Autosomal dominant progressive nephropathy with hypertension
- Type
- Disease
- Group
- Disorder
- Inheritance
- Autosomal dominant
- Age of onset
- Adult
- Status
- active
A rare, genetic hypertension characterized by an adult onset of increased blood pressure associated with nephropathy progressing to end-stage renal disease. Renal biopsy may show interstitial fibrosis, glomerulosclerosis and mild tubular atrophy. Increased serum creatinine and proteinuria have also been reported.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Autosomal dominant progressive nephropathy with hypertension | |
| fr | Néphropathie progressive avec hypertension autosomique dominante | |
| de | Nephropathie, progressive mit Hypertension, autosomal-dominante Form | |
| es | Nefropatía progresiva con hipertensión autosómica dominante | |
| it | Nefropatia progressiva con ipertensione autosomica dominante | |
| nl | Autosomaal dominante progressieve nefropathie met hypertensie | |
| pt | Nefropatia progressiva com hipertensão autossómica dominante | |
| pl | Autosomalna dominująca prostępująca nefropatia z nadciśnieniem | |
| cs | Autozomálně dominantní progresivní nefropatie s hypertenzí | |
| uk | Аутосомно-домінантна прогресуюча нефропатія з гіпертензією | |
| zh | 常染色体显性遗传进行性肾病伴高血压 | |
| tr | Hipertansiyon ile seyreden otozomal dominant ilerleyici nefropati |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| OMIM | 161900 | E | validated | |
| ICD-10 | I15.1 | NTBT | attributed | validated |
| ICD-11 | BA04.Y | NTBT | index | validated |
| MONDO | 0008071 | E | validated | |
| UMLS | C3839782 | 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:506213Rare disorder potentially indicated for kidney transplant
rare transplant-related disorders - ORPHA:156629Rare genetic cause of hypertension
rare genetic diseases; rare renal diseases
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 | 14 cases | Worldwide | Case(s) | Validated | 10930359 [PMID] | |
| Point prevalence | <1 / 1 000 000 | — | Worldwide | Class only | Validated | ORPHANET_10930359 [PMID] |
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/88659
- This record as JSON: /data/d/88659.json