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Due to a lack of United States (US)\u2010specific guidance on ATTRv amyloidosis with polyneuropathy, a panel of US\u2010based expert clinicians convened to address identification, monitoring, and treatment of this disease. ATTRv amyloidosis with polyneuropathy should be suspected in unexplained progressive neuropathy, especially if associated with systemic symptoms or family history. The diagnosis is confirmed through genetic testing, biopsy, or cardiac technetium\u2010based scintigraphy. Treatment should be initiated as soon as possible after diagnosis, with gene\u2010silencing therapeutics recommended as a first\u2010line option. Consensus is lacking on what represents \u201cdisease progression\u201d during treatment; however, the aggressive natural history of this disease should be considered when evaluating the effectiveness of any therapy.<\/jats:p>","DOI":"10.1002\/mus.28026","type":"journal-article","created":{"date-parts":[[2024,1,4]],"date-time":"2024-01-04T07:53:49Z","timestamp":1704354829000},"page":"273-287","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":40,"title":["Diagnosis and treatment of hereditary transthyretin amyloidosis with polyneuropathy in the\n                    <scp>United States<\/scp>\n                    :\n                    <scp>Recommendations<\/scp>\n                    from a panel of experts"],"prefix":"10.1002","volume":"69","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-3868-2994","authenticated-orcid":false,"given":"Chafic","family":"Karam","sequence":"first","affiliation":[{"name":"Department of Neurology 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