ReviewOrphanet journal of rare diseases2015
Idiopathic non-cirrhotic portal hypertension: a review.
Review in Orphanet journal of rare diseases, 2015. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 62 papers, 2 of them syntheses that pooled it.
What it found
Each row is one number read from the abstract, on the scale the paper reported it, with its interval. Left of the dashed line favours the treatment, right favours the comparator. Under each row is the sentence it came from. New to these charts? A ten-minute tutorial.
The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.
The trial behind it
Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.
Neither the registry nor the abstract names a trial number. If this is a trial report, that itself is worth knowing.
Who cites it
62 citing papers in PubMed, 2 syntheses or guidelines pooled it, 120 citations in OpenAlex.
- Non-cirrhotic Idiopathic portal hypertension in systemic sclerosis patients: report of one case and a systematic review of previous case reports.Advances in rheumatology (London, England) · 2025Pooled it
- Nodular Regenerative Hyperplasia: Report of 82 Patients and Systematic Review of Literature.United European gastroenterology journal · 2024Pooled it
- Overview of Non-Cirrhotic Portal Hypertension in Pediatric Patients.Journal of clinical medicine · 2026Review
- Life-Threatening Variceal Bleeding in Antisynthetase Syndrome: Nodular Regenerative Hyperplasia and Porto-Sinusoidal Vascular Disorder.ACG case reports journal · 2026Article
- Diagnostic Pitfalls and Lessons Learned in Portal Hypertension.ACG case reports journal · 2026Article
- Pathophysiology of cystic fibrosis-related liver disease.Current opinion in gastroenterology · 2026Review
- Noncirrhotic Portal Hypertension in Systemic Lupus Erythematosus Complicated by Hepatic Microabscesses After Transjugular Intrahepatic Portosystemic Shunt.ACG case reports journal · 2026Article
- An unusual presentation of portal biliopathy manifesting as chronic abdominal pain as a delayed post-splenectomy complication: a case report.International journal of surgery case reports · 2026Article
- Case Report: Primary myelofibrosis presenting with non-cirrhotic portal hypertension and initially misdiagnosed as cirrhosis.Frontiers in medicine · 2026Article
- A rare case of splenic arteriovenous fistula causing portal hypertension, treated by embolisation.SA journal of radiology · 2026Article
- Utility of splenic transient elastography in assessing the presence of portal hypertension: A review.World journal of methodology · 2025Review
- Case Report: Partial Splenic Artery Embolization for the Treatment of Painful Splenomegaly Secondary to Noncirrhotic Portal Hypertension.Case reports in hepatology · 2025Article
- Case Report: Successful surgical intervention for portal hypertension caused by primary hypoplasia of the portal vein in a dog.Frontiers in veterinary science · 2025Article
- Epidemiological Study of Pathogens in Spontaneous Bacterial Peritonitis in 2017-2024-A Preliminary Report of the University Hospital in South-Eastern Poland.Microorganisms · 2024Article
- Article
- [Idiopathic portal hypertension: a case report].Zhonghua gan zang bing za zhi = Zhonghua ganzangbing zazhi = Chinese journal of hepatology · 2024Article
- Common variable immunodeficiency disorder-related liver disease is common and results in portal hypertension and an increased risk of death.Hepatology communications · 2024Article
- Management of liver disease and portal hypertension in common variable immunodeficiency (CVID).JHEP reports : innovation in hepatology · 2023Review
- A Narrative Review on Non-Cirrohotic Portal Hypertension: Not All Portal Hypertensions Mean Cirrhosis.Diagnostics (Basel, Switzerland) · 2023Review
- Molecular Mechanisms Underlying Vascular Liver Diseases: Focus on Thrombosis.International journal of molecular sciences · 2023Review
2 more citing papers are in PubMed but not listed here.
Corrections and comments
PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.
Authors and funding
3 authors at 3 institutions in 3 countries.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Idiopathic non-cirrhotic portal hypertension (INCPH) is a rare disease characterized of intrahepatic portal hypertension in the absence of cirrhosis or other causes of liver disease and splanchnic venous thrombosis. The etiology of INCPH can be classified in five categories: 1) immunological disorders (i.e. association with common variable immunodeficiency syndrome, connective tissue diseases, Crohn's disease, etc.), 2) chronic infections, 3) exposure to medications or toxins (e.g. azathioprine, 6- thioguanine, arsenic), 4) genetic predisposition (i.e. familial aggregation and association with Adams-Oliver syndrome and Turner disease) and 5) prothrombotic conditions (e.g. inherited thrombophilias myeloproliferative neoplasm antiphospholipid syndrome). Roughly, INCPH diagnosis is based on clinical criteria and the formal exclusion of any other causes of portal hypertension. A formal diagnosis is based on the following criteria: 1) presence of unequivocal signs of portal hypertension, 2) absence of cirrhosis, advanced fibrosis or other causes of chronic liver diseases, and 3) absence of thrombosis of the hepatic veins or of the portal vein at imaging. Patients with INCPH usually present with signs or symptoms of portal hypertension such as gastro-esophageal varices, variceal bleeding or splenomegaly. Ascites and/or liver failure can occur in the context of precipitating factors. The development of portal vein thrombosis is common. Survival is manly limited by concomitant disorders. Currently, treatment of INCPH relies on the prevention of complications related to portal hypertension, following current guidelines of cirrhotic portal hypertension. No treatment has been studied aimed to modify the natural history of the disease. Anticoagulation therapy can be considered in patients who develop portal vein thrombosis.
Indexed as
Identifiers
What OpenQuestion holds
Registered trials
Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.