Cardiac amyloidosis-A review of current literature for the practicing physician.
Cardiac amyloidosis-A review of current literature for the practicing physician.
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心脏淀粉样变性：执业医师当前文献综述。
DOI：
10.1002/clc.23572
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发表时间：
2021-03
影响因子：
2.7
通讯作者：
Jain R
中科院分区：
文献类型：
--
作者：
Ash S;Shorer E;Ramgobin D;Vo M;Gibbons J;Golamari R;Jain R;Jain R
淀粉样变性是一个疾病家族，其中错误折叠的前体蛋白聚集形成淀粉样蛋白并存款在身体组织中。这种疾病的一种非常严重但未被充分认识的形式是心脏淀粉样变性，其中淀粉样蛋白沉积到心肌的细胞外空间中，导致心室壁增厚和硬化，从而导致心力衰竭和传导功能障碍。本文综述了心脏淀粉样变性亚型的发病机制和临床表现，以及最新的诊断和治疗方法。近年来，关于这种疾病的诊断和治疗已经取得了重大进展，但预后仍然严重依赖于疾病的早期检测。两种类型的前体蛋白是大多数心脏淀粉样变性病例的原因：甲状腺素运载蛋白淀粉样蛋白和免疫球蛋白衍生的轻链淀粉样蛋白。心脏淀粉样变性的早期诊断可以允许新的治疗方式开始与改善预后的潜力。
The amyloidoses are a family of diseases in which misfolded precursor proteins aggregate to form amyloid and deposit in body tissues. A very serious yet underrecognized form of this disease is cardiac amyloidosis, in which amyloid deposits into the extracellular space of the myocardium, resulting in thickening and stiffening of ventricular walls with resultant heart failure and conductive dysfunction. This review provides a discussion of the pathogenesis and clinical presentation of cardiac amyloidosis subtypes, as well as an up‐to‐date approach to diagnosis and treatment. Significant progress has been made in recent years regarding diagnosis and treatment of this condition, but prognosis remains heavily reliant on early detection of the disease. Two types of precursor protein are responsible for most cardiac amyloidosis cases: transthyretin amyloid, and immunoglobulin‐derived light chain amyloid. An early diagnosis of cardiac amyloidosis can allow for novel treatment modalities to be initiated with the potential to improve prognosis.
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影响因子：
120.7
作者：
Berk, John L.;Suhr, Ole B.;Obici, Laura;Sekijima, Yoshiki;Zeldenrust, Steven R.;Yamashita, Taro;Heneghan, Michael A.;Gorevic, Peter D.;Litchy, William J.;Wiesman, Janice F.;Nordh, Erik;Corato, Manuel;Lozza, Alessandro;Cortese, Andrea;Robinson-Papp, Jessica;Colton, Theodore;Rybin, Denis V.;Bisbee, Alice B.;Ando, Yukio;Ikeda, Shu-ichi;Seldin, David C.;Merlini, Giampaolo;Skinner, Martha;Kelly, Jeffery W.;Dyck, Peter J.
通讯作者：
Dyck, Peter J.
影响因子：
37.8
作者：
Kittleson, Michelle M.;Maurer, Mathew S.;Ruberg, Frederick L.
通讯作者：
Ruberg, Frederick L.
影响因子：
24
作者：
Martinez-Naharro, Ana;Treibel, Thomas A.;Fontana, Marianna
通讯作者：
Fontana, Marianna
影响因子：
39.3
作者：
Gillmore, Julian D.;Damy, Thibaud;Hawkins, Philip N.
通讯作者：
Hawkins, Philip N.
影响因子：
5.7
作者：
Dubrey, S. W.;Hawkins, P. N.;Falk, R. H.
通讯作者：
Falk, R. H.