Congenital pulmonary sequestration
Synonyms: Congenital bronchopulmonary sequestration
- Type
- Malformation syndrome
- Group
- Disorder
- Age of onset
- Neonatal
- Status
- active
Congenital pulmonary sequestration is a rare respiratory malformation characterized by a cystic or solid mass of nonfunctioning primitive segmental lung tissue that does not communicate with the tracheobronchial tree and has anomalous systemic blood supply. Intralobar pulmonary sequestration may be asymptomatic or may present with recurrent pulmonary infections, hemoptysis, chest pain, cough and is usually diagnosed in older children and adults. Extralobar pulmonary sequestration present with respiratory distress, cyanosis, difficulty feeding or infection, may be associated with other anomalies and is mostly diagnosed in neonates or infants.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Congenital pulmonary sequestration | Congenital bronchopulmonary sequestration |
| fr | Séquestration pulmonaire congénitale | Séquestration broncho-pulmonaire congénitale |
| de | Lungensequestration, kongenitale | Bronchopulmonale Sequester, kongenitale · Pulmonale Sequestration, kongenitale |
| es | Secuestro pulmonar congénito | Secuestro broncopulmonar congénito |
| it | Sequestro polmonare congenito | Sequestro broncopolmonare congenito |
| nl | Congenitaal longsekwester | Congenitale bronchopulmonale sequestratie |
| pt | Sequestro pulmonar congénito | Sequestro broncopulmonar congénito |
| pl | Wrodzona sekwestracja płuc | Wrodzona sekwestracja oskrzelowo-płucna |
| cs | Vrozená plicní sekvestrace | Vrozená bronchopulmonální sekvestrace |
| uk | Вроджена легенева секвестрація | Вроджена бронхолегенева секвестрація |
| zh | 先天性肺隔离症 | 先天性支气管肺隔离症 |
| tr | Doğumsal pulmoner sekestrasyon | Doğumsal bronkopulmoner sekestrasyon |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | Q33.2 | E | specific | validated |
| ICD-11 | LA75.6 | E | specific | validated |
| MONDO | 0017843 | E | validated | |
| MeSH | D001998 | E | validated | |
| UMLS | C0006288 | E | validated | |
| GARD | 4593 | E | validated | |
| MedDRA | 10059160 | 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 (2):
- ORPHA:108993Non-syndromic respiratory or mediastinal malformation
rare developmental anomalies during embryogenesis; rare surgical thoracic diseases - ORPHA:182111Respiratory malformation
rare respiratory diseases
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/3161
- This record as JSON: /data/d/3161.json