Prognostic role of cardiopulmonary exercise testing in wild-type transthyretin amyloid cardiomyopathy patients treated with tafamidis [0.03%]
野生型转甲状腺素蛋白淀粉样变性心肌病患者接受tafamidis治疗的预后心肺运动试验的作用
Tarun Dalia,Zafar Ali,Stefano H Byer et al.
Tarun Dalia et al.
Objectives: Tafamidis has demonstrated survival benefits in transthyretin amyloid cardiomyopathy (ATTR-CM), yet variability in therapeutic response underscores the need for reliable tools to predict outcomes. This study e...
Clinical and neurophysiological features of neuropathic pain in hereditary transthyretin amyloidosis associated polyneuropathy [0.03%]
遗传性转甲状腺素蛋白淀粉样变性周围神经病变的神经病理性疼痛的临床和神经电生理特征
Isabel Conceição,Isabel de Castro,José Castro
Isabel Conceição
Background: Hereditary transthyretin amyloidosis with polyneuropathy (ATTRv-PN) is often associated with neuropathic pain (NP), involving developing mechanisms across different nerve fibres. This study aimed to explore th...
Impact of autonomic dysfunction in light chain amyloidosis patient with nephrotic syndrome and cardiac involvement [0.03%]
轻链型淀粉样变性肾病合并心脏受累患者的自主神经功能障碍影响分析
Hamza Sakhi,Amira Zaroui,Mounira Kharoubi et al.
Hamza Sakhi et al.
Hereditary transthyretin amyloidosis with cardiomyopathy and polyneuropathy associated with a novel pathogenic TTR Tyr105His (p.Tyr125His) mutation [0.03%]
与新型致病TTR酪氨酸105组氨酸（p.酪氨酸125组氨酸）突变相关的遗传性转甲状腺素蛋白淀粉样变性心脏病和多发性神经病变
Masahiro Nakamori,Keisuke Tachiyama,Naoe Matsumura et al.
Masahiro Nakamori et al.
Marie Robert,Yasmine Serrar,Thibaud Mathis et al.
Marie Robert et al.
A case of acute myelopathy in ATTR-Val30Met amyloidosis with leptomeningeal involvement: pathophysiological insights and upcoming therapeutic challenges [0.03%]
特异性的ATTR变异型淀粉样变性合并脑膜受累的急性髓内病变病例报告：病理生理学见解和即将面临的治疗挑战
Obay Alalousi,Thierry Gendre,Blanche Bapst et al.
Obay Alalousi et al.
Olesia O Kalmukova,Liudmyla M Surzhko,Ruth C Campbell et al.
Olesia O Kalmukova et al.
Genetic landscape of hereditary transthyretin amyloidosis in Spain: a multicentric retrospective study [0.03%]
西班牙遗传性转甲状腺素蛋白淀粉样变性的基因图谱：一项多中心回顾性研究
Marta Domínguez-Martínez,Alfonso Caro-Llopis,Carla Martín-Grau et al.
Marta Domínguez-Martínez et al.
Background: Hereditary transthyretin amyloidosis (ATTRv) is a rare, progressive disorder caused by TTR gene variants, leading to amyloid fibril deposition and clinical manifestations like cardiomyopathy and polyneuropathy...
Impact of autonomic dysfunction on cardiovascular outcomes among patients with ATTR cardiomyopathy: insights from the COMPASS-31 [0.03%]
自主神经功能障碍对ATTR心肌病患者心血管预后的影响：COMPASS-31研究启示
Ariel Weinsaft,Sergio Teruya,Alfonsina Mirabal Santos et al.
Ariel Weinsaft et al.
Background: ATTR is a systemic disease, causing significant morbidity and mortality, manifesting with symptoms affecting both the heart and nervous system. This study employed the Composite Autonomic Symptom Scale 31 (COM...
Hepatic involvement in light chain amyloidosis: analysis of 130 patients and predictors of hepatic response and survival [0.03%]
轻链型淀粉样变性肝受累的临床特征、生存及相关危险因素分析（附130例报告）
Matthew J Rees,Nadia Toumeh,Angela Dispenzieri et al.
Matthew J Rees et al.
Organ response is key to improving outcomes in light chain (AL) amyloidosis. We investigated factors associated hepatic response (HepR) in a large cohort of patients with hepatic AL amyloidosis. ...