Cardiac involvement in Fabry's disease

被引：0
|
作者：
Weidemann, Frank [1 ]
Breunig, Frank [1 ]
机构：
[1] Univ Wurzburg, Med Klin & Poliklin 1, Univ Klinikum, D-97080 Wurzburg, Germany
来源：
MEDIZINISCHE KLINIK | 2008年 / 103卷 / 03期
关键词：
Fabry; cardiomyopathy;
D O I：
10.1007/s00063-008-1023-1
中图分类号：
R5 [内科学];
学科分类号：
1002 ; 100201 ;
摘要：
Fabry's disease is a rare X-linked lysosomal storage disorder leading to an accumulation of globotriaosylceramides in the lysosomes of all tissues. The disease is characterized by a progressive involvement of important vital organs like the kidneys, the cerebrovascular system and the heart. Within the scope of this article an overview of Fabry's cardiomyopathy, the necessary cardiac diagnostic tests and, in addition, the new concept of enzyme replacement therapy is given.
引用
收藏
页码：161 / 165
页数：5
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