{"orpha":1303,"name":"Bronchiolitis obliterans","type":"Clinical group","group":"Group of disorders","status":"active","flags":[],"synonyms":["Constrictive bronchiolitis","Obliterative bronchiolitis"],"definition":"A rare lung disorder that is mainly associated with chronic allograft dysfunction after lung transplantation and that is characterized by inflammation and fibrosis of bronchiolar walls that reduce the diameter of the bronchioles and result in progressive and irreversible airflow obstruction.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-1303/","orphanet_url":"https://www.orpha.net/en/disease/detail/1303","names":{"fr":{"name":"Bronchiolite oblitérante","synonyms":["Bronchiolite constrictive","Bronchiolite obstructive"],"definition":"Une maladie pulmonaire surtout associée à un dysfonction chronique de l'allogreffe après une transplantation pulmonaire. Elle se caractérise par une inflammation et une fibrose des parois des bronchioles, ce qui réduit leur diamètre et conduit à une obstruction progressive et irréversible des voies aériennes."},"de":{"name":"Bronchiolitis obliterans","synonyms":["Bronchiolitis, konstriktive","Bronchiolitis, obliterative"],"definition":"Eine seltene Lungenerkrankung, die hauptsächlich mit einer chronischen Transplantatdysfunktion nach einer Lungentransplantation in Verbindung gebracht wird und durch eine Entzündung und Fibrose der Bronchiolenwände gekennzeichnet ist, die den Durchmesser der Bronchiolen verringert und zu einer fortschreitenden und irreversiblen Obstruktion der Atemwege führt."},"es":{"name":"Bronquiolitis obliterante","synonyms":["Bronquiolitis constrictiva"],"definition":"Es una enfermedad pulmonar poco frecuente mayormente asociada a disfunción crónica del aloinjerto después de un trasplante de pulmón y que está caracterizada por inflamación y fibrosis de las paredes bronquiolares con reducción del diámetro de los bronquiolos, resultando en obstrucción progresiva e irreversible del flujo aéreo."},"it":{"name":"Bronchiolite obliterante","synonyms":["Bronchiolite costrittiva","Bronchiolite obliterativa"]},"nl":{"name":"Bronchiolitis obliterans","synonyms":["Constrictieve bronchiolitis","Obliteratieve bronchiolitis"],"definition":"Een zeldzame aandoening van long, voornamelijk geassocieerd met chronische disfunctie van allotransplantaat na longtransplantatie, en gekenmerkt door inflammatie en fibrose van bronchiolaire wanden, die de diameter van bronchioli verkleinen en resulteren in progressieve en onomkeerbare obstructie van de luchtstroom."},"pt":{"name":"Bronquiolite obliterante","synonyms":["Bronquiolite constritiva","Bronquiolite obliterativa"]},"pl":{"name":"Zarostowe zapalenie oskrzelików z obturacyjną chorobą płuc","synonyms":["BOOP","Zaciskające zapalenie oskrzelików","Zarostowe zapalenie oskrzelików"],"definition":"Rzadka choroba płuc związana głównie z przewlekłą dysfunkcją alloprzeszczepu po przeszczepie płuc, która charakteryzuje się stanem zapalnym i zwłóknieniem ścian oskrzelików, powodującym zmniejszenie średnicy oskrzelików i prowadzącym do postępującej i nieodwracalnej blokady przepływu powietrza."},"cs":{"name":"Obliterující bronchiolitida s obstrukční plicní nemocí","synonyms":["BOOP","Konstriktivní bronchiolitida","Obliterující bronchiolitida"]},"uk":{"name":"Облітеруючий бронхіоліт","synonyms":["Констриктивний бронхіоліт","Облітеруючий бронхіоліт"],"definition":"A rare lung disorder that is mainly associated with chronic allograft dysfunction after lung transplantation and that is characterized by inflammation and fibrosis of bronchiolar walls that reduce the diameter of the bronchioles and result in progressive and irreversible airflow obstruction."},"zh":{"name":"闭塞性细支气管炎伴阻塞性肺疾病","synonyms":["闭塞性细支气管炎"]},"tr":{"name":"Obstrüktif akciğer hastalığı ile seyreden bronşiyolit obliterans","synonyms":["Konstriktif bronşiolit","Obliteratif bronşiolit"]}},"xrefs":[{"source":"ICD-10","code":"J44.8","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"ICD-11","code":"CA26.0","qualifier":"E","icd_relation":"specific","validated":true,"icd11_uri":"592711730"},{"source":"MONDO","code":"15265","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C0006272","qualifier":"E","icd_relation":null,"validated":true},{"source":"GARD","code":"5961","qualifier":"E","icd_relation":null,"validated":true},{"source":"MedDRA","code":"10029888","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":97955,"name":"Rare respiratory disease"},"parents":[{"orpha":506222,"name":"Rare disorder potentially indicated for lung transplant","classifications":[233]},{"orpha":101944,"name":"Rare pulmonary disease","classifications":[184]}],"children":[{"orpha":658612,"name":"Non-transplant-related bronchiolitis obliterans","type":"Disease"},{"orpha":658602,"name":"Transplant-related bronchiolitis obliterans","type":"Disease"}],"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":"2021-08-26","source":"30038355[PMID]_24806161[PMID]_26335918[PMID]","terms":[{"hpo":"HP:0006536","label":"Airway obstruction","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0011946","label":"Bronchiolitis obliterans","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0012735","label":"Cough","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0032359","label":"Decreased forced expiratory flow 25-75%","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0002094","label":"Dyspnea","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0012418","label":"Hypoxemia","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0030877","label":"Reduced FEV1/FVC ratio","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0032342","label":"Reduced forced expiratory volume in one second","frequency":"Very frequent (99-80%)","criteria":"Diagnostic criterion"},{"hpo":"HP:0032341","label":"Reduced forced vital capacity","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0045051","label":"Decreased DLCO","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0031246","label":"Nonproductive cough","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0002090","label":"Pneumonia","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0011947","label":"Respiratory tract infection","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0002960","label":"Autoimmunity","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0002110","label":"Bronchiectasis","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0025179","label":"Ground-glass opacification on pulmonary HRCT","frequency":"Very rare (<4-1%)","criteria":null}]}}