{"orpha":280847,"name":"Congenital pulmonary airway malformation type 3","type":"Clinical subtype","group":"Subtype of disorder","status":"active","flags":[],"synonyms":["CCAM type 3","CPAM type 3","Congenital cystic adenomatoid malformation of the lung type 3","Congenital cystic adenomatous malformation of the lung type 3","Congenital cystic disease of the lung type 3"],"definition":"A rare subtype of congenital pulmonary airway malformation characterized by a multicystic mass of non-functioning lung tissue consisting of numerous microcysts of less than 0.5 cm in diameter. The lesions have intracystic communications, can be connected to the tracheobronchial tree, and are usually unilateral, involving an entire lobe. The condition may be associated with polyhydramnios, fetal hydrops, and stillbirth, or present with severe respiratory distress in the neonatal period.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-280847/","orphanet_url":"https://www.orpha.net/en/disease/detail/280847","names":{"fr":{"name":"Malformation congénitale des voies aériennes pulmonaires type 3","synonyms":["CCAM type 3","CPAM type 3","MAPK type 3","MCVAP type 3","Maladie adénomatoïde kystique du poumon type 3","Malformation adénomatoïde kystique du poumon type 3"],"definition":"Sous-type rare de malformation congénitale des voies aériennes pulmonaires caractérisé par la présence d'une masse multikystique de tissu pulmonaire non fonctionnel, constituée de petits kystes de moins de 0,5 cm de diamètre. On observe des communications intrakystiques, parfois avec l'arbre trachéobronchique, généralement unilatérales et impliquant le lobe dans son ensemble. Elle est fréquemment associée à d'autres malformations congénitales graves, telles qu'une agénésie ou une dysgénésie rénale, une séquestration pulmonaire ou des malformations cardiaques."},"de":{"name":"Kongenitale Fehlbildung der Atemwege Typ 3","synonyms":["CCAM Typ 3","CPAM Typ 3","Kongenitale zystische Krankheit der Lunge Typ 3","Kongenitale zystische adenomatoide Fehlbildung der Lunge Typ 3"],"definition":"Ein seltener Subtyp einer angeborenen Fehlbildung der pulmonalen Atemwege, der durch eine multizystische Gewebeverdichtung von nicht funktionierendem Lungengewebe gekennzeichnet ist, die aus zahlreichen Mikrozysten mit einem Durchmesser von weniger als 0,5 cm besteht. Die Läsionen haben intrazystische Verbindungen, können mit dem Tracheobronchialbaum verbunden sein und sind in der Regel einseitig und betreffen einen ganzen Lappen. Die Erkrankung kann mit Polyhydramnion, fetalem Hydrops und Totgeburt einhergehen oder mit schwerer Atemnot in der Neugeborenenperiode einhergehen."},"es":{"name":"Malformación congénita de las vías aéreas pulmonares tipo 3","synonyms":["CCAM tipo 3","CCAM tipo III","Enfermedad quística congénita del pulmón tipo 3","Malformación adenomatoide quística congénita del pulmón tipo 3","Malformación adenomatosa quística congénita del pulmón tipo 3"],"definition":"Es un subtipo poco frecuente de malformación congénita de las vías aéreas pulmonares caracterizado por una masa multiquística de tejido pulmonar no funcionante, compuesta por numerosos microquistes menores de 0,5 cm de diámetro. Las lesiones presentan comunicaciones intraquísticas, pueden estar conectadas al árbol traqueobronquial y, por lo general, son unilaterales, afectando a un lóbulo completo. El trastorno puede estar asociado a polihidramnios, hidropesía fetal y mortinatalidad, o presentarse con distrés respiratorio grave en el período neonatal."},"it":{"name":"Malformazione congenita delle vie aeree polmonari, tipo 3","synonyms":["CCAM, tipo 3","CPAM, tipo 3","Malattia cistica congenita del polmone, tipo 3","Malformazione adenomatoide cistica congenita del polmone, tipo 3"],"definition":"Si tratta di un sottotipo raro di malformazione congenita delle vie aeree del polmone, caratterizzato da una massa multicistica di tessuto polmonare non funzionante costituita da numerose microcisti di diametro inferiore a 0,5 cm. Le lesioni presentano comunicazioni intracistiche, possono essere connesse all'albero tracheobronchiale, in genere sono monolaterali, e interessano un intero lobo. La malattia può associarsi a polidramnios, idrope fetale, morte perinatale, o a distress respiratorio nel periodo neonatale."},"nl":{"name":"Congenitale malformatie van pulmonale luchtwegen type 3","synonyms":["CCAM type 3","CPAM type 3","Congenitale cystische adenomateuze malformatie van long type 3","Congenitale cystische adenomatoïde malformatie van long type 3","Congenitale cystische ziekte van long type 3"],"definition":"Een zeldzaam subtype van congenitale malformatie van pulmonale luchtwegen, gekarakteriseerd door een multicystische massa van niet-functioneel longweefsel bestaande uit talloze microcysten met een diameter kleiner dan 0,5 cm. De laesies vertonen intracystische communicatie, kunnen verbonden zijn met tracheobronchiale boom, en zijn meestal unilateraal, met betrokkenheid van een hele kwab. De aandoening kan geassocieerd zijn met polyhydramnion, hydrops foetalis, en doodgeboorte, of zich presenteren met ernstige ademnood in de neonatale periode."},"pt":{"name":"Malformação congénita das vias aéreas pulmonares, tipo 3","synonyms":["CCAM tipo III","CPAM tipo 3"]},"pl":{"name":"Wrodzona malformacja dróg oddechowych płuc, typu 3","synonyms":["CCAM typu 3","CPAM typu 3","Wrodzona choroba torbielowata płuc typu 3","Wrodzona torbielowatość gruczolakowata płuc typu 3"]},"cs":{"name":"Vrozená cystická adenomatozní malformace plic, typ 3","synonyms":["CCAM, typ 3","CPAM, typ 3","Vrozená cystická adenomatoidní malformace plic, typ 3","Vrozená malformace plicních bronchů, typ 3","Vrozené cystické onemocnění plic, typ 3"]},"uk":{"name":"Вроджена вада розвитку легеневих дихальних шляхів, тип 3","synonyms":["CCAM тип 3","Вроджена кістозна аденоматоїдна мальформація легень 3 типу","Вроджена кістозно-аденоматозна мальформація легень 3 типу","Вроджена кістозна хвороба легень 3 типу","CPAM тип 3"],"definition":"A rare subtype of congenital pulmonary airway malformation characterized by a multicystic mass of non-functioning lung tissue consisting of numerous microcysts of less than 0.5 cm in diameter. The lesions have intracystic communications, can be connected to the tracheobronchial tree, and are usually unilateral, involving an entire lobe. The condition may be associated with polyhydramnios, fetal hydrops, and stillbirth, or present with severe respiratory distress in the neonatal period."},"zh":{"name":"先天性肺气道畸形3型","synonyms":["先天性肺囊性腺瘤性畸形3型"]},"tr":{"name":"Doğumsal pulmoner hava yolu malformasyonu tip 3","synonyms":["CCAM tip 3","CPAM tip 3","Akciğer tip 3'ün doğumsal kistik hastalığı","Akciğer tipi 3'ün doğumsal kistik adenomatoid malformasyonu","Akciğer tip 3'ün doğumsal kistik adenomatöz malformasyonu"]}},"xrefs":[{"source":"ICD-10","code":"Q33.0","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"ICD-11","code":"LA75.4","qualifier":"NTBT","icd_relation":"index","validated":true,"icd11_uri":"1342201768"},{"source":"MONDO","code":"0017251","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C5437762","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":93890,"name":"Rare developmental defect during embryogenesis"},"parents":[{"orpha":2444,"name":"Congenital pulmonary airway malformation","classifications":[147,184,186]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":[],"onset":["Antenatal","Neonatal"],"prevalence":[],"phenotypes":null}