ORPHA:48372
Nodular regenerative hyperplasia of the liver
Synonyms: Non-cirrhotic nodulation
- Type
- Histopathological subtype
- Group
- Subtype of disorder
- Inheritance
- Not applicable
- Age of onset
- Adult
- Status
- active
A form of portosinusoidal vascular disease characterized histologically by the absence of cirrhosis and diffuse benign transformation of the hepatic parenchyma into multiple small nodules (typically 1-3 mm).
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Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Nodular regenerative hyperplasia of the liver | Non-cirrhotic nodulation |
| fr | Hyperplasie nodulaire régénérative du foie | Nodules d'origine non cirrhotique · Transformation nodulaire du foie |
| de | Noduläre regenerative Hyperplasie der Leber | Nicht-zirrhotische Nodulation |
| es | Hiperplasia nodular regenerativa del hígado | Nódulo no cirrótico |
| it | Iperplasia rigenerativa nodulare del fegato | Nodulazione non cirrotica |
| nl | Nodulaire regeneratieve hyperplasie van lever | Niet-cirrotische nodulatie |
| pt | Hiperplasia hepática regenerativa nodular | Hipertensão portal idiopática não-cirrótica · Nodularidade não-cirrótica |
| pl | Ogniskowy rozrost guzkowy wątroby | = en |
| cs | Nodulární regenerativní hyperplázie jater | Non-cirhotická nodulace |
| uk | Вузлова регенеративна гіперплазія печінки | Нециротична нодуляція |
| zh | 肝脏结节性再生性增生 | 非肝硬化性肝脏结瘤 |
| tr | Karaciğerin nodüler rejeneratif hiperplazisi | Sirotik olmayan nodülasyon |
Definition in:
2
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | K76.8 | NTBT | attributed | validated |
| ICD-11 | DB98.2 | E | specific | validated |
| MONDO | 0018835 | E | validated | |
| UMLS | C5779783 | E | validated | |
| GARD | 10929 | E | validated |
E exact · NTBT this ORPHAcode is narrower than the target · BTNT broader than the target · ND not decided.
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Classification
Preferential parent (Orphanet linearisation):
Parents in the classifications (2):
- ORPHA:596937Portosinusoidal vascular disease
rare hepatic diseases - ORPHA:506210Rare disorder potentially indicated for liver transplant
rare transplant-related disorders
No children.
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Genes
0 directOrphanet records no gene directly on this code.
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Prevalence and incidence
3 records| Type | Class | Value | Area | Qualification | Validation | Source |
|---|---|---|---|---|---|---|
| Annual incidence | 1-9 / 1 000 000 | 0.34 / 100 000 | Spain | Value and class | Validated | 2586240[PMID] |
| Point prevalence | 1-9 / 100 000 | 3.1 / 100 000 | Spain | Value and class | Validated | 2586240[PMID] |
| 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.
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HPO phenotypes
2 annotations · validated 2016-06-01| HPO | Term (as annotated by Orphanet) | Diagnostic |
|---|---|---|
| Very frequent (99-80%) | ||
| HP:0006707 | Abnormality of the hepatic vasculature | |
| Occasional (29-5%) | ||
| HP:0001409 | Portal hypertension | |
Run the differential on the 1 obligate / very frequent terms
Annotation source: 26415006[PMID]
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Sources for this card
- Orphanet page: orpha.net/en/disease/detail/48372
- This record as JSON: /data/d/48372.json