Rare diseases · Sign or symptom
Portal hypertension
HP:0001409
What it means
Increased pressure in the portal vein.
Portal hypertension is defined as portal vein pressures exceeding 5 mm Hg or portal vein to hepatic vein gradient of greater than 10 mm Hg.
Rare diseases that can present with this33
Very common80–99%
2Common30–79%
10- Autosomal recessive polycystic kidney disease
- Cirrhosis-dystonia-polycythemia-hypermanganesemia syndrome
- Enteric anendocrinosis
- Hepatoportal sclerosis
- Hereditary hemorrhagic telangiectasia
- Isolated sedoheptulokinase deficiency
- MPI-CDG
- Primary biliary cholangitis
- Primary sclerosing cholangitis
- Senior-Boichis syndrome
The range is how often this sign appears among people diagnosed with each disease — it is not a probability of having any of them. Many of these signs also occur in common, non-rare conditions. A sign on this page is a conversation to have with a clinician, not a conclusion.
Part of the broader category
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Start free with EleplanThis page uses the Human Phenotype Ontology (2026-06-23) — Human Phenotype Ontology Consortium, hpo.jax.org. Disease associations from Orphanet (INSERM), CC BY 4.0. Content is educational and is not medical advice.