Renal arteriovenous malformation
Synonyms: Arteriovenous malformation of the kidney · rAVM
- Type
- Malformation syndrome
- Group
- Disorder
- Inheritance
- Not applicable
- Age of onset
- Adolescent, Adult, Childhood
- Status
- active
A rare visceral arteriovenous malformation characterized by abnormal communications between renal arteries and the venous system. Most cases are asymptomatic but patients may present with clinical symptoms including hematuria, nausea, lumbar and abdominal pain, hypertension, cardiomegaly and high output heart failure. This type of malformation is more commonly reported in females.
Names in Orphadata languages
9 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Renal arteriovenous malformation | Arteriovenous malformation of the kidney · rAVM |
| fr | Malformation artérioveineuse rénale | |
| de | Renale arteriovenöse Fehlbildung | Arteriovenöse Fehlbildung der Nieren · rAVM |
| es | Malformación arteriovenosa renal | MAV renal · Malformación arteriovenosa del riñón |
| it | Malformazione arterovenosa renale | Malformazione arterovenosa del rene · rAVM |
| nl | Arterioveneuze malformatie van nier | RAVM · Renale arterioveneuze malformatie |
| pt | Malformação arteriovenosa renal | Malformação arteriovenosa do rim · rAVM |
| pl | Malformacja tętniczo-żylna nerki | = en |
| cs | Renal arteriovenous malformation | = en |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | Q27.3 | NTBT | attributed | validated |
| ICD-11 | LA90.3Y | NTBT | attributed | 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 (3):
- ORPHA:93618Rare cause of hypertension
rare renal diseases - ORPHA:506213Rare disorder potentially indicated for kidney transplant
rare transplant-related disorders - ORPHA:693855Visceral fast-flow vascular malformation
rare developmental anomalies during embryogenesis; rare circulatory system 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 | Unknown | — | Worldwide | Class only | Validated | ORPHANET |
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
Orphanet has no HPO annotation for this code in this release.
Sources for this card
- Orphanet page: orpha.net/en/disease/detail/693839
- This record as JSON: /data/d/693839.json