An updated AL-base reveals ranked enrichment of immunoglobulin light chain variable genes in AL amyloidosis [0.03%]
更新后的AL-base显示免疫球蛋白轻链可变基因在AL淀粉样变性中排名丰富
Gareth J Morgan,Allison N Nau,Sherry Wong et al.
Gareth J Morgan et al.
Background: Each monoclonal antibody light chain associated with AL amyloidosis has a unique sequence. Defining how these sequences drive amyloid deposition could facilitate faster diagnosis and lead to new treatments. ...
Pathogenesis, manifestations, diagnosis, and management of CNS complications in hereditary ATTR amyloidosis [0.03%]
遗传性ATTR淀粉样变性中枢神经系统并发症的发病机制、表现、诊断和管理
Yoshiki Sekijima,Luísa Sousa
Yoshiki Sekijima
The clinical efficacy of transthyretin (TTR) tetramer stabilisers and TTR gene silencers in addition to liver transplantation has been established for hereditary ATTR (ATTRv) amyloidosis. Accordingly, non-central nervous system (CNS) system...
Clinical impact of beta-blocker withdrawal in transthyretin amyloid cardiomyopathy [0.03%]
特异性甲状腺素转运蛋白心肌病中β受体阻滞剂撤药的临床影响
Eva Cabrera-Romero,Leidy Alexandra Serrao-Faria,Nerea Mora-Ayestarán et al.
Eva Cabrera-Romero et al.
Involvement of bile acid in diarrhoea and therapeutic effect of colestimide in hereditary ATTR amyloidosis [0.03%]
胆酸参与遗传性ATTR淀粉样变性病腹泻的发病机制及其与考来刺激胺治疗反应的关系
Yusuke Mochizuki,Nagaaki Katoh,Akira Matsushima et al.
Yusuke Mochizuki et al.
Background: Diarrhoea is one of the most serious complications in hereditary ATTR (ATTRv) amyloidosis. However, its precise pathomechanism remains unknown. The present study investigated the involvement of bile acid in di...
Effects of eplontersen on symptoms of autonomic neuropathy in hereditary transthyretin-mediated amyloidosis: secondary analysis from the NEURO-TTRansform trial [0.03%]
NEURO-TTRansform试验的二次分析：eplontersen对遗传性转甲状腺素蛋白介导的淀粉样变性自主神经病变症状的影响
Jonas Wixner,John L Berk,David Adams et al.
Jonas Wixner et al.
Background: The NEURO-TTRansform trial showed that after 66 weeks of treatment, eplontersen significantly reduced neuropathic impairment and improved quality of life (QoL) in patients with hereditary transthyretin-mediate...
High frequency of occult transthyretin and apolipoprotein AI-type amyloid in aortic valves removed by valve replacement for aortic stenosis [0.03%]
在主动脉瓣置换术中切除的主动脉瓣中隐匿性转铁蛋白和载脂蛋白AI型淀粉样变高频发生
Kohei Honda,Masayoshi Tasaki,Tetsuhiro Yamano et al.
Kohei Honda et al.
Background: A high incidence of valvular involvement of amyloid in the setting of aortic stenosis (AS) has been reported. Amyloid derived from ApoAI (AApoAI) can form local amyloid deposits in the aortic valve. Although a...
Cardiac amyloidosis: the possibilities and challenges in the Ghanaian setting [0.03%]
心脏淀粉样变性：加纳环境下的机遇与挑战
Andrew Sefenu Dzebu,Magalys López Cuba
Andrew Sefenu Dzebu
Amyloidosis can be diagnosed by cardiologists in Africa: now they should be given the medicine to treat it [0.03%]
非洲的心脏病专家能够诊断出淀粉样变性病，现在应该提供治疗药物了
Lucio Luzzatto
Lucio Luzzatto
Anomalous colours, not interference colours or 'apple-green birefringence', in Congo red-stained amyloid [0.03%]
异常颜色，不是干涉色或"苹果绿色双折射"，在刚果红染色的淀粉样物中的出现
Alexander J Howie
Alexander J Howie
PRX004 in variant amyloid transthyretin (ATTRv) amyloidosis: results of a phase 1, open-label, dose-escalation study [0.03%]
新型ATTR变体淀粉样变性病治疗药物PRX004的I期开放标签剂量递增式研究结果
Ole B Suhr,Martha Grogan,Ana Martins da Silva et al.
Ole B Suhr et al.
Background: The investigational monoclonal antibody PRX004 is designed to specifically target and deplete TTR amyloid. Here, we report on the safety, tolerability, pharmacokinetics, pharmacodynamics and preliminary clinic...