ArticleOrphanet journal of rare diseases2020
Gender differences in hereditary hemorrhagic telangiectasia severity.
Article in Orphanet journal of rare diseases, 2020. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 23 papers.
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Who cites it
23 citing papers in PubMed, 29 citations in OpenAlex.
- Ruptured pulmonary arteriovenous malformation causing hemothorax during pregnancy: a case report.Journal of cardiothoracic surgery · 2026Article
- Genotype-driven cerebrovascular risk across age and sex in hereditary hemorrhagic telangiectasia.Neurological sciences : official journal of the Italian Neurological Society and of the Italian Society of Clinical Neurophysiology · 2026Article
- Validation and clinical application of the hereditary hemorrhagic telangiectasia-specific quality of life scale.Journal of thrombosis and haemostasis : JTH · 2026Article
- Endoscopic Vascular Grading Directs Stepwise Therapy in Hereditary Hemorrhagic Telangiectasia.Laryngoscope investigative otolaryngology · 2025Article
- Assessing racial differences in North American hereditary hemorrhagic telangiectasia study recruitment and care.Orphanet journal of rare diseases · 2025Article
- Quantifying the burden of hereditary hemorrhagic telangiectasia on quality of life and psychological health: a cross-sectional study.Orphanet journal of rare diseases · 2025Article
- Minimal encephalopathy in hereditary hemorrhagic telangiectasia patients with portosystemic vascular malformations.Orphanet journal of rare diseases · 2024Observational
- Diagnostic and Prognostic Value of Angiogenic Status in Hereditary Hemorrhagic Telangiectasia.Diagnostics (Basel, Switzerland) · 2024Article
- Pulmonary vascular manifestations of hereditary haemorrhagic telangiectasia.Pulmonary circulation · 2024Review
- Hereditary hemorrhagic telangiectasia may be the most morbid inherited bleeding disorder in women.Blood advances · 2024Observational
- [Prevalencia de la Telangiectasia Hemorrágica Hereditaria: estudio de base poblacional en la Comunitat Valenciana (España).]Revista espanola de salud publica · 2023Article
- Sex-biased TGFβ signalling in pulmonary arterial hypertension.Cardiovascular research · 2023Review
- Current guidelines for diagnosis and management of hepatic involvement in hereditary hemorrhagic teleangiectasia.World journal of hepatology · 2023Review
- Improving Hereditary Hemorrhagic Telangiectasia Molecular Diagnosis: A Referral Center Experience.Genes · 2023Article
- Perioperative Complications and Long-Term Follow-Up of Liver Transplantation in Hemorrhagic Hereditary Telangiectasia: Report of Three Cases and Systematic Review.Journal of clinical medicine · 2022Review
- HHT-Related Epistaxis and Pregnancy-A Retrospective Survey and Recommendations for Management from an Otorhinolaryngology Perspective.Journal of clinical medicine · 2022Article
- Rethinking growth factors: the case of BMP9 during vessel maturation.Vascular biology (Bristol, England) · 2022Review
- Case report: Diagnosis of hereditary hemorrhagic telangiectasia (Osler Weber Rendu Syndrome) in a 23-year-old male presented with anemia and thrombocytopenia and its response to bevacizumab.Frontiers in medicine · 2022Article
- Hereditary haemorrhagic telangiectasia and pulmonary arteriovenous malformations.BMJ case reports · 2021Article
- Pulmonary Arteriovenous Malformation in a Patient with Suspected Hereditary Hemorrhagic Telangiectasia: A Case Report.Journal of education & teaching in emergency medicine · 2021Article
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Authors and funding
11 authors at 1 institution in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
backgroundGender differences in organ involvement and clinical severity have been poorly described in hereditary hemorrhagic telangiectasia (HHT). The aim of this study was to describe differences in the severity of HHT manifestations according to gender.
methodsSeverity was measured according to Epistaxis Severity Score (ESS), Simple Clinical Scoring Index for hepatic involvement, a general HHT-score, needing for invasive treatment (pulmonary or brain arteriovenous malformations -AVMs- embolization, liver transplantation or Young's surgery) or the presence of adverse outcomes (severe anemia, emergency department -ED- or hospital admissions and mortality).
resultsOne hundred forty-two (58.7%) women and 100 (41.3%) men were included with a mean age of 48.9 ± 16.6 and 49 ± 16.5 years, respectively. Women presented hepatic manifestations (7.1% vs 0%) and hepatic involvement (59.8% vs 47%), hepatic AVMs (28.2% vs 13%) and bile duct dilatation (4.9% vs 0%) at abdominal CT, and pulmonary AVMs at thoracic CT (35.2% vs 23%) more often than men. The Simple Clinical Scoring Index was higher in women (3.38 ± 1.2 vs 2.03 ± 1.2), and more men were considered at low risk of harboring clinically significant liver disease than women (61% vs 25.3%). These differences were mantained when considering HHT1 and HHT2 patients separetely. Duodenal telangiectasia were more frequent in men than women (21% vs 9.8%). Invasive treatments were more frequently needed in women (28.2% vs 16%) but men needed attention at the ED more often than women (48% vs 28.2%), with no differences in ESS, HHT-score, anemia hospital admissions or mortality.
conclusionsHHT women showed more severe hepatic involvement than men, also among HHT1 and HHT2 patients. Women had higher prevalence of pulmonary AVMs and needed invasive procedures more frequently, while men needed attention at the ED more often. These data might help physicians to individualize HHT patients follow-up.
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