Supernumerary kidney
Synonyms: Accessory kidney
- Type
- Morphological anomaly
- Group
- Disorder
- Inheritance
- Unknown
- Age of onset
- Neonatal
- Status
- active
A rare non-syndromic renal/urinary tract malformation characterized by the presence of more than two kidneys (up to five), each of which mostly has its own collecting system, vascular supply, and distinct encapsulated parenchyma. The additional kidney(s), smaller than native ones, mostly occurs ipsilateral and caudal to the left kidney and may be fused to the native kidneys. Patients are usually asymptomatic but they may present with symptoms such as pain, a palpable abdominal mass, fever and sometimes with urinary symptoms (such as urinary incontinence) when supernumerary kidneys occur with an ectopic ureter. Additional congenital abnormalities including horseshoe kidney malformation, ventricular septal defect, and cloacal abnormalities (such as urethral atresia, vaginal atresia, ectopic ureter implantation, imperforate anus, and duplication of urethra) may also be present in some patients.
Names in Orphadata languages
10 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Supernumerary kidney | Accessory kidney |
| fr | Rein surnuméraire | Rein accessoire |
| de | Akzessorische Niere | Niere, überzählige |
| es | Riñón supernumerario | Riñón adicional |
| it | Reni soprannumerari | Rene accessorio |
| nl | Accessoire nier | Extra nier |
| pt | Rins supranumerários não-sindrómico | Rim acessório não-sindrómico · Rim supranumerário não-sindrómico · Rins acessórios não-sindrómico |
| pl | Niesyndromiczne nerki dodatkowe | = en |
| cs | Nesyndromické nadpočetné ledviny | = en |
| uk | Несиндромальні додаткові нирки | Несиндромальні додаткові нирки · Несиндромальна додаткова нирка · Несиндромальна додаткова нирка |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | Q63.0 | E | specific | validated |
| ICD-11 | LB30.5 | E | specific | not yet validated |
| MONDO | 0958093 | E | validated | |
| MONDO | 958093 | E | validated | |
| UMLS | C5925122 | 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:93546Non-syndromic renal or urinary tract malformation
rare developmental anomalies during embryogenesis; rare renal diseases; rare transplant-related disorders
No children.
Genes
0 directOrphanet records no gene directly on this code.
Prevalence and incidence
2 records| Type | Class | Value | Area | Qualification | Validation | Source |
|---|---|---|---|---|---|---|
| Cases/families | 99 cases | Worldwide | Case(s) | Validated | 30761238[PMID] | |
| Point prevalence | <1 / 1 000 000 | — | Worldwide | Class only | Validated | 30761238[PMID]_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/652528
- This record as JSON: /data/d/652528.json