{"orpha":73260,"name":"Paracoccidioidomycosis","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":[],"definition":"A rare mycosis characterized by an acute form mostly occurring in children and young adults presenting with fever, weight loss, lymph node enlargement, hepatosplenomegaly, and bone marrow dysfunction, versus a chronic form which usually involves the lungs and mucosae of the upper respiratory tract, skin, lymph nodes, and adrenal glands, but may affect any part of the body. The most common sequelae are chronic respiratory insufficiency and Addison's disease. The infectious agent, <i>Paracoccidioides brasiliensis</i>, is a fungus limited to Latin America.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-73260/","orphanet_url":"https://www.orpha.net/en/disease/detail/73260","names":{"fr":{"name":"Paracoccidioïdomycose","definition":"Mycose rare caractérisée par une forme aiguë survenant principalement chez les enfants et les jeunes adultes. Elle se caractérise par de la fièvre, une perte de poids, une hypertrophie des ganglions lymphatiques, une hépatosplénomégalie et un dysfonctionnement de la moelle osseuse, et une forme chronique qui touche généralement les poumons et les muqueuses des voies respiratoires supérieures, la peau, les ganglions lymphatiques et les glandes surrénales, mais qui peut affecter n'importe quelle partie de l'organisme. Les séquelles les plus courantes sont l'insuffisance respiratoire chronique et la maladie d'Addison. L'agent infectieux, <i>Paracoccidioides brasiliensis</i>, est un champignon dont la répartition géographique est limitée à l'Amérique latine."},"de":{"name":"Parakokzidioidomykose","definition":"Eine seltene Pilzerkrankung, die sich durch eine akute Form auszeichnet, die meist bei Kindern und jungen Erwachsenen auftritt und mit Fieber, Gewichtsverlust, Lymphknotenvergrößerung, Hepatosplenomegalie und Knochenmarkstörungen einhergeht, und eine chronische Form, die in der Regel die Lunge und die Schleimhäute der oberen Atemwege, die Haut, die Lymphknoten und die Nebennieren befällt, aber jeden Teil des Körpers befallen kann. Die häufigsten Folgen sind chronische respiratorische Insuffizienz und die Addisonsche Krankheit. Der Infektionserreger, <i>Paracoccidioides brasiliensis</i>, ist ein auf Lateinamerika beschränkter Pilz."},"es":{"name":"Paracoccidiomicosis","definition":"Es una micosis poco frecuente caracterizada por una forma aguda que aparece principalmente en niños y adultos jóvenes, presentándose con fiebre, pérdida de peso, agrandamiento de los ganglios linfáticos, hepatoesplenomegalia y disfunción de la médula ósea, frente a una forma crónica que suele afectar a los pulmones y a las mucosas del tracto respiratorio superior, la piel, los ganglios linfáticos y las glándulas suprarrenales, aunque puede afectar a cualquier parte del cuerpo. Las secuelas más frecuentes son la insuficiencia respiratoria crónica y la enfermedad de Addison. El agente infeccioso, <i>Paracoccidioides brasiliensis</i>, es un hongo endémico de América Latina."},"it":{"name":"Paracoccidioidomicosi","definition":"Si tratta di una micosi rara, che può essere acuta (soprattutto nei bambini e nei giovani adulti; si manifesta con febbre, perdita di peso, ingrossamento dei linfonodi, epatosplenomegalia e disfunzione del midollo osseo), oppure cronica (di solito interessa i polmoni e le mucose delle vie aeree superiori, la cute, i linfonodi, le ghiandole surrenali, ma può interessare tutte le parti del corpo). Le sequele più comuni sono l'insufficienza respiratoria cronica e la malattia di Addison. L'agente infettivo, il Paracoccidioides brasiliensis, è un fungo presente solo in America Latina."},"nl":{"name":"Paracoccidioïdomycose","definition":"Een zeldzame mycose, gekarakteriseerd door een acute vorm die vooral voorkomt bij kinderen en jonge volwassenen en zich presenteert met koorts, gewichtsverlies, vergroting van lymfeklieren, hepatosplenomegalie, en disfunctie van beenmerg, alsook een chronische vorm die elke regio van het lichaam kan aantasten maar meestal betrokkenheid van long en slijmvlies van bovenste luchtwegen, huid, lymfeklieren, en bijnier vertoont. De meest gangbare restverschijnselen zijn chronische respiratoire insufficiëntie en ziekte van Addison. Het infectieuze agens, <i>Paracoccidioides brasiliensis</i>, is een schimmel die enkel in Latijns-Amerika voorkomt."},"pt":{"name":"Paracoccidioidomicose"},"pl":{"name":"Parakokcydioidomikoza"},"cs":{"name":"Paracokcidioidomykóza"},"uk":{"name":"Паракокцидіоїдомікоз","definition":"A rare mycosis characterized by an acute form mostly occurring in children and young adults presenting with fever, weight loss, lymph node enlargement, hepatosplenomegaly, and bone marrow dysfunction, versus a chronic form which usually involves the lungs and mucosae of the upper respiratory tract, skin, lymph nodes, and adrenal glands, but may affect any part of the body. The most common sequelae are chronic respiratory insufficiency and Addison's disease. The infectious agent, <i>Paracoccidioides brasiliensis</i>, is a fungus limited to Latin America."},"zh":{"name":"副球孢子菌病"},"tr":{"name":"Parakoksidioidomikoz"}},"xrefs":[{"source":"ICD-10","code":"B41.0","qualifier":"BTNT","icd_relation":"specific","validated":true},{"source":"ICD-10","code":"B41.7","qualifier":"BTNT","icd_relation":"specific","validated":true},{"source":"ICD-10","code":"B41.8","qualifier":"BTNT","icd_relation":"specific","validated":true},{"source":"ICD-10","code":"B41.9","qualifier":"BTNT","icd_relation":"specific","validated":true},{"source":"ICD-11","code":"1F2E","qualifier":"E","icd_relation":"specific","validated":true,"icd11_uri":"268777657"},{"source":"ICD-11","code":"1F2E.0","qualifier":"BTNT","icd_relation":"specific","validated":true,"icd11_uri":"1220568868"},{"source":"ICD-11","code":"1F2E.1","qualifier":"BTNT","icd_relation":"specific","validated":true,"icd11_uri":"992933048"},{"source":"ICD-11","code":"1F2E.Y","qualifier":"BTNT","icd_relation":"attributed","validated":true},{"source":"MONDO","code":"0005894","qualifier":"E","icd_relation":null,"validated":true},{"source":"MeSH","code":"D010229","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C0030409","qualifier":"E","icd_relation":null,"validated":true},{"source":"GARD","code":"7323","qualifier":"E","icd_relation":null,"validated":true},{"source":"MedDRA","code":"10061906","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":null}