{"orpha":70589,"name":"Bronchopulmonary dysplasia","type":"Malformation syndrome","group":"Disorder","status":"active","flags":[],"synonyms":["BPD"],"definition":"Bronchopulmonary dysplasia is a chronic respiratory disease that results from complications related to lung injury during the treatment of infant acute respiratory distress syndrome in low-birth-weight premature infants or from abnormal lung development in older infants. Clinical signs are tachypnea, tachycardia and signs of respiratory distress such as intercostal recession, grunting and nasal flaring.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-70589/","orphanet_url":"https://www.orpha.net/en/disease/detail/70589","names":{"fr":{"name":"Dysplasie broncho-pulmonaire","definition":"La dysplasie broncho-pulmonaire est une maladie respiratoire chronique secondaire au traitement du syndrome de détresse respiratoire aigüe de l'enfant à faible poids, ou d'un développement pulmonaire anormal chez des nourrissons plus âgés. Les signes cliniques sont une tachypnée, une tachycardie et des signes de détresse respiratoire tels qu'une rétraction intercostale, un geignement expiratoire et des battements des ailes nasales."},"de":{"name":"Dysplasie, bronchopulmonale","synonyms":["BPD"],"definition":"Die Bronchopulmonale Dysplasie ist eine chronische Atemwegserkrankung, die entweder bei Frühgeborenen als Komplikation (Schädigung der Lunge) während der Behandlung des Atemnotsyndroms oder bei älteren Kindern als Folge einer abnormen Entwicklung der Lunge auftritt. Klinische Zeichen sind Tachypnoe, Tachykardie und Zeichen von Atemnot wie interkostale Einziehungen, expiratorisches Stöhnen und Nasenflügeln."},"es":{"name":"Displasia broncopulmonar","synonyms":["DBP"],"definition":"La displasia broncopulmonar es una enfermedad respiratoria crónica que resulta de las complicaciones relacionadas con lesiones pulmonares durante el tratamiento del síndrome de distrés respiratorio en niños prematuros con bajo peso al nacer o del desarrollo anormal del pulmón en niños mayores. Los signos clínicos son taquipnea, taquicardia y signos de distrés respiratorio tales como recesión intercostal, gruñidos y aleteo nasal."},"it":{"name":"Displasia broncopolmonare","synonyms":["BPD"],"definition":"La displasia broncopolmonare è una malattia respiratoria cronica che origina dalle complicazioni associate alle lesioni respiratorie presenti nei neonati prematuri, con basso peso alla nascita, durante il trattamento della sindrome da distress respiratorio, o dallo sviluppo anomalo dei polmoni nei neonati più grandi. I segni clinici sono la tachipnea, la tachicardia, il distress respiratorio, la recessione intercostale, il grugnito e la distensione delle narici."},"nl":{"name":"Bronchopulmonale dysplasie","synonyms":["BPD"],"definition":"Een chronische respiratoire aandoening, ten gevolge van complicaties gerelateerd aan beschadiging van long tijdens behandeling van infantiele acute ademnood-syndroom bij premature zuigelingen met laag geboortegewicht, of van abnormale ontwikkeling van long bij oudere zuigelingen. Klinische verschijnselen zijn tachypneu, tachycardie en verschijnselen van ademnood zoals intercostale recessie, kreunende ademhaling, en opengesperde neusgaten."},"pt":{"name":"Displasia broncopulmonar","synonyms":["DBP"]},"pl":{"name":"Dysplazja oskrzelowo-płucna","synonyms":["BPD"]},"cs":{"name":"Bronchopulmonální dysplázie","synonyms":["BPD"]},"uk":{"name":"Бронхолегенева дисплазія","synonyms":["BPD"],"definition":"Bronchopulmonary dysplasia is a chronic respiratory disease that results from complications related to lung injury during the treatment of infant acute respiratory distress syndrome (see these terms) in low-birth-weight premature infants or from abnormal lung development in older infants. Clinical signs are tachypnea, tachycardia and signs of respiratory distress such as intercostal recession, grunting and nasal flaring."},"zh":{"name":"支气管肺发育不良","synonyms":["BPD"]},"tr":{"name":"Bronkopulmoner displazi","synonyms":["BPD"]}},"xrefs":[{"source":"ICD-10","code":"P27.1","qualifier":"E","icd_relation":"specific","validated":true},{"source":"ICD-11","code":"KB29.0","qualifier":"E","icd_relation":"specific","validated":true,"icd11_uri":"1462855296"},{"source":"MONDO","code":"0019091","qualifier":"E","icd_relation":null,"validated":true},{"source":"MeSH","code":"D001997","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C0006287","qualifier":"E","icd_relation":null,"validated":true},{"source":"GARD","code":"5962","qualifier":"E","icd_relation":null,"validated":true},{"source":"MedDRA","code":"10006475","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":97955,"name":"Rare respiratory disease"},"parents":[{"orpha":108993,"name":"Non-syndromic respiratory or mediastinal malformation","classifications":[147,186]},{"orpha":182111,"name":"Respiratory malformation","classifications":[184]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":["Not applicable"],"onset":["Infancy","Neonatal"],"prevalence":[{"type":"Point prevalence","qualification":"Value and class","class":"1-5 / 10 000","value":13.0,"area":"Europe","status":"Validated","source":"European Medicines Agency[OTHER]"}],"phenotypes":{"validation_status":"y","online":"y","validation_date":"2024-02-01","source":"26593076[PMID]_28067830[PMID]_28090255[PMID]_27806990[PMID]_26593086[PMID]_34670756[PMID]_32854293[PMID]","terms":[{"hpo":"HP:0002088","label":"Abnormal lung morphology","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0006528","label":"Chronic lung disease","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0012735","label":"Cough","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0002094","label":"Dyspnea","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0002097","label":"Emphysema","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0012419","label":"Hyperoxemia","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0001622","label":"Premature birth","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0002098","label":"Respiratory distress","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0004887","label":"Respiratory failure requiring assisted ventilation","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0001518","label":"Small for gestational age","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0012252","label":"Abnormal respiratory system morphology","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0002795","label":"Abnormal respiratory system physiology","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0006536","label":"Airway obstruction","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0002780","label":"Bronchomalacia","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0002871","label":"Central apnea","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0006597","label":"Diaphragmatic paralysis","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0004890","label":"Elevated pulmonary artery pressure","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0001708","label":"Right ventricular failure","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0001667","label":"Right ventricular hypertrophy","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0002360","label":"Sleep abnormality","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0001607","label":"Subglottic stenosis","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0030828","label":"Wheezing","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0030878","label":"Abnormality on pulmonary function testing","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0100750","label":"Atelectasis","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0003546","label":"Exercise intolerance","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0100632","label":"Pulmonary sequestration","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0002786","label":"Tracheobronchomalacia","frequency":"Occasional (29-5%)","criteria":null}]}}