Non-amyloid fibrillary glomerulopathy
Synonyms: Congo red-negative amyloidosis-like glomerulopathy · Non-amyloid fibrillary glomerulonephritis
- Type
- Disease
- Group
- Disorder
- Inheritance
- Not applicable
- Age of onset
- Adult
- Status
- active
Non-amyloid fibrillary glomerulopathy (non-amyloid FGP) is a rare cause of glomerulonephritis (GN) characterized by glomerular accumulation of non-amyloid fibrils in the mesangium and the glomerular (and rarely tubular) basement membrane, that mainly presents with renal insufficiency, micro-hematuria and nephrotic range proteinuria. Non-amyloid FGP and immunotactoid glomerulopathy (ITG) are often grouped together as pathogenetically related diseases.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Non-amyloid fibrillary glomerulopathy | Congo red-negative amyloidosis-like glomerulopathy · Non-amyloid fibrillary glomerulonephritis |
| fr | Glomérulonéphrite à dépôts fibrillaires pseudoamyloïdes | Glomérulopathie à dépôts fibrillaires pseudoamyloïdes |
| de | Glomerulopathie, fibrilläre nicht-amyloide | |
| es | Glomerulopatía fibrilar no amiloide | |
| it | Glomerulopatia fibrillare non amiloide | |
| nl | Niet-amyloïde fibrillaire glomerulopathie | |
| pt | Glomerulopatia fibrilar, não-amilóide | |
| pl | Nieamyloidowa glomerulopatia włókienkowa | = en |
| cs | Neamyloidová fibrilární glomerulopatie | Neamyloidová fibrilární glomerulonefritida · Kongo-červeň negativní glomerulopatie podobná amyloidóze |
| uk | Неамілоїдна фібрилярна гломерулопатія | Конго червоно-негативна амілоїдозоподібна гломерулопатія · Неамілоїдний фібрилярний гломерулонефрит |
| zh | 非淀粉样纤维样肾小球病 | 刚果红阴性淀粉样变性样肾小球病 |
| tr | Amiloid olmayan fibriller glomerülopati | Amiloid olmayan fibriller glomerülonefrit · Kongo kırmızısı-negatif amiloidoz benzeri glomerülopati |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | N03.8 | NTBT | attributed | validated |
| ICD-11 | GB4Y | NTBT | attributed | validated |
| MONDO | 0019990 | E | validated | |
| UMLS | C4273674 | E | validated | |
| GARD | 12740 | 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:91137Immunotactoid or fibrillary glomerulopathy
rare renal diseases; rare transplant-related disorders
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/97566
- This record as JSON: /data/d/97566.json