RARE DISEASERESEARCH ATLAS

ORPHA:64743

Hepatoportal sclerosis

high confidenceSubtype of disorder

Also known as: Obliterative portal venopathy

Publications

573

85.8th percentile

Trials

1

Interventional, condition-specific

Researchers

1,123

Distinct authors in sample

Gene link

Readiness

2/6

Stages with a signal

Clinical definition (Orphanet)

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.

How rare: How common this is has not been clearly measured.

Orphanet entry

Cross-references

Joined from Mondo / Orphanet. MeSH labels may enter searches; UMLS / OMIM / NCIT are stored for reference.

Additional Mondo synonyms (1)

obliterative portal venopathy

Research stages

Trial readiness signals

Where this condition sits on an open-data research pipeline — not how close a treatment is, and not medical advice. Empty stages often mean “not in these databases under this Mondo ID,” not “impossible.”

2/6 stages with a signal

An interventional trial matched this condition name on ClinicalTrials.gov — see trials below.

  1. Gene identifiedNot found

    No GenCC disease–gene assertion in this build

  2. LiteraturePresent

    573 matched papers (324 in last 10 years) Source

  3. Phenotype characterisedNot checked

    Not yet enriched from Monarch / HPO

  4. Animal modelNot checked

    Not yet enriched from Monarch / Alliance

  5. Orphan designationNot checked

    FDA/EMA orphan-drug designation not enriched yet

  6. Interventional trialPresent

    1 matched on ClinicalTrials.gov

Biology

Genes and phenotypes

Gene–disease validity from GenCC, plus phenotypes and animal models joined from Monarch Initiative via Mondo ID — not a clinical diagnosis aid.

Do we know what causes it?

Not yet — the cause hasn't been pinned down in GenCC.

No strong gene–disease assertion joined for this Orphanet entity.

Phenotypes (Monarch / HPO)

Not enriched in this build — Monarch phenotype joins were not run for this record.

Animal models (Monarch / Alliance)

Not enriched in this build.

Literature

Is anyone studying this?

573

573 papers have been published on this condition. That is a real research literature — still far smaller than common diseases (breast cancer: over 700,000 papers). Median papers in the last 10 years for a rare disease in this dataset (publications denominator n=5449) is 41.

573 papers since the earliest indexed year in this search — median last-10-year count for a rare disease in this dataset is 41 (publications denominator n=5449).

324 in the last 10 years · high confidence · 85.8th percentile (publications denominator)

Phrase hits: 573 · MeSH hits: 0

Open Europe PMC search

Who's working on it?

1,123

Distinct author names in 200 sampled papers — named people below.

Who's working on it?

People publishing on this condition (sampled Europe PMC records). Affiliation is the most recent found in that sample.

  1. 01
    Fiel MI14 papers · 2025

    Department of Pathology , Mount Sinai School of Medicine , New York City , New York , USA.

    Papers in Europe PMC
  2. 02
    Kleiner DE10 papers · 2026

    National Cancer Institute, Bethesda, MD, USA.

    Papers in Europe PMC
  3. 03
    Heller T9 papers · 2026

    Liver Diseases Branch, National Institute of Diabetes and Digestive and Kidney Diseases, National Institutes of Health, Bethesda, Maryland, USA.

    Papers in Europe PMC
  4. 04
    Paradis V9 papers · 2026

    Department of Pulmonology, Centre de Resources et de Compétence de la Mucoviscidose, Hôpital Foch, Suresnes, France.

    Papers in Europe PMC
  5. 05
    Rautou PE9 papers · 2026

    Service d'Hépatologie, DMU DIGEST, Centre de Référence des Maladies Vasculaires du Foie, FILFOIE, ERN RARE-LIVER, Centre de Recherche sur l'inflammation, Inserm, UMR 1149, Université de Paris, AP-HP, Hôpital Beaujon, Paris, France.

    Papers in Europe PMC
  6. 06
    Schiano TD9 papers · 2025

    Department of Medicine, Division of Liver Diseases, Recanati/Miller Transplantation Institute, Icahn School of Medicine at Mount Sinai, New York, NY, USA.

    Papers in Europe PMC
  7. 07
    Schinoni MI7 papers · 2026

    Federal University of Bahia, School of Medical Sciences, Salvador, Bahia, Brazil.

    Papers in Europe PMC
  8. 08
    Sempoux C7 papers · 2026

    Service d'Anatomie pathologique, Cliniques universitaires Saint-Luc, Avenue Hippocrate, 10, B-1200 Brussels, Belgium.

    Papers in Europe PMC
  9. 09
    Elkrief L6 papers · 2026

    Service d'Hépato-gastroentérologie, Hôpitaux Universitaires de Genève, Geneva, Switzerland.

    Papers in Europe PMC
  10. 10
    Gioia S6 papers · 2025

    Dipartimento di Medicina Traslazionale e di Precisione, "Sapienza" Università di Roma, Roma 00185, Italy, stefania.gioia@uniroma1.it.

    Papers in Europe PMC

Clinical research

Is a treatment being tested?

1

interventional trials for this specific condition

1 interventional trial matched this specific condition name; none in our sample are currently recruiting.

Data as of 27 July 2026

1 interventional trial — more than 73% of diseases in the trials denominator have none at all (5114 of 7003; this disease is at the 76.8th percentile).

high confidence · 76.8th percentile (trials denominator)

Recruiting interventional trials

From the matched ClinicalTrials.gov set

1 interventional trials matched after quoted-phrase search and title/condition post-filter.

No currently recruiting studies in the matched set. Open the same search on ClinicalTrials.gov.

Where to find support

We do not yet link condition-specific patient organisations. These umbrella groups support undiagnosed and ultra-rare families:

India — NPRD

Last verified 2026-07-26

This ORPHAcode is not on our curated NPRD list (direct or Mondo-parent match). That does not decide clinical eligibility; families in India should ask a notified Centre of Excellence about current coverage.

Hand-curated for this project. ORPHAcode mappings are best-effort and may be incomplete or imprecise for umbrella categories. Parent (Mondo) matches mean the policy lists a broader category — confirm eligibility with a Centre of Excellence. Financial entitlements summarised from public policy statements and may change. This is not official government guidance.

How we counted this

Europe PMC query (preferred label + any corrected label + Orphanet and Mondo exact synonyms, stoplisted; unioned with resolved MeSH labels when available). UMLS / OMIM / NCIT cross-references are stored on the overview but are not added to the query string.

"Hepatoportal sclerosis" OR "Obliterative portal venopathy"

Run this search on Europe PMC

ClinicalTrials.gov query (quoted phrases + MeSH via query.cond, plus recall-expansion terms when used):

"Hepatoportal sclerosis" OR "Obliterative portal venopathy"

Interventional trials matched via: phrase (mesh = registered under a MeSH descriptor no name phrase would catch; recall-expansion = gene / selected parent terms used only for trials).

Study-type breakdown: 1 interventional · 0 observational · 0 expanded access. Only interventional studies enter the trial headline.

Query health: ok — strategies attempted: phrase; with hits: phrase

Run this search on ClinicalTrials.gov

Confidence reasoning

  • Preferred label is multi-word and distinctive
  • No synonyms dropped by stoplist
  • No label/synonym collisions with other diseases in this corpus

Ingested 2026-07-27T01:11:35.660Z