Congenital renal artery stenosis
Synonyms: Congenital renovascular hypoplasia
- Type
- Disease
- Group
- Disorder
- Age of onset
- Neonatal
- Status
- active
A rare renal disease characterized by congenital unilateral or bilateral narrowing of the renal artery leading to severe arterial hypertension and progressive renal failure in the neonate. Manifestations include hypertensive encephalopathy and/or neurological signs and symptoms due to hyponatremia, polyuria, renal electrolyte loss, proteinuria, and hematuria.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Congenital renal artery stenosis | Congenital renovascular hypoplasia |
| fr | Sténose congénitale de l'artère rénale | Hypoplasie rénovasculaire congénitale |
| de | Nierenarterienstenose, kongenitale | |
| es | Estenosis arterial renal congénita | Hipoplasia renovascular congénita · Hipoplasia vascular-renal congénita |
| it | Stenosi congenita dell'arteria renale | Ipoplasia renovascolare congenita |
| nl | Congenitale nierarteriestenose | Congenitale renovasculaire hypoplasie · Congenitale stenose van arteria renalis |
| pt | Estenose congénita da artéria renal | |
| pl | Wrodzone zwężenie tętnicy nerkowej | Wrodzona hipoplazja nerkowo-naczyniowa |
| cs | Vrozená stenóza renální arterie | Vrozená renovaskulární hypoplázie |
| uk | Вроджений стеноз ниркової артерії | Вроджена реноваскулярна гіпоплазія |
| zh | 先天性肾动脉狭窄 | 先天性肾血管发育不良 |
| tr | Doğumsal renal arter darlığı | Doğumsal renovasküler hipoplazi |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | Q27.1 | E | specific | validated |
| ICD-11 | LA90.40 | E | specific | validated |
| MONDO | 0019993 | E | validated | |
| UMLS | C0495523 | 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 (3):
- ORPHA:93618Rare cause of hypertension
rare renal diseases - ORPHA:506213Rare disorder potentially indicated for kidney transplant
rare transplant-related disorders - ORPHA:458844Rare vascular malformation of major vessels
rare developmental anomalies during embryogenesis; 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/97598
- This record as JSON: /data/d/97598.json