Idiopathic nephrotic syndrome
- Type
- Clinical group
- Group
- Group of disorders
- Age of onset
- All ages
- Status
- active
A rare primary glomerular group of diseases characterized by the triad of edema, massive, or nephrotic-range, proteinuria and hypoalbuminemia, for which there is no known cause. Depending on response to treatment, disease is distinguished into steroid-sensitive nephrotic syndrome (SSNS) and steroid-resistant nephrotic syndrome (SRNS), with the latter being further divided, depending on occurrence, into familial or sporadic forms.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Idiopathic nephrotic syndrome | |
| fr | Syndrome néphrotique idiopathique | Néphrose idiopathique |
| de | Nephrotisches Syndrom, idiopathisches | |
| es | Síndrome nefrótico idiopático | |
| it | Sindrome nefrosica idiopatica | |
| nl | Idiopathisch nefrotisch syndroom | |
| pt | Síndrome nefrótica idiopática | |
| pl | Idiopatyczny zespół nefrotyczny | Idiopatyczny zespół nerczycowy |
| cs | Idiopatický nefrotický syndrom | |
| uk | Ідіопатичний нефротичний синдром | |
| zh | 特发性肾病综合征 | |
| tr | İdiyopatik nefrotik sendrom |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| MONDO | 0018170 | E | validated | |
| MONDO | 18170 | E | validated | |
| UMLS | C3496337 | 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:567564Nephrotic syndrome without extrarenal manifestations
rare renal diseases; rare transplant-related disorders
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 | — | Europe | Class only | Not yet 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/357502
- This record as JSON: /data/d/357502.json