RARE DISEASERESEARCH ATLAS

ORPHA:596941

Incomplete septal cirrhosis

high confidenceSubtype of disorder

Also known as: Incomplete septal fibrosis

Publications

185

68.7th percentile

Trials

1

Interventional, condition-specific

Researchers

1,110

Distinct authors in sample

Gene link

Readiness

2/6

Stages with a signal

Clinical definition (Orphanet)

A histopathological form of portosinusoidal vascular disease characterized by the presence of incomplete, thin, perforated, or blind-ended septa, which intermittently delimit rudimentary nodules, although complete cirrhotic-type regenerative nodules are not seen. Isolated collagen bundles can also be observed within the parenchyma.

Orphanet entry

Cross-references

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

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

    185 matched papers (114 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?

185

185 papers have ever been published on this condition (under this name). For scale, breast cancer has over 700,000. Median papers in the last 10 years for a rare disease in this dataset (publications denominator n=5449) is 41.

185 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).

114 in the last 10 years · high confidence · 68.7th percentile (publications denominator)

Phrase hits: 185 · MeSH hits: 0

Open Europe PMC search

Who's working on it?

1,110

Distinct author names in 185 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
    Bioulac-Sage P5 papers · 2019

    Inserm U1053, Université de Bordeaux, Bordeaux, France.

    Papers in Europe PMC
  2. 02
    Dhiman RK5 papers · 2024

    Department of Hepatology, Postgraduate Institute of Medical Education and Research, Chandigarh, India. rkpsdhiman@hotmail.com

    Papers in Europe PMC
  3. 03
    Fiel MI5 papers · 2025

    Department of Pathology, The Mount Sinai Medical Center, New York, NY, USA.

    Papers in Europe PMC
  4. 04
    Rautou PE5 papers · 2026

    Service d'Hépatologie, Hôpital Beaujon, Clichy, France; Centre de Recherche de l'Inflammation, Inserm and Université Paris Diderot, Paris, France.

    Papers in Europe PMC
  5. 05
    Sarin SK5 papers · 2025

    Department of Gastroenterology, G B Pant Hospital, University of Delhi, Room 201, Academic Block, New Delhi, 110 002, India, sksarin@nda.vsnl.net.in.

    Papers in Europe PMC
  6. 06
    Valla D5 papers · 2026

    Service d'Hépatologie, Hôpital Beaujon, Clichy, France; Centre de Recherche de l'Inflammation, Inserm and Université Paris Diderot, Paris, France.

    Papers in Europe PMC
  7. 07
    Bernard PH4 papers · 2000

    Unité Fonctionnelle d'Hépatologie, Hôpital Saint-André, Bordeaux.

    Papers in Europe PMC
  8. 08
    Fevery J4 papers · 1999
    Papers in Europe PMC
  9. 09
    Gioia S4 papers · 2025

    Department of Translational and Precision Medicine, Sapienza University of Rome, Rome, Italy.

    Papers in Europe PMC
  10. 10
    Jain D4 papers · 2025

    Department of Pathology, Yale School of Medicine, New Haven, CT.

    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.

"Incomplete septal cirrhosis" OR "Incomplete septal fibrosis"

Run this search on Europe PMC

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

"Incomplete septal cirrhosis" OR "Incomplete septal fibrosis"

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 2026-07-27T18:54:29.678Z