{"orpha":35062,"name":"Severe disseminated cytomegalovirus infection in immunocompetent patients","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":["Severe disseminated CMV infection in immunocompetent patients"],"definition":"A rare viral disease characterized by fulminant cytomegalovirus infection with multiple organ involvement including the brain, lung, liver, and/or heart, among others, and marked constitutional symptoms in immunocompetent patients. The condition is associated with a high case fatality rate.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-35062/","orphanet_url":"https://www.orpha.net/en/disease/detail/35062","names":{"fr":{"name":"Infection disséminée sévère à cytomégalovirus chez les patients immunocompétents","synonyms":["Infection disséminée sévère à CMV chez les patients immunocompétents"],"definition":"Maladie virale rare caractérisée par une infection fulminante par le cytomégalovirus avec une atteinte de plusieurs organes dont, entre autres, le cerveau, les poumons, le foie et/ou le coeur, et des symptômes constitutionnels marqués chez les patients immunocompétents. La maladie est associée à un taux de létalité élevé."},"de":{"name":"Schwere disseminierte Zytomegalievirus-Infektion bei immunkompetenten Patienten","synonyms":["Schwere disseminierte CMV-Infektion bei immunkompetenten Patienten"],"definition":"Eine seltene Viruserkrankung, die durch eine fulminante Cytomegalovirus-Infektion mit Beteiligung mehrerer Organe, u. a. Gehirn, Lunge, Leber und/oder Herz, und ausgeprägten konstitutionellen Symptomen bei immunkompetenten Patienten gekennzeichnet ist. Die Erkrankung ist mit einer hohen Sterblichkeitsrate verbunden."},"es":{"name":"Infección diseminada por citomegalovirus grave en pacientes inmunocompetentes","synonyms":["Infección diseminada por CMV grave en pacientes inmunocompetentes"],"definition":"Es una enfermedad viral poco frecuente caracterizada por una infección fulminante por citomegalovirus con afectación de múltiples órganos, incluyendo el cerebro, los pulmones, el hígado y/o el corazón, entre otros, y síntomas constitucionales marcados en pacientes inmunocompetentes. La enfermedad se asocia con una elevada tasa de letalidad."},"it":{"name":"Infezione grave disseminata da citomegalovirus nei pazienti immunocompetenti","synonyms":["Infezione grave disseminata da CMV nei pazienti immunocompetenti"],"definition":"Si tratta di una malattia virale rara, caratterizzata da un'infezione fulminante da citomegalovirus che interessa diversi organi (cervello, polmoni, fegato e/o cuore) e marcati sintomi costituzionali in pazienti immunocompetenti. Il tasso di mortalità è elevato."},"nl":{"name":"Ernstige gedissemineerde infectie door cytomegalovirus bij immuuncompetente patiënten","synonyms":["Ernstige gedissemineerde CMV-infectie bij immuuncompetente patiënten"],"definition":"Een zeldzame virale ziekte, gekenmerkt door fulminante infectie door cytomegalovirus met betrokkenheid van meerdere organen, waaronder hersenen, long, lever en/of hart, en opmerkelijke constitutionele symptomen bij immuuncompetente patiënten. De aandoening is geassocieerd met een hoog sterftepercentage."},"pt":{"name":"Infeção disseminada grave por citomegalovirus em doentes sem imunodeficiência","synonyms":["Infeção disseminada grave por CMV em doentes sem imunodeficiência"],"definition":"Doença vírica rara caracterizada por uma infeção fulminante por citomegalovírus com envolvimento de múltiplos órgãos, incluindo cérebro, pulmão, fígado e/ou coração, entre outros, e sintomas constitucionais graves em pacientes imunocompetentes. A doença está associada a uma elevada taxa de mortalidade."},"pl":{"name":"Idiopatyczna rosiane zakażenie cytomegalowirusem","synonyms":["Ciężkie rozsiane zakażenie cytomegalowirusem u pacjentów immunokompetentnych"]},"cs":{"name":"Těžká diseminovaná cytomegalovirová infekce u imunokompetentních pacientů","synonyms":["Idiopatická diseminovaná CMV infekce","Idiopatická diseminovaná cytomegalovirová infekce"]},"uk":{"name":"Тяжка дисемінована цитомегаловірусна інфекція в імунокомпетентних пацієнтів","synonyms":["Тяжка дисемінована ЦМВ-інфекція в імунокомпетентних пацієнтів"],"definition":"A rare viral disease characterized by fulminant cytomegalovirus infection with multiple organ involvement including the brain, lung, liver, and/or heart, among others, and marked constitutional symptoms in immunocompetent patients. The condition is associated with a high case fatality rate."},"zh":{"name":"特发性播散性巨细胞病毒感染","synonyms":["特发性播散性巨细胞病毒感染"]},"tr":{"name":"İdiyopatik yaygın sitomegalovirüs enfeksiyonu","synonyms":["İdiyopatik yaygın CMV enfeksiyonu"]}},"xrefs":[{"source":"ICD-10","code":"B25.8","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"ICD-11","code":"1D82.Y","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"UMLS","code":"C5680152","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":68416,"name":"Rare infectious disease"},"parents":[{"orpha":163585,"name":"Rare viral disease","classifications":[203]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":["Not applicable"],"onset":["All ages"],"prevalence":[{"type":"Point prevalence","qualification":"Class only","class":"Unknown","value":0.0,"area":"Worldwide","status":"Not yet validated","source":"ORPHANET"}],"phenotypes":{"validation_status":"y","online":"y","validation_date":"2024-11-25","source":"18371229[PMID]_25390262[PMID]","terms":[{"hpo":"HP:0002583","label":"Colitis","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0033431","label":"Cytomegalovirus colitis","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0002027","label":"Abdominal pain","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0002039","label":"Anorexia","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0430087","label":"Anti-cytomegalovirus antibody positivity","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0001945","label":"Fever","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0012115","label":"Hepatitis","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0006515","label":"Interstitial pneumonitis","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0001287","label":"Meningitis","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0012486","label":"Myelitis","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0002017","label":"Nausea and vomiting","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0002090","label":"Pneumonia","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0032118","label":"Retinitis","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0000554","label":"Uveitis","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0033993","label":"Viral encephalitis","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0001824","label":"Weight loss","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0025143","label":"Chills","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0001289","label":"Confusion","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0033117","label":"Duodenitis","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0012378","label":"Fatigue","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0001878","label":"Hemolytic anemia","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0003326","label":"Myalgia","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0001733","label":"Pancreatitis","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0000979","label":"Purpura","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0000988","label":"Skin rash","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0012223","label":"Splenic rupture","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0001873","label":"Thrombocytopenia","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0004936","label":"Venous thrombosis","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0000572","label":"Visual loss","frequency":"Occasional (29-5%)","criteria":null}]}}