{"orpha":171700,"name":"Diffuse panbronchiolitis","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":[],"definition":"Diffuse panbronchiolitis is a rare chronic inflammatory obstructive pulmonary disease primarily affecting the respiratory bronchioles throughout both lungs and inducing sinobronchial infection. Onset occurs in the second to fifth decade of life and manifests by chronic cough, exertional dyspnea, and sputum production. Most patients also have chronic paranasal sinusitis","note":null,"url":"https://raremap.alftech.space/d/ORPHA-171700/","orphanet_url":"https://www.orpha.net/en/disease/detail/171700","names":{"fr":{"name":"Panbronchiolite diffuse","definition":"La panbronchiolite diffuse est une pneumopathie inflammatoire chronique obstructive rare affectant en premier lieu les bronchioles des deux poumons, induisant une infection sinobronchique. La maladie commence à se manifester entre la deuxième et la cinquième décennie de vie par une toux chronique productive et une dyspnée d'effort. La plupart des patients ont aussi une sinusite paranasale chronique."},"de":{"name":"Panbronchiolitis, diffuse","definition":"Die Diffuse Panbronchiolitis ist eine seltene chronisch-entzündlich-obstruktive Lungenerkrankung. Primär betroffen sind die Bronchiolen beider Lungenflügel. Die Krankheit beginnt im zweiten bis fünften Lebensjahrzehnt mit chronischem Husten, Belastungsdyspnoe und Auswurf. Bei den meisten Patienten besteht auch eine chronische Entzündung der Nasennebenhöhlen."},"es":{"name":"Panbronquiliolitis difusa","definition":"La panbronquiolitis difusa es una enfermedad pulmonar inflamatoria obstructiva crónica que afecta principalmente a los bronquiolos respiratorios a través de ambos pulmones e induce a infecciones sinobronquiales. Su aparición se produce de la segunda a la quinta década de vida y se manifiesta mediante tos crónica, disnea de esfuerzo, y producción de esputo. La mayoría de los pacientes también presentan sinusitis paranasal crónica."},"it":{"name":"Panbronchiolite diffusa","definition":"La panbronchiolite diffusa è una rara malattia polmonare ostruttiva infiammatoria cronica, che interessa primariamente i bronchioli di entrambi i polmoni, provocando un'infezione sinobronchiale. La malattia esordisce nella seconda-quinta decade di vita e si presenta con tosse cronica, dispnea da sforzo e produzione di espettorato. La maggior parte dei pazienti presenta anche una sinusite paranasale cronica."},"nl":{"name":"Diffuse panbronchiolitis","definition":"Een zeldzame chronische inflammatoire obstructieve aandoening van long, die hoofdzakelijk respiratoire bronchioli in beide longen aantast, en sinobronchiale infectie induceert. De aandoening treedt op in het tweede tot vijfde levensdecennium, en manifesteert zich met chronische hoest, inspanningsdyspneu, en productie van sputum. De meeste patiënten lijden ook aan chronische paranasale sinusitis."},"pt":{"name":"Panbronquiolite difusa"},"pl":{"name":"Rozlane zapalenie oskrzelików"},"cs":{"name":"Difuzní panbronchiolitida"},"uk":{"name":"Дифузний панбронхіоліт","definition":"Diffuse panbronchiolitis is a rare chronic inflammatory obstructive pulmonary disease primarily affecting the respiratory bronchioles throughout both lungs and inducing sinobronchial infection. Onset occurs in the second to fifth decade of life and manifests by chronic cough, exertional dyspnea, and sputum production. Most patients also have chronic paranasal sinusitis"},"zh":{"name":"弥漫性全细支气管炎","definition":"弥漫性泛细支气管炎是一种罕见的慢性炎性阻塞性肺病，主要累及整个双肺的呼吸性细支气管，并诱发鼻窦支气管感染。主要临床表现为慢性咳嗽、劳累性呼吸困难和多痰，大部分患者伴有慢性副鼻窦炎，发病年龄多在20-50岁。"},"tr":{"name":"Yaygın panbronşiolit"}},"xrefs":[{"source":"OMIM","code":"604809","qualifier":"E","icd_relation":null,"validated":true},{"source":"ICD-10","code":"J44.8","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"ICD-11","code":"CA26.1","qualifier":"E","icd_relation":"specific","validated":true,"icd11_uri":"291357751"},{"source":"MONDO","code":"0011490","qualifier":"E","icd_relation":null,"validated":true},{"source":"MeSH","code":"C536174","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C0878555","qualifier":"E","icd_relation":null,"validated":true},{"source":"GARD","code":"8526","qualifier":"E","icd_relation":null,"validated":true},{"source":"MedDRA","code":"10062952","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":97955,"name":"Rare respiratory disease"},"parents":[{"orpha":101944,"name":"Rare pulmonary disease","classifications":[184]}],"children":[],"genes":[{"symbol":"MUC5B","name":"mucin 5B, oligomeric mucus/gel-forming","gene_type":"gene with protein product","association":"Major susceptibility factor in","status":"Not yet assessed","source":null,"hgnc":"7516","ensembl":"ENSG00000117983","omim":"600770","loci":["11p15.5"]},{"symbol":"MUCL3","name":"mucin like 3","gene_type":"gene with protein product","association":"Major susceptibility factor in","status":"Not yet assessed","source":null,"hgnc":"21666","ensembl":"ENSG00000168631","omim":"613928","loci":["6p21.33"]}],"genes_via_subtypes":[],"inheritance":["Multigenic/multifactorial"],"onset":["Adult"],"prevalence":[{"type":"Point prevalence","qualification":"Class only","class":"<1 / 1 000 000","value":0.0,"area":"Europe","status":"Not yet validated","source":"ORPHANET"}],"phenotypes":null}