{"orpha":26790,"name":"Pseudomyxoma peritonei","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":["Adenomucinosis","Gelatinous ascites","PMP"],"definition":"Pseudomyxoma peritonei is characterized by disseminated intra-peritoneal mucinous tumors and mucinous ascites in the abdomen and pelvis.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-26790/","orphanet_url":"https://www.orpha.net/en/disease/detail/26790","names":{"fr":{"name":"Maladie gélatineuse du péritoine","synonyms":["Ascite gélatineuse","PMP","Pseudomyxome péritonéal"],"definition":"La maladie gélatineuse du péritoine se caractérise par des tumeurs mucineuses disséminées du péritoine et des ascites mucineuses de l'abdomen et du pelvis."},"de":{"name":"Pseudomyxoma peritonei","synonyms":["Aszites, gallertiger","PMP","Adenomuzinose"],"definition":"Das Pseudomyxoma peritonei ist gekennzeichnet durch disseminierte intra-peritoneale muzinöse Tumoren und schleimigen Aszites im Abdomen und Beckenraum."},"es":{"name":"Pseudomixoma peritoneal","synonyms":["Adenomucinosis","Ascitis gelatinosa","PMP"],"definition":"El pseudomixoma peritoneal se caracteriza por la presencia de tumores mucinosos intraperitoneales diseminados y de ascitis mucinosa en el abdomen y la pelvis."},"it":{"name":"Pseudomixoma del peritoneo","synonyms":["Adenomucinosi","Ascite gelatinosa","PMP"]},"nl":{"name":"Pseudomyxoma peritonei","synonyms":["Adenomucinosis","Gelatineuze ascites","PMP"],"definition":"Pseudomyxoma peritonei wordt gekenmerkt door verspreide intraperitoneale mucineuze tumoren en mucineuze ascites in de buik en het bekken."},"pt":{"name":"Pseudo-mixoma do peritoneu","synonyms":["Ascite gelatinosa"],"definition":"O pseudomixoma peritoneal é caracterizado por tumores mucinosos intraperitoneais disseminados e ascite mucinosa no abdómen e na pélvis."},"pl":{"name":"Śluzak rzekomy otrzewnej","synonyms":["Adenomucinosis","Gelatinous ascites","PMP"]},"cs":{"name":"Pseudomyxom peritonea","synonyms":["Adenomucinóza","Želatinózní ascites","PMP"]},"uk":{"name":"Псевдоміксома очеревини","synonyms":["Аденомуциноз","Желатинозний асцит","PMP"],"definition":"Pseudomyxoma peritonei is characterized by disseminated intra-peritoneal mucinous tumors and mucinous ascites in the abdomen and pelvis."},"zh":{"name":"腹膜假粘液瘤","synonyms":["明胶性腹水"]},"tr":{"name":"Psödomiksoma peritonei","synonyms":["PMP","Adenomüsinoz","Jelatinimsi asit"]}},"xrefs":[{"source":"ICD-10","code":"C78.6","qualifier":"NTBT","icd_relation":"index","validated":true},{"source":"ICD-11","code":"2D91","qualifier":"NTBT","icd_relation":"index","validated":true,"icd11_uri":"1523083931"},{"source":"MONDO","code":"0017048","qualifier":"E","icd_relation":null,"validated":true},{"source":"MeSH","code":"D011553","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C0033822","qualifier":"E","icd_relation":null,"validated":true},{"source":"GARD","code":"7488","qualifier":"E","icd_relation":null,"validated":true},{"source":"MedDRA","code":"10037138","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":250908,"name":"Rare neoplastic disease"},"parents":[{"orpha":168807,"name":"Primary malignant peritoneal tumor","classifications":[182,202,233]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":["Unknown"],"onset":["Adult"],"prevalence":[{"type":"Annual incidence","qualification":"Value and class","class":"1-9 / 1 000 000","value":0.1,"area":"Worldwide","status":"Validated","source":"19838447[PMID]_17524597[PMID]_16678994[PMID]_[EXPERT]"},{"type":"Annual incidence","qualification":"Value and class","class":"1-9 / 1 000 000","value":0.2,"area":"Netherlands","status":"Validated","source":"17524597[PMID]"},{"type":"Point prevalence","qualification":"Class only","class":"Unknown","value":0.0,"area":"Worldwide","status":"Not yet validated","source":"ORPHANET"},{"type":"Point prevalence","qualification":"Value and class","class":"1-9 / 100 000","value":2.0,"area":"Europe","status":"Validated","source":"European Medicines Agency 2018[OTHER]"}],"phenotypes":{"validation_status":"y","online":"y","validation_date":"2016-06-01","source":null,"terms":[{"hpo":"HP:0004298","label":"Abnormality of the abdominal wall","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0002585","label":"Abnormality of the peritoneum","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0001541","label":"Ascites","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0002037","label":"Inflammation of the large intestine","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0002027","label":"Abdominal pain","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0002019","label":"Constipation","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0100790","label":"Hernia","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0005214","label":"Intestinal obstruction","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0002716","label":"Lymphadenopathy","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0002017","label":"Nausea and vomiting","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0002093","label":"Respiratory insufficiency","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0001824","label":"Weight loss","frequency":"Occasional (29-5%)","criteria":null}]}}