Quality of life in patients with hereditary haemorrhagic telangiectasia (HHT)

9 p.-3 fig.-7 tab.

Detalhes bibliográficos
Autores: Zarrabeitia, Roberto, Fariñas-Álvarez, Concepción, Santibáñez, Miguel, Señaris, Blanca, Fontalba, Ana, Botella, Luisa María, Parra, José A.
Formato: artículo
Estado:Versión publicada
Fecha de publicación:2017
País:España
Recursos:Consejo Superior de Investigaciones Científicas (CSIC)
Repositorio:DIGITAL.CSIC. Repositorio Institucional del CSIC
OAI Identifier:oai:digital.csic.es:10261/151035
Acesso em linha:http://hdl.handle.net/10261/151035
Access Level:acceso abierto
Palavra-chave:HHT
Hereditary hemorrhagic telangiectasia
Rendu Osler
Euroqol
Qol
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spelling Quality of life in patients with hereditary haemorrhagic telangiectasia (HHT)Zarrabeitia, RobertoFariñas-Álvarez, ConcepciónSantibáñez, MiguelSeñaris, BlancaFontalba, AnaBotella, Luisa MaríaParra, José A.HHTHereditary hemorrhagic telangiectasiaRendu OslerEuroqolQol9 p.-3 fig.-7 tab.Background: There are very few studies about general quality of life parameters, standards for the description of health status and comparison with general population data on patients with Hereditary hemorrhagic telangiectasia(HHT), a rare disease in which epistaxis is a cardinal symptom.Purpose: To assess the quality of life in a population of Spanish patients with HHT and compare it with the general population.Design and methods: Between January 1st 2005 and December 31st 2013, 187 adult patients diagnosed with HHT who were admitted to the HHT Unit of the Hospital Sierrallana, completed on their first visit, the EuroQol 5D-3L(five dimensions and three levels) quality of life descriptive test and the visual analog scale (VAS). The numerical social index value was also determined and the subjective effect of the nasal epistaxis on their quality of life was estimated classified as mild, moderate or severe.Results: Patients with HHT had greater problems than the general population in the five dimensions of the EuroQol 5D-3L, particularly considering pain/discomfort and anxiety/depression. In the VAS and the social index value, patients with HHT also scored lower than the general population, particularly older patients, males, and patients with HHT2. They also had values similar to those of populations with chronic illnesses. The subjective perception of the severity of epistaxis correlated strongly with the VAS and social index values.Conclusions: The quality of life of patients with HHT, estimated using the EuroQol 5D-3L scale, is affected across all dimensions. The scores are similar to those seen in cases of other chronic diseases. Older patients, males and the carriers of the ACVRL1 mutation generally have worse scores on these scales. The VAS and the social index value are index that correlate well with the severity of the clinical symptoms associated mainly with epistaxis.This study has been supported by grants from Instituto de Salud Carlos III(ISCIII; PI11/0246) also subsidiary to be cofunded with FEDER funding to Jose Antonio Parra, Ministerio de Economía y Competitividad of Spain (SAF2011-23475) to Luisa Botella and Centro de Investigación Biomédica en Red de Enfermedades Raras (CIBERER) to Luisa Botella and Roberto Zarrabeitia.Peer reviewedBioMed CentralInstituto de Salud Carlos IIIMinisterio de Economía y Competitividad (España)Centro de Investigación Biomédica en Red Enfermedades Raras (España)Consejo Superior de Investigaciones Científicas [https://ror.org/02gfc7t72]201720172017info:eu-repo/semantics/articlehttp://purl.org/coar/resource_type/c_6501Publisher's versioninfo:eu-repo/semantics/publishedVersionhttp://hdl.handle.net/10261/151035reponame:DIGITAL.CSIC. Repositorio Institucional del CSICinstname:Consejo Superior de Investigaciones Científicas (CSIC)Ingléshttp://dx.doi.org/ 10.1186/s12955-017-0586-zSíinfo:eu-repo/semantics/openAccessoai:digital.csic.es:10261/1510352026-05-22T06:33:51Z
dc.title.none.fl_str_mv Quality of life in patients with hereditary haemorrhagic telangiectasia (HHT)
title Quality of life in patients with hereditary haemorrhagic telangiectasia (HHT)
spellingShingle Quality of life in patients with hereditary haemorrhagic telangiectasia (HHT)
Zarrabeitia, Roberto
HHT
Hereditary hemorrhagic telangiectasia
Rendu Osler
Euroqol
Qol
title_short Quality of life in patients with hereditary haemorrhagic telangiectasia (HHT)
title_full Quality of life in patients with hereditary haemorrhagic telangiectasia (HHT)
title_fullStr Quality of life in patients with hereditary haemorrhagic telangiectasia (HHT)
title_full_unstemmed Quality of life in patients with hereditary haemorrhagic telangiectasia (HHT)
title_sort Quality of life in patients with hereditary haemorrhagic telangiectasia (HHT)
dc.creator.none.fl_str_mv Zarrabeitia, Roberto
Fariñas-Álvarez, Concepción
Santibáñez, Miguel
Señaris, Blanca
Fontalba, Ana
Botella, Luisa María
Parra, José A.
author Zarrabeitia, Roberto
author_facet Zarrabeitia, Roberto
Fariñas-Álvarez, Concepción
Santibáñez, Miguel
Señaris, Blanca
Fontalba, Ana
Botella, Luisa María
Parra, José A.
author_role author
author2 Fariñas-Álvarez, Concepción
Santibáñez, Miguel
Señaris, Blanca
Fontalba, Ana
Botella, Luisa María
Parra, José A.
author2_role author
author
author
author
author
author
dc.contributor.none.fl_str_mv Instituto de Salud Carlos III
Ministerio de Economía y Competitividad (España)
Centro de Investigación Biomédica en Red Enfermedades Raras (España)
Consejo Superior de Investigaciones Científicas [https://ror.org/02gfc7t72]
dc.subject.none.fl_str_mv HHT
Hereditary hemorrhagic telangiectasia
Rendu Osler
Euroqol
Qol
topic HHT
Hereditary hemorrhagic telangiectasia
Rendu Osler
Euroqol
Qol
description 9 p.-3 fig.-7 tab.
publishDate 2017
dc.date.none.fl_str_mv 2017
2017
2017
dc.type.none.fl_str_mv info:eu-repo/semantics/article
http://purl.org/coar/resource_type/c_6501
Publisher's version
info:eu-repo/semantics/publishedVersion
format article
status_str publishedVersion
dc.identifier.none.fl_str_mv http://hdl.handle.net/10261/151035
url http://hdl.handle.net/10261/151035
dc.language.none.fl_str_mv Inglés
language_invalid_str_mv Inglés
dc.relation.none.fl_str_mv http://dx.doi.org/ 10.1186/s12955-017-0586-z
Sí
dc.rights.none.fl_str_mv info:eu-repo/semantics/openAccess
eu_rights_str_mv openAccess
dc.publisher.none.fl_str_mv BioMed Central
publisher.none.fl_str_mv BioMed Central
dc.source.none.fl_str_mv reponame:DIGITAL.CSIC. Repositorio Institucional del CSIC
instname:Consejo Superior de Investigaciones Científicas (CSIC)
instname_str Consejo Superior de Investigaciones Científicas (CSIC)
reponame_str DIGITAL.CSIC. Repositorio Institucional del CSIC
collection DIGITAL.CSIC. Repositorio Institucional del CSIC
repository.name.fl_str_mv
repository.mail.fl_str_mv
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score 15,812429

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