Visceral calciphylaxis
- Type
- Clinical subtype
- Group
- Subtype of disorder
- Status
- active
A rare, life-threatening, non-inflammatory vasculopathy characterized by diffuse precipitation of calcium in viscera (mainly in the heart or lungs, but also in the stomach or kidneys) leading to fibrosis and thrombosis, which eventually causes tissue necrosis. Depending on the affected organ, patients may present with dyspnea, cough and respiratory failure or acute heart block and subsequent sudden cardiac death. The disease predominantly affects patients with end-stage kidney disease (ESKD) on dialysis.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Visceral calciphylaxis | |
| fr | Calciphylaxie viscérale | |
| de | Kalziphylaxie, viszerale | |
| es | Calcifilaxis visceral | |
| it | Calcifilassi viscerale | |
| nl | Viscerale calciphylaxis | Viscerale calcifylaxie |
| pt | Calcifilaxia visceral | |
| pl | Kalcyfilaksja trzewna | |
| cs | Viscerální kalcifylaxe | |
| uk | Вісцеральна кальцифілаксія | |
| zh | 内脏钙化防御 | |
| tr | Viseral kalsifilaksi |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | E83.5 | NTBT | attributed | validated |
| ICD-11 | EB90.42 | NTBT | attributed | validated |
| MONDO | 0017217 | E | validated | |
| UMLS | C4749793 | 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:280062Calciphylaxis
rare systemic and rheumatological diseases; rare circulatory system 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/280068
- This record as JSON: /data/d/280068.json