hepatoportal sclerosis
MONDO:0018991Mondo
Findings
No curated finding names hepatoportal sclerosis yet. Everything below is reference data from Mondo, HPO and GenCC, not evidence about what affects it.
Definition
Hepatoportal sclerosis (HPS) is a rare disorder characterized by sclerosis of the intrahepatic portal veins, non-cirrhotic portal hypertension, asymptomatic splenomegaly and recurrent variceal bleeding.
Definition from the Mondo Disease Ontology (MONDO:0018991), read 2026-09-29. CC BY 4.0.
Features
30 features
What the disease is reported to present with, and how often among people who have it — never how often a feature means the disease. HPO, annotations 2026-09-02.
- AnemiaHPOHP:0001903
- Very frequent (80% to 99% of cases)
- SplenomegalyHPOHP:0001744
- Very frequent (80% to 99% of cases)
- Abnormal bleedingHPOHP:0001892
- Frequent (30% to 79% of cases)
- Abnormal liver parenchyma morphologyHPOHP:0030146
- Frequent (30% to 79% of cases)
- Abnormality of the hepatic vasculatureHPOHP:0006707
- Frequent (30% to 79% of cases)
- Anti-smooth muscle antibody positivityHPOHP:0003262
- Frequent (30% to 79% of cases)
- Decreased total leukocyte countHPOHP:0001882
- Frequent (30% to 79% of cases)
- Esophageal varixHPOHP:0002040
- Frequent (30% to 79% of cases)
- Gastric varixHPOHP:0030169
- Frequent (30% to 79% of cases)
- HypersplenismHPOHP:0001971
- Frequent (30% to 79% of cases)
- Intrahepatic portal vein sclerosisHPOHP:0031015
- Frequent (30% to 79% of cases)
- Portal hypertensionHPOHP:0001409
- Frequent (30% to 79% of cases)
Show the remaining 18
- Prolonged prothrombin timeHPOHP:0008151
- Frequent (30% to 79% of cases)
- Anticardiolipin IgG antibody positivityHPOHP:0020136
- Occasional (5% to 29% of cases)
- Anticardiolipin IgM antibody positivityHPOHP:0020137
- Occasional (5% to 29% of cases)
- AscitesHPOHP:0001541
- Occasional (5% to 29% of cases)
- Cognitive impairmentHPOHP:0100543
- Occasional (5% to 29% of cases)
- Elevated circulating hepatic transaminase concentrationHPOHP:0002910
Where it sits
- A kind of
Other names
1 name
Resolves to: hepatoportal sclerosis
- Also called
- obliterative portal venopathy