Treatment of amyloidosis: present and future

التفاصيل البيبلوغرافية
العنوان: Treatment of amyloidosis: present and future
المؤلفون: Maria Teresa Mallus, Vittoria Rizzello
المصدر: European Heart Journal Supplements. 25:B99-B103
بيانات النشر: Oxford University Press (OUP), 2023.
سنة النشر: 2023
مصطلحات موضوعية: Cardiology and Cardiovascular Medicine
الوصف: Cardiac amyloidosis (CA) is an infiltrative heart disease resulting from the deposition of amyloid fibrils in the interstitial spaces of the myocardium. The two main forms of CA are represented by light chain amyloidosis (AL) and transthyretin amyloidosis (ATTR) in the two forms familial or variant or wild-type or senile. Although considered a rare disease, CA is an underdiagnosed disease. Delay in diagnosis has a negative impact on the prognosis, delaying the initiation of specific therapy. The treatment of both forms of CA is based on: (i) prevention and slowing of the generation and deposition of amyloid fibrils and (ii) supportive care of complications. The main success of recent years has been the development of effective therapies that have been possible thanks to the understanding of the pathophysiology of amyloidosis. For the AL form, new therapeutic combinations between a proteasome inhibitor and a monoclonal antibody have been developed. For ATTR forms, the main strategies are transthyretin (TTR) production ‘silencers’ and TTR tetramer stabilizers. Supportive care of patients with CA involves various clinical aspects including treatment of heart failure, arrhythmias, conduction disturbances, thrombo-embolism, and the concomitant presence of aortic stenosis.
تدمد: 1554-2815
1520-765X
DOI: 10.1093/eurheartjsupp/suad082
URL الوصول: https://explore.openaire.eu/search/publication?articleId=doi_________::949024b7901a5ffda4840ffb2e2dcff3
https://doi.org/10.1093/eurheartjsupp/suad082
Rights: OPEN
رقم الانضمام: edsair.doi...........949024b7901a5ffda4840ffb2e2dcff3
قاعدة البيانات: OpenAIRE
الوصف
تدمد:15542815
1520765X
DOI:10.1093/eurheartjsupp/suad082