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파브리병에서의 심장 자기공명영상의 역할

Other Titles
 파브리병에서의 심장 자기공명영상의 역할 
Authors
 홍유진  ;  김영진 
Citation
 Journal of the Korean Radiological Society (대한영상의학회지), Vol.81(2) : 302-309, 2020-03 
Journal Title
Journal of the Korean Society of Radiology(대한영상의학회지)
ISSN
 1738-2637 
Issue Date
2020-03
Keywords
Fabry Disease ; Magnetic Resonance Imaging ; Cardiomyopathies ; Genes ; Alpha-Galactosidase
Abstract
Fabry disease is a rare X-linked metabolic disorder that is characterized by the accumulation of glycosphingolipids in various organs, resulting from the deficiency of alpha-galactosidase A. Cardiac involvement is relatively common; myocardial inflammation, left ventricular hypertrophy, and myocardial fibrosis secondary to abnormal lipid deposition in myocytes are often observed. Hence, the diagnosis of cardiac involvement is crucial for evaluating patient prognosis. Cardiac MRI is the standard technique for measuring the function, volume, and mass of the ventricles. It is also useful for myocardial tissue characterizations. The evaluation of native myocardial T1 values can facilitate early diagnosis of cardiac involvement, while measurements of left ventricular myocardial mass can be used to monitor treatment outcomes, in patients with Fabry disease. Consequently, cardiac MRI can provide useful information for diagnosing, monitoring, and treating patients with Fabry disease.
Files in This Item:
T202007352.pdf Download
DOI
10.3348/jksr.2020.81.2.302
Appears in Collections:
1. College of Medicine (의과대학) > Dept. of Radiology (영상의학교실) > 1. Journal Papers
Yonsei Authors
Kim, Young Jin(김영진) ORCID logo https://orcid.org/0000-0002-6235-6550
Hong, Yoo Jin(홍유진) ORCID logo https://orcid.org/0000-0002-7276-0944
URI
https://ir.ymlib.yonsei.ac.kr/handle/22282913/187357
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