Congenital pulmonary airway malformation type 4
Synonyms: CPAM type 4 · Congenital cystic adenomatoid malformation of the lung type 4 · Congenital cystic adenomatous malformation of the lung type 4
- Type
- Clinical subtype
- Group
- Subtype of disorder
- Age of onset
- Antenatal, Neonatal
- Status
- active
A rare subtype of congenital pulmonary airway malformation characterized by a multicystic mass of non-functioning lung tissue, with peripheral, large, thin-walled, often multiloculated cysts, which may be 8 cm in diameter. The lesions have intracystic communications, can be connected to the tracheobronchial tree, and are usually unilateral, involving a single lobe. Patients present with respiratory distress or respiratory infections in the neonatal period or in infancy. The condition is often associated with tension pneumothorax, signs of mediastinal shift, and malignant transformation to pleuropulmonary blastoma type 1.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Congenital pulmonary airway malformation type 4 | CPAM type 4 · Congenital cystic adenomatoid malformation of the lung type 4 · Congenital cystic adenomatous malformation of the lung type 4 |
| fr | Malformation congénitale des voies aériennes pulmonaires type 4 | CPAM type 4 · Maladie adénomatoïde kystique du poumon type 4 · Malformation adénomatoïde kystique du poumon type 4 |
| de | Kongenitale Fehlbildung der Atemwege Typ 4 | CCAM Typ 4 · CPAM Typ 4 · Kongenitale zystische Krankheit der Lunge Typ 4 · Kongenitale zystische adenomatoide Fehlbildung der Lunge Typ 4 |
| es | Malformación congénita de las vías aéreas pulmonares tipo 4 | CPAM tipo 4 · Malformación adenomatoide quística congénita del pulmón tipo 4 · Malformación adenomatosa quística congénita del pulmón tipo 4 |
| it | Malformazione congenita delle vie aeree polmonari, tipo 4 | CPAM, tipo 4 · Malformazione adenomatoide cistica congenita del polmone, tipo 4 |
| nl | Congenitale malformatie van pulmonale luchtwegen type 4 | CCAM type IV · CPAM type 4 · Congenitale cystische adenomateuze malformatie van long type 4 · Congenitale cystische adenomatoïde malformatie van long type 4 |
| pt | Malformação congénita das vias aéreas pulmonares, tipo 4 | CPAM tipo 4 |
| pl | Wrodzona malformacja dróg oddechowych płuc, typu 4 | CPAM typu 4 · Wrodzona torbielowatość gruczolakowata płuc typu 4 |
| cs | Vrozená cystická adenomatozní malformace plic, typ 4 | CCAM, typ 4 · Vrozená cystická adenomatoidní malformace plic, typ 4 · Vrozená malformace plicních bronchů, typ 4 |
| uk | Вроджена вада розвитку легеневих дихальних шляхів, тип 4 | Вроджена кістозна аденоматоїдна мальформація легень 4 типу · Вроджена кістозно-аденоматозна мальформація легень 4 типу · CPAM тип 4 |
| zh | 先天性肺气道畸形4型 | 先天性肺囊性腺瘤样畸形4型 |
| tr | Doğumsal pulmoner hava yolu malformasyonu tip 4 | CPAM tip 4 · Akciğer tipi 4'ün doğumsal kistik adenomatoid malformasyonu · Akciğer tipi 4'ün doğumsal kistik adenomatöz malformasyonu |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | Q33.0 | NTBT | attributed | validated |
| ICD-11 | LA75.4 | NTBT | index | validated |
| MONDO | 0017252 | E | validated | |
| UMLS | C5437760 | E | validated |
E exact · NTBT this ORPHAcode is narrower than the target · BTNT broader than the target · ND not decided.
Classification
Preferential parent (Orphanet linearisation):
Parents in the classifications (1):
- ORPHA:2444Congenital pulmonary airway malformation
rare developmental anomalies during embryogenesis; rare respiratory diseases; rare surgical thoracic diseases
No children.
Genes
0 directOrphanet records no gene directly on this code.
Prevalence and incidence
0 recordsNo prevalence record in this release.
HPO phenotypes
Orphanet has no HPO annotation for this code in this release.
Sources for this card
- Orphanet page: orpha.net/en/disease/detail/280854
- This record as JSON: /data/d/280854.json