ArticleJournal of clinical medicine2020
Hereditary Hemorrhagic Telangiectasia (HHT) and Survival: The Importance of Systematic Screening and Treatment in HHT Centers of Excellence.
Article in Journal of clinical medicine, 2020. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 19 papers.
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Who cites it
19 citing papers in PubMed, 33 citations in OpenAlex.
- Expected survival is decreased in hereditary hemorrhagic telangiectasia: Results from a population-based registry study.Journal of internal medicine · 2026Article
- Imaging features, diagnosis, and clinical management of hepatic involvement in hereditary hemorrhagic telangiectasia: a case series report and literature review.Frontiers in medicine · 2026Article
- A case of hereditary hemorrhagic telangiectasia with hypoxemia onset in the neonatal period.BMC pulmonary medicine · 2025Article
- Imaging manifestations of hereditary hemorrhagic telangiectasia with pulmonary arterial hypertension: a case report.Frontiers in cardiovascular medicine · 2025Article
- Updates on the Pathogenesis and Therapeutic Approaches for Hereditary Hemorrhagic Telangiectasia.Current vascular pharmacology · 2025Review
- Brain and lung arteriovenous malformation rescreening practices for children and adults with hereditary hemorrhagic telangiectasia.Orphanet journal of rare diseases · 2024Article
- Hereditary Hemorrhagic Telangiectasia (Osler's Disease): Systemic, Interdisciplinary, Relatively Common—and Often Missed.Deutsches Arzteblatt international · 2024Review
- Outcomes of patients with Juvenile Polyposis-Hereditary Haemorrhagic Telangiectasia caused by pathogenic SMAD4 variants in a pan-Scotland cohort.European journal of human genetics : EJHG · 2024Article
- Updates on diagnostic criteria for hereditary haemorrhagic telangiectasia in the light of whole genome sequencing of 'gene-negative' individuals recruited to the 100 000 Genomes Project.Journal of medical genetics · 2024Article
- Pathogenic Variant Frequencies in Hereditary Haemorrhagic Telangiectasia Support Clinical Evidence of Protection from Myocardial Infarction.Journal of clinical medicine · 2023Article
- Hereditary Hemorrhagic Telangiectasia: Genetics, Pathophysiology, Diagnosis, and Management.Journal of clinical medicine · 2022Review
- The Impact on COVID-19 by Intravenous Bevacizumab Used for Hereditary Hemorrhagic Telangiectasia: A Case Report.Journal of medical cases · 2022Article
- A Rare Case of Upper Gastrointestinal Bleeding: Osler-Weber-Rendu Syndrome.Medicina (Kaunas, Lithuania) · 2022Article
- Review
- Recurrent erysipelas led to diagnosis of hereditary hemorrhagic telangiectasia.Le infezioni in medicina · 2022Article
- Imaging to intervention: a review of what the Interventionalist needs to Know about Hereditary Hemorrhagic Telangiectasia.CVIR endovascular · 2021Review
- Mutational and clinical spectrum of Japanese patients with hereditary hemorrhagic telangiectasia.BMC medical genomics · 2021Article
- Hereditary Hemorrhagic Telangiectasia: Success of the Osler Calendar for Documentation of Treatment and Course of Disease.Journal of clinical medicine · 2021Article
- Arteriovenous Malformations-Current Understanding of the Pathogenesis with Implications for Treatment.International journal of molecular sciences · 2021Review
Corrections and comments
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Authors and funding
7 authors at 3 institutions in 3 countries.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Hereditary hemorrhagic telangiectasia (HHT), an autosomal dominant disease, is characterized by telangiectases and arteriovenous malformations (AVMs). Untreated AVMs, especially in the lungs-pulmonary AVMs (PAVMs)-can result in morbidity with a decreased life expectancy. We have investigated whether HHT patients, systematically screened for HHT-related organ involvement and treated if needed, have a similar survival as persons without HHT. We included all individuals screened for HHT between 2004 and 2016 with a genetically or clinically confirmed diagnosis (HHT group) or excluded diagnosis (non-HHT control group). The social security number was used to confirm status as dead or alive in December 2019. We included 717 HHT patients and 471 controls. There was no difference in survival between the HHT and the non-HHT control group. The HHT group had a life expectancy of 75.9 years (95% confidence interval (CI) 73.3-78.6), comparable to the control group (79.3 years, 95% CI 74.8-84.0, Mantel-Cox test:
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