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Management of gastrointestinal complications in hereditary transthyretin amyloidosis: a single-center experience over 40 years
Umeå University, Faculty of Medicine, Department of Public Health and Clinical Medicine, Section of Medicine.
Umeå University, Faculty of Medicine, Department of Public Health and Clinical Medicine, Section of Medicine.
Umeå University, Faculty of Medicine, Department of Public Health and Clinical Medicine, Section of Medicine.
2018 (English)In: Expert Review of Gastroenterology & Hepatology, ISSN 1747-4124, E-ISSN 1747-4132, Vol. 12, no 1, p. 73-81Article, review/survey (Refereed) Published
Abstract [en]

Introduction: Hereditary transthyretin amyloidosis (ATTRm amyloidosis) is a rare disease caused by the deposition and accumulation of insoluble non-native transthyretin fibrils in the body. The disease inevitably results in widespread organ disruption, and poor life expectancy. The GI tract is one organ system vulnerable to disruption and, although the clinical presentation of the disease varies, GI involvement affects most patients with ATTRm amyloidosis.

Areas covered: This article presents our experience with diagnosing and treating the GI symptoms of ATTRm amyloidosis patients at our center over the last 40 years, in the Swedish clustering area of the disease. Our aim is to help other physicians to better manage GI complications in patients with this rare but widespread condition.

Expert commentary: GI symptoms are debilitating complications for ATTRm amyloidosis patients to experience, yet with the appropriate questioning and diagnosis methods, symptomatic treatments of these symptoms can be implemented to provide relief. Further, patients with fewer GI complications and a good nutritional status are also better candidates for liver transplantation which, in selected cases, is the best disease-modifying treatment of ATTRm amyloidosis to date.

Place, publisher, year, edition, pages
Taylor & Francis, 2018. Vol. 12, no 1, p. 73-81
Keywords [en]
amyloidosis, familial amyloid neuropathy, gastric emptying, gastrointestinal tract, liver, therapeutics, ansthyretin
National Category
Gastroenterology and Hepatology
Identifiers
URN: urn:nbn:se:umu:diva-146467DOI: 10.1080/17474124.2018.1397511ISI: 000427553900008PubMedID: 29073801OAI: oai:DiVA.org:umu-146467DiVA, id: diva2:1196628
Available from: 2018-04-10 Created: 2018-04-10 Last updated: 2019-05-16Bibliographically approved

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Wixner, JonasSuhr, Ole B.Anan, Intissar

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