{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,4,27]],"date-time":"2026-04-27T13:52:53Z","timestamp":1777297973185,"version":"3.51.4"},"reference-count":0,"publisher":"S. Karger AG","issue":"3-4","license":[{"start":{"date-parts":[[2018,1,1]],"date-time":"2018-01-01T00:00:00Z","timestamp":1514764800000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/www.karger.com\/Services\/SiteLicenses"},{"start":{"date-parts":[[2018,1,1]],"date-time":"2018-01-01T00:00:00Z","timestamp":1514764800000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.karger.com\/Services\/SiteLicenses"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Neuroepidemiology"],"published-print":{"date-parts":[[2018]]},"abstract":"<jats:p>&lt;b&gt;&lt;i&gt;Background:&lt;\/i&gt;&lt;\/b&gt; Transthyretin-associated familial amyloid polyneuropathy (TTR-FAP) is a rare, hereditary, progressive and neurodegenerative disease. We aimed to study \u00adTTR-FAP epidemiology in Portugal. &lt;b&gt;&lt;i&gt;Methods:&lt;\/i&gt;&lt;\/b&gt; National, observational, prospective and retrospective, case identification of adults with TTR-FAP. Countrywide patient multiple identification sources included reference centers registries and centralized medical electronic prescription database. Crude rates were reported per 100,000 adult inhabitants. &lt;b&gt;&lt;i&gt;Results:&lt;\/i&gt;&lt;\/b&gt; Over 2010\u20132016 period, mean incidence rates was 0.87\/100,000 (95% CI 0.68\u20131.10) corresponding to 71 new patients yearly, that has decreased 31% in the last 7 years. The proportion of late-onset cases (age \u226550 years) among incident cases was 28.7%. Estimated crude 2016 prevalence was 22.93\/100,000 adult inhabitants (95% CI 21.90\u201323.99) corresponding to 1,865 TTR-FAP individuals in Portugal (45.8% male; mean age: 52.3 \u00b1 15.4 years). In 2016, the Portuguese region with the highest TTR-FAP prevalence shows a 16% prevalence increase over the last 25 years. &lt;b&gt;&lt;i&gt;Conclusions:&lt;\/i&gt;&lt;\/b&gt; In Portugal, TTR-FAP affects both genders and mainly young adults. TTR-FAP incidence appears to be decreasing while prevalence is increasing. In comparison to previous studies, there is an increased representativeness of late-onset patients. This epidemiological setting poses future and complex challenges for the social and healthcare system, strengthening the relevance of regular epidemiologic surveillance.<\/jats:p>","DOI":"10.1159\/000490553","type":"journal-article","created":{"date-parts":[[2018,8,28]],"date-time":"2018-08-28T21:46:37Z","timestamp":1535492797000},"page":"177-182","source":"Crossref","is-referenced-by-count":70,"title":["Epidemiology of Transthyretin Familial Amyloid Polyneuropathy in Portugal: A Nationwide Study"],"prefix":"10.1159","volume":"51","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-3631-4995","authenticated-orcid":false,"given":"M\u00f3nica","family":"In\u00eas","sequence":"first","affiliation":[]},{"given":"Teresa","family":"Coelho","sequence":"additional","affiliation":[]},{"given":"Isabel","family":"Concei\u00e7\u00e3o","sequence":"additional","affiliation":[]},{"given":"Filipa","family":"Duarte-Ramos","sequence":"additional","affiliation":[]},{"given":"Mamede","family":"de\u00a0Carvalho","sequence":"additional","affiliation":[]},{"given":"Jo\u00e3o","family":"Costa","sequence":"additional","affiliation":[]}],"member":"127","published-online":{"date-parts":[[2018,8,28]]},"container-title":["Neuroepidemiology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.karger.com\/Article\/Pdf\/490553","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/www.karger.com\/Article\/Pdf\/490553","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,4,23]],"date-time":"2025-04-23T16:40:10Z","timestamp":1745426410000},"score":1,"resource":{"primary":{"URL":"https:\/\/karger.com\/article\/doi\/10.1159\/000490553"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2018]]},"references-count":0,"journal-issue":{"issue":"3-4","published-online":{"date-parts":[[2018,11,16]]},"published-print":{"date-parts":[[2018,11,22]]}},"URL":"https:\/\/doi.org\/10.1159\/000490553","archive":["Portico"],"relation":{"is-supplemented-by":[{"id-type":"doi","id":"10.6084\/m9.figshare.7000508","asserted-by":"subject"}]},"ISSN":["0251-5350","1423-0208"],"issn-type":[{"value":"0251-5350","type":"print"},{"value":"1423-0208","type":"electronic"}],"subject":[],"published":{"date-parts":[[2018]]}}}