Immunotactoid glomerulopathy
Synonyms: Immunotactoid glomerulonephritis
- Type
- Disease
- Group
- Disorder
- Inheritance
- Not applicable
- Age of onset
- Adult
- Status
- active
Immunotactoid glomerulopathy (ITG) is a very rare condition characterized by glomerular accumulation of microtubules in the mesangium and the glomerular basement membrane, that mainly presents with proteinuria, micro-hematuria, nephrotic syndrome, renal insufficiency and hematologic malignancy. ITG and non-amyloid fibrillary glomerulopathy (non-amyloid FGP) are often grouped together as pathogenetically related diseases.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Immunotactoid glomerulopathy | Immunotactoid glomerulonephritis |
| fr | Glomérulonéphrite à dépôts organisés microtubulaires | Glomérulopathie à dépôts organisés microtubulaires |
| de | Glomerulopathie, immunotaktoide | |
| es | Glomerulopatía inmunotactoide | |
| it | Glomerulopatia immunotattoide | |
| nl | Immunotactoïde glomerulopathie | |
| pt | Glomerulopatia imunotactoide | |
| pl | Glomerulopatia immunotaktoidalna | = en |
| cs | Imunotaktoidní glomerulopatie | Imunotaktoidní glomerulonefritida |
| uk | Імунотактоїдна гломерулопатія | Імунотактоїдний гломерулонефрит |
| zh | 免疫触发性肾小球肾病 | 免疫触发性肾小球肾炎 |
| tr | İmmünotaktoid glomerülopati | İmmünotaktoid glomerülonefrit |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | N03.6 | NTBT | attributed | validated |
| ICD-11 | 5D00.Y | NTBT | index | validated |
| MONDO | 0019991 | E | validated | |
| UMLS | C2242534 | E | validated | |
| GARD | 12048 | E | validated | |
| MedDRA | 10067871 | 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
2 records| Type | Class | Value | Area | Qualification | Validation | Source |
|---|---|---|---|---|---|---|
| Cases/families | 200 cases | Worldwide | Case(s) | Validated | ORPHANET | |
| Point prevalence | <1 / 1 000 000 | — | 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
Orphanet has no HPO annotation for this code in this release.
Sources for this card
- Orphanet page: orpha.net/en/disease/detail/97567
- This record as JSON: /data/d/97567.json