ORPHA:69061
Idiopathic steroid-sensitive nephrotic syndrome
- Type
- Clinical syndrome
- Group
- Disorder
- Inheritance
- Unknown
- Age of onset
- All ages
- Status
- active
A rare primary glomerulopathy of unknown cause characterized by edema, nephrotic-range proteinuria and hypoalbuminemia that responds to standard prednisone treatment within 4-6 weeks.
1
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Idiopathic steroid-sensitive nephrotic syndrome | |
| fr | Syndrome néphrotique idiopathique corticosensible | |
| de | Nephrotisches Syndrom, steroid-sensitives | |
| es | Síndrome nefrótico idiopático sensible a esteroides | |
| it | Sindrome nefrosica idiopatica sensibile agli steroidi | |
| nl | Idiopathisch steroïdgevoelig nefrotisch syndroom | |
| pt | Síndrome nefrótico sensível aos esteróides | |
| pl | Idiopatyczny zespół nerczycowy wrażliwy na steroidy | |
| cs | Idiopatický steroid-sensitivní nefrotický syndrom | |
| uk | Ідіопатичний стероїд-чутливий нефротичний синдром | |
| zh | 特发性类固醇敏感性肾病综合征 | |
| tr | İdiyopatik steroide duyarlı nefrotik sendrom |
Definition in:
2
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| OMIM | 615861 | NTBT | validated | |
| ICD-10 | N04.0 | NTBT | attributed | validated |
| ICD-11 | GB41 | NTBT | index | validated |
| MONDO | 0019067 | E | validated | |
| UMLS | C0403396 | E | validated |
E exact · NTBT this ORPHAcode is narrower than the target · BTNT broader than the target · ND not decided.
3
Classification
Preferential parent (Orphanet linearisation):
Parents in the classifications (1):
- ORPHA:357502Idiopathic nephrotic syndrome
rare renal diseases; rare transplant-related disorders
No children.
Codes that Orphanet moved or referred to this one:
- ORPHA:93206OBSOLETE: Idiopathic steroid-sensitive nephrotic syndrome with focal segmental hyalinosis referred to · inactive
- ORPHA:93207OBSOLETE: Idiopathic steroid-sensitive nephrotic syndrome with minimal change referred to · inactive
- ORPHA:93209OBSOLETE: Idiopathic steroid-sensitive nephrotic syndrome with diffuse mesangial proliferation referred to · inactive
- ORPHA:97552Steroid-sensitive nephrotic syndrome without renal biopsy moved to · inactive
4
Genes
0 directOrphanet records no gene directly on this code.
5
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.
6
HPO phenotypes
2 annotations · validated 2016-06-01| HPO | Term (as annotated by Orphanet) | Diagnostic |
|---|---|---|
| Very frequent (99-80%) | ||
| HP:0001004 | Lymphedema | |
| HP:0000100 | Nephrotic syndrome | |
Run the differential on the 2 obligate / very frequent terms
7
Sources for this card
- Orphanet page: orpha.net/en/disease/detail/69061
- This record as JSON: /data/d/69061.json