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Characteristics of ...
Characteristics of patients with autonomic dysfunction in the Transthyretin Amyloidosis Outcomes Survey (THAOS)
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- Barroso, Fabio A. (författare)
- Institute for Neurological Research, FLENI, Buenos Aires, Argentina
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- Coelho, Teresa (författare)
- Unidade Corino Andrade, Hospital Santo António, Centro Hospitalar Universitário do Porto, Porto, Portugal
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- Dispenzieri, Angela (författare)
- Division of Hematology, Mayo Clinic, MN, Rochester, United States
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- Conceição, Isabel (författare)
- Hospital de Santa Maria–CHULN, FML Universidade de Lisboa, Lisbon, Portugal
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- Waddington-Cruz, Marcia (författare)
- CEPARM, University Hospital, Federal University of Rio de Janeiro, Rio de Janeiro, Brazil
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- Wixner, Jonas (författare)
- Umeå universitet,Avdelningen för medicin
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- Maurer, Mathew S. (författare)
- Columbia University College of Physicians and Surgeons, NY, New York, United States
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- Rapezzi, Claudio (författare)
- Cardiological Center, University of Ferrara, Ferrara, Italy; Maria Cecilia Hospital, GVM Care & Research, Cotignola (RA), Italy
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- Planté-Bordeneuve, Violaine (författare)
- Department of Neurology, East-Paris University, Hospital Henri Mondor, Assistance Publique Hopitaux de Paris, INSERM U955 Team 10 “Biology of the Neuro-Muscular System”, Crétei, France
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- Kristen, Arnt V. (författare)
- Department of Cardiology, Angiology, and Respiratory Medicine, Medical University of Heidelberg, Heidelberg, Germany
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- González-Duarte, Alejandra (författare)
- Instituto Nacional De Ciencias Medicas y Nutricion Salvador Zubiran, Mexico City, Mexico
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- Chapman, Doug (författare)
- Pfizer Inc, NY, New York, United States
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- Stewart, Michelle (författare)
- Pfizer Inc, CT, Groton, United States
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- Amass, Leslie (författare)
- Pfizer Inc, CT, Groton, United States
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(creator_code:org_t)
- 2022-04-22
- 2022
- Engelska.
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Ingår i: Amyloid. - : Taylor & Francis. - 1350-6129 .- 1744-2818. ; 29:3, s. 175-183
- Relaterad länk:
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https://doi.org/10.1...
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https://umu.diva-por... (primary) (Raw object)
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https://urn.kb.se/re...
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https://doi.org/10.1...
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Abstract
Ämnesord
Stäng
- 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.Trial registration: ClinicalTrials.gov: NCT00628745.
Ämnesord
- MEDICIN OCH HÄLSOVETENSKAP -- Klinisk medicin -- Neurologi (hsv//swe)
- MEDICAL AND HEALTH SCIENCES -- Clinical Medicine -- Neurology (hsv//eng)
Nyckelord
- Amyloidosis
- autonomic nervous system
- neuropathy
- registry
- transthyretin
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Amyloid
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Barroso, Fabio A ...
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Coelho, Teresa
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Dispenzieri, Ang ...
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Conceição, Isabe ...
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Waddington-Cruz, ...
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Wixner, Jonas
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Maurer, Mathew S ...
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Rapezzi, Claudio
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Planté-Bordeneuv ...
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Kristen, Arnt V.
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González-Duarte, ...
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Chapman, Doug
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Stewart, Michell ...
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Amass, Leslie
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