Brazilian consensus for diagnosis, management and treatment of transthyretin familial amyloid polyneuropathy

Author:

Pinto Marcus Vinicius1,Barreira Amilton Antunes2,Bulle Acary Souza3,Freitas Marcos Raimundo Gomes de1,França Jr Marcondes Cavalcante4,Gondim Francisco de Assis Aquino5,Marrone Carlo Domenico6,Marques Jr Wilson2,Nascimento Osvaldo J. M.7,Rotta Francisco Tellechea8,Pupe Camila7,Waddington-Cruz Márcia1

Affiliation:

1. Universidade Federal do Rio de Janeiro, Brasil

2. Universidade de São Paulo, Brasil

3. Universidade Federal de São Paulo, Brasil

4. Universidade de Campinas, Brasil

5. Universidade Federal do Ceará, Brasil; Centro Universitário Unichristus, Brasil

6. PUCRS, Brasil

7. Universidade Federal Fluminense, Brasil

8. Hospital Moinhos de Vento, Brasil; Santa Casa de Misericórdia de Porto Alegre, Brasil

Abstract

ABSTRACT Transthyretin familial amyloid polyneuropathy is an autosomal dominant inherited sensorimotor and autonomic polyneuropathy, which if untreated, leads to death in approximately 10 years. In Brazil, liver transplant and tafamidis are the only disease-modifying treatments available. This review consists of a consensus for the diagnosis, management and treatment for transthyretin familial amyloid polyneuropathy from the Peripheral Neuropathy Scientific Department of the Brazilian Academy of Neurology. The first and last authors produced a draft summarizing the main views on the subject and emailed the text to 10 other specialists. Relevant literature on this subject was reviewed by each participant and used for the individual review of the whole text. Each participant was expected to review the text and send a feedback review by e-mail. Thereafter, the 12 panelists got together at the city of Fortaleza, discussed the controversial points, and reached a consensus for the final text.

Publisher

FapUNIFESP (SciELO)

Subject

Neurology,Clinical Neurology

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