De novo thrombotic microangiopathy after kidney transplantation
- Type
- Particular clinical situation in a disease or syndrome
- Group
- Disorder
- Age of onset
- All ages
- Status
- active
A rare renal disease characterized by thrombotic microangiopathy developing <i>de novo</i> in kidney transplant recipients with no evidence of occurrence of the disease prior to transplantation. Precipitating factors include antibody-mediated rejection, immunosuppressive medication, viral infections, and genetic abnormalities in the complement cascade, among others. The condition most commonly occurs within the first 3-6 months post-transplantation. Clinical presentation is highly variable and ranges from a limited form confined to the kidney with relatively good prognosis to a systemic variant consisting of the classic triad of thrombocytopenia, microangiopathic hemolytic anemia, and acute kidney injury.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | De novo thrombotic microangiopathy after kidney transplantation | |
| fr | Microangiopathie thrombotique de novo après transplantation rénale | |
| de | De-novo thrombotische Mikroangiopathie nach Nierentransplantation | |
| es | Microangiopatía trombótica de novo post-trasplante renal | |
| it | Microangiopatia trombotica de novo successiva a trapianto renale | |
| nl | De novo trombotische microangiopathie na niertransplantatie | |
| pt | Microangiopatia trombótica de novo após transplante renal | |
| pl | Mikroangiopatia zakrzepowa powstająca de novo po transplantacji nerki | |
| cs | De novo vznik trombotické mikroangiopatie po transplantaci ledviny | |
| uk | Тромботична мікроангіопатія de novo після трансплантації нирки | |
| zh | 肾移植后新发血栓性微血管病 | |
| tr | Böbrek nakli sonrası de novo trombotik mikroanjiyopati |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | M31.1 | NTBT | attributed | not yet validated |
| ICD-11 | NE8Y | NTBT | attributed | validated |
| UMLS | C5680696 | 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 (2):
- ORPHA:565779Rare disorder potentially indicated for transplant or complication after transplantation
rare transplant-related disorders - ORPHA:93573Thrombotic microangiopathy
rare renal diseases
No children.
Genes
2 direct| Gene | Association | Status | HGNC | Ensembl | OMIM | Locus | Name | Source of validation |
|---|---|---|---|---|---|---|---|---|
| CFH | Biomarker tested in | Assessed | HGNC:4883 | ENSG00000000971 | 134370 | 1q31.3 | complement factor H | |
| CFI | Biomarker tested in | Assessed | HGNC:5394 | ENSG00000205403 | 217030 | 4q25 | complement factor I |
Prevalence and incidence
1 record| Type | Class | Value | Area | Qualification | Validation | Source |
|---|---|---|---|---|---|---|
| Point prevalence | Unknown | — | Worldwide | 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.
HPO phenotypes
Orphanet has no HPO annotation for this code in this release.
Sources for this card
- Orphanet page: orpha.net/en/disease/detail/244275
- This record as JSON: /data/d/244275.json