title: Diagnosis, Prognosis, and Therapy of Transthyretin Amyloidosis.
abstract: Transthyretin amyloidosis is a fatal disorder that is characterized primarily by progressive neuropathy and cardiomyopathy. It occurs in both a mutant form (with autosomal dominant inheritance) and a wild-type form (with predominant cardiac involvement). This article guides clinicians as to when the disease should be suspected, describes the appropriate diagnostic evaluation for those with known or suspected amyloidosis, and reviews the interventions currently available for affected patients.
authors.affiliation: Division of Hematology, Mayo Clinic, Rochester, Minnesota. Electronic address: gertz.morie@mayo.edu.
authors.forename: Morie A
authors.initials: MA
authors.lastname: Gertz
authors.affiliation: Indiana University School of Medicine, Indianapolis, Indiana.
authors.forename: Merrill D
authors.initials: MD
authors.lastname: Benson
authors.affiliation: Division of Peripheral Nerve, Mayo Clinic, Rochester, Minnesota.
authors.forename: Peter J
authors.initials: PJ
authors.lastname: Dyck
authors.affiliation: Division of Cardiovascular Diseases, Mayo Clinic, Rochester, Minnesota.
authors.forename: Martha
authors.initials: M
authors.lastname: Grogan
authors.affiliation: Hospital de Santo Antonio, Porto, Portugal.
authors.forename: Terresa
authors.initials: T
authors.lastname: Coelho
authors.affiliation: Federal University of Rio de Janeiro of Brazil, University Hospital, Rio de Janeiro, Brazil.
authors.forename: Marcia
authors.initials: M
authors.lastname: Cruz
authors.affiliation: Amyloidosis Center, Boston University School of Medicine, Boston, Massachusetts.
authors.forename: John L
authors.initials: JL
authors.lastname: Berk
authors.affiliation: University Hospital, Henri Mondor, Créteil, France.
authors.forename: Violaine
authors.initials: V
authors.lastname: Plante-Bordeneuve
authors.affiliation: Universitätsklinikum Münster, Münster, Germany.
authors.forename: Hartmut H J
authors.initials: HHJ
authors.lastname: Schmidt
authors.affiliation: Department of Molecular Medicine, University of Pavia, Pavia, Lombardy, Italy.
authors.forename: Giampaolo
authors.initials: G
authors.lastname: Merlini
mesh_terms: D028227:Amyloid Neuropathies, Familial / Q000175:diagnosis* / Q000453:epidemiology / Q000628:therapy*; D000818:Animals; D009202:Cardiomyopathies / Q000175:diagnosis / Q000453:epidemiology / Q000628:therapy; D006801:Humans; D011379:Prognosis
keywords: familial amyloid cardiomyopathy; familial amyloid polyneuropathy; genetics; liver transplantation