Hepatoportal sclerosis
Synonyms: Obliterative portal venopathy
- Type
- Histopathological subtype
- Group
- Subtype of disorder
- Age of onset
- All ages
- Status
- active
A form of portosinusoidal vascular disease characterized histologically by varying degrees of phlebosclerosis, primarily involving the small and medium branches of the portal vein with heterogeneous distribution, in the absence of cirrhosis.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Hepatoportal sclerosis | Obliterative portal venopathy |
| fr | Veinopathie portale oblitérante | Sclérose hépatoportale |
| de | Hepatoportale Sklerose | Venopathie, obliterative portale |
| es | Esclerosis hepatoportal | Venopatía portal obliterante |
| it | Sclerosi epatoportale | Venopatia portale obliterativa |
| nl | Hepatoportale sclerose | Obliteratieve portale venopathie |
| pt | Esclerose hepato-portal | Fibrose portal não-cirrótica · Venopatia portal obliterante |
| pl | Stwardnienie wątrobowo-wrotne | = en |
| cs | Hepatoportální skleróza | Obliterující portální venopatie |
| uk | Гепатопортальний склероз | Облітеруюча портальна венопатія |
| zh | 肝门脉硬化症 | 闭塞性门静脉病变 |
| tr | Hepatoportal skleroz | Obliteratif portal venopati |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | K74.1 | NTBT | attributed | validated |
| MONDO | 0018991 | E | validated | |
| UMLS | C4273756 | 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:596937Portosinusoidal vascular disease
rare hepatic diseases
No children.
Genes
0 directOrphanet records no gene directly on this code.
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
30 annotations · validated 2021-09-14| HPO | Term (as annotated by Orphanet) | Diagnostic |
|---|---|---|
| Very frequent (99-80%) | ||
| HP:0001903 | Anemia | |
| HP:0001744 | Splenomegaly | |
| Frequent (79-30%) | ||
| HP:0001892 | Abnormal bleeding | |
| HP:0030146 | Abnormal liver parenchyma morphology | |
| HP:0006707 | Abnormality of the hepatic vasculature | |
| HP:0002040 | Esophageal varix | |
| HP:0030169 | Gastric varix | |
| HP:0001971 | Hypersplenism | |
| HP:0031015 | Intrahepatic portal vein sclerosis | |
| HP:0001882 | Leukopenia | |
| HP:0001409 | Portal hypertension | |
| HP:0008151 | Prolonged prothrombin time | |
| HP:0003262 | Smooth muscle antibody positivity | |
| Occasional (29-5%) | ||
| HP:0020136 | Anticardiolipin IgG antibody positivity | |
| HP:0020137 | Anticardiolipin IgM antibody positivity | |
| HP:0001541 | Ascites | |
| HP:0100543 | Cognitive impairment | |
| HP:0002910 | Elevated circulating hepatic transaminase concentration | |
| HP:0002239 | Gastrointestinal hemorrhage | |
| HP:0002480 | Hepatic encephalopathy | |
| HP:0002904 | Hyperbilirubinemia | |
| HP:0003073 | Hypoalbuminemia | |
| HP:0000952 | Jaundice | |
| HP:0011954 | Nodular regenerative hyperplasia of liver | |
| HP:0001405 | Periportal fibrosis | |
| HP:0030242 | Portal vein thrombosis | |
| HP:0000989 | Pruritus | |
| HP:0002719 | Recurrent infections | |
| HP:0001873 | Thrombocytopenia | |
| Very rare (<4-1%) | ||
| HP:0001402 | Hepatocellular carcinoma | |
Run the differential on the 2 obligate / very frequent terms
Annotation source: 27555800 [PMID]_24133357[PMID]_29043403[PMID]
Sources for this card
- Orphanet page: orpha.net/en/disease/detail/64743
- This record as JSON: /data/d/64743.json