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英文誌(2004-)

Journal of Medical Ultrasonics

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2016 - Vol.43

Vol.43 No.Supplement

特別プログラム 循環器
ワークショップ 循環器 Joint1(JSUM・AFSUMB Joint Session)(English) 二次性心筋症の診療における心エコー図の役割を明らかにする

(S294)

Fabry病

Fabry disease

湯淺 敏典1, 堀添 善尚1, 茶圓 秀人1, 高崎 州亜1, 水上 尚子2, 木佐貫 彰3, 樋口 公嗣1, 宮田 昌明1, 大石 充1

Toshinori YUASA1, Yoshihisa HORIZOE1, Hideto CYAEN1, Kunitsugu TAKASAKI1, Naoko MIZUKAMI2, Akira KISANUKI3, Kouji HIGUCHI1, Masaaki MIYATA1, Mitsuru OHISHI1

1鹿児島大学医歯学総合研究科心臓血管・高血圧内科学, 2鹿児島大学病院臨床検査部, 3鹿児島大学病院保健学科

1Department of Cardiovascular Medicine and Hypertension, Graduate School of Medical and Dental Sciences, Kagoshima University, 2Clinical Laboratory, Kagoshima University Hospital, 3Department of Health Science, Kagoshima University Hospital

キーワード :

Classic Fabry disease is a disorder firstly reported in 1898 by German and British dermatologist. The accumulation of sphingoglycolipid to an organ by loss of α-galactosidase A causes various disorders. Enzyme activity slightly remains in cardiac Fabry disease whose obstacle limits to heart. The onset age in cardiac Fabry disease is older than classic one. Furthermore, Fabry disease develop even in females who should be a carrier while taking an X chromosome-related hereditary form. Echocardiographic findings in Fabry disease is left ventricle (LV) hypertrophy at early and middle period of illness. When a stage of this disease progresses, LV wall abnormality develops with wall thinning on the basal posterior wall. Since enzyme replacement therapy spreads as basic treatment of this disease in Japan after 2004, the start of therapy before the decrement of cardiac function is expected. Echocardiography is one of the important tools in clinical practice of Fabry disease. Therefore, I want to show the roles of echocardiography in early checkup, follow-up and the curative effect judgment in Fabry disease.