Ratio of Fabry disease in patients with idiopathic left ventricular hypertrophy: A single-center study in Turkey
ANATOLIAN JOURNAL OF CARDIOLOGY, cilt.23, sa.2, ss.79-85, 2020 (SCI-Expanded, Scopus, TRDizin)
- Yayın Türü: Makale / Tam Makale
- Cilt numarası: 23 Sayı: 2
- Basım Tarihi: 2020
- Doi Numarası: 10.14744/anatoljcardiol.2019.84782
- Dergi Adı: ANATOLIAN JOURNAL OF CARDIOLOGY
- Derginin Tarandığı İndeksler: Science Citation Index Expanded (SCI-EXPANDED), Scopus, Academic Search Premier, CINAHL, EMBASE, MEDLINE, Directory of Open Access Journals, TR DİZİN (ULAKBİM)
- Sayfa Sayıları: ss.79-85
- Anahtar Kelimeler: hypertrophic cardiomyopathy, echocardiography, Fabry disease, EUROPEAN-SOCIETY, PREVALENCE, CARDIOMYOPATHY, MANIFESTATIONS, MANAGEMENT, DIAGNOSIS
- İstanbul Üniversitesi-Cerrahpaşa Adresli: Evet
Özet
Objective: Fabry disease (FD) is a progressive, X-linked inherited disorder of glycosphingolipid metabolism which arises due to deficient or absent activity of lysosomal alpha-galactosidase A (alpha-Gal A). This may be associated with increased left ventricular (LV) wall thickness and may mimic the morphological features of hypertrophic cardiomyopathy. The purpose of this study was to define the ratio of occurrence of FD to the manifestation of unexplained left ventricular hypertrophy (LVH).