ArticleDigestive diseases and sciences2011
Natural history and outcome of hepatic vascular malformations in a large cohort of patients with hereditary hemorrhagic teleangiectasia.
Article in Digestive diseases and sciences, 2011. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 59 papers.
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Who cites it
59 citing papers in PubMed, 127 citations in OpenAlex.
- Can Iron Treatments Aggravate Epistaxis in Some Patients With Hereditary Hemorrhagic Telangiectasia?The Laryngoscope · 2016Trial
- Complications and clinical outcome of hepatic artery embolisation in patients with hereditary haemorrhagic telangiectasia.European radiology · 2013Trial
- Incidence and diagnostic yield of repeat imaging-based screening for hepatic arteriovenous malformations in patients with HHT.Hepatology communications · 2026Article
- Haemodynamic alterations associated with hepatic vascular malformations in hereditary haemorrhagic telangiectasia: A cohort study.JHEP reports : innovation in hepatology · 2026Article
- Targeting stiffness-dependent YAP/TAZ restores angiogenesis dynamics impaired by ALK1 knockout in silico.PLoS computational biology · 2026Article
- Availability, use, efficacy and safety of bevacizumab in European hereditary haemorrhagic telangiectasia centres.British journal of clinical pharmacology · 2026Article
- Unmasking a Rare Genetic Puzzle: Hereditary Hemorrhagic Telangiectasia in a Black Kenyan Woman: A Case Report.Case reports in gastrointestinal medicine · 2026Article
- Epistaxis Prevention, Treatment, and Future Perspectives for Hereditary Hemorrhagic Telangiectasia.Journal of clinical medicine · 2025Review
- Hereditary haemorrhagic telangiectasia.Nature reviews. Disease primers · 2025Review
- Liver Transplantation Using a Whole-Liver Graft Donated from a Brain-Dead Donor Complicated with Rendu-Osler-Weber Syndrome: A Case Report.Surgical case reports · 2025Article
- Liver transplantation for hepatocellular carcinoma in a patient with hereditary hemorrhagic telangiectasia: a case report.Frontiers in surgery · 2025Article
- Pulmonary vascular manifestations of hereditary haemorrhagic telangiectasia.Pulmonary circulation · 2024Review
- Ischemic cholangitis: Lethal complication of Osler-Weber-Rendu disease.Radiology case reports · 2024Article
- Pathogenic Variant Frequencies in Hereditary Haemorrhagic Telangiectasia Support Clinical Evidence of Protection from Myocardial Infarction.Journal of clinical medicine · 2023Article
- Pharmacogenomic Considerations for Anticoagulant Prescription in Patients with Hereditary Haemorrhagic Telangiectasia.Journal of clinical medicine · 2023Article
- Gastrointestinal bleeding in patients with hereditary hemorrhagic telangiectasia: Long-term results of endoscopic treatment.Endoscopy international open · 2023Article
- Hepatic Vascular Variants in Hereditary Haemorrhagic Telangiectasia: Imaging findings.Sultan Qaboos University medical journal · 2023Article
- Article
- Current guidelines for diagnosis and management of hepatic involvement in hereditary hemorrhagic teleangiectasia.World journal of hepatology · 2023Review
- Obstetrical and neonatal complications in hereditary haemorrhagic telangiectasia: A retrospective study.BJOG : an international journal of obstetrics and gynaecology · 2023Article
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Authors and funding
23 authors at 10 institutions in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
backgroundHereditary hemorrhagic telangiectasia is a genetic disease characterized by teleangiectasias involving virtually every organ. There are limited data in the literature regarding the natural history of liver vascular malformations in hemorrhagic telangiectasia and their associated morbidity and mortality.
aimThis prospective cohort study sought to assess the outcome of liver involvement in hereditary hemorrhagic telangiectasia patients.
methodsWe analyzed 16 years of surveillance data from a tertiary hereditary hemorrhagic telangiectasia referral center in Italy. We considered for inclusion in this study 502 consecutive Italian patients at risk of hereditary hemorrhagic telangiectasia who presented at the hereditary hemorrhagic telangiectasia referral center and underwent a multidisciplinary screening protocol for the diagnosis of hereditary hemorrhagic telangiectasia. Of the 502 individuals assessed in the center, 154 had hepatic vascular malformations and were the subject of the study; 198 patients with hereditary hemorrhagic telangiectasia and without hepatic vascular malformations were the controls. Additionally, we report the response to treatment of patients with complicated hepatic vascular malformations.
resultsThe 154 patients were included and followed for a median period of 44 months (range 12-181); of these, eight (5.2%) died from VM-related complications and 39 (25.3%) experienced complications. The average incidence rates of death and complications were 1.1 and 3.6 per 100 person-years, respectively. The median overall survival and event-free survival after diagnosis were 175 and 90 months, respectively. The rate of complete response to therapy was 63%.
conclusionsThis study shows that substantial morbidity and mortality are associated with liver vascular malformations in hereditary hemorrhagic telangiectasia patients.
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