{"orpha":73263,"name":"Zygomycosis","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":["Mucormycosis"],"definition":"A rare mycosis caused by ubiquitous, opportunistic fungi of the order Mucorales, characterized by tissue infarction and necrosis due to invasion of the vasculature by hyphae. The spectrum of clinical manifestations depends on the route of infection and includes rhinocerebral, pulmonary, cutaneous, gastrointestinal, renal, and disseminated forms. The disease is usually rapidly progressive and associated with high mortality.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-73263/","orphanet_url":"https://www.orpha.net/en/disease/detail/73263","names":{"fr":{"name":"Zygomycose","synonyms":["Mucormycose"],"definition":"Mycose rare causée par des champignons opportunistes ubiquistes de l'ordre des Mucorales, caractérisée par un infarctus et une nécrose des tissus dus à l'invasion du système vasculaire par les hyphes. Le spectre des signes cliniques dépend de la voie de transmission et inclue des formes rhinocérébrales, pulmonaires, cutanées, gastro-intestinales, rénales et disséminées. La maladie est généralement rapidement progressive et associée à une létalité élevée."},"de":{"name":"Zygomykose","synonyms":["Mukormykose"],"definition":"Eine seltene Mykose, die durch ubiquitäre, opportunistische Pilze der Ordnung Mucorales verursacht wird und durch Gewebsinfarkte und -nekrosen infolge der Invasion von Hyphen in das Gefäßsystem gekennzeichnet ist. Das Spektrum der klinischen Manifestationen hängt vom Infektionsweg ab und umfasst rhinozerebrale, pulmonale, kutane, gastrointestinale, renale und disseminierte Formen. Die Krankheit schreitet in der Regel rasch voran und ist mit einer hohen Sterblichkeit verbunden."},"es":{"name":"Zigomicosis","synonyms":["Mucormicosis"],"definition":"Es una micosis poco frecuente causada por hongos oportunistas, ubicuos del orden Mucorales, caracterizada por infarto y necrosis tisular debido a la invasión vascular por las hifas. El espectro de las manifestaciones clínicas depende de la vía de infección, incluyendo la forma rinocerebral, la pulmonar, la cutánea, la gastrointestinal, la renal y la diseminada. La enfermedad suele progresar rápidamente, asociándose a una elevada mortalidad."},"it":{"name":"Zigomicosi","synonyms":["Mucormicosi"],"definition":"Si tratta di una micosi rara, causata dai funghi opportunistici ubiquitari dell'ordine dei Mucorales. È caratterizzata dall'infarto e dalla necrosi dei tessuti secondarie all'invasione del sistema vascolare da parte delle ife. Il quadro clinico dipende dalla via di infezione e comprende la forma rinocerebrale, polmonare, cutanea, gastrointestinale, renale e disseminata. In genere la progressione è rapida e la malattia si associa a una mortalità elevata."},"nl":{"name":"Zygomycose","synonyms":["Mucormycose"],"definition":"Een zeldzame mycose, veroorzaakt door alomtegenwoordige, opportunistische schimmels van de orde Mucorales, en gekarakteriseerd door infarct en necrose van weefsel als gevolg van invasie van de vasculatuur door hyfen. Het spectrum van klinische manifestaties hangt af van de route van infectie en omvat rinocerebrale, pulmonale, cutane, gastro-intestinale, renale, en gedissemineerde vormen. De ziekte toont doorgaans snelle progressie en is geassocieerd met hoge mortaliteit."},"pt":{"name":"Zigomicose","synonyms":["Mucormicose"]},"pl":{"name":"Mukormykoza","synonyms":["Mukormikoza"]},"cs":{"name":"Zygomykóza","synonyms":["Mukormykóza"]},"uk":{"name":"Зигомікоз","synonyms":["Мукормікоз"],"definition":"A rare mycosis caused by ubiquitous, opportunistic fungi of the order Mucorales, characterized by tissue infarction and necrosis due to invasion of the vasculature by hyphae. The spectrum of clinical manifestations depends on the route of infection and includes rhinocerebral, pulmonary, cutaneous, gastrointestinal, renal, and disseminated forms. The disease is usually rapidly progressive and associated with high mortality."},"zh":{"name":"接合菌病","synonyms":["毛霉菌病"]},"tr":{"name":"Zigomikoz","synonyms":["Mukormikoz"]}},"xrefs":[{"source":"ICD-10","code":"B46.0","qualifier":"BTNT","icd_relation":"specific","validated":true},{"source":"ICD-10","code":"B46.1","qualifier":"BTNT","icd_relation":"specific","validated":true},{"source":"ICD-10","code":"B46.2","qualifier":"BTNT","icd_relation":"specific","validated":true},{"source":"ICD-10","code":"B46.3","qualifier":"BTNT","icd_relation":"specific","validated":true},{"source":"ICD-10","code":"B46.4","qualifier":"BTNT","icd_relation":"specific","validated":true},{"source":"ICD-10","code":"B46.5","qualifier":"BTNT","icd_relation":"specific","validated":true},{"source":"ICD-10","code":"B46.8","qualifier":"BTNT","icd_relation":"specific","validated":true},{"source":"ICD-10","code":"B46.9","qualifier":"BTNT","icd_relation":"specific","validated":true},{"source":"ICD-11","code":"1F2C","qualifier":"E","icd_relation":"specific","validated":true,"icd11_uri":"1676389165"},{"source":"MONDO","code":"0019136","qualifier":"E","icd_relation":null,"validated":true},{"source":"MeSH","code":"D020096","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C0043541","qualifier":"E","icd_relation":null,"validated":true},{"source":"GARD","code":"10224","qualifier":"E","icd_relation":null,"validated":true},{"source":"MedDRA","code":"10061418","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":68416,"name":"Rare infectious disease"},"parents":[{"orpha":163591,"name":"Rare mycosis","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":"2020-04-21","source":"17848885[PMID]_19754758[PMID]_16568297[PMID]","terms":[{"hpo":"HP:0012735","label":"Cough","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0012378","label":"Fatigue","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0001945","label":"Fever","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0012531","label":"Pain","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0000246","label":"Sinusitis","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0032162","label":"Unusual skin infection","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0002027","label":"Abdominal pain","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0008066","label":"Abnormal blistering of the skin","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0001291","label":"Abnormal cranial nerve morphology","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0045026","label":"Abnormal mediastinum morphology","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0011949","label":"Acute infectious pneumonia","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0032172","label":"Air crescent sign","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0100750","label":"Atelectasis","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0000622","label":"Blurred vision","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0100658","label":"Cellulitis","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0100749","label":"Chest pain","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0002583","label":"Colitis","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0032674","label":"Cutaneous wound","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0001875","label":"Decreased total neutrophil count","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0000819","label":"Diabetes mellitus","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0002014","label":"Diarrhea","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0004387","label":"Enterocolitis","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0100537","label":"Fasciitis","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0005263","label":"Gastritis","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0002315","label":"Headache","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0002248","label":"Hematemesis","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0004377","label":"Hematological neoplasm","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0002105","label":"Hemoptysis","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0032564","label":"Ileitis","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0002383","label":"Infectious encephalitis","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0001993","label":"Ketoacidosis","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0100721","label":"Mediastinal lymphadenopathy","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0001742","label":"Nasal congestion","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0002018","label":"Nausea","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0032177","label":"Parenchymal consolidation","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0100539","label":"Periorbital edema","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0000629","label":"Periorbital fullness","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0002202","label":"Pleural effusion","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0002107","label":"Pneumothorax","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0001622","label":"Premature birth","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0002113","label":"Pulmonary infiltrates","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0200039","label":"Pustule","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0031417","label":"Rhinorrhea","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0200035","label":"Skin plaque","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0032166","label":"Unusual gastrointestinal infection","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0000572","label":"Visual loss","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0002013","label":"Vomiting","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0004420","label":"Arterial thrombosis","frequency":"Very rare (<4-1%)","criteria":null},{"hpo":"HP:0030049","label":"Brain abscess","frequency":"Very rare (<4-1%)","criteria":null},{"hpo":"HP:0012375","label":"Chemosis","frequency":"Very rare (<4-1%)","criteria":null},{"hpo":"HP:0031369","label":"Colon perforation","frequency":"Very rare (<4-1%)","criteria":null},{"hpo":"HP:0004944","label":"Dilatation of the cerebral artery","frequency":"Very rare (<4-1%)","criteria":null},{"hpo":"HP:0000651","label":"Diplopia","frequency":"Very rare (<4-1%)","criteria":null},{"hpo":"HP:0100584","label":"Endocarditis","frequency":"Very rare (<4-1%)","criteria":null},{"hpo":"HP:0000421","label":"Epistaxis","frequency":"Very rare (<4-1%)","criteria":null},{"hpo":"HP:0000544","label":"External ophthalmoplegia","frequency":"Very rare (<4-1%)","criteria":null},{"hpo":"HP:0002239","label":"Gastrointestinal hemorrhage","frequency":"Very rare (<4-1%)","criteria":null},{"hpo":"HP:0002573","label":"Hematochezia","frequency":"Very rare (<4-1%)","criteria":null},{"hpo":"HP:0012115","label":"Hepatitis","frequency":"Very rare (<4-1%)","criteria":null},{"hpo":"HP:0020101","label":"Invasive fungal infection","frequency":"Very rare (<4-1%)","criteria":null},{"hpo":"HP:0007185","label":"Loss of consciousness","frequency":"Very rare (<4-1%)","criteria":null},{"hpo":"HP:0000265","label":"Mastoiditis","frequency":"Very rare (<4-1%)","criteria":null},{"hpo":"HP:0002249","label":"Melena","frequency":"Very rare (<4-1%)","criteria":null},{"hpo":"HP:0012819","label":"Myocarditis","frequency":"Very rare (<4-1%)","criteria":null},{"hpo":"HP:0000123","label":"Nephritis","frequency":"Very rare (<4-1%)","criteria":null},{"hpo":"HP:0002797","label":"Osteolysis","frequency":"Very rare (<4-1%)","criteria":null},{"hpo":"HP:0001733","label":"Pancreatitis","frequency":"Very rare (<4-1%)","criteria":null},{"hpo":"HP:0001701","label":"Pericarditis","frequency":"Very rare (<4-1%)","criteria":null},{"hpo":"HP:0002586","label":"Peritonitis","frequency":"Very rare (<4-1%)","criteria":null},{"hpo":"HP:0000520","label":"Proptosis","frequency":"Very rare (<4-1%)","criteria":null},{"hpo":"HP:0000508","label":"Ptosis","frequency":"Very rare (<4-1%)","criteria":null},{"hpo":"HP:0000083","label":"Renal insufficiency","frequency":"Very rare (<4-1%)","criteria":null},{"hpo":"HP:0025326","label":"Retinal arterial occlusion","frequency":"Very rare (<4-1%)","criteria":null},{"hpo":"HP:0000541","label":"Retinal detachment","frequency":"Very rare (<4-1%)","criteria":null},{"hpo":"HP:0025059","label":"Splenic abscess","frequency":"Very rare (<4-1%)","criteria":null},{"hpo":"HP:0004418","label":"Thrombophlebitis","frequency":"Very rare (<4-1%)","criteria":null}]}}