{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,2]],"date-time":"2026-10-02T16:42:24Z","timestamp":1790959344560,"version":"4.1.0"},"reference-count":42,"publisher":"Wiley","issue":"5","license":[{"start":{"date-parts":[[2025,9,17]],"date-time":"2025-09-17T00:00:00Z","timestamp":1758067200000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0\/"}],"funder":[{"DOI":"10.13039\/501100002960","name":"V\u00e4sterbotten L\u00e4ns Landsting","doi-asserted-by":"publisher","award":["RV\u2010995135"],"award-info":[{"award-number":["RV\u2010995135"]}],"id":[{"id":"10.13039\/501100002960","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["J Intern Med"],"published-print":{"date-parts":[[2025,11]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Background<\/jats:title><jats:p>Hereditary transthyretin (ATTRv) amyloidosis was first described in Sweden in the late 1960s. Selected patient data have been collected since then and have now been transferred to a national quality registry.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>This is the first report from SveATTR\u2014a longitudinal Swedish web\u2010based registry open for <jats:italic>TTR<\/jats:italic> variant carriers and patients with ATTR amyloidosis. The registry covers basic background information, as well as relevant clinical follow\u2010up measures and data on disease\u2010modifying therapies. Data from all ATTRv amyloidosis patients registered through December 2022 were included.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>In total, 1055 patients were included, of whom 65% were males and 95% carried the V30M variant. Median age of onset was 64 years, and 79% had a late disease onset (\u226550 years). Eighty\u2010seven percent of the patients had peripheral polyneuropathy at onset, whereas 10% had cardiac symptoms, 8% had visual disturbances, and 6% had gastrointestinal symptoms. A total of 159 patients had undergone liver transplantation, and 233 had received a disease\u2010modifying drug. Improved survival was seen for transplanted patients and for patients on drug therapy.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>This report highlights the importance of SveATTR for further characterization of the Swedish ATTRv amyloidosis population as well as for evaluating the efficacy of disease\u2010modifying therapies.<\/jats:p><\/jats:sec>","DOI":"10.1111\/joim.70020","type":"journal-article","created":{"date-parts":[[2025,9,17]],"date-time":"2025-09-17T10:14:27Z","timestamp":1758104067000},"page":"478-488","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":7,"title":["Sixty years of experience with hereditary transthyretin amyloidosis: Insights from the Swedish transthyretin amyloidosis registry"],"prefix":"10.1111","volume":"298","author":[{"given":"Lotta","family":"Stenberg","sequence":"first","affiliation":[{"name":"Department of Public Health and Clinical Medicine Ume\u00e5 University  Ume\u00e5 Sweden"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Bj\u00f6rn","family":"Pilebro","sequence":"additional","affiliation":[{"name":"Department of Public Health and Clinical Medicine Ume\u00e5 University  Ume\u00e5 Sweden"},{"name":"Department of Medicine Norrland's University Hospital  Ume\u00e5 Sweden"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Intissar","family":"Anan","sequence":"additional","affiliation":[{"name":"Department of Public Health and Clinical Medicine Ume\u00e5 University  Ume\u00e5 Sweden"},{"name":"Department of Medicine Norrland's University Hospital  Ume\u00e5 Sweden"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jorge Mejia","family":"Baranda","sequence":"additional","affiliation":[{"name":"Department of Medicine Pite\u00e5 Hospital  Pite\u00e5 Sweden"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kristin","family":"Samuelsson","sequence":"additional","affiliation":[{"name":"Department of Clinical Neuroscience Karolinska Institutet  Stockholm Sweden"},{"name":"Department of Neurology Karolinska University Hospital  Stockholm Sweden"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Per","family":"Eldhagen","sequence":"additional","affiliation":[{"name":"Department of Medicine Karolinska Institutet  Stockholm Sweden"},{"name":"Unit of Cardiology, Karolinska University Hospital  Stockholm Sweden"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Rolf","family":"Backlund","sequence":"additional","affiliation":[{"name":"Department of Medicine Norrland's University Hospital  Ume\u00e5 Sweden"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jonas","family":"Wixner","sequence":"additional","affiliation":[{"name":"Department of Public Health and Clinical Medicine Ume\u00e5 University  Ume\u00e5 Sweden"},{"name":"Department of Medicine Norrland's University Hospital  Ume\u00e5 Sweden"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2025,9,17]]},"reference":[{"key":"e_1_2_9_2_1","doi-asserted-by":"publisher","DOI":"10.14797\/mdcvj.1066"},{"key":"e_1_2_9_3_1","doi-asserted-by":"publisher","DOI":"10.1111\/joim.12585"},{"key":"e_1_2_9_4_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0025-6196(12)60388-7"},{"key":"e_1_2_9_5_1","doi-asserted-by":"publisher","DOI":"10.1093\/eurheartj\/ehr383"},{"key":"e_1_2_9_6_1","doi-asserted-by":"publisher","DOI":"10.3109\/13506129.2015.1019610"},{"key":"e_1_2_9_7_1","first-page":"1","article-title":"Familial amyloidosis with polyneuropathy. A clinical study based on patients living in northern Sweden","volume":"590","author":"Andersson R","year":"1976","journal-title":"Acta Med Scand Suppl."},{"key":"e_1_2_9_8_1","unstructured":"CoutinhoP Martins da SilvaA Lopes LimaJ et\u00a0al.Forty years of experience with type I amyloid neuropathy. Review of 483 cases. In: Amyloid and amyloidosis. Amsterdam: Excerpta Medica;1980. p.88\u201398."},{"key":"e_1_2_9_9_1","doi-asserted-by":"publisher","DOI":"10.1212\/WNL.0000000000006543"},{"key":"e_1_2_9_10_1","doi-asserted-by":"publisher","DOI":"10.3390\/brainsci11050545"},{"key":"e_1_2_9_11_1","doi-asserted-by":"publisher","DOI":"10.1097\/TP.0000000000000574"},{"key":"e_1_2_9_12_1","doi-asserted-by":"publisher","DOI":"10.1212\/WNL.0b013e3182661eb1"},{"key":"e_1_2_9_13_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00415-013-7051-7"},{"key":"e_1_2_9_14_1","doi-asserted-by":"publisher","DOI":"10.1001\/jama.2013.283815"},{"key":"e_1_2_9_15_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMoa1805689"},{"key":"e_1_2_9_16_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMoa1716153"},{"key":"e_1_2_9_17_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMoa1716793"},{"key":"e_1_2_9_18_1","doi-asserted-by":"publisher","DOI":"10.1080\/13506129.2022.2091985"},{"key":"e_1_2_9_19_1","doi-asserted-by":"publisher","DOI":"10.1161\/CIRCHEARTFAILURE.120.008193"},{"key":"e_1_2_9_20_1","doi-asserted-by":"publisher","DOI":"10.1080\/13506129.2023.2177986"},{"key":"e_1_2_9_21_1","doi-asserted-by":"publisher","DOI":"10.1016\/S1474-4422(20)30368-9"},{"key":"e_1_2_9_22_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1755-3768.1968.tb02827.x"},{"key":"e_1_2_9_23_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jacc.2005.05.073"},{"key":"e_1_2_9_24_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2796.1994.tb01106.x"},{"key":"e_1_2_9_25_1","doi-asserted-by":"publisher","DOI":"10.3109\/13506129708995279"},{"key":"e_1_2_9_26_1","doi-asserted-by":"publisher","DOI":"10.1097\/WCO.0000000000000288"},{"key":"e_1_2_9_27_1","doi-asserted-by":"publisher","DOI":"10.1080\/13506129.2022.2065191"},{"key":"e_1_2_9_28_1","doi-asserted-by":"publisher","DOI":"10.1080\/13506129.2022.2026323"},{"key":"e_1_2_9_29_1","article-title":"Hereditary transthyretin amyloidosis \u2010 from symptomatic to curative treatment?","volume":"119","author":"Wixner J","year":"2022","journal-title":"Lakartidningen."},{"key":"e_1_2_9_30_1","doi-asserted-by":"publisher","DOI":"10.1080\/13506129.2020.1841623"},{"key":"e_1_2_9_31_1","doi-asserted-by":"publisher","DOI":"10.1186\/s13023-023-02962-5"},{"key":"e_1_2_9_32_1","doi-asserted-by":"publisher","DOI":"10.3109\/13506129.2010.483114"},{"key":"e_1_2_9_33_1","doi-asserted-by":"publisher","DOI":"10.1371\/journal.pone.0143456"},{"key":"e_1_2_9_34_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jchf.2021.05.005"},{"key":"e_1_2_9_35_1","doi-asserted-by":"publisher","DOI":"10.1002\/ejhf.2674"},{"key":"e_1_2_9_36_1","doi-asserted-by":"publisher","DOI":"10.1080\/13506120600876849"},{"key":"e_1_2_9_37_1","doi-asserted-by":"publisher","DOI":"10.3109\/13506129.2012.673184"},{"key":"e_1_2_9_38_1","doi-asserted-by":"publisher","DOI":"10.1159\/000490553"},{"key":"e_1_2_9_39_1","doi-asserted-by":"publisher","DOI":"10.1002\/path.2411"},{"key":"e_1_2_9_40_1","doi-asserted-by":"publisher","DOI":"10.3109\/13506129.2012.670813"},{"key":"e_1_2_9_41_1","doi-asserted-by":"publisher","DOI":"10.3109\/13506129.2012.674580"},{"key":"e_1_2_9_42_1","doi-asserted-by":"publisher","DOI":"10.1002\/mus.20644"},{"key":"e_1_2_9_43_1","doi-asserted-by":"publisher","DOI":"10.3109\/13506129.2010.527068"}],"container-title":["Journal of Internal Medicine"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1111\/joim.70020","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,10,15]],"date-time":"2025-10-15T19:35:27Z","timestamp":1760556927000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1111\/joim.70020"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2025,9,17]]},"references-count":42,"journal-issue":{"issue":"5","published-print":{"date-parts":[[2025,11]]}},"alternative-id":["10.1111\/joim.70020"],"URL":"https:\/\/doi.org\/10.1111\/joim.70020","archive":["Portico"],"relation":{},"ISSN":["0954-6820","1365-2796"],"issn-type":[{"value":"0954-6820","type":"print"},{"value":"1365-2796","type":"electronic"}],"subject":[],"published":{"date-parts":[[2025,9,17]]},"assertion":[{"value":"2025-09-17","order":3,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}]}}