3.8 Article

Transthyretin Amyloidosis (ATTR Amyloidosis): Recommendations for the Management in Germany and Austria Recommendations of the German Society of Amyloid Diseases

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AKTUELLE NEUROLOGIE
卷 45, 期 8, 页码 605-616

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GEORG THIEME VERLAG KG
DOI: 10.1055/a-0649-0724

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amyloidosis; transthyretin; polyneuropathy; heart; therapy

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Transthyretin amyloidosis (ATTR amyloidosis) is a rare and rapidly progressive disease caused by mutations within the transthyretin gene. Because of its rarity, the disease is little-known with the result of a frequently missed diagnosis and, therefore, appropriate therapy often being delayed. Liver transplantation has become a treatment option since the mid 90 s. In 2011, an oral drug stabilizing transthyretin and, subsequently, stopping amyloid production has been introduced. Additional substances are currently being studied, some of which are awaiting approval. The present paper written by German speaking experts gives recommendations for diagnosis, management and therapy of ATTR amyloidosis and is intended to increase awareness for this hereditary but treatable disease within the medical community.

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