Idiopathic steroid-sensitive nephrotic syndrome with secondary steroid resistance
Synonyms: Idiopathic SSNS with secondary steroid resistance · Secondary SRNS · Secondary steroid-resistant nephrotic syndrome
- Type
- Clinical syndrome
- Group
- Disorder
- Inheritance
- Not applicable
- Age of onset
- All ages
- Status
- active
A rare, idiopathic nephrotic syndrome characterized by pediatric onset of proteinuria, hypoalbuminemia and edema. Patients respond successfully to the initial standard course of corticosteroids, but are resistant to standard therapy for a subsequent relapse and following this relapse remain steroid-resistant.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Idiopathic steroid-sensitive nephrotic syndrome with secondary steroid resistance | Idiopathic SSNS with secondary steroid resistance · Secondary SRNS · Secondary steroid-resistant nephrotic syndrome |
| fr | Syndrome néphrotique corticosensible idiopathique avec résistance secondaire aux stéroïdes | SNCS idiopathique avec résistance secondaire aux stéroïdes · Syndrome néphrotique sensible aux stéroïdes avec résistance secondaire |
| de | Idiopathisches Steroid-sensitives nephrotisches Syndrom mit sekundärer Steroid-Resistenz | Idiopathisches SSNS mit sekundärer Steroid-Resistenz · Nephrotisches Syndrom, sekundär Steroid-resistentes · Sekundäres SRNS |
| es | Síndrome nefrótico idiopático sensible a esteroides con resistencia secundaria a esteroides | SNRE secundario · SNSE idiopático con resistencia secundaria a esteroides · Síndrome nefrótico secundario resistente a esteroides |
| it | Sindrome nefrosica idiopatica sensibile agli steroidi con resistenza secondaria agli steroidi | SRNS secondaria · SSNS idiopatica con resistenza secondaria agli steroidi · Sindrome nefrosica con resistenza secondaria agli steroidi |
| nl | Idiopathisch steroïdsensitief nefrotisch syndroom met secundaire steroïdresistentie | Idiopathisch SSNS met secundaire steroïdresistentie · Secundair SRNS · Secundair steroïdresistent nefrotisch syndroom |
| pt | Síndrome nefrótico cortico-resistente idiopático com resistência secundária aos corticoides | |
| pl | Idiopatyczny zespół nerczycowy sterydo-wrażliwy z wtórną opornością na sterydy | = en |
| cs | Idiopatický nefrotický syndrom citlivý na steroidy se sekundární rezistencí na steroidy | = en |
| uk | Ідіопатичний стероїдчутливий нефротичний синдром із вторинною стероїдною резистентністю | Ідіопатичний SSNS із вторинною стероїдною резистентністю · Вторинна SRNS · Вторинний стероїдорезистентний нефротичний синдром |
| zh | 特发性类固醇敏感肾病综合征伴继发性类固醇抵抗 | Idiopathic SSNS with secondary steroid resistance |
| tr | Sekonder steroid direnci ile seyreden idiyopatik steroide duyarlı nefrotik sendrom | Sekonder SRNS · Sekonder steroide-dirençli nefrotik sendrom · Sekonder steroid direnci ile seyreden idiyopatik SSNS |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | N04.8 | NTBT | attributed | validated |
| ICD-11 | GB41 | NTBT | attributed | validated |
| MONDO | 0035764 | E | validated | |
| UMLS | C5680131 | 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:357502Idiopathic nephrotic syndrome
rare renal diseases; rare transplant-related disorders
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
28 annotations · validated 2021-06-17| HPO | Term (as annotated by Orphanet) | Diagnostic |
|---|---|---|
| Very frequent (99-80%) | ||
| HP:0012592 | Albuminuria | |
| HP:0000969 | Edema | |
| Frequent (79-30%) | ||
| HP:0012622 | Chronic kidney disease | |
| HP:0000282 | Facial edema | |
| HP:0012378 | Fatigue | |
| HP:0000097 | Focal segmental glomerulosclerosis | |
| HP:0100724 | Hypercoagulability | |
| HP:0003077 | Hyperlipidemia | |
| HP:0003073 | Hypoalbuminemia | |
| HP:0100540 | Palpebral edema | |
| HP:0010741 | Pedal edema | |
| Occasional (29-5%) | ||
| HP:0002027 | Abdominal pain | |
| HP:0012393 | Allergy | |
| HP:0002094 | Dyspnea | |
| HP:0000034 | Hydrocele testis | |
| HP:0000822 | Hypertension | |
| HP:0002907 | Microscopic hematuria | |
| HP:0012579 | Minimal change glomerulonephritis | |
| HP:0011947 | Respiratory tract infection | |
| HP:0003774 | Stage 5 chronic kidney disease | |
| Very rare (<4-1%) | ||
| HP:0012050 | Anasarca | |
| HP:0004420 | Arterial thrombosis | |
| HP:0001541 | Ascites | |
| HP:0004431 | Complement deficiency | |
| HP:0012587 | Macroscopic hematuria | |
| HP:0002202 | Pleural effusion | |
| HP:0002204 | Pulmonary embolism | |
| HP:0004936 | Venous thrombosis | |
Run the differential on the 2 obligate / very frequent terms
Annotation source: 28117080[PMID]_29169714[PMID]_29058154[PMID]_24511128[PMID]
Sources for this card
- Orphanet page: orpha.net/en/disease/detail/567546
- This record as JSON: /data/d/567546.json