hepatoportal sclerosis
ORPHA: 64743
Overview
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
Available Treatments (0)
No treatments linked yet
Clinical trials or compassionate use may be available — consult a specialist.
Clinical Presentation
Signs and symptoms associated with hepatoportal sclerosis, sourced from HPO and Orphanet clinical annotations.
Prolonged prothrombin timeAbnormal liver parenchyma morphologyGastric varixIntrahepatic portal vein sclerosisJaundicePruritusPeriportal fibrosisAscitesThrombocytopeniaGastrointestinal hemorrhageHepatic encephalopathyRecurrent infectionsHyperbilirubinemiaElevated circulating hepatic transaminase concentrationHypoalbuminemiaNodular regenerative hyperplasia of liverAnticardiolipin IgG antibody positivityAnticardiolipin IgM antibody positivityPortal vein thrombosisCognitive impairmentHepatocellular carcinomaSplenomegalyAnemiaPortal hypertensionDecreased total leukocyte countAbnormal bleedingHypersplenismEsophageal varixAnti-smooth muscle antibody positivityAbnormality of the hepatic vasculature
Classification & Codes
Orphanet Code
ORPHA:64743hepatoportal sclerosis
| Orphanet | ORPHA:64743 |
| Treatments | 0 drug(s) |
| Symptoms on record | 30 signs |
| Status | published |
Factual Authority
Last Updated3/20/2026
Clinical DataHPO · Orphanet
Drug DataFDA · EMA · CDSCO