著者
Kanae Hasegawa Hiroyasu Uzui Yoshitomo Fukuoka Dai Miyanaga Yuichiro Shiomi Naoto Tama Hiroyuki Ikeda Kentaro Ishida Shinsuke Miyazaki Yoshiki Sekijima Hironobu Naiki Hiroshi Tada
出版者
International Heart Journal Association
雑誌
International Heart Journal (ISSN:13492365)
巻号頁・発行日
vol.63, no.1, pp.49-55, 2022-01-29 (Released:2022-01-29)
参考文献数
29
被引用文献数
2

The diagnosis of cardiac amyloidosis is frequently delayed because histological confirmation is often challenging. Few studies have attempted to clarify the utility and safety of abdominal fat pad fine-needle aspiration (FPFNA) for an initial screening test in patients with suspected cardiac amyloidosis.This study included 77 consecutive patients with suspected non-ischemic cardiomyopathy who had left ventricular dysfunction and/or hypertrophy. All patients underwent abdominal FPFNA and an endomyocardial biopsy. In all patients, the abdominal FPFNA could be performed within less than 5 minutes with no complications; however, in 1 patient (1.3%), the obtained specimen was too small to evaluate. Among the remaining 76 patients, 5 (6.6%) were positive for amyloid (FPFNA[+]) and 7 (9.2%), including the 5 FPFNA[+], were diagnosed with cardiac amyloidosis (AL = 1, ATTR = 6) by endomyocardial biopsy. Positive abdominal FPFNAs indicated cardiac amyloidosis with high accuracy (sensitivity, 71.4%; specificity, 100%).Positive abdominal FPFNAs are directly linked to diagnoses of cardiac amyloidosis. Abdominal FPFNA is simple and useful for the initial screening test for cardiac amyloidosis in patients with non-ischemic cardiomyopathy.
著者
Jin Endo Motoaki Sano Yasuhiro Izumiya Kenichi Tsujita Kazufumi Nakamura Nobuhiro Tahara Koichiro Kuwahara Takayuki Inomata Mitsuharu Ueda Yoshiki Sekijima Yukio Ando Hiroyuki Tsutsui Mitsuaki Isobe Keiichi Fukuda
出版者
The Japanese Circulation Society
雑誌
Circulation Journal (ISSN:13469843)
巻号頁・発行日
pp.CJ-19-0811, (Released:2019-11-16)
参考文献数
4
被引用文献数
18

Transthyretin cardiac amyloidosis is a progressive and life-threating disease that is significantly underdiagnosed, and the actual number of patients with the disease is presently unknown. Accumulation of wild-type transthyretin-derived amyloid in the heart is a common finding in very elderly patients. Recent clinical trials demonstrated that tafamidis reduced all-cause death and the number of cardiovascular hospitalizations when compared with placebo. The Japanese Ministry of Health, Labour and Welfare approved tafamidis (Vyndaqel®, Pfizer Inc.) for the treatment of cardiomyopathy caused by both wild-type and mutated transthyretin-derived amyloidoses. This scientific statement on transthyretin-derived cardiac amyloidosis summarizes the conditions for reimbursement of the cost of tafamidis therapy, and the institutional and physician requirements for the introduction of tafamidis.
著者
Junji Ikeda Akira Matsushima Wataru Ishii Tetuya Goto Kenta Takahashi Kazuo Nakamichi Masayuki Saijo Yoshiki Sekijima Shu-ichi Ikeda
出版者
一般社団法人 日本内科学会
雑誌
Internal Medicine (ISSN:09182918)
巻号頁・発行日
vol.56, no.10, pp.1231-1234, 2017-05-15 (Released:2017-05-15)
参考文献数
22
被引用文献数
17

The current standard diagnostic approach for progressive multifocal leukoencephalopathy (PML) is to perform a DNA test to identify the presence of the JC virus in cerebrospinal fluid (CSF). A 32-year-old woman with a 5-year history of systemic lupus erythematosus developed right hemiplegia and motor aphasia. MRI revealed a large white matter lesion in the left frontal lobe. JC virus DNA was undetectable in the CSF, but a brain biopsy showed typical histopathology and a high DNA load of the JC virus. The patient was treated with mefloquine and mirtazapine, and is currently alive at 24 months after onset. An early brain biopsy may therefore be important for making a timely diagnosis of PML.