High altitude pulmonary edema
Synonyms: HAPE
- Type
- Particular clinical situation in a disease or syndrome
- Group
- Disorder
- Inheritance
- Not applicable
- Age of onset
- All ages
- Status
- active
A rare pulmonary condition characterized by non-cardiogenic pulmonary edema occurring in otherwise healthy individuals within days of an ascent above 2500-3000 m. Early symptoms include exertional dyspnea, non-productive cough, chest tightness, and reduced exercise performance, followed by dyspnea at rest and possibly orthopnea, as well as gurgling in the chest and pink frothy sputum in advanced cases. Clinical signs are cyanosis, tachypnea, tachycardia, crackles or wheezing, and elevated body temperature (generally not exceeding 38.5°C). Signs of concomitant high-altitude cerebral edema may also be observed. Chest x-rays typically show patchy opacities predominantly in the right middle lobe.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | High altitude pulmonary edema | HAPE |
| fr | Oedème pulmonaire de haute altitude | |
| de | Höhenlungenödem | = en |
| es | Edema pulmonar de gran altitud | EPGA |
| it | Edema polmonare da elevata altitudine | = en |
| nl | Hoogtelongoedeem | HAPE · Longoedeem door hoogteziekte |
| pt | Edema pulmonar de grande altitude | = en |
| pl | Obrzęk płuc na dużej wysokości | Wysokościowy obrzęk płuc |
| cs | Výškový plicní edém | = en |
| uk | Високий набряк легенів | = en |
| zh | 高原性肺水肿 | |
| tr | Yüksek irtifa akciğer ödemi |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | J81 | NTBT | attributed | validated |
| ICD-11 | CB01 | NTBT | attributed | validated |
| MONDO | 0031257 | E | validated | |
| MeSH | C535833 | E | validated | |
| UMLS | C0340100 | 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:101944Rare pulmonary disease
rare respiratory diseases
No children.
Genes
0 directOrphanet records no gene directly on this code.
Prevalence and incidence
1 record| Type | Class | Value | Area | Qualification | Validation | Source |
|---|---|---|---|---|---|---|
| Point prevalence | 1-9 / 1 000 000 | — | Europe | Class only | Validated | European Medicines Agency 2013[OTHER] |
Value is Orphadata's ValMoy: the mean estimate per 100 000 for prevalence and incidence, the number of cases or families for case reports.
HPO phenotypes
21 annotations · validated 2023-04-06| HPO | Term (as annotated by Orphanet) | Diagnostic |
|---|---|---|
| Obligate (100%) | ||
| HP:0012418 | Hypoxemia | |
| HP:0100598 | Pulmonary edema | |
| Very frequent (99-80%) | ||
| HP:0002094 | Dyspnea | Diagnostic criterion |
| Frequent (79-30%) | ||
| HP:0002039 | Anorexia | |
| HP:0012735 | Cough | Diagnostic criterion |
| HP:0030830 | Crackles | Diagnostic criterion |
| HP:0000961 | Cyanosis | Diagnostic criterion |
| HP:0003546 | Exercise intolerance | Diagnostic criterion |
| HP:0002315 | Headache | |
| HP:0001974 | Increased total leukocyte count | |
| HP:0002017 | Nausea and vomiting | |
| HP:0031457 | Pulmonary opacity | Diagnostic criterion |
| HP:0002360 | Sleep abnormality | |
| HP:0001649 | Tachycardia | Diagnostic criterion |
| Occasional (29-5%) | ||
| HP:0032016 | Abnormal sputum | |
| HP:0100749 | Chest pain | |
| HP:0001945 | Fever | |
| HP:0012764 | Orthopnea | |
| HP:0002789 | Tachypnea | Diagnostic criterion |
| HP:0002321 | Vertigo | |
| Very rare (<4-1%) | ||
| HP:0002105 | Hemoptysis | |
Run the differential on the 3 obligate / very frequent terms
Annotation source: 15183846[PMID]_31005280[PMID]_35611432[PMID]_34106165[PMID]_36562650[PMID]_35384735[PMID]
Sources for this card
- Orphanet page: orpha.net/en/disease/detail/330012
- This record as JSON: /data/d/330012.json