominal pain, nausea, and vomiting, jaundice, or cholangitis. Cholecystobiliary or -enteric fistulae can arise due to chronic inflammation and ulceration."},"zh":{"name":"米里齐综合征","synonyms":["胆道外压迫综合症"],"definition":"一种少见的胆道疾病，其特征是肝外胆管被一个或多个胆囊管或胆囊中的胆结石外力压迫并继而发生阻塞。患者可出现急性或慢性胆囊炎，伴有右上腹痛、恶心呕吐、黄疸或胆管炎。由于慢性炎症和溃疡，可出现胆囊胆管瘘或胆囊小肠瘘。"},"tr":{"name":"Mirizzi sendromu","synonyms":["Ekstrinsik biliyer kompresyon sendromu"]}},"xrefs":[{"source":"ICD-10","code":"K80.8","qualifier":"NTBT","icd_relation":"attributed","validated":false},{"source":"ICD-11","code":"DC11.Y","qualifier":"NTBT","icd_relation":"index","validated":true,"icd11_uri":"381742412"},{"source":"MONDO","code":"0043330","qualifier":"E","icd_relation":null,"validated":true},{"source":"MeSH","code":"D057792","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C0267878","qualifier":"E","icd_relation":null,"validated":true},{"source":"GARD","code":"10177","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":57146,"name":"Rare hepatic disease"},"parents":[{"orpha":101941,"name":"Rare biliary tract disease","classifications":[183]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":["Not applicable"],"onset":["Adult","Elderly"],"prevalence":[{"type":"Point prevalence","qualification":"Class only","class":"Unknown","value":0.0,"area":"Worldwide","status":"Validated","source":"ORPHANET_29494098[PMID]"}],"phenotypes":{"validation_status":"y","online":"y","validation_date":"2021-06-02","source":"29494098[PMID]_28653000[PMID]","terms":[{"hpo":"HP:0002027","label":"Abdominal pain","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0001081","label":"Cholelithiasis","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0011980","label":"Cholesterol gallstones","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0040319","label":"Dark urine","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0003155","label":"Elevated circulating alkaline phosphatase concentration","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0002904","label":"Hyperbilirubinemia","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0000952","label":"Jaundice","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0011848","label":"Abdominal colic","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0003270","label":"Abdominal distention","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0002039","label":"Anorexia","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0025143","label":"Chills","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0002910","label":"Elevated circulating hepatic transaminase concentration","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0410019","label":"Epigastric pain","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0001945","label":"Fever","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0002018","label":"Nausea","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0001733","label":"Pancreatitis","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0001649","label":"Tachycardia","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0002013","label":"Vomiting","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0100889","label":"Abnormality of the ductus choledochus","frequency":"Very rare (<4-1%)","criteria":null},{"hpo":"HP:0030154","label":"Gallbladder perforation","frequency":"Very rare (<4-1%)","criteria":null}]}}