Observational studyJournal of immunology research2014
Idiopathic non cirrhotic portal hypertension and spleno-portal axis abnormalities in patients with severe primary antibody deficiencies.
Observational study in Journal of immunology research, 2014. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 19 papers, 2 of them syntheses that pooled it.
What it found
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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.
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Who cites it
19 citing papers in PubMed, 2 syntheses or guidelines pooled it, 45 citations in OpenAlex.
- Non-cirrhotic portal fibrosis/idiopathic portal hypertension: APASL recommendations for diagnosis and management.Hepatology international · 2024Guideline
- Histopathological features of idiopathic portal hypertension: A systematic review and meta-analysis.Science progressPooled it
- Overview of Non-Cirrhotic Portal Hypertension in Pediatric Patients.Journal of clinical medicine · 2026Review
- Porto-sinusoidal Vascular Disease is Associated with Gastrointestinal Disorders in Common Variable Immunodefiency.Journal of clinical immunology · 2026Article
- Idiopathic portal hypertension misdiagnosed as hepatitis B cirrhosis: A case report and review of the literature.World journal of hepatology · 2025Article
- Common variable immunodeficiency disorder-related liver disease is common and results in portal hypertension and an increased risk of death.Hepatology communications · 2024Article
- Molecular Mechanisms Underlying Vascular Liver Diseases: Focus on Thrombosis.International journal of molecular sciences · 2023Review
- Portal hypertension in common variable immunodeficiency disorders - a single center analysis on clinical and immunological parameter in 196 patients.Frontiers in immunology · 2023Article
- Severe Ascites in Common Variable Immunodeficiency.Cureus · 2022Article
- Living donor liver transplantation for idiopathic portal hypertension with focal nodular hyperplasia.Surgical case reports · 2022Article
- Liver disease accompanied by enteropathy in common variable immunodeficiency: Common pathophysiological mechanisms.Frontiers in immunology · 2022Article
- Nodular regenerative hyperplasia in X-linked agammaglobulinemia: An underestimated and severe complication.The Journal of allergy and clinical immunology · 2022Article
- Idiopathic Non-Cirrhotic Portal Hypertension and Porto-Sinusoidal Vascular Disease: Review of Current Data.Gastroenterology research · 2021Review
- [Progress in the diagnosis and treatment of idiopathic non-cirrhotic portal hypertension].Zhonghua gan zang bing za zhi = Zhonghua ganzangbing zazhi = Chinese journal of hepatology · 2021Article
- Editorial: The Complexity of Primary Antibody Deficiencies.Frontiers in immunology · 2021Article
- Heterogeneity of Liver Disease in Common Variable Immunodeficiency Disorders.Frontiers in immunology · 2020Review
- Idiopathic portal hypertension and extrahepatic portal venous obstruction.Hepatology international · 2018Review
- Idiopathic noncirrhotic portal hypertension: current perspectives.Hepatic medicine : evidence and research · 2016Review
- Comprehensive Screening of Gene Function and Networks by DNA Microarray Analysis in Japanese Patients with Idiopathic Portal Hypertension.Mediators of inflammation · 2015Article
Corrections and comments
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Authors and funding
9 authors at 1 institution in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
BACKGROUND AND
aimPortal hypertension has been reported in association with acquired and primary immune deficiencies without a comprehensive description of associated spleno-portal axis abnormalities. Pathological mechanisms are poorly defined.
methodsObservational, single centre study with the aim of assessing the prevalence of spleno-portal axis abnormalities in an unselected cohort of 123 patients with primary antibody deficiencies and without known causes of liver diseases regularly followed up for a mean time of 18 ± 14 years. A cumulative period of 1867 patients-year was analysed. Clinical and immunological data, abdominal ultrasounds, CT scans, and endoscopy features were included in the analysis.
resultsTwenty-five percent of patients with primary antibody deficiencies had signs of portal vein enlargement but only 4% of them had portal hypertension, with portal systemic collaterals. Liver biopsies showed liver sinusoids congestive dilatation, endothelization, and micronodularity fulfilling the criteria for noncirrhotic portal hypertension. Patients with portal vein enlargement had severe clinical and immunological phenotypes.
conclusionsIn primary antibody deficient patients, infections, inflammations, splenomegaly, increased blood venous flow, and lymphocyte abnormalities contribute to establishment of liver damage possibly leading to noncirrhotic portal hypertension. Patients with primary antibody deficiency should be considered a good model to give insight into the pathological mechanisms underlying noncirrhotic portal hypertension.
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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.