Collagen type III glomerulopathy
Synonyms: Collagenofibrotic glomerulopathy
- Type
- Disease
- Group
- Disorder
- Status
- active
A rare non-immune-mediated glomerular disease characterized by abnormal accumulation of type III collagen within the mesangium and subendothelial space of the glomerulus. Clinically it usually manifests with proteinuria (often in the nephrotic range), microscopic hematuria, peripheral edema and/or hypertension. Progression to end-stage kidney failure is possible.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Collagen type III glomerulopathy | Collagenofibrotic glomerulopathy |
| fr | Glomérulopathie à dépôts de collagène de type III | |
| de | Kollagen Typ III-Glomerulopathie | Kollagenofibrotische Glomerulopathie |
| es | Glomerulopatía por colágeno tipo III | Glomerulopatía colagenofibrótica |
| it | Glomerulopatia da deposito di collagene tipo 3 | |
| nl | Collageen type III-glomerulopathie | Collagenofibrotische glomerulopathie |
| pt | Glomerulopatia de colagénio tipo III | |
| pl | Glomerulopatia kolagenu typu III | = en |
| cs | Glomerulopatie související s kolagenem typu III | = en |
| uk | Колагенова гломерулопатія, тип III | Колагенофіброзна гломерулопатія |
| zh | 胶原3型肾小球病 | 胶原纤维性肾小球病 |
| tr | Kollajen tip III glomerülopati | Kolajenofibrotik glomerülopati |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | N07.6 | NTBT | attributed | validated |
| ICD-11 | GB4Y | NTBT | attributed | validated |
| MONDO | 0019396 | E | validated | |
| UMLS | C3872695 | 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:93548Glomerular disease
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/84087
- This record as JSON: /data/d/84087.json