ORPHA:420259
Secondary pulmonary alveolar proteinosis
Synonyms: Secondary PAP
- Type
- Disease
- Group
- Disorder
- Inheritance
- Not applicable
- Status
- active
A rare, acquired, interstitial lung disease, characterized by alveolar surfactant accumulation, cough, progressive dyspnea and respiratory insufficiency. The disease may be secondary to hematological disorder, toxic inhalation, and infection or may occur within the setting of immunosuppression after transplantation.
1
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Secondary pulmonary alveolar proteinosis | Secondary PAP |
| fr | Protéinose alvéolaire pulmonaire secondaire | PAP secondaire |
| de | Pulmonale Alveolarproteinose, sekundäre | Sekundäre PAP |
| es | Proteinosis alveolar pulmonar secundaria | PAP secundaria |
| it | Proteinosi alveolare polmonare secondaria | PAP secondaria |
| nl | Secundaire pulmonale alveolaire proteïnose | Secundaire PAP |
| pt | Proteinose pulmonar alveolar secundária | |
| pl | Wtórna proteinoza pęcherzyków płucnych | Wtórna PAP |
| cs | Sekundární plicní alveolární proteinóza | Sekundární PAP |
| uk | Вторинний альвеолярний протеїноз легень | Вторинний ПАП |
| zh | 继发性肺泡蛋白沉着症 | 继发型PAP |
| tr | Sekonder pulmoner alveolar proteinoz | Sekonder PAP |
Definition in:
2
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | J84.0 | NTBT | attributed | validated |
| ICD-11 | CB04.31 | NTBT | index | validated |
| MONDO | 0018483 | E | validated | |
| UMLS | C3873302 | E | validated |
E exact · NTBT this ORPHAcode is narrower than the target · BTNT broader than the target · ND not decided.
3
Classification
Preferential parent (Orphanet linearisation):
Parents in the classifications (1):
- ORPHA:264944Secondary interstitial lung disease in childhood and adulthood
rare respiratory diseases; rare transplant-related disorders
No children.
4
Genes
0 directOrphanet records no gene directly on this code.
5
Prevalence and incidence
0 recordsNo prevalence record in this release.
6
HPO phenotypes
Orphanet has no HPO annotation for this code in this release.
7
Sources for this card
- Orphanet page: orpha.net/en/disease/detail/420259
- This record as JSON: /data/d/420259.json