{"orpha":1164,"name":"Allergic bronchopulmonary aspergillosis","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":["ABPA","Allergic aspergillosis","Hinson-Pepys disease"],"definition":"A rare immunologic pulmonary disorder caused by hypersensitivity to <i>Aspergillus fumigatus</i>, clinically manifesting with poorly controlled asthma and recurrent pulmonary infiltrates.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-1164/","orphanet_url":"https://www.orpha.net/en/disease/detail/1164","names":{"fr":{"name":"Aspergillose broncho-pulmonaire allergique","synonyms":["Maladie de Hinson-Pepys","ABPA","Aspergillose allergique"],"definition":"L'aspergillose broncho-pulmonaire allergique (ABPA) est une maladie pulmonaire rare causée par une hypersensibilité à l'<i>Aspergillus fumigatus</i> dont les signes principaux sont un asthme mal contrôlé et des infiltrats pulmonaires récidivants."},"de":{"name":"Aspergillose, allergische bronchopulmonale","synonyms":["ABPA","Hinson-Pepys-Krankheit","Lungenaspergillose, allergische"],"definition":"Die Allergische bronchiopulmonale Aspergillose (ABPA) ist eine seltene, immunbedingte Lungenerkrankung, verursacht durch Hypersensitivität gegenüber Aspergillus fumigatus. Sie äußert sich klinisch mit schlecht therapierbarem Asthma und rezidivierenden Lungeninfiltraten."},"es":{"name":"Aspergilosis bronco-pulmonar alérgica","synonyms":["ABPA","Aspergilosis alérgica","Enfermedad de Hinson-Pepys"],"definition":"Es un trastorno pulmonar poco frecuente de origen inmunológico mediado por una reacción de hipersensibilidad al hongo Aspegillus fumigatus, que se manifiesta clínicamente por un asma mal controlado e infiltrados pulmonares recurrentes."},"it":{"name":"Aspergillosi broncopolmonare allergica","synonyms":["ABPA","Aspergillosi allergica","Malattia di Hinson-Pepsy"],"definition":"L'aspergillosi bronco-polmonare allergica (ABPA) è una pneumopatia immunologica rara causata dall'ipersensibilità all'<i>Aspergillus fumigatus</i>, che si manifesta, clinicamente, con asma difficilmente controllabile e infiltrati polmonari ricorrenti."},"nl":{"name":"Allergische bronchopulmonale aspergillose","synonyms":["ABPA","Allergische aspergillose","Ziekte van Hinson-Pepys"],"definition":"Een zeldzame immunologische pulmonale aandoening veroorzaakt door overgevoeligheid voor <i>Aspergillus fumigatus</i>, die zich klinisch manifesteert met moeilijk te behandelen astma en terugkerende longinfiltraten."},"pt":{"name":"Aspergilose broncopulmonar alérgica","synonyms":["Doença de Hinson-Pepys"],"definition":"A aspergilose broncopulmonar alérgica (ABPA) é uma doença pulmonar imunológica rara causada por hipersensibilidade a <i> Aspergillus fumigatus </ i>, manifestando-se clinicamente com asma de difícil controlo e infiltrados pulmonares recorrentes."},"pl":{"name":"Alergiczna aspegiloza oskrzelowo-płucna","synonyms":["ABPA","Aspergiloza alergiczna","Choroba Hinsona i Pepysa"]},"cs":{"name":"Alergická bronchopulmonální aspergilóza","synonyms":["ABPA","Alergická aspergilóza","Hinsonova-Pepysova nemoc","Hinson-Pepysova nemoc"]},"uk":{"name":"Алергічний бронхолегеневий аспергільоз","synonyms":["ABPA","Алергічний аспергільоз","Хвороба Хінсона-Пепіса"],"definition":"A rare immunologic pulmonary disorder caused by hypersensitivity to <i>Aspergillus fumigatus</i>, clinically manifesting with poorly controlled asthma and recurrent pulmonary infiltrates."},"zh":{"name":"变应性支气管肺曲霉菌病","synonyms":["变应性曲霉菌病"]},"tr":{"name":"Alerjik bronkopulmoner aspergilloz","synonyms":["ABPA","Hinson-Pepys hastalığı","Alerjik aspergilloz"]}},"xrefs":[{"source":"OMIM","code":"103920","qualifier":"BTNT","icd_relation":null,"validated":true},{"source":"ICD-10","code":"B44.1+","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"ICD-10","code":"J99.8*","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"ICD-11","code":"CA82.4","qualifier":"NTBT","icd_relation":"index","validated":true,"icd11_uri":"1591607082"},{"source":"MONDO","code":"0015243","qualifier":"E","icd_relation":null,"validated":true},{"source":"MeSH","code":"D001229","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C0004031","qualifier":"E","icd_relation":null,"validated":true},{"source":"GARD","code":"602","qualifier":"E","icd_relation":null,"validated":true},{"source":"MedDRA","code":"10006474","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":97955,"name":"Rare respiratory disease"},"parents":[{"orpha":98052,"name":"Rare allergic respiratory disease","classifications":[212]},{"orpha":101944,"name":"Rare pulmonary disease","classifications":[184]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":["Not applicable"],"onset":["Childhood"],"prevalence":[{"type":"Point prevalence","qualification":"Class only","class":"Unknown","value":0.0,"area":"Worldwide","status":"Validated","source":"ORPHANET"}],"phenotypes":{"validation_status":"y","online":"y","validation_date":"2016-06-01","source":null,"terms":[{"hpo":"HP:0001879","label":"Abnormality of eosinophils","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0002715","label":"Abnormality of the immune system","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0002099","label":"Asthma","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0012735","label":"Cough","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0002110","label":"Bronchiectasis","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0002120","label":"Cerebral cortical atrophy","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0011134","label":"Low-grade fever","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0001824","label":"Weight loss","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0025426","label":"Abnormal bronchus morphology","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0001231","label":"Abnormal fingernail morphology","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0002097","label":"Emphysema","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0002105","label":"Hemoptysis","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0002092","label":"Pulmonary arterial hypertension","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0002093","label":"Respiratory insufficiency","frequency":"Occasional (29-5%)","criteria":null}]}}