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Characteristics of patients with autonomic dysfunction in the Transthyretin Amyloidosis Outcomes Survey (THAOS)

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dc.contributor.author Barroso, Fabio Adrián
dc.contributor.author Coelho, Teresa
dc.contributor.author Dispenzieri, Angela
dc.contributor.author Conceição, Isabel
dc.contributor.author Waddington Cruz, Marcia
dc.contributor.author Wixner, Jonas
dc.contributor.author Maurer, Mathew S.
dc.contributor.author Rapezzi, Claudio
dc.contributor.author Planté-Bordeneuve, Violaine
dc.contributor.author Kristen, Arnt V.
dc.contributor.author González-Duarte, Alejandra
dc.contributor.author Chapman, Doug
dc.contributor.author Stewart, Michelle
dc.contributor.author Amass, Leslie
dc.contributor.author THAOS investigators
dc.date.accessioned 2023-01-03T11:07:18Z
dc.date.available 2023-01-03T11:07:18Z
dc.date.issued 2022-09
dc.identifier.citation Barroso FA, Coelho T, Dispenzieri A, Conceição I, Waddington-Cruz M, Wixner J, Maurer MS, Rapezzi C, Planté-Bordeneuve V, Kristen AV, González-Duarte A, Chapman D, Stewart M, Amass L; THAOS investigators. Characteristics of patients with autonomic dysfunction in the Transthyretin Amyloidosis Outcomes Survey (THAOS). Amyloid. 2022 Sep;29(3):175-183. doi: 10.1080/13506129.2022.2043270. Epub 2022 Apr 22. es_ES
dc.identifier.uri https://doi.org/10.1080/13506129.2022.2043270
dc.identifier.uri https://repositorio.fleni.org.ar/xmlui/handle/123456789/754
dc.description.abstract Background: Autonomic dysfunction is common in transthyretin amyloidosis (ATTR amyloidosis), but its frequency, characteristics, and quality-of-life (QoL) impact are not well understood. Methods: The Transthyretin Amyloidosis Outcomes Survey (THAOS) is an ongoing, global, longitudinal survey of patients with ATTR amyloidosis, including patients with inherited (ATTRv) and wild-type (ATTRwt) disease and asymptomatic patients with TTR mutations (ClinicalTrials.gov: NCT00628745). In a descriptive analysis, characteristics and Norfolk QoL-DN total (TQoL) scores at enrolment were compared in patients with vs without autonomic dysfunction (analysis cut-off: 1 August 2020). Results: Autonomic dysfunction occurred in 1181/2922 (40.4%) symptomatic patients, and more commonly in ATTRv (1107/1181 [93.7%]) than ATTRwt (74/1181 [6.3%]) amyloidosis. Time (mean [SD]) from ATTR amyloidosis symptom onset to first autonomic dysfunction symptom was shorter in ATTRv (3.4 [5.7] years) than ATTRwt disease (9.7 [10.4]). In ATTRv disease, patients with vs without autonomic dysfunction had worse QoL (TQoL, 47.3 [33.2] vs 16.1 [18.1]); in ATTRwt disease, those with vs without autonomic dysfunction had similar QoL (23.0 [18.2] vs 19.9 [20.5]). Conclusions: Autonomic dysfunction was more common and presented earlier in symptomatic ATTRv than ATTRwt amyloidosis and adversely affected QoL in ATTRv disease. These THAOS findings may aid clinicians in diagnosing and treating patients with ATTR amyloidosis. es_ES
dc.language.iso eng es_ES
dc.publisher Taylor & Francis es_ES
dc.rights info:eu-repo/semantics/openAccess
dc.rights.uri https://creativecommons.org/licenses/by/2.5/ar/
dc.subject Amyloidosis es_ES
dc.subject Amiloidosis es_ES
dc.subject Autonomic Nervous System es_ES
dc.subject Sistema Nervioso Autónomo es_ES
dc.title Characteristics of patients with autonomic dysfunction in the Transthyretin Amyloidosis Outcomes Survey (THAOS) es_ES
dc.type info:eu-repo/semantics/article es_ES
dc.type info:eu-repo/semantics/publishedVersion
dc.description.fil Fil: Barroso, Fabio Adrián. Fleni. Departamento de Neurología. Sección de Enfermedades Neuromusculares; Argentina.
dc.description.fil Fil: Coelho, Teresa. Centro Hospitalar Universitário do Porto. Hospital Santo António. Unidade Corino Andrade; Portugal.
dc.description.fil Fil: Dispenzieri, Angela. Mayo Clinic. Division of Hematology; Estados Unidos.
dc.description.fil Fil: Conceição, Isabel. FML Universidade de Lisboa. Hospital de Santa Maria-CHULN; Portugal.
dc.description.fil Fil: Waddington-Cruz, Marcia. Federal University of Rio de Janeiro. University Hospital; Brasil.
dc.description.fil Fil: Wixner, Jonas. Umeå University. Department of Public Health and Clinical Medicine; Suecia.
dc.description.fil Fil: Maurer, Mathew S. Columbia University College of Physicians and Surgeons; Estados Unidos.
dc.description.fil Fil: Rapezzi, Claudio. University of Ferrara. Cardiological Center; Italia. GVM Care & Research. Maria Cecilia Hospital; Italia.
dc.description.fil Fil: Planté-Bordeneuve, Violaine. Assistance Publique Hopitaux de Paris. Hospital Henri Mondor; Francia. East-Paris University. Department of Neurology; Francia.
dc.description.fil Fil: Kristen, Arnt V. Medical University of Heidelberg. Department of Cardiology, Angiology, and Respiratory Medicine; Alemania.
dc.description.fil Fil: González-Duarte, Alejandra. Instituto Nacional De Ciencias Medicas y Nutrición Salvador Zubiran; México.
dc.description.fil Fil: Chapman, Doug. Pfizer Inc; Estados Unidos.
dc.description.fil Fil: Stewart, Michelle. Pfizer Inc; Estados Unidos.
dc.description.fil Fil: Amass, Leslie. Pfizer Inc; Estados Unidos.
dc.relation.ispartofVOLUME 29
dc.relation.ispartofNUMBER 3
dc.relation.ispartofPAGINATION 175-183.
dc.relation.ispartofCOUNTRY Inglaterra
dc.relation.ispartofCITY Oxford
dc.relation.ispartofTITLE Amyloid : the international journal of experimental and clinical investigation : the official journal of the International Society of Amyloidosis
dc.relation.ispartofISSN 1744-2818
dc.type.snrd info:ar-repo/semantics/artículo es_ES


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