Epidemiology of Transthyretin Familial Amyloid Polyneuropathy in Portugal: A Nationwide Study

Neuroepidemiology
Mónica InêsJoão Costa

Abstract

Transthyretin-associated familial amyloid polyneuropathy (TTR-FAP) is a rare, hereditary, progressive and neurodegenerative disease. We aimed to study -TTR-FAP epidemiology in Portugal. 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. Over 2010-2016 period, mean incidence rates was 0.87/100,000 (95% CI 0.68-1.10) corresponding to 71 new patients yearly, that has decreased 31% in the last 7 years. The proportion of late-onset cases (age ≥50 years) among incident cases was 28.7%. Estimated crude 2016 prevalence was 22.93/100,000 adult inhabitants (95% CI 21.90-23.99) corresponding to 1,865 TTR-FAP individuals in Portugal (45.8% male; mean age: 52.3 ± 15.4 years). In 2016, the Portuguese region with the highest TTR-FAP prevalence shows a 16% prevalence increase over the last 25 years. 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...Continue Reading

Citations

May 10, 2019·BMJ : British Medical Journal·Kelly Graham Gwathmey, Kathleen T Pearson
Feb 20, 2020·Journal of Clinical Neuromuscular Disease·Coreen Schwartzlow, Mohamed Kazamel
Jul 23, 2020·Amyloid : the International Journal of Experimental and Clinical Investigation : the Official Journal of the International Society of Amyloidosis·Massimo RussoGiuseppe Vita
Jun 19, 2019·Nature Reviews. Neurology·David AdamsTeresa Coelho
Dec 20, 2019·Amyloid : the International Journal of Experimental and Clinical Investigation : the Official Journal of the International Society of Amyloidosis·Mónica InêsJoão Costa
Sep 29, 2020·Journal of Neuromuscular Diseases·Josef FinstererStefan Iglseder
Dec 18, 2020·Frontiers in Neurology·Maria do Carmo Vilas-BoasJoão Paulo Silva Cunha
Jan 19, 2021·Muscle & Nerve·Marcus V PintoTeerin Liewluck
Feb 26, 2021·The Canadian Journal of Neurological Sciences. Le Journal Canadien Des Sciences Neurologiques·Monica AlcantaraVera Bril

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