التفاصيل البيبلوغرافية
| العنوان: |
Ascending aortic remodelling in Fabry disease after long-term enzyme replacement therapy |
| المؤلفون: |
Pierre Monney, Salah D. Qanadli, Steven D. Hajdu, Christel Tran, Juerg Schwitter, Olivier Dormond, Frédéric Barbey |
| المصدر: |
Swiss Medical Weekly, Vol 147, Iss 4142 (2017) |
| بيانات النشر: |
SMW supporting association (Trägerverein Swiss Medical Weekly SMW), 2017. |
| سنة النشر: |
2017 |
| المجموعة: |
LCC:Medicine |
| مصطلحات موضوعية: |
aorta, atherosclerosis, dilation, Enzyme replacement therapy, Fabry disease, Remodelling, Medicine |
| الوصف: |
BACKGROUND Previous cross-sectional studies reported a high prevalence of ascending aorta dilations/aneurysms in male adults with Fabry disease, independently of cardiovascular risk factors. AIMS OF THE STUDY To characterise the remodelling of the ascending aorta in classic Fabry disease under long-term enzyme replacement therapy. METHODS Diameter of the ascending aorta was measured with magnetic resonance imaging at the sino-tubular junction (STJ), and proximal (pAsAo), and distal ascending aorta (dAsAo) at baseline, and after 5 and 10 years of enzyme replacement therapy in 15 adult Fabry patients (10 males; 5 females). RESULTS Over a mean follow-up of 9.5 years, the annual expansion rates measured in 10 males with Fabry disease were 0.41 ± 0.16, 0.36 ± 0.25 and 0.41 ± 0.26 mm/y at the STJ, pAsAo and dAsAo, respectively. Expansion rate at the pAsAo level in male patients was significantly higher than the expected expansion projected from theoretical normal values: 0.36 ± 0.25 vs 0.13 ± 0.05, p = 0.017. In 5 females, the annual expansion rates at the STJ, pAsAo and dAsAo were 0.14 ± 0.11, 0.21 ± 0.18 and 0.26 ± 0.24 mm/y, respectively. There was no significant difference from the projected normal expansion rate at the level of the pAsAo: 0.21 ± 0.18 vs 0.13 ± 0.04, p = 0.39. CONCLUSION Our data suggest that the remodelling of the ascending aorta is more pronounced in male patients with Fabry disease under long-term enzyme replacement therapy compared with the progression observed in a large population study. |
| نوع الوثيقة: |
article |
| وصف الملف: |
electronic resource |
| اللغة: |
English |
| تدمد: |
1424-3997 |
| Relation: |
https://www.smw.ch/index.php/smw/article/view/2379; https://doaj.org/toc/1424-3997 |
| DOI: |
10.4414/smw.2017.14517 |
| URL الوصول: |
https://doaj.org/article/d75cf88c0d4a4fd9b1c2e3c45f85f902 |
| رقم الانضمام: |
edsdoj.75cf88c0d4a4fd9b1c2e3c45f85f902 |
| قاعدة البيانات: |
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