Nationwide Survey of 741 Patients with Systemic Amyloid Light-chain Amyloidosis in Japan.
Nationwide Survey of 741 Patients with Systemic Amyloid Light-chain Amyloidosis in Japan.
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DOI：
10.2169/internalmedicine.9206-17
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发表时间：
2018-01-15
期刊：
影响因子：
--
通讯作者：
Ando Y
中科院分区：
文献类型：
--
作者：
Shimazaki C;Hata H;Iida S;Ueda M;Katoh N;Sekijima Y;Ikeda S;Yazaki M;Fukushima W;Ando Y
回顾性探讨日本患者全身性轻链淀粉样变性的临床表现及治疗策略。我们对2012年1月1日至2014年12月31日期间接受治疗的日本AL淀粉样变性患者进行了调查。本研究共纳入741例AL淀粉样变患者（男性436例，女性305例，中位年龄65岁，范围31-93岁）。最常见的受累器官是肾脏（542例），其次是心脏（252例）、胃肠道（164例）、自主神经系统（131例）、肝脏（71例）和周围神经系统（71例）。诊断结果最常见于胃肠道（上消化道：350例，下消化道：167例），其次是骨髓和肾脏。仅在128例患者中进行了腹部脂肪垫活检。自体干细胞移植（ASCTs）和硼替佐米分别用于126例和276例患者。日本全身性AL淀粉样变性患者的临床特征与此前在美国和欧洲报道的病例相似。在治疗方面，在日本和西方国家进行了大量的asct。令人惊讶的是，相当数量的患者接受硼替佐米作为AL淀粉样变的治疗。
To retrospectively investigate the clinical manifestations of systemic amyloid light-chain (AL) amyloidosis in Japanese patients and the treatment strategy for the condition. We conducted a survey of Japanese AL amyloidosis patients, who were treated between January 1, 2012, and December 31, 2014. A total of 741 AL amyloidosis patients were included in this study (436 men and 305 women; median age: 65 years old, range: 31-93). The most frequently affected organ was the kidneys (n=542), followed by the heart (n=252), gastrointestinal (GI) tract (n=164), autonomic nervous system (n=131), liver (n=71), and peripheral nervous system (n=71). Diagnostic findings were most commonly detected in the GI tract (upper GI tract: 350 cases, lower GI tract: 167 cases), followed by the bone marrow and kidneys. An abdominal fat-pad biopsy was only conducted in 128 patients. Autologous stem cell transplants (ASCTs) and bortezomib were used to treat 126 and 276 patients, respectively. The clinical features of Japanese patients with systemic AL amyloidosis are similar to those reported previously for cases in the US and Europe. Regarding treatment, a significant number of ASCTs were performed in Japan as well as in Western countries. Surprisingly, a marked number of patients received bortezomib as a treatment for AL amyloidosis.
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影响因子：
2.1
作者：
Katoh, Nagaaki;Ueno, Akihiro;Ikeda, Shu-ichi
通讯作者：
Ikeda, Shu-ichi
影响因子：
20.3
作者：
Muchtar, Eli;Gertz, Morie A.;Dispenzieri, Angela
通讯作者：
Dispenzieri, Angela
影响因子：
20.3
作者：
Palladini, Giovanni;Hegenbart, Ute;Schoenland, Stefan
通讯作者：
Schoenland, Stefan
影响因子：
45.3
作者：
Kastritis, Efstathios;Wechalekar, Ashutosh D.;Palladini, Giovanni
通讯作者：
Palladini, Giovanni
影响因子：
1.2
作者：
Matsuda, Masayuki;Katoh, Nagaaki;Ikeda, Shu-ichi
通讯作者：
Ikeda, Shu-ichi