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dc.contributor.authorMora-Luján, José María
dc.contributor.authorIriarte, Adriana
dc.contributor.authorAlba, E.
dc.contributor.authorSánchez-Corral, M. A.
dc.contributor.authorCerdà, Pau
dc.contributor.authorCruellas, F.
dc.contributor.authorOrdi, Q.
dc.contributor.authorCorbella Virós, Xavier
dc.contributor.authorRibas, Jesús
dc.contributor.authorCastellote, J.
dc.contributor.authorRiera-Mestre, Antoni
dc.date.accessioned2021-09-03T15:21:36Z
dc.date.available2021-09-03T15:21:36Z
dc.date.issued2020
dc.identifier.citationMora-Luján, J. M.; Iriarte, A.; Alba, E. [et al.]. Gender differences in hereditary hemorrhagic telangiectasia severity. Orphanet Journal of Rare Diseases, 2020, 15, 63. Disponible en: <https://ojrd.biomedcentral.com/articles/10.1186/s13023-020-1337-5>. Fecha de acceso: 3 sep. 2021. DOI: 10.1186/s13023-020-1337-5ca
dc.identifier.issn1750-1172ca
dc.identifier.urihttp://hdl.handle.net/20.500.12328/2754
dc.description.abstractBackground: Gender 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. Methods: Severity 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). Results: One 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. Conclusions: HHT 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.en
dc.format.extent10ca
dc.language.isoengca
dc.publisherSpringer Natureca
dc.relation.ispartofOrphanet Journal of Rare Diseasesca
dc.relation.ispartofseries15;
dc.rightsThis article is licensed under a Creative Commons Attribution 4.0 International License, which permits use, sharing, adaptation, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons licence, and indicate if changes were made. The images or other third party material in this article are included in the article's Creative Commons licence, unless indicated otherwise in a credit line to the material. If material is not included in the article's Creative Commons licence and your intended use is not permitted by statutory regulation or exceeds the permitted use, you will need to obtain permission directly from the copyright holder. To view a copy of this licence, visit http://creativecommons.org/licenses/by/4.0/. The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated in a credit line to the data.en
dc.rights.urihttps://creativecommons.org/licenses/by/4.0/
dc.subject.otherTelangiectasia hemorràgica hereditàriaca
dc.subject.otherGènereca
dc.subject.otherMalformacions arteriovenosesca
dc.subject.otherMalalties raresca
dc.subject.otherTelangiectasia hemorrágica hereditariaes
dc.subject.otherGéneroes
dc.subject.otherMalformaciones arteriovenosases
dc.subject.otherEnfermedades rarases
dc.subject.otherHereditary hemorrhagic telangiectasia,en
dc.subject.otherGenderen
dc.subject.otherArteriovenous malformationsen
dc.subject.otherRare diseasesen
dc.titleGender differences in hereditary hemorrhagic telangiectasia severityen
dc.typeinfo:eu-repo/semantics/articleca
dc.description.versioninfo:eu-repo/semantics/publishedVersionca
dc.rights.accessLevelinfo:eu-repo/semantics/openAccess
dc.embargo.termscapca
dc.subject.udc61ca
dc.identifier.doihttps://dx.doi.org/10.1186/s13023-020-1337-5ca


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This article is licensed under a Creative Commons Attribution 4.0 International License, which permits use, sharing, adaptation, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons licence, and indicate if changes were made. The images or other third party material in this article are included in the article's Creative Commons licence, unless indicated otherwise in a credit line to the material. If material is not included in the article's Creative Commons licence and your intended use is not permitted by statutory regulation or exceeds the permitted use, you will need to obtain permission directly from the copyright holder. To view a copy of this licence, visit http://creativecommons.org/licenses/by/4.0/. The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated in a credit line to the data.
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