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Assessment of patients with hereditary transthyretin amyloidosis : understanding the impact of management and disease progression

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Resumo:Timely diagnosis of hereditary variant transthyretin (ATTRv) amyloidosis is critical for appropriate treatment and optimal outcomes. Significant differences are seen between patients receiving treatment and those who are not, though disease progression may continue despite treatment in some patients. Healthcare professionals caring for patients with ATTRv amyloidosis therefore need reliable ongoing assessments to understand the continuing course of disease and make appropriate treatment choices on an individual basis. Various signs and symptoms experienced by patients may be evaluated as indicators of disease progression, though there is currently no validated score that can be used for such ongoing assessment. Recognizing this situation, a group of clinicians highly experienced in ATTR amyloidosis developed an approach to understand and define disease progression in diagnosed and treated patients with ATTRv amyloidosis. The suggested approach is based on the recognition of distinct phenotypes which may usefully inform the particular tools, tests and investigations that are most likely to be appropriate for individual patients. It is aimed at implementing appropriate and ongoing assessment of patients being treated for ATTRv amyloidosis, such that the effectiveness of management can be usefully assessed throughout the course of disease and management can be tailored according to the patient’s requirements.
Autores principais:Conceição, isabel
Outros Autores:Coelho, Teresa; Rapezzi, Claudio; Parman, Yeşim; Obici, Laura; Galán, Lucía; Rousseau, Antoine
Assunto:ATTR Amyloidosis Follow-up Hereditary Monitoring Progression Transthyretin
Ano:2019
País:Portugal
Tipo de documento:artigo
Tipo de acesso:acesso aberto
Instituição associada:Universidade de Lisboa
Idioma:inglês
Origem:Repositório da Universidade de Lisboa
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author Conceição, isabel
author2 Coelho, Teresa
Rapezzi, Claudio
Parman, Yeşim
Obici, Laura
Galán, Lucía
Rousseau, Antoine
author2_role author
author
author
author
author
author
author_facet Conceição, isabel
Coelho, Teresa
Rapezzi, Claudio
Parman, Yeşim
Obici, Laura
Galán, Lucía
Rousseau, Antoine
author_role author
contributor_name_str_mv Repositório Científico de Acesso Aberto da ULisboa
country_str PT
creators_json_txt [{\"Person.name\":\"Conceição, isabel\",\"Person.identifier.orcid\":\"0000-0003-0934-9631\"},{\"Person.name\":\"Coelho, Teresa\"},{\"Person.name\":\"Rapezzi, Claudio\"},{\"Person.name\":\"Parman, Yeşim\"},{\"Person.name\":\"Obici, Laura\"},{\"Person.name\":\"Galán, Lucía\"},{\"Person.name\":\"Rousseau, Antoine\"}]
datacite.contributors.contributor.contributorName.fl_str_mv Repositório Científico de Acesso Aberto da ULisboa
datacite.creators.creator.creatorName.fl_str_mv Conceição, isabel
Coelho, Teresa
Rapezzi, Claudio
Parman, Yeşim
Obici, Laura
Galán, Lucía
Rousseau, Antoine
datacite.date.Accepted.fl_str_mv 2019-01-01T00:00:00Z
datacite.date.available.fl_str_mv 2021-02-19T14:32:38Z
datacite.date.embargoed.fl_str_mv 2021-02-19T14:32:38Z
datacite.rights.fl_str_mv http://purl.org/coar/access_right/c_abf2
datacite.subjects.subject.fl_str_mv ATTR
Amyloidosis
Follow-up
Hereditary
Monitoring
Progression
Transthyretin
datacite.titles.title.fl_str_mv Assessment of patients with hereditary transthyretin amyloidosis : understanding the impact of management and disease progression
dc.contributor.none.fl_str_mv Repositório Científico de Acesso Aberto da ULisboa
dc.creator.none.fl_str_mv Conceição, isabel
Coelho, Teresa
Rapezzi, Claudio
Parman, Yeşim
Obici, Laura
Galán, Lucía
Rousseau, Antoine
dc.date.Accepted.fl_str_mv 2019-01-01T00:00:00Z
dc.date.available.fl_str_mv 2021-02-19T14:32:38Z
dc.date.embargoed.fl_str_mv 2021-02-19T14:32:38Z
dc.format.none.fl_str_mv application/pdf
dc.identifier.none.fl_str_mv http://hdl.handle.net/10451/46443
dc.language.none.fl_str_mv eng
dc.publisher.none.fl_str_mv Taylor & Francis Group
dc.rights.cclincense.fl_str_mv http://creativecommons.org/licenses/by-nc-nd/4.0/
dc.rights.none.fl_str_mv http://purl.org/coar/access_right/c_abf2
dc.subject.none.fl_str_mv ATTR
Amyloidosis
Follow-up
Hereditary
Monitoring
Progression
Transthyretin
dc.title.fl_str_mv Assessment of patients with hereditary transthyretin amyloidosis : understanding the impact of management and disease progression
dc.type.none.fl_str_mv http://purl.org/coar/resource_type/c_6501
description Timely diagnosis of hereditary variant transthyretin (ATTRv) amyloidosis is critical for appropriate treatment and optimal outcomes. Significant differences are seen between patients receiving treatment and those who are not, though disease progression may continue despite treatment in some patients. Healthcare professionals caring for patients with ATTRv amyloidosis therefore need reliable ongoing assessments to understand the continuing course of disease and make appropriate treatment choices on an individual basis. Various signs and symptoms experienced by patients may be evaluated as indicators of disease progression, though there is currently no validated score that can be used for such ongoing assessment. Recognizing this situation, a group of clinicians highly experienced in ATTR amyloidosis developed an approach to understand and define disease progression in diagnosed and treated patients with ATTRv amyloidosis. The suggested approach is based on the recognition of distinct phenotypes which may usefully inform the particular tools, tests and investigations that are most likely to be appropriate for individual patients. It is aimed at implementing appropriate and ongoing assessment of patients being treated for ATTRv amyloidosis, such that the effectiveness of management can be usefully assessed throughout the course of disease and management can be tailored according to the patient’s requirements.
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instname_str Universidade de Lisboa
language eng
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oai_identifier_str oai:repositorio.ulisboa.pt:10451/46443
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person_str_mv Conceição, isabel
Conceição, isabel
http://orcid.org/0000-0003-0934-9631
0000-0003-0934-9631
Coelho, Teresa
Rapezzi, Claudio
Parman, Yeşim
Obici, Laura
Galán, Lucía
Rousseau, Antoine
publishDate 2019
publisher.none.fl_str_mv Taylor & Francis Group
reponame_str Repositório da Universidade de Lisboa
repository_id_str urn:repositoryAcronym:ul
service_str_mv urn:repositoryAcronym:ul
spelling engTaylor & Francis Grouppt_PTTimely diagnosis of hereditary variant transthyretin (ATTRv) amyloidosis is critical for appropriate treatment and optimal outcomes. Significant differences are seen between patients receiving treatment and those who are not, though disease progression may continue despite treatment in some patients. Healthcare professionals caring for patients with ATTRv amyloidosis therefore need reliable ongoing assessments to understand the continuing course of disease and make appropriate treatment choices on an individual basis. Various signs and symptoms experienced by patients may be evaluated as indicators of disease progression, though there is currently no validated score that can be used for such ongoing assessment. Recognizing this situation, a group of clinicians highly experienced in ATTR amyloidosis developed an approach to understand and define disease progression in diagnosed and treated patients with ATTRv amyloidosis. The suggested approach is based on the recognition of distinct phenotypes which may usefully inform the particular tools, tests and investigations that are most likely to be appropriate for individual patients. It is aimed at implementing appropriate and ongoing assessment of patients being treated for ATTRv amyloidosis, such that the effectiveness of management can be usefully assessed throughout the course of disease and management can be tailored according to the patient’s requirements.application/pdfpt_PTAssessment of patients with hereditary transthyretin amyloidosis : understanding the impact of management and disease progressionPersonalConceição, isabelDSpacehttp://dspace.org/items/52ba30d8-6cbf-424d-b334-4d06a2ecc6e5DSpacehttp://dspace.org/items/52ba30d8-6cbf-424d-b334-4d06a2ecc6e5CONCEICAOISABELORCIDhttp://orcid.org0000-0003-0934-9631Scopus Author IDhttps://www.scopus.com6701694419Coelho, TeresaRapezzi, ClaudioParman, YeşimObici, LauraGalán, LucíaRousseau, AntoineHostingInstitutionOrganizationalRepositório Científico de Acesso Aberto da ULisboae-mailmailto:repositorio@reitoria.ulisboa.ptrepositorio@reitoria.ulisboa.ptISSNIsPartOf1350-6129ISSNIsPartOf1744-2818DOIIsPartOf10.1080/13506129.2019.16273122021-02-19T14:32:38Z20192019-01-01T00:00:00ZHandlehttp://hdl.handle.net/10451/46443http://purl.org/coar/access_right/c_abf2open accessATTRAmyloidosisFollow-upHereditaryMonitoringProgressionTransthyretin1210423 bytesliteraturehttp://purl.org/coar/resource_type/c_6501journal article2019http://creativecommons.org/licenses/by-nc-nd/4.0/http://purl.org/coar/access_right/c_abf2application/pdffulltexthttps://repositorio.ulisboa.pt/bitstreams/1c87f9d9-3421-4b7e-8acf-42f63962c1f3/downloadAmyloid263103111
spellingShingle Assessment of patients with hereditary transthyretin amyloidosis : understanding the impact of management and disease progression
Conceição, isabel
ATTR
Amyloidosis
Follow-up
Hereditary
Monitoring
Progression
Transthyretin
status SINGLETON
subject.fl_str_mv ATTR
Amyloidosis
Follow-up
Hereditary
Monitoring
Progression
Transthyretin
title Assessment of patients with hereditary transthyretin amyloidosis : understanding the impact of management and disease progression
title_full Assessment of patients with hereditary transthyretin amyloidosis : understanding the impact of management and disease progression
title_fullStr Assessment of patients with hereditary transthyretin amyloidosis : understanding the impact of management and disease progression
title_full_unstemmed Assessment of patients with hereditary transthyretin amyloidosis : understanding the impact of management and disease progression
title_short Assessment of patients with hereditary transthyretin amyloidosis : understanding the impact of management and disease progression
title_sort Assessment of patients with hereditary transthyretin amyloidosis : understanding the impact of management and disease progression
topic ATTR
Amyloidosis
Follow-up
Hereditary
Monitoring
Progression
Transthyretin
topic_facet ATTR
Amyloidosis
Follow-up
Hereditary
Monitoring
Progression
Transthyretin
url http://hdl.handle.net/10451/46443
visible 1